Part 20. Cardiovascular System

PART 20. The Cardiovascular System

Section 1. Evaluation of the Cardiovascular System

Chap. 380. History and Physical Examination

# cardiovascular disorders가 의심되는 환아에 있어서 history & physical examination의 중요성을 아무리 강조해도 지나치지 않다.

History

# history taking해야 할 사항

  : details of the perinatal period - cyanosis, respiratory distress, or prematurity

  : maternal complication, such as gestational diabetes, medication exposure, or

    substance use

  : during infancy, the timing of 1st presentation

# *Sx of congestive heart failure - age specific

# Infant

    ; *feeding diffculities

           - *▲common

        - less volume per feeding

           - dyspneic or diaphoretic while sucking

           - awaken after brief period of time

    ; frequent G-E reflux

    ; respiratiory distress

           - rapid brething, nasal flaring, chest retraction

# older children

    ; exercise intolerance

           - *initially Sx

    ; fatigue

    ; orthopnea, nocturnal dyspnea

; cyanosis

   -정상인에 있어 deep coloring

   -운동시 blueness로 잘 관찰됨

; chest pain

    - *usually not Sx of cardiac disease

★Table 380-1

# cardiac disease may be a congenital malformation syndrome

★Table 380-2

# cardiac disease may be a generalized disorder affecting the heart and other organ system

★Table 380-3

# Congenital HD

    ; *25%- extracardic malformation

    ; *10%- chromosomal abnormality

# family history로서 알 수 있는 질환

1) early coronary a.ds(familial hypercholesterolemia)

2) generalized m.ds(muscular dystrophy,dermatomyositis)

3) prior congenital heart ds.

General Physical Examination

; ※96객 begin with a general assessment of the patient

; height and weight

; cyanosis

    - best observed over the nail beds, lip, tongue, and mucus membranes.

; difference cyanosis

    - right-to-left shunting across a ductus arterious in the presence of a coraction of aorta.

; circumoral cyanosis or blueness about forehead

    - by *prominant venous plexus than arterial oxygen saturation

; heart failure

  - failure to thrive, tachypnea, liver and less so spleen enlargement, pulmonary rales, peripheral edema.

; Heart rate

★Table 380-4

    1) newborn infants에서는 rapid & wide fluctuation

    2) average range ; 120-140beats/min

    3) crying & activity때는 170까지 증가하고 잠자는 동안은 70-90까지 감소한다.

    4) persistent tachycardia

           - neonate > 200 beats/min

           - infant > 150 beats/min

           - old child > 120 beats/min

# character of pulse

    1) congenital heart ds.의 physical Dx에 있어 early step으로서 중요.

    2) *wide pulse Pr.with bounding pulse(Water-Hammer pulse)

       - aortic runoff lesion

               ; *PDA, aortic insufficiency, A-V communication

       - increased cardiac output secondary to anemia, anxiety

       - increased catecholamine secretion

    3) *diminished pulse

       - heart failure

       - pericardial tamponade

       - left ventricular outflow obstruction

       - cardiomyopathy

# ★blood pressure

    1) arm & leg

    2) decreased femoral and/or dorsalis pedis pulse

           - not reliable finding of coarctation

    3) 2/3 covered cuff of upper arms & legs

    4) small cuff -> high reading, large cuff -> low reading

    5) use popliteal a. in measuring legs pressure

    6) legs B.P - *10mmHg higher than upper arms

    7) in infant, ausculation, palpation, ultrasonic(doppler), oscillometric(dinamap)devices, flush method

    8) 나이에 따라 변하며 신장,체중과도 밀접한 관계

    9) exercise,excitment,coughing,straining등은 정상보다 수축기 혈압을 40-50mmHg 올린다.

# normal jugular phlebogram- information about CVP, RA pressure

  ; three positive component

1) a wave; atrial systole

2) v wave; atrial diastole

3) c wave; early ventricular systole

  - external jugular vein should not be visible above the clavicle unless venous

    pressure is elevated.

Cardiac Examination

# Precardial bulge to the left of the sternum with increased precordial activity

  ; cardiac enlargement

# *Substernal thrust

  ; *right ventricular enlargement

# Apical heave

  ; left ventricular hypertrophy

# Hyperdynamic precordiun

  ; volume load like that found with a large left to right shunt

# Silent precordium with a barely detectable apical impulse

  ; pericardial effusion or severe cardiomyopathy

# Right sided apical impulses

  ; dextrocardia, tension pneumothorax, left sided thoracic space occupying lesions

    ; e.g.diaphragmatic hernia

# Thrills

  ; areas of maximum intensity of the auscultatory murmurs

# *Aortic bruits

    ; palpate suprasternal notch and neck

  ; *aortic stenosis

    ; less prominent pulmonary stenosis

# *apical systolic thrills on Rt. lower sternal border

  ; VSD, mitral insufficience

# diastolic thrills

  ; A-V vavular stenosis

# Stethoscopy

  ; diaphragm-high pitched sounds, bell-low pitched sounds

# 1st heart sound

  ; A-V valves closure에 의해 생성, heart apex에서 가장 잘 들림

# 2nd heart sound

  ; semilunar valves closure에 의해 생성, left upper sternal border에서 가장 잘 들림

# Inspiration과 heart의 right side에 filling이 증가하는 동안에는 rt.vent.ejection time이 증가하고 pul.valve closure는 delayed

# heart sound의 intensity에 영향을 주는 요소

  ; age if pt.,thickness of chest wall,cardiac output

# ♥ Wide splitting of S2

    1) PS

    2) Ebstein anomaly

    3) total anomalous venous rturn

    4) ASD

    5) TOF

    6) RBBB

※96 single S2

    ; pulmonary or aortic atresia

    ; severe stenosis

    ; truncus arteriosus

    ; TGA

# Gallop rhythm

    ; sign of congestive heart failure and tachycardia

    ; poor compliance of ventricle

※96주 Accentuated 2nd sound with narrow splitting

  ; pul.hypertension

# 3rd heart sound

    ; mid-diastolic때 bell로서 심첨부에서 가장 잘 들림

  ; narrow in adolescent with a relatively slow heart rate

  ; CHF and tachycardia 임상증상을 가진 환아에서도 나타남

# 4th heart sound

    ; late diastole때 1st heart sound 바로 직전에 들림

# ♥ Ejection clicks

    1) heard in early systole

    2) related to *dilation of or hypertension in the aorta and pul.a.

    3) heard so close to 1st heart sound

           - mistaken for a split 1st sound

    4) Aortic systolic clicks

           - left mid to right upper sternal border

           - constant

           - AS,TOF,truncus arterious

    5) pulmonary ejection clicks

           - left mid to upper sternal border

           - vary with respiration, disappearing with inspiration

    6) midsystolic click at apex - mitral valve prolapse

# description of murmur

  ; intensity, pitch, timing(systolic and diastolic), area of maximal intensity, radiation to other areas

# Systolic murmurs

    1) ★Intensity

        1-barely audible

    2-medium intensity

    3-loud but no thrill

    4-loud with a thrill

    5-very loud but still requires the stesthoscope to be on the chest

    6-so loud the murmur can be heard with the stethoscope off the chest

    2) 분류

           ; ejection

                - 1st heart sound이후에 시작하여 2nd sound 이전에 끝난다.

                   - increased flow or stenosis across semilunar valve

                   - 1st heart sound appreciated --> ejection in nature

             - severe aortic or pulmonary stenosis

           ; pansystolic

                - 1st sound와 동시에 시작하여 systole동안에 지속된다

             - VSD or A-V valve insufficience

       ; late systolic

               - 1st sound이후에 시작하는 bruit로서 end systole까지 지속된다

               - mitral valve prolapse

    3) continuous murmur

        ; 수축기부터 시작하여 2nd sound를 지나서 확장기까지 들린다

    ; PDA, A-P communication

           ; DDx

                   - to and fro mummur

                   - aortic or pulmonary stenosis with insufficiency

※객 Diastolic murmurs

    1) high pitched, blowing, decrescendo diastolic murmur along left sternal border

       ; *aortic insuffiency

           ; pul. insuffiency(if high pul. pressure)

    2) early, short, lower-pitched diastolic murmurs along left mid & upper sternal border

       ; pul. insuffiency

           ; after surgical repair of pul. outflow defect such as TOF

    3) rumbling mid-diastolic murmur at the left mid and lower sternal border

       ; increased blood flow across TV

           ; ASD

           ; tricuspid valve stenosis

    4) ★rumbling mid-diastolic murmur at the apex follow the 3rd heart sound

       ; *increased transmitral flow

           ; *large right to left shunts

           ; mitral insuffiency

    5) longer diastolic rumbling murmur at apex

       ; anatomic mitral stenosis

# *absence of precordial mummur --> no rule-out congenital or acquired heart disease

    ; pulmonary or tricuspid valve atresia & TGA

# insignificant mummur

  ; severe AS, ASD, anomalous pul.venous return, A-V septal defects, COA

# loud mummur in absence structual anomaly

  ; large noncardiac arteriovenous malformation, myocarditis, severe anemia, hypertension

# ♥ Innocent(functional,normal,insignificant) murmur

    1) over 30% of children during routine random ausculation

    2) increase percentage under nonbasal circumstances

        ; high cardiac output due to fever, infection, anxiety

    3) *▲common innocent murmur

           ; *still murmur

           ; from 3 to 7yr of age

        ; *medium-pitched,vibratory, or "musical" relatively short systolic ejection murmur along left lower and midsternal border

           ; *attenuated in sitting or prone position

    4) innocent pulmonic murmurs

       - common in children,adolescents

       - normal turbulence during ejection into the pulmonary artery

       - high pitched, blowing, brief, early systolic murmurs(grade 1-2)

       - best detected in 2nd left parasternal space in supine position

    5) *venous hum

           - *turbulence of blood in the jugular venous system

       - no pathologic significance

       - heared in neck or ant.part of upper chest

       - soft huming sound in both systole and diastole

       - head position에 따라 악화되거나 사라짐

       - *jugular venous system을 가볍게 압박함으로서 약화됨

               /organic cardiovascular ds에 의해 발생하는 murmur와 구분 할 수있다.

Chap. 381. Laboratory Evaluation

381.1 Radiologic Assessment

# 흉부사진에서 알 수 있는 사항

 ; cardiac size and shape, pul.blood flow(vascularity), pul.edema, lung and thorax anomaly(skeletal dysplasia,extra or deficient numbers of ribs)

# 흉부사진에서 variation이 생기는 원인

  1) difference in body build

  2) phase of respiration or cardiac cycle

  3) abnormality of thoracic cage

  4) position of diaphragm

  5) pul.ds

# cardiac size 측정에 가장 흔히 사용하는 방법

 ; PA사진에서 midinsperation때 cardiac shadow의 maximal width

# maximal cardiac width

 ; sternum의 중간을 연결하는 수직선을 긋고, 이 수직선에서 직각으로 심장의 extreme

   right and left border로 선을 그어 그 두 선의 합으로 표시한다.

# maximal chest width

 ; right diaphragm의 top level에서 rib cage의 right and lett inner border를 연결한

   horizontal line

# ★Cardiac Enlargements

    ; maximal cardiac width가 maximal chest width의 1/2이상이면 심장은 enlarged

    ; *evaluation in upright and inspiration

    ; *less useful index in infancy

           - ♥why ?

                   / horizontal position of heart

                           --> increase ratio more than 50%

                   / thymus overlap entire mediasternum & heart base

# Chest PA View

   Fig 381-1

    ; heart의 left border를 구성하는 요소

       - aortic knob, main and left pul.arteries, left ventricle

    ; heart의 right border를 구성하는 요소

        - superior vena cava, ascending aorta, right atrium

# ECG

  ; more sensitive and accurate index of ventricular hypertrophy

# pl. overcirculation

   1) left to right shunts

   2) stenosis or atresia of the outflow tract of the right ventricle or of the pul.valve

381.2  Electrocardiogram(ECG)

# 출생시에는 pulmonary vascular resistance와 systemic vascular resistance는 거의

  동일하다. 출생이후에는 systemic vascular resistance가 증가하게 된다.

# ECG는 QRS and T wave morphology로서 anatomic and hemodynamic feature를

  묘사하게 된다

# right precordial lead(V3R or V4R)

  - RVH 평가에 중요

  - dominant R or S pattern을 반영함

# ♥생후 첫날의 ECG 특징

    1) RAD

  2) large R wave

  3) upright T wave in the right precordial leads(V3R or V4R and V1)

# 생후 48시간내에 대체로 pulmonary resistance는 감소되고 right ventricular pressure     는 정상에 도달하게 되어 right precordial T wave는 negative가 된다. 만일 1주일이 넘어서도 V3R and/or V1에 upright T wave가 지속되면 비정상이다.

# newborn에 있어 mean QRS axis;+110 to +180

# infancy에 있어서는 우심실이 상대적으로 뚜껍기 때문에 수개월 혹은 수년동안은 right

   sided chest leads는 larger positive (R) than negative (S) wave를 나타내게 된다.

# 세월이 흐르면 QRS axis는 왼쪽으로 이동하게 되고 right ventricular forces는 감소

   하게 된다.

# 6개월에서 8세까지는 lead V1 and V4R에서 현저한 R wave를 나타낸다.

  4세까지는 lead V4R에서 RS ratio는 1이상을 나타낸다

# infancy 동안에는 V4R,V1,V2,V3에서 inverted T wave를 나타내며, 이것은 10대중반

  혹은 그 이후까지 지속될 수 있다.

# 우심실이 얇아지고 좌심실이 뚜꺼워지는 과정은 right precordial leads의 QRS-T

   pattern에서 잘 볼 수 있다.

# ventricular hypertrophy되면 chest leads의 R and S wave의 voltage가 증가하게 된다

# physiologic right ventricular hypertrophy는 정상소견이므로 생후 일주일까지는

  pathologic  right ventricular hypertrophy의 진단은 어렵다

# Pathologic RVH

    ; physiologic RVH in neonate

           --> serial tracings이 필요하다.

    ; adult ECG pattern in neonate

           --> left ventricular enlargement

           - ★exception) premature infant - mature ECG pattern

                   / *result of lower pulmonary vscular resistance due to underdevelopment of medial muscular layer of pulmonary arterioles

※97짝짓기 P wave

1. tall (2.5mm이상), narrow, spiked P wave (P-pulmonale)

    ; *congenital pul. stenosis, Ebstein anomaly of the tricuspid valve, tricuspid atresia, cor pulmonale, thyrotoxicosis

  ; Right atrial hypertrophy and/or dilatation

  ; *obvious in lead II, V4R, V3R, V1

2. widened P wave(bifid) (P-mitrale)

  ; *large VSD with communication between the aorta and pul.circulation, severe MS

  ; Left atrial enlargement

3. flat P wave

  ; ★hyperkalemia

# normal P wave

    ; upright in lead I, AVF

    ; inverted in AVR

# ★inverted P wave in lead I, AVF

  ; atrial inversion(situs inversus) in lead I

  ; *nodal or junctional rhythm in lead I and AVF

QRS Complex

Right Ventricular Hypertrophy

# ♥RVH Criteria

    ; at least two of following

   - qR pattern in right ventricular surf. leads

   - positive T wave in leads V3-4R and V1-3 between 6days and 6yr

   - *monophasic R wave in V3-4R and/or V1

   - *rsR' in right precordial leads, often with a tall secondary R wave

   - age-related increased voltage criteria of R wave in V3-4R and/or of S wave in V6-7

   - marked RAD(>*120degrees)

   - *complete reversal of the normal adult precordial RS pattern

   - RAE

2) Right vent.systolic overload pattern

   - PS에서 볼 수 있다.PS에서 rsR' in right precordial lead 보임

   - right precordial lead에서 tall pure  R wave보이며 처음에는 upright T wave 나

     중에는 inverted T wave보임

3) Right vent.diastolic overload pattern

   - ASD에서 볼 수 있다

   - rsR' pattern and right ventricular conduction delay

Left Ventricular Hypertrophy

# ♥LVH Criteria

    ; S-T segment depression and T wave inversion in left precordial leads (V5-7)

         - left ventricular strain pattern

    - severe lesion and significant myocardial abnormality

    ; *increase in magnitude of initial forces to the right (i.e., deep Q in left precordial leads)

    ; voltage criteria in V3R & V1(S) and/or V6-7(R)

2) severe systolic overload of the left ventricles 소견

 a) straightening of the ST segment

 b) inverted T wave over the left precordial leads

3) diastolic overload 소견

 a) tall R wave,large Q wave,normal T waves over the left precordium

Q-T Interval

1) cardiac rate에 따라 변화

2) normal Q-TC < 0.45 sec

# ♥Prolonged Q-Tc Interval

  ; hypokalemia

  ; hypocalcemia

    ; *Jervell-Lange-Nielsen syndrome or Romano-Ward syndrome

        - high risk of ventricular arrhythmia such as torsade de pointes

        - sudden death

           - *mutation in Harvey ras-1 gene, encoding G protein

ST Segment And T Wave Abnormalities

1) normal teenengers에서 ST segment elevation은 심장의 repolarization을 유발

2) pericarditis에서 superficial epicardial involvement는 abnormal T wave inversion후에

   ST segment elevation을 유발한다

3) Digitalis 투여 효과

  - sagging of ST segment,abnormal T wave inversion

4) Depression of ST segment

  - muocardial damage를 유발하는 상황에서 발생

  - anemia, CO poisoning, endocardial fibroelastosis, aberrant origin of the left 

    coronary a. from the pul.a., glycogen storage disease of the heart, myocardial

    tumors, mucopolysaccharidoses.

5) carditis에서는 T wave inversion을 보임

6) hypothyroidism

   - flat or inverted T waves,generalized low voltage

7) hyperkalemia(Fig.381-14)

   - high voltage,tent shaped T waves

381.3 Hematologic Data

# acyanotic infants with large Lt-to-Rt shunts

    ; CHF coincides with nadir of normal physiologic anemia of infancy

           --> Tx : increasing Hct > 40%

# Persistent Polycythemia

    ; *▲frequent abnormalities

       - accelerated fibrinolysis

       - thrombocytopenia

       - abnormal clot retraction

       - hypofibrinogenemia

       - prolonged PT,PTT

    ; Complication

           - *vascular thrombosis esp. cerebral veins

           - ♥Predisposings Factors

                   / dehydration

                   / IDA

    ; Treatment

           - *epsilon-aminocaproic acid

                   / suppress fibrinolysis

           - correction of predisposing factors of CVA

           - phlebotomy

# Hematologic F/U of cyanotic patients

    ; increasing polycythemia, asso. with headache, fatigue and/or dyspnea

           --> indication of palliative or corrective surgical intervention

    ; *phlebotomy, if Hct 65-70%   

           - replacement of fresh frozen plasma or albumin

           - desired level : 60%

           - a week until desired level and then at interval of only 3-5wks

381.4 Echocardiography

# ※82,87 기능

  1) cardiac contractility(performance)

  2) gradients across stenotic valves

  3) direction of flow across a shunt

  4) patency of coronary arteries

  5) prescence of vegetations due to endocarditis

  6) prescence of pericardial fluid,cardiac tumors,chamber thrombi

  7) prosthetic valve function

  8) septal hypertrophy

  9) aortic root dimensions

  10) affects of cardiotonic or cardiotoxic drugs

  11) assist in performance of pericardiocentesis

M Mode Echocardiography

; ★Use

    - *identifies dimensions & motion of intracardiac structure

           /opening, closing of valves, movement of septa

    - anatomy of valves

    - *presence of endocarditis vegetations larger than 2-3mm

    - presence or absence of individual structure and their relationships

    - cardiac function 

Two-Dimensional Real-Time Imaging

; better,more coherent,realistic image of cardiac structures

; technique of choice for diagnosing structural heart disease

; superior to angiography in several areas

    - *imaging AV valves and their chordal attachments

Doppler Echocardiography

; ★Use

    - identifies blood flow than morphology

    - *estimate systemic or pulmonary blood flow, pressure

; color doppler

    - *presence & direction of intracardiac shunt

Transesophageal Echocardiography

; ★Use

    - *clearer view of smaller lesion such as vegetation in endocarditis

    - *visualize posteriorly located structures

           / atria, aortic root, atrioventricular valves

    - intraoperative monitoring of cardiac function

    - intraoperative screening for residual cardiac defects after cardiopulmonary bypass

Fetal Echocardiography

Overview

; *ASD or PDA등은 cardiac cath.를 하지 않고 2-D and doppler echo.만으로도 수술

381.5 Exercise Testing

# 기능

  1) evaluating symptoms

  2) quantitating the severity of cardiac abnormality

  3) assisting in the management of patients

# Bruce protocol로 시행

# exercise에 대한 주 반응은 heart rate,stroke volume,systemic venous return,pul.pr.의

  증가로 인해 cardiac output이 증가하며, 또한 systemic vascular resistance가 감소

  한다

# 정상아에 있어 exercise동안에 ECG의 변화는 P-R interval의 감소(심박수 증가)이다

# ※Abnormal Exercise ECG

    ; *ST segment depression > 2mm & extends for at least 0.06sec after J point in conjunction with a horizontal-, upward- or downward-sloping ST segment

# ※87 Indication

    1) LV outflow obstruction

    - valvular,subvalvular,supravalvular aortic stenosis

    - hypertrophic cardiomyopathy

    - CoA

    2) chronic vol. overload of the left or right ventricle

       - atrioventricular or semilunar valve incompetence

       - left to right shunts

  3) arrhythmia

  4) hypertension

    5) patients who have undergone open heart surgical correction of complex congenital heart lesions

           - fontan op.

# ♣Indication For Termination Of A Exercise Test

  1) failure or inadequacy of the ECG monitoring

  2) onset of serious arrythmia

  3) premature beats(more than 25% of beats) precipitated or aggrevated by exercise

  4) development of heart block

  5) precipitation of pain,headache,dizziness,syncope

  6) ST segmental depression or elevation of 3mm or more

  7) inappropriate hypertension

    - syst. pr. > 230mmHg

       - dias. pr. > 120mmHg

  8) inappropriate fall of BP

  9) development of cutaneous vascular insufficiency(e.g.,pallor)

  10) severe fatigue

381.6 Magnetic esonance Imaging(MRI) and Radionuclide Studies

# gay-scale intensity에 관계하는 요소

  ; concentration,motion,and chemical microenvironment of hydrogen nuclei

# excellent contrast resolution of fat,myocardium,lung,moving blood from blood vessel

  wall을 얻을 수가 있다.

# 진단적 가치가 있는 질환

  1) malformation of great vessels

   - COA, proximal brench pul.a.stenosis, TGA

  2) simple and complex cardiac malformations

   -AS, PS, ASD, VSD, TOF, single ventricle,inversion of the ventricles

# 종류

  1) cine MRI

   - wall thickening,chamber volume,valve function의 변화를 나타냄

  2) phosphorous MR spectroscopy

   - high-energy metabolites(ATP,ADP,Pi,phosphocreatine)의 상대적 농도를 묘사함

     으로써 영상을 얻게된다

# Radionuclide angiography

  ; detect and quantify shunts

  ; analyze distribution of blood flow to each lung

# ★Gated Blood Pool Scanning

  ; calculate hemodynamic measurements

  ; quantify valvular regurgitation

  ; detects regional wall motion abnormalities

# ★Thalium Imaging

  ; cardiac muscle perfusion

381.7 Cardiac Catheterization

# ♥Major Indication

    1) presurgical evaluation of cardiac anatomy and shunt size

    2) evaluation of pulmonary vascular resistance and its reactivity to vasodilators or oxygen

    3) F/U after surgical repair or palliation of complex CHD

    4) myocardial biopsy for diagnosis of cardiomyopathy or screening for cardiac rejection after trnsplantation

    5) interventional cardiac catherization

    6) electrophysiologic study and/or transcatheter ablation

# *avoidance of deep anesthesia

    ; distort calculation of hemodynamic measurements

           - cardiac output, pul. and systemic resistance, shunt ratio

# postangiographic care

    ; thermally neutral environment, correction of hypothermia, acidemia, excess blood loss

# ♥Complication

    1) severe arrhythmia

    2) cardiac perforation

    3) intramyocardial injection of contrast material

           ; soft, flow-directed ballon-tipped catheters의 개발로 빈도가 감소됨

    4) anaphylaxis

           ; nausea, generalized burning sensation, CNS symptoms, allergic rashs

Indicator Dilution and Appearance Technique

# indicator meterial을 heart의 right side 혹은 I.V로 주입하면 pul.circulation을 거쳐heart left side로 들어가 arterial circulation을 하게 되는데 이러한 indicator material은 arterial circulation에서 detect된다

# A contious record of the circulation of indicator in normal subjects shows two peaks

    a. 1st peak는 passage of indicator past the arterial detectors에 의해 발생

  b. 2nd peak는 recirculation through the systemic arterial and venous system,the pul. circulation, reappearance in the arterial tree에 의해 발생

# thermodilution method

  ; cardiac output측정을 위해 가장 흔히 사용하는 indicator dilution technique

  ; dye dilution과 같이 사용하게 되면 diseased mitral or aortic valves를 가로지르는 regurgitant volume을 측정할 수 있다

★Table 381-2

Fig. 381-23

Fig. 381-24

Angiocardiography

1) intramyocardial injection은 피한다

2) hypertonic contrast medium 부작용

    ; tansient myocardial depression, drop in BP, tachycardia, cadiac output증가, shift of interstitial fluid into the circulation

3) idealized diagrams of the normal angiocardiogram

Interventional Catheterization

# balloon angioplasty로 치료가 가능한 질환

    ; valvular PS, AS, restenotic of CoA after early surgery, amelioration of MS of subaortic stenosis, dilatation of surgical conduits(atrial baffles), relief of branch pul. a. narrowing, dilatation of venous obstruction, long utilized balloon atrial septostomy for TGA, obliteration of temporary A-V shunts as well as pul. collateral vessels, PDA, secundum ASD

section 2. the transitional circulation

Chap 382. Fetal And Neonatal Circulation

Fetal Circulation

# three CV sturcture for maintaining parallel circulation

    ; ductus venosus, foramen ovale, ductus arteriosus

# placental oxygenation ; *PO₂ 30-50mmHg

    --> umbilical vein --> IVC via ductus venosus (*PO₂ 26-28mmHg) --> RA

    --> hepatic circulation (*50%)

    --> RA

    --> foramen ovale

    --> LA

    --> LV

    --> ascending aorta

# fetal SVC blood ; *PO₂ 12-14mmHg

    --> RA

    --> RV

    --> pulmonrary artery --> pulmonary circulation (*10%)

    --> ducturs arteriosus : PO₂ 18-22mmHg

    --> descending arota --> umbilical artery (*65%) --> placenta

    --> fetal organ

4) Rt ventricular output은 Lt ventricular output 의 1.3배.

   Total cardiac output(combined both ventricular output) : 450ml/kg/min 

5) effective fetal cardiac output

   ; sum of left ventricular output and the ductal flow

   ; 220mL/kg/min

   ; 65%는 placenta로 return하고, 나머지 35%는 fetal organ and tissues로 perfusion된다.

6) left ventricular output 구성요소

   ; mixture of venous return from IVC,foramen ovale,left atrium,minimal pul.venous          return

7) fetal life 동안에는 right ventricle이 dominant하기 때문에 right ventricle output이 left

   ventricular output보다 50%정도 크다.

Transitional Circulation

Neonatal Circulation

# ♥Neonatal Circulation과 Older Infancy와의 차이점

    ; Rt-to-Lt or Lt-to-Rt shunting across foramen ovale

    ; in presence of cardiopulmonary disease, Lt-to-Rt, Rt-to-Lt or bidirectional shunting across ductus arteriosus

    ; neonate pul. vasculature - more vigorouly constriction in response to hypoxemia, hypercapnea, acidosis

    ; almost equal of Rt. and Lt.ventricle muscular mass & wall thickness

    ; high oxygen consumption asso. with high cardiac output

           - *350ml/kg/min

           - cf.) 150 ml/kg/min by 2mo, gradually 75 ml/kg/min of adult level

# ★Functional Closure

  ; foramen ovale

           - 3rd month

  ; ductus arteriosus

           - 10-15hrs

# ductus arteriosus closure에 영향을 미치는 인자

    ; ★Oxygen

           - ▲important

    - *PO2 > 50mmHg

           - direct or mediated by its effect on PG synthesis

    ; gestational age

           - less response to oxygen

Chapter 383. Persistence Of Fetal Circulatory Pathways

# pul.a.pr.= pul.blood flow # pul.vascular resistance (P=F#R)

Persistent Fetal Circulation

(=Persistent Pulominary Hypertension Of The Newborn)

 ; hypoxemia후에 pul.vasoconstriction and hypertension이 오면 Rt. to Lt. patent foramen

   ovale and ductus arteriosus shunting이 발생

Pul.Venous Hypertension

을 유발하는 질환

  (생후 첫 수일내에 pul.venous obstruction을 유발할 수 있는 질환)

 ; stenosis of pul.veins,cor triatriatum,congenital MS,supravalvular webs.

Pul. A. Hypertension

을 유발하는 질환

 ; Lt.ventricular failure,COA,aortic valve disease,cardiomyopathy

Hyperviscosity Syndrome

 ; maternal-fetal or fetal-fetal transfusion 혹은 perinatal hypoxemia에 의한 polycythemia

  가진 환아에서 잘 발생

Persistence Of Fetal Circulation

 ; pul.vascular constriction이 있으면서 parenchymal pul.disease or cardiac lesion이 없는      경우

# decreased pul.vascular bed

 1) elevated pul.resistance and persistent pul.hypertension of newborn을 유발   

 2) 유발할 수 있는 경우

  - congenital pul.hypoplasia

  - diaphragmatic hernia

  - space occupying intrathoracic masses

# systemic Rt.ventricles or single ventricles을 가진 환아는 pul.hypertension과 medial

   muscular hypertrophy of small pul.vessels을 유발하게 된다.

# anatomic and physiologic abnormalities이 동반된 perinatal hypoxemia는 복합적 원인의    persistent pul.hypertension을 유발하게 된다. 예를 들어 diaphragmatic hernia를 가진 환    아는 ipsilateral pul.hypoplasia and contralateral pul.vasoconstriction을 지니게 되는데 이     두 가지는  high pul.resistance,hypertension,Rt.to Lt.shunting을 유발하게 된다.

# severe RDS를 가진 미숙아가 cyanotic한 이유

  1) pul.vasoconstriction

  2) pul.hypertension

  3) Rt.to Lt.ductus arteriosus

  4) foramen ovale shunting in the first few days of life

section 3. CONGENITAL HEART DISEASE

Chapter 384. Epidemiology Of Congenital Heart Disease

 

Incidence

    ; 8/1000 live births

    ; stillborns(2%), abortuses(10-25%), premature infants (about 2% including VSD, excluding tansient PDA)에서 높다.

3) 1000명당 2-3명은 1년내에 증상을 나타낸다.

4) 진단은 1주일째 되는 경우가 40-50%, 1달   되는 경우가 50-60%이다.

5) pul.vascular resistance는 생후 1주일   감소하고,Lt.to Rt.shunts는 보다 현저해진다. 여     러가지 defects는 성장에 따라 변화가 생기는데, 예를  들어 large VSD는 성장에 따라      small communication이 되고, aortic or pul.valve stenosis는 성장에 따라 valve orifice가     커지지 아니하면 오히려 더 악화된다.

Etiology

1) genetic factors

  ; supracristal VSD는 동양인에 많다

2) single gene defect

  ; 3%

  ; Marfan or Noonan syndrome

3) chromosome anomalies

  ; 5-8%

4) environmental or adverse maternal conditions and teratogenic influences

  ; 2-4%

    ; maternal DM, phenylketonuria, SLE, congenital rubella syndrome, drugs (lithium, ethanol, thalidomide, anticonvulsant agents)

5) ★Polygenetic Or Multifactorial inheritance

  ; mostly

㉿표 15-3, 15-4, 15-5 (p540-541)

Genetic Counseling

Chapter 385. Evaluation Of The Infnat Or Child With Congenital Heart Disease

Acynotic Congenital Heart Lesions

- most common lesion : volume overload에 의한 것, 주로  Lt to Rt shunt lesion

- second most common lesion : pressure load에 의한 것,

   1) PS, aortic valve steenosis ( ventricular outflowobstruction에 의한 것 )  

   2) coactation of aorta ( great vessel의 한군데가 좁아진 것에 의한 것)

Lesions Resulting In Increased Volume Overload

- m/c : Lt to Rt shunt를 유발하는 병변 --> ASD, VSD, AVSD, PDA

Lesions Resulting In Increased Pressure Overload

- m/c : obstruction to ventricular outflow --> valvar pulmonary stenosis, valvar aortic stenosis, CoA

- less common lesion --> tricuspid stenosis, mitral stenosis, cor triatrium

- ventricular outflow obstruction의 level에 따라

  ① below the valve : double-chambered Rt ventricle, subaortic menbane

  ② above the valve : branch pulmonary stenosis, supravalvular aotic stenosis       

Cyanotic Congenital Heart Lesions

Cyanotic Lesions With Decreased Pulmonary Blood Flow

- obstruction to pulmonary blood flow(at the tricuspid valve, Rt. ventricle, pulmonary       valve level) : tricuspid atresia, single ventricle with pulmonary stenosis, TOF

- Rt to Lt shunt lesions : PFO, ASD, VSD

Cyanotic Lesions With Increased Pulmonary Blood Flow

- pulmonary blood flow obstruction은 없이

  ① 비정상적인 ventricular-arterial connection이 있는 경우 : TGA

  ② heart내에서 systemic venous, pulmonary venous blood의 systemic venous,

     pulmonary  venous blood의 total mixing으로 인해 cyanosis가 일어나는 것

     : cadiac defect with a common atria or ventricle

       TAPVR, truncus arteriosus

Chapter 386. Acynotic Congenital Heart Disease

THE LEFT-TO-RIGHT SHUNT LESIONS

386.1 ASD

; occur in any portion of atrial septum

    - secundum, primum, sinus venosus

386.2 Ostium Secundum Defect

# 남:여=1:3

# associated lesion

    ; *partial anomalous venous return

Pathophysiology

# ※81♥Lt-to-Rt shunt 결정 요소

    ; size of the defect

    ; relative compliances of the Rt. and Lt. ventricles

    ; relative vascular resistances in the pulmonary and systemic circulations

# large defects

    ; pulmonary blood flow 2-4 times systemic blood flow

    ; in infancy, paucity of symptom

           - why ?

                   / *RV is thick and less compliant

  <5> infant가 성장할수록 right ventricular wall이 얇아져 left to right shunt가 증가한다

  <6> large pul.blood flow에도 불구하고 pul.a.pr.는 normal로 유지한다. 왜냐하면 pul.vascular

      resistance가 극히 낮기 때문이다

  <7> LV and aorta;normal size

  <8> cyanosis;극히 드물고 pul.vascular disease가 동반된 성인에서 볼 수 있다.

Clinical Manifestations

; *most often aymptomatic

    - discovered inadvertently during P/E

; normal pulse

; right ventricular systolic lift

    - palpable from left sternal border to midclavicular line

# Å청진소견

    ; loud 1st heart sound & sometimes pulmonary ejection click

    ; ㉿widely fixed splitting 2nd heart sound

    ; ejection type systolic murmur

        - medium pitched, without harsh qualities, seldom thill

           - best heard in left mid. and upper sternal border

           - *produced by increased blood flow across RVOT

    ; short rumbling mid-diastolic murmur

           - *produced by increased volume of blood flow across TV

           - audible at lower left sternal border

           - best with bell of stethoscope

           - *excellent diagnostic sign of shunt ratio of at least 2:1

Diagnosis

# X-ray

  ; Shunt 양에 따라 RA, RV의 enlargement가 다양하다.

  ; LV and aorta ; normal size

  ; *large pulmonary artery & increased pulmonary vascularity

  ; RV cardiomegaly

           - best seen on lat.view

       - due to ant. protrusion of RV enlargement

# EKG

  1) volume overload of RV with RAD or normal axis

  2) minor right ventricular conduction delay (rsR' in right precordial leads)

# ★Echcardiogram

    ; right ventricular volume overload

           - increased right ventricular end-diastolic dimension

           - *paradoxical septal motion

# catheterization

    ; O2 content

           - *RA >SVC

           - not diagnostic

       - ★DDx

                   / partial anomalous pul.venous return to RA

             / VSD and tricuspid insufficiency

             / atrioventricular septal defect associated with left ventricular-right atrial shunts

             / aortic-right atrial communication (eg.ruptured sinus of Valsalva)

    ; indicator dilution curves

       - left to right shunt의 위치와 anomalous pul.v.의 존재를 밝힌다.

 3) right sided heart pr.;normal

 4) pul.a.resistance;normal or lower than normal

 5) shunt volume ; as high as 20 l/min/m2

Prognosis And Complications

<1> childhood때에는 well tolerable

<2> 증상은 3rd decade 혹은 그 이후에 나타난다.

<3> late manifestation

     1) pul.hypertension

     2) atrial dysrhythmias

     3) tricuspid or mitral incompetence

     4) heart failure

<4> 임신 중에도 증상이 나타날 수 있다.

<5> infectious endocarditis;extremely rare

<6> 20세 이후에 수술했었던 환자에서 post op. Cx.으로서 late heart failure와 atrial fibrillation

     이 more common

<7> 동반되는 질환

     1) partial anomalous pul.venous return

     2) pul.valvular stenosis

     3) VSD

     4) pul. a. branch stenosis

     5) persistent left SVC

     6) mitral valve prolapse and insufficiency

Treatment 

# surgical operation

    ; *symptomatic or shunt ratio > 2:1

    ; op. mortality < 1%

    ; prior to entry into school

 <3> pregnancy시 risk증가

 <4> early surgical repair로서 운동시에 발생하는 mild symptom을 예방할 수 있다.

 <5> large shunt라도 수술후 결과는 좋다.

 <6> 수술후 결과

      1) 증상이 신속히 사라짐

      2) physical development가 좋아짐

      3) heart size는 정상적으로 감소됨

      4) EKG;decreased right ventricular forces

 <7> late arrhythmias;less frequent and less important

386.3 Sinus Venosus Defect

  # SVC 입구에 근접하여 atrial septum의 upper part에 위치한다

  # 1개 이상의 pul.v.(usually from right lung) SVC로 drain된다

  # 때때로 SVC는 defect에 걸쳐서 위치하여 systemic venous blood는 LA로 들어가게 된다.

  # abnormal hemodynamics(RV의 volume overload)는 secundum ASD와 유사

  # DX. ; two-dimensional echocardiography

  # catheter는 SVC에서 pul.v.으로 유입

  # 증상,EKG,X-ray는 secundum ASD와 유사

386.4 Partial Anomalous Pulmonary Venous Return

# more often right lung origin pulmonary vein

# associated ASD

    ; *sinus venosus type

# ★Scimtar syndrome

    ; *anomalous pul. v. draining into IVC

           --> crescentric shadow of vascular density along the right border of cardiac silhouette

    ; usually not present ASD

# prognosis ; excellent

386.5  Atrioventricular Septal Defect (Ostium Primum And Av Canal Or  Endocardial Cushion Defects)

# Classification

    1) ostium primum defect

        ; lower portion of atrial septum & overlies MV and TV

           ; *mostly MV ant. leaflet cleft

    ; TV - functionally normal

       ; intact ventricular septum

    2) A-V septal defect (=AV canal defect, endocardial cushion defect)

       ; contiguous atrial and ventricular septal defect with markedly abnormal A-V valves

       ; variable valve abnormalities

            - *complete form : single AV valve

                           / common to both ventricle

                           / *ant. post. bridging leaflet with lat. leaflet

                     / *common in Down syndrome

                           / *occasionally with PS

    3) Transitional Varieties

           ; 종류

    - ostium primum defect with clefts in the ant. mitral and septal tricuspid valve leaflets, mild ventricular septal deficiencies

        - ostium primum defects with normal A-V valves

           - atrial septum ; intact

Pathophysiology

# ostium primum defect

    1) left to right shunt across the atrial defect with mitral incompetence

    2) shunt;moderate to large

    3) degree of mitral insufficiency; mild to moderate

    4) pul.a.pr.;normal or only mildly increased

# A-V canal

    1) left to right shunt

      ; both transatrial and transventricular

    2) pul.hypertension and increased pul.vascular resistance

    3) A-V valvular,incompetence로 인해 ventricle에서 both atrium으로 blood가 regurgitated

    4) 때로 both atrial and ventricular level에서 right to left shunt가 발생

    5) mild,significant arterial unsaturation

    6) progressive pul.vascular disease는 right to left shunt를 증가시켜 cyanosis를 유발한다

Clinical Manifestations

# ostium primum defect

    1) 대개는 asymptomatic하고 anomaly는 P/E때 발견된다

    2) moderate shunt and trivial mitral incompetence가 있으면 physical sign은 secundum

       type과 유사

    3) apical systolic murmur

    4) large left to right shunts and severe mitral incompetence가 있는 경우

      (1) effort intolerance, easy fatigability, recurrent pneumonitis 나타남

      (2) cardiomegaly

      (3) hyperdynamic precordium

    5) left to right shunt에 의한 청진소견

      (1) normal or accentuated S1

      (2) wide,fixed splitting S2

      (3) pul.ejection systolic murmur

      (4) low-pitched early diastolic murmur at LLSB or apex

    6) mitral incompetence가 있으면 left axilla로 radiation하는 apical pansystolic murmur 발생

# A-V canal

    ; CHF, intermittent pul. infection

    ; minimal cyanosis

  ; epatomegaly, prominent neck vein, failure to thrive, cardiomegaly, palpable systolic thrill

    ; 청진소견

      (1) S1 ; normal or accentuated

      (2) S2 ; pul.flow가 massive하면 widely split

      (3) low pitched mid diastolic rumbling murmur(lower sternal edge)

      (4) pul.systolic ejection murmur; large pul.flow에 의해 발생

      (5) mitral insufficiency시 apical holosystolic murmur발생

Diagnosis

# X-ray

    1) cardiomegaly;both ventricles and RA의 prominence에 의해 발생

    2) pul.a.;large

    3) pul.vascularity;증가

# ※EKG

    1) sup. orientation of the mean frontal QRS axis with LAD to the left or right upper quadrant

           ; *▲important

    2) counter-clockwise inscription of the superiorly oriented QRS vector loop

  3) signs of biventricular hypertrophy or isolated RVH

    4) *right ventricular conduction delay (RSR’ in leads V3R and V1)

    5) normal or tall P waves

  6) occasional prolongation of the P-R interval

# echocardiogram

    1) RVH

    2) encroachment of the MV echo on the left ventricular outflow

# catheterization and angiocardiography

    ; selective left ventriculography

        1) deformity of mitral or common A-V valve

    2) distortion of the outflow of the left ventricle

    3) *goose-neck deformity

    4) MV의 abnormal ant.leaflet;serrated

    5) mitral incompetence

    6) regurgitation of blood to both the left and right atrium

Prognosis

# ★결정 요소

    1) magnitude of left and right shunt

    2) degree of pul.vascular resistance

    3) severity of mitral incompetence

# infancy때 op.하지 않으면 CHF로 종종 사망함

# op.하지 않고 생존하고 있는 환자는 pul.vascular obstructive disease가 잘 발생

# 대개 환자는 무증상이거나 3rd-4th decade가 될 때까지 minor, nonprogressive symptom이 나타난다.

Treatment

<1> surgical mortality; low

<2> CHF and pul. hypertension이 있으면 수술이 보다 어렵다.

<3> Pul. a. bending

386.6  Ventricular Septal Defect

; *▲common cardiac malformation - 25%

# 대개는 membranous type

  동양인에서는 supracristal type(30%)이 흔하다

# defects between the crista supraventricularis and the papillary muscle of conus

  ; PS and TOF와 동반됨

# defects superior to the crista supraventricularis

  <1> less common

  <2> pul.valve 바로 밑에 위치

  <3> aorta sinus에 침범하여 aortic insufficiency 유발

# defects in the midposition or apical region of the ventricular septum or apical area

  <1> muscular type

  <2> single or multiple (Swiss-cheese type)

Pathophysiology

; small defect (restrictive)

    - *< 0.5 cm2

    - *pul. to systemic flow ratio < 1.75:1

    - normal cardiac chamber and pul.vascular bed

; large defect (nonrestrictive)

    - *> 1.0cm2

    - *flow ratio > 2.5:1

    - LV volume overload, RV and pul. a. hypertension

    - LA and LV enlargement & large pul.a.trunk

; 출생후 large VSD가 있는 경우

  - 처음에는 normal infant에 비해 pul.resistance가 비교적 높아 left to right shunt가 limited

  - 수주이내에 pul.a.& arterioles의 muscular media가 normal involution함에 따라 large left to right shunt and clinical symptom이 나타남

<4> large VSD있는 some patients에서는 medial thickness가 시간이 흐름에 따라 intimal

     arteriolar pathologic change가 발생하여 right to left shunt가 발생 ( Eisenmenger

     syndrome ) 그러나 large VSD 있는 대부분의 환자에서는 massive left to right shunt가 발생

# Hemodynamics

  ; ★left to right shunt의 정도를 결정하는 요소

        - size of defect

    - systemic resistance와 비교하여 pul.vascular resistance의 정도

  2> 대개 pul.resistance는 약간 증가

  3> pul. hypertension 유발요소

      ; pul.a. and heart right side를 통한 extremely large blood flow

  4> small communication인 경우

      1) defects;restictive

      2) RV ; normal

Clinical Menifestation

; varies according to defect size and pul.blood flow and pressure

# small defects with trival left to right shunts and normal pul. a. pr.

    ; *▲common

  ; asymptomatic

  ; 청진 소견

           - *loud, harsh, or blowing left parasternal holosystolic murmur

           - *frequently accompanied by thrill

    ; prematurity에서 murmur가 빨리 들리는 이유

    - pul.vascular resistance가 more rapidly decrease

    ; X-ray

        - normal or minimal cardiomegaly

        - border increase in pul. vasculature

  ; EKG

     - normal

# Large Defects With Excessive Pul. Blood Flow And Pul. Hypertention

    ; dyspnea, feeding difficulty, poor growth, profuse perspiration, recurrent pul. infection, cardiac failure

    ; *cyanosis (-)

         - sometimes duskiness during infetion or crying

    ; *prominent left precordium and sternum --> cardiomegaly

    ; palpable parastenal lift, apical thrust, systolic thrill

    ; 청진 소견

           - holosystolic murmur

                   / less harsh more blowing

           - accentuated P2

           - *mid-diastolic low-pitched rumble at apex

                   / increased blood flow across MV

                   / *indicate Lt to Rt shunt 2:1 or greater

    ;  X-ray

       - gross cardiomegaly

       - prominence of both ventricles, LA, pul.a.

    ; EKG

        - *biventricular hypertrophy

       - P wave ; norched or peaked

Diagnosis

# two dementional echocardiogram

  ; volume overload of LA and LV

# catheterizaton

  ; RV가 RA보다 O2 content가 높다.

     2) small defect associated with

        ; normal Rt. sided heart pressure and pul. vascular resistance

     3) 동일한 pul. and systemic pr. 가진 large VSD 환아에서 pul. bl. flow는 systemic blood

        flow보다 3배 이상이다.

Prognosis And Complication

# Small VSD

    ; *30-50% spontaneous closure during 1st yr

           - *대부분 4세이전에 closure

    ; asymptomatic without evidence of a increase in heart size, pul. artery pressure, resistance

    ; infective endocarditis

           - fewer than 2%

           - *rare in 2yr, more common in adolescent

           - *independent of VSD size

# Moderate to large VSD

    ; less common spontaneous closure

    ; *repeated episodes of respiratory infection & CHF

    ; pulmonary hypertension

           - result of high pulmonary blood flow

           - *risk of developing pul. vscular disease if not repaired

# Acquired infundibular stenosis

Treatment

# Small defects

# Large defects

    1) medical management

           ; *control of CHF, prevention of pul. vascular disease

    2) surgical management

           ; symptomatic infants

           ; *asso. with pul. hypertension

                   - *surgery electively 6 - 12mo or earlier if symptom

left to right shunt가 사라지고 나면

 ① quiet heart

 ② heart size는 정상적으로 감소

 ③ thrills and murmurs 사라짐

 ④ pul. a. hypertension regress

 ⑤ 임상 증상의 현저한 개선

㉿Table 386-1

386.7  VSD With Aortic Insufficiency

 # VSD 환자의 5%에서 발생됨. 동양인에게서 많다.

 # VSD ; small and moderate size ant. and subpulmonary ( outlet septum )

         때때로 infracristal

 # AI는 1st decade말 혹은 그 이후에도 잘 발견되지 않는다.

 # early CHF는 잘 발생하지 않으나 수술하지 않으면 severe AI and LV failure가 발생한다

 # DDx

    ① PDA

    ② other defects associated with aortic runoff

 # Clinical manifestations

    ① asymptomatic child에서 trivial AR and small left to right shunt

    ② symptomatic adolescent에서 floride aortic incompetence and massive cardiomegaly

Treatment

    ; supracristal VSD(=subarterial juxta-arterial VSD)

           - *진단 당시 수술

386.8 PDA

; pul.vascular resistance가 감소한 이후에도 출생 후 ductus가 닫히지 않는 경우,

   aortic blood가 pul. a.로 shunting됨

; congenital rubella syndrome때 호발

; 남:여 = 1:2

; ★Term Infant

    - deficiency of mucoid endocardial layer and muscular media of ductus

           --> *no spontanous closure if persisting beyond 1wks

; premature infant

    - normal structural anatomy of ductus

           --> *mostly spontaneous closure if not early pharmacologic or surgical intervention

; CHF의 10%

; isolated PDA는 high altitude에서 출생한 어린이에 흔하다

Pathophysiology

  <1> high aortic pr.의 결과로서 aorta에서 ductus를 통해 pul.a.로 blood flow가 발생한다

  <2> shunt extent를 결정하는 요소

       1) ductus size

       2) ratio of pulm. to systemic vascular resistance

  <3> 심한 경우에는 LV output의 70%가 diastole동안에 ductus를 통해 pul.circulation으로

       shunting -> wide pulse pressure가 발생

   <4> small PDA인 경우에는 pul. a., RV, RA의 pr.는 정상. 그러나 large PDA인 경우에 pulm.

       a. pr.는  중등도  혹은 systemic level까지 증가하게 된다.

  <5> total blood volume 증가

Clinical Manifestations

# small PDA

    ; asymptomatic

# large PDA

       1) retardation of physical growth( 주증상 )

       2) left ventricular failure

       3) wide pulse pr. -> 두드러진 bounding arterial  pr.

       4) moderately or grossly enlarged heart

# 청진 소견

    ;  apical impulse;prominent

  ; thrill

       1) 2nd left intercostal space에서 잘 느껴짐

       2) radiate toward left clavicle,down left sternal border,toward apex

  ; classic murmur

        - machinery,a humming top,a mill wheel,rolling thunder in quality로 묘사됨

       -> S1이 onset하고 난 후에 시작되어 end-systole때 maximal intensity에 도달된 후 late diastole때 작아진다

       - localized to 2nd left intercostal space 또는 radiate down the left sternal border or left clavicle

    ; *increased pul. vascular resistance

           --> *less prominent or absent diastolic component of murmur

cf) large left to right shunt가 있으면 MV를 가로지르는 large blood flow에 의해

          low-pitched mitral diastolic murmur가 발생

# EKG

       1) small PDA;normal

       2) large;left ventricular or biventricular hypertrophy

# X-ray

       1) prominent pul.a.

       2) intrapul.vascular marking;증가

       3) involved chamber;LA and LV

       4) aortic knob;normal or prominent

# echocardiography

       1) small PDA ; cardiac chamber는 normal

       2) left atrial and ventricular dimension;증가

       3) isovolumic contraction time;감소

       4) aortic runoff in diastole

# catheterization

       1) RV and pul.a.pr.;정상 혹은 증가

       2) pul. a.로 oxygenated blood가 shunting되면 left to right shunt가 있음을 증명

       3) vena cava,RA,RV;normal oxygen content

       4) catheter는 ductus를 통해 descending aorta로 간다

       5) ascending aorta로 dye를 주입하면 aorta로부터 pul.a.의 opacification을 볼 수 있다.

Diagnosis

# ★DDx

    ; venous hum

    ; aorticopulmonary window defect

    ; sinus of valsalva aneurysm that has ruptured into the Rt. side of heart or pulmonary artery

    ; coronary artery fistulas

    ; aberrant left coronary artery with massive collaterals from Rt. coronary artery

    ; Truncus arteriosus with torrential pulmonary flow, pul. branch stenosis

           - normal pulse pressure

    ; peripheral arteriovenous fistula

           - distinctive murmur from PDA

    ; VSD with AI, combined rheumatic aortic and mitral insufficiency

           - distinctive murmur from PDA

Prognosis & Complication

  <1> small PDA는 정상 수명을 살 수 있다

  <2> infancy를 지나서 spontaneous closure는 극히 드물다

# ★Complications

    ; CHF

           - *▲often in early infancy

  ; infective endocarditis

           - *at any age

    ; pul. or systemic emboli

    ; rare

           - aneurysmal dilatation of pul. artery or ductus, calcification of ductus, non-infective thrombosis of ductus with embolization, paradoxic emboli, pul. hypertension( Eisenmenger syndrome )

Treatment

# surgical operation

    ; irrespective of age

    ; *case fatality < 1%

           --> *operation before 1yr

  ; *pul.hypertension이 있어도 reverse shunt가 없으면 수술할 수 있다.

# ligation and diversion

386.9  Aorticopulmonary Window Defect

; ascending aorta와 main pul.a.사이의 communication

; *pul. and aortic valve가 존재하며 ventricular septum은 intact

    - DDx with truncus arteriosus

# Symptom

  <1> large VSD and PDA appearing early infant와 유사

  <2> recurrent pul.infection,CHF,minimal cyanosis

# defect;large

# 청진 소견

  <1> systolic with a mid-diastolic rumbling murmur

      - MV을 가로지르는 increased blood flow에 의해 발생

# Sign

  <1> PDA와 유사

  <2> wide pulse pr.,cardiac enlargement,continuous right and left upper sternal border

       systolic murmur

# EKG

  ; left or biventricular hypertrophy

# X-ray

  <1> cardiac enlargement

  <2> prominence of pul.a. and intrapul.vascularity

# echocardiogram

  ; large volume left sided heart chamber, and the window

# catheterization

  <1> pul.a.level에서 left to right shunt

  <2> hyperkinetic pul.hypertension

  ; large defect에 의해 발생

# Tx.

  ; infant때 cardiopul.bypass

386.10 Coronary Artery Fistula

# coronary a.와 atrium,ventricle(esp.right),pul.a.사이에 congenital fistula가 존재하는 것

# coronary a.에서 blood가 right sided heart로 향할 때 atrial or ventricular level에서 단지

  small left to right shunt가 존재한다

# involved coronary a.;dilated or aneurysmal

386.11 Ruptured Sinus Of Valsalva

# aorta의 valsalva sinus중의 하나가 congenital or acquired disease에 의해 약해졌을 때

  aneurysm이 rupture되어 RA 혹은 RV로 향한다

# extremely rare in childhood, sudden onset

# Dx.

  ; 환자가 갑자기 new loud to-and-fro murmur를 동반하면서 acute CHF가 발생했을 때

# catheterization

  ; atrial or ventricular level에서 left to right shunt

# Tx.

  ; urgent surgical repair

THE OBSTRUCTIVE LESIONS

386.12 Pulmonary Valve Stenosis With Intact Ventricular Septum

# 여러 형태의 right ventricular outflow obstruction이 있으면서 ventricular septum은 intact하다

# the most common;valvular pulmonary stenosis

# systole동안에 dome-like obstruction이 발생

# 때때로 PS와 ASD가 동시에 나타남

Pathophysiology

  <1> right ventricular outflow obstruction은 systolic pr.을 증가시키고 RVH을 유발시킨다

  <2> severity 결정요소

       ; size of restricted valvular opening

  <3> severe case;RV pr.> systemic systolic pr.

       mild case ; RV pr.가 경도 혹은 중등도로 증가

  <4> pul. a. pr. ; 정상 혹은 감소

  <5> arterial oxygen saturation

       ; RV compliance 감소와 intra-cardiac communication에 의해 right to left shunting이 생기는 심한 경우 ( neonate 혹은 small infant에서 볼수 있음 )를 제외하고는 정상이다

Clinical Manifestaitions

  <1> 경도 혹은 중등도의 stenosis는 무증상

  <2> severe stenosis는 exercise intolerance

  <3> critical pulm. stenosis를 가진 neonate와 young infant에서의 obstuction; RV failure가 더

      현저하고 foramen ovale에서의 shunting 때문에 cyanosis 유발

  <4> PS환아

       ; growth and development는 정상

  <5> valve dysplasia에 의한 PS;Noonan syndrome의 가장 흔한 cardiac anomaly

  <6> mild PS시

       1) venouse pressure and pulse ; 정상

       2) not enlarged heart

       3) apical impulse ;  정상

       4) RV ; not palpable

       5) pulmonic area에서 relativly short pul systolic ejection murmur가 들림

       6) S2 ; split (pul. element는 delayed)

       7) EKG ; mild RVH

       8) X-ray ; pul.a.의 poststenotic dilatation

       9) two-dimentional echocardiography ; domed valve

  <7> Moderate PS

       1) venouse pressure ; 약간 증가

       2) jugular pulse에 intrinsic "a"가 현저

       3) palpable RV sternal lift

       4) systolic ejetion murmur ; prolonged later into systolic

       5) S2 ; split (pul. component는 delayed and diminished)

       6) EKG

         (1) RVH (systolic over load)

         (2) prominent spiked P wave

       7) X-ray

         (1) heart ; 정상이거나 혹은 RV prominence에 의해서 약간증가

         (2) pul. vascularity ; 감소

  <8> severe PS

       1) interarterial communication이 있으면 mild to moderate cyanosis

       2) RV failure시 hepatomegalry and peripheral edema

       3) elevation of venouse pr.

          ; large presystolic jugular "a" wave에 의해 발생

       4) heart

          ; moderatly or greatly enlarged

       5) loud systolic ejection murmur

          (1) trill과 동반

          (2) pul. area에서 잘 들림

          (3) entire precordium에서 neck과 back으로 radiation

          (4) late systolic accentuation을 동반함

       6) S2의 pul. element가 들리지 않는다.  

       7) EKG

          (1) RVH

          (2) tall spiked P wave

       8) two-dimensional echocardiogram

          (1) severe pul.valve deformity

          (2) intact ventricular septum

          (3) RVH

       9) X-ray

          (1) cardiomegaly

          (2) prominence of RV and RA

          (3) prominence of pul.a.segment

              ; poststenotic dilatation에 의해 발생

          (4) pul.vascularity는 감소

      10) catheterization

          (1) pul. a. pr. ; 정상 혹은 감소

          (2) RV pr. ; mild case에서는 30-50mmHg

                      moderate case에서는 30-50mmHg

                      severe case에서는 systemic systolic pr.보다 크다

          (3) severe or moderate case에서는 RA.pr.는 prominent하며 giant "a" wave가 나타남

Prognosis And Complication

# Complication

  <1> CHF

       1) the most common

       2) severe case에서만 발생하며 생후 첫 1개월에 발생

  <2> cyanosis

       1) foramen ovale를 가로지르는 right to left shunt에 의해 발생

       2) infancy and severe case에서만 발생

  <3> infective endocarditis

        ; not common

# Course and prognosis

  <1> mild to moderate case

      1) 정상 생활 가능

      2) 정기 검진 받아야 함

  <2> small gradient가진 환자

      ; 병이 진행하지 않으며 치료할 필요 없다

  <3> moderate case

      ; 성장함에 따라 more significant gradient

  <4> obstruction을 악화시키는 요소

      ; subvalvular muscular and fibrous tissue hypertrophy

  <5> severe case를 치료하지 않으면 RV dysfunction and cardiac failure 발생

  <6> severe case Tx.

      ; urgent catheter balloon valvuloplasty or surgical valvotomy

Treatment

# ★Balloon valvuloplasty

 ; *moderate or severe isolated PS인 경우 Tx. of choice

# Emergency Closed or Open Valvotomy

 ; obstruction이 심한 neonate or infant에 실시

386.13 Infundibular Pulmonary Stenosis And Double Right Ventricle

# infundibular pul. stenosis

  <1> RV outflow tract의 muscular or fibrous obstruction에 의해 발생

  <2> obstruction site;pul.valve 근처 혹은 바로 밑

  <3> infundibular chamber는 RV cavity and pul.valve사이에 발생

  <4> 초기에 VSD 나타났다가 나중에 spontaneously closed

  <5> hemodynamics and 임상증상

       ; valvular PS와 유사

# double right ventricle

  <1> pul.valve의 밑에 위치하는 RV outflow obstruction의 more common variation

  <2> mid right ventricular region에 muscular band가 있어 chamber를 two part로 나누고

       inlet에서 outlet으로 obstruction을 유발시킨다

  <3> VSD가 잘 동반되며 이것은 spontaneously closed

  <4> obstruction은 초기에는 잘 나타나지 않으며 빠르게 진행

386.14 PS In Combination With An Intracardiac Shunt

# valvular or infundibular PS는 ASD or VSD를 가로지르는 left to right shunt가 잘 동반됨

# 임상증상 결정요소

  <1> degree of stenosis

  <2> magnitude of left to right shunt

# left to right shunt가 있으면 PS는 mild하고 증상이 심해지면 (severe PS) right to left

   shunt가 발생

386.15 Peripheral Pulmonary Arterial Stenosis

# ♥Associated Other Type Congenital Heart Diseases

    ; pul. valvular stenosis

  ; TOF

  ; PDA

  ; VSD

  ; ASD

  ; supravalvular aortic stenosis

; *familial tendency

# high risk condition

    ; ★congenital rubella syndrome

# Williams syndrome

    ; supravalvular AS with pulmonary arterial branch stenosis

    ; idiopathic hypercalcemia

# multiple severe constriction시 RV and obstruction 근위부위 pul.a.pr.는 증가한다

# systolic, continuous murmur

# EKG

  ; RVH and RAH (severe case인 경우)

# X-ray

  <1> cardiomegaly and prominence of main pul.a.

  <2> pul.vascularity;정상

386.16  Aortic Stenosis

Pathophysiology

; *남:여=3:1

; *mostly valvualr stenosis

# Type

    1) valvular

       ; *▲common

    ; thickened leaflet

       ; fused commissure

    2) subvalvular(subaortic)

       ; LV ourflow obstruction의 important form

       ; rapid progression in severity

    ; 다른 CHD(COA,PDA,VSD)의 successful surgery후에 나타남

  3) supravalvular

       ; less common type

       ; sporadic,familial or ass. with williams syndrome

    ; ★Williams Syndrome

                   - mental retardation, elfin facies (full face, broad forehead, flattened bridge of nose, long upper lip, rounded cheek)

                   - idiopathic hypercalcemia in infancy

Clinical Manifestations

  <1> early infancy때 critical aortic stenosis and severe left ventricular failure 나타냄.

    -> low cardiac output sign으로서, congestive heart failure,  cardiomegaly, pulm edema가

       심하고,  사지에서 pulse가 약해지며, urine output이 작아짐.  

       대개 children은 무증상으로서 normal growth and development

  <2> murmur ; routine P/E에서는 나타나지 않음

  <3> sudden death

       ; severe left ventricular outflow obstruction시 볼 수 있다.

  <4> mild or moderate case

       ; heart size and apical impulse은 정상

  <5> severe case

       ; cardiomegaly with left ventricular apical thrust

  <6> 청진소견

      1> rough systolic ejection murmur

         (1) right upper sternal border에서 잘 들림

         (2) suprasternal notch  thrill을 동반

         (3) neck and down the left sternal border로 radiate

      2> diastolic murmur

         (1) mild aortic insufficiency 의미

         (2) subvalvular obstruction 혹은 bicuspid aortic valve시 나타남

      3> apical short mid-diastolic rumbling murmur

         ; normal MV시에도 나타남

      4> S2

        (1) mild case시 normal splitting

        (2) severe case시 aortic valve closure는 diminished, paradoxic splitting S2나타남

      5> S4

          ; severe case시 나타남

      6> subvalvular type시 murmur는 left sternal border or apex에서 maximal intensity

      7> valvular type시 aortic ejection click이 선행됨

  <7> infancy시 critical AS의 증상

      1> CHF ; cardiomegaly,pul.edema;severe

      2> pulse ; weak

Diagnosis

  <1> EKG

      1) severe case시 normal

      2) 오랫동안 severe stenosis가 지속되면 LVH and strain이 나타남(Lt precordial lesd에서

         inverted T-wave 나타냄.)

  <2> X-ray

      1) prominent ascending aorta

      2) aortic knob;mormal

      3) heart size;normal

      4) valvular calcification;old children 시

  <3> M-mode echocardiography

      1) multiple diastolic echoes of aortic valve

      2) eccentric aortic valve closure

      3) increased thickness of ventricular septum and free wall of LV

  <4> Graded exercise testing

      1) old children에서 left ventricular outflow obstruction의 severity를 평가

      2) gradient severity가 증가할수록

        (1) working capacity;감소

        (2) systolic pr.;fails to rise adequately

        (3) diastolic pr.;증가

        (4) ST segment;depression

  <5> left cardiac catheterization

      1) obstruction이 심하면 abnormal aortic pr.curve 나타냄

        (1) early-appearing anacrotic notch

        (2) slow,prolonged,delayed systolic upstroke

        (3) narrow pulse pr.

        (4) delayed dicrotic notch

Prognosis

  <1> mild to moderate 시 good

  <2> severe 시 sudden death;gross LVH의 evidence가 보임

  <3> neonate에서의 사망원인

       ; LV의 endocardial fibroelastosis를 동반한 CHF

Treatment

  <1> progressive left ventricular dysfunction을 예방하기 위해 severe valvular AS가진 어린이

      는 surgical valvotomy 실시한다   

  <2> Balloon valvuloplasty

      ; infant and older children에 성공적 실시

  <3> OP Ix.

      1> LVH의 definitive evidence

      2> significant gradient ( rest시 normal cardiac output이 있으면서 좌심실과 대돔막과의

         압력차가 60mmHg 이상 ) across the aortic valve

         => surgery or balloon dilatation을 실시함

   <4> careful follow-up이 필수적이다

        왜냐하면 ventricular obstruction의 severe recurrence가 early symptom과 관계가 없기

        때문이다.

   <5> infective endocarditis에 대해 prophylaxis가 필요

 386.17 Coarctation Of The Aorta

; constriction of aorta

    - any point from transverse aorta to iliac bifurcation

    - *98% just below origin of left subclavian a. at origin of ductus arteriosus(=justaductal coarctation)

; 남:여=2:1

; Turner syndrome(XO)에 많다

; ★동반 anomaly

  - *bicuspid aortic valve(70%)

  - *MV anomaly : supravalvular mitral ring, parachute mitral valve

  - *subaortic stenosis

Pathology

# type

    1) preductal segmental tubular hypoplasia

    ventricular hypertension and hypertrophy→ PDA widening→ relief of

    obstruction→ acyanotic

    2) discrete juxtaductal obstruction

    RV blood→ ductus→ descending aorta→ lower body→ femoral pulse are

    palpable→ severe pulmonary hypertension and vascular rssistence→ signs of heart failure 

# hypertension의 원인

  ; mechanical obstruction

  ; renal mechanism

 COA는 extensive collateral circulation을 유발한다

 1> branches of subclavian a.

 2> sup.intercostal a.

 3> internal mammary a.

 4> axillary a.의 thoracic and subscapular branches

 infancy을 지나면 대개는 무증상

Clinical Manifestations

# *After infancy, mostly asymptomatic even severe coarctation

# Older Children

    ; *hypertensive on routine P/E

           --> frequently brought to cardiologist

# classic sign

  ; disparity in pulsations and blood pressures of arms and legs

           - *radial-femoral delay

                   / normal에서는 femoral before radial pulse

           - lower BP in legs

                   / normal에서는 lower BP in arms 10-20mmHg

                   / common over 1yr

           - *exercise에 반응하여 systemic blood pr의 증가가 현저하다

# *90% systolic hypertension in upper extremities

# higher pressure in right arm

    - suggest involvement of left subclavian artery

# 청진 소견

    ; usually normal

    ; *apical systolic ejection click or thrill in suprasternal notch

           - suggest *bicuspid aortic valve

    ; short systolic murmur

       - along left sternal border at 3rd and 4th ICS

    - *well transmitted to left infrascapular area & neck

    ; typical murmur of mild AS in 3rd Rt. intercostal space

    ; *low-pitched mid-diastolic murmur at apex

           - *suggest MS

    ; *systolic or continuous murmur over left & right side of chest

           - *well developed collarteral blood flow

Diagnosis

# X - Ray

    ; cardiac enlargement

           - CHF or LV prominence에 의해 발생

    ; prominence shadow in left sup. mediastinum

       - enlarged left subclavian a.에 의해 발생

    ; ★notching of inf. border of ribs

       - pressure erosion by enlarged collateral vessel

           - *except upper and lower 2 to 3 ribs

           - *8세이후에 나타난다.

    ; displacement of barium filled esophagus and discontinuity of lateral margin of aorta below the arch

           - post stenotic dilatation of descending aorta 에 의해 발생 ( E sign or inverted 3 sign. )

# EKG

           ① NEONATE AND INFANT 때에는 RVH or BVH

           ② Young child때에는 normal

           ③ Old child때에는 LVH

Prognosis And Complicatoin

# ㉿Associated Anomalies

    ; *aortic valve anormality

           - *most patients

           - *biscuspid valve is ▲common

           - no clincal sign in mostly

    ; PDA

    ; VSD and ASD

        - left to right shunt 있을 때 의심

    ; mitral valve abnormality

  ; *congenital aneurysm of wilis circle, defective elastic & medial tissue of vessels

           --> *subarachnoid or intracerebal hemorrage

                   / secondary to hypertensive state

    ; subclavian a. abnormality

       - involvement of left subclavian a. in the area of coarctation

    - *stenosis of orifice of Lt. subclavian a., anormalous origin of Rt.subclavian a.

# *치료 않으면 20-40세 사이에 사망

# ★Common Serious Complication

    ; related hypertensive state

           - *premature coronary a. disease

           - CHF    

           - hypertensive encephalopathy

           - intracranial hemorrage

    ; infective endocarditis or endarteritis

    ; *aneurysm of the descending aorta or of the enlarged collateral vessels

Treatment

  in neonate severe COA + ductal closure→ hypoperfusion→ acidosis rapid

  deterioration→ PGE1

# 수술 시기

    ; *2-4세 (mortality rate는 1% 미만)

    ; 2nd decade시 수술이 비성공적인 이유

    1> decreased LV function

    2> degenerative change

# Choice OP

  ; excision of the area of coarctation and primary anastomosis

# ★Postop. Complication

    ; striking increase in amplitude of pulsation in lower extremities

    ; rebound hypertension

    ; residual murmur

           - due to asso. cardiac anomalies

    ; rare operative problem

           - spinal cord injury, chylothorax, diaphragm injury, laryngeal nerve injury

           - diminished or absent pulse if left subclavian flap

           - aortic aneurysm if balloon angioplasty

    ; recoarctation

           - *common in end-to-end anastomosis within 1st mo

           - *balloon angioplasty가 choice procedure

    ; premature cardiovascular disease

           - early onset adult hypertension

           - *repair of 2nd decade or beyond시 증가한다.

    ; postcoarctectomy syndrome

Postcoarctectomy Syndrome 

; *postoperative mesenteric arteritis

    --> *hypertension, abdominal pain

1) Sx and Sg

  - anorexia, nausea, vomiting, leukocytosis, intestinal hemorrhage, small bowel obstruction, bowel necrosis. 

2) Tx

   ① antihypertensive drug ; nitroprusside, labetalol

   ② intestinal decompression

   ③ corticosteroid

386. 18 Coarctation with VSD

       infancy   isolated COA는 드물게 CHF를 유발한다.

         <1> LV의 preload and afterload를 증가시킴

         <2> 생후 첫 1개월에 발생

         <3> intractable cardiac failure을 유발

      Sx.

         <1> tachypnea, failure to thrive, heart failure

         <2> cardiac output가 낮아 상하지 압력차이가 현저하다

386. 19 Coarctation With Orher Cardiac Anomaly

동반 Anomaly

         ① hypoplastic Lt Ht

         ② severe aortic valvular disease

         ③ TGA

         ④ Variation of single ventricle

         ⑤ endocardial fibroelastosis

      ductus를 가로지르는 blood flow를 결정하는 요소 

         ① positipon

         ② severity of obstruction at the site of coarctation

         ③ pul. vascular resistance

386. 20 Congenital MS

# associated defects

  ; AS

  ; CoA

      MV

         <1> funnel shape

         <2> leaflet;thickened

         <3> chordae tendineae;shortened and deformed

         <4> parachute MV and double orifice MV

      Sx.

         <1> 첫 2년 내에 나타남

         <2> infant : underdevelopment, dyspnea secondary to CHF, cyanosis, pallor

      청진소견

         <1> rumbling diastolic murmur

         <2> S2 : loud and split

         <3> opening snap of MV

      EKG

         <1> RVH

         <2> normal, bifid, or spiked P waves

      X-ray

         <1> LA and RV enlargement

         <2> pul. congestion

      echocardiogram : 특징적

         <1> thickened MV leaflets

         <2> diminished E-F slope

         <3> enlarge LA with normal or small LV

         <4> two-dimensional (short axis)

               significant reduction of MV orifice in diastole

 

      catheterization

              RV, pul.a., pul. capillary wedge pr. : 증가

      Doppler study : pressure gradient across the mitral orifice

      PROGNOSIS

         <1> 대개 치료하지 않으면 예후가 poor

         <2> 대개 생후 첫 2년 내에 사망

386.21 Pulmonary Venous Hypertension

  chronic pul.venous hypertension을 유발하고 이것이 심해지면 pul. a. hypertension and

      right sided heart failure를 유발한다

      pul.venous hypertension을 유발하는 질환

         <1> congenital MS

         <2> MI

         <3> some varieties of total anomalous pul.venous return with obstruction

         <4> left atrial myxomas

         <5> cor triatriatum (stenosis of common pul.v.)

         <6> indivisual pul.venous stenosis

         <7> supravalvular mitral ring or web

      EKG

         <1> RVH

         <2> spiked P waves

      X-ray

         <1> cardiomegaly

         <2> pul. v. , RV, RA, main pul. a. : prominent

         <3> LA : normal size or slightly enlarged

      Echocardiogram

         <1> left atrial myxoma

         <2> cor triatriatum

         <3> mitral valve abnormality

      Catheterization

         <1> pul. hypertension

         <2> pul. a. wedge pr.가 증가

         <3> left atrial pr. : lesion이 proximal side이면 normal

      DDx.

        pul.veno-occlusive disease

         <1> children과 young adult에서 pul.v.에 obstructive lesion을 유발하는 idiopathic

              process

         <2> toxin or viral agent에 의해 local injury 받은 후에 obstruction발생

         <3> 초기에 pul.edema가 있으면서 left sided heart failure

         <4> dyspnea, fatigue, pleural effusion : common

              cyanosis, digital clubbing, syncope, hemoptysis : variable

         <5> LA pr. : normal

              pul. a. wedge pr. : normal or elevated

              pul. venous return의 anatomic abnormality는 없다

      예후

         <1> infant : 수 주에서 수 개월간 생존

         <2> adult : 수 개월에서 수 년간 생존

THE REGURGETANT LESIONS

386.22 Pulmonary Valvular Insufficiency And Congenital  Absence Of The Pulmonary Valve

     

      여러 cardiovascular disease를 잘 동반하며 severe pul.hypertension에 이차적으로 온다

      수술 후 합병증

         incompetence of valve

         prominent physical sign

         diastolic murmur

          <1> upper and mil left sternal border에서 잘 들림

          <2> AI때보다 lower pitch

      X-ray

         prominence of main pul. a.

    

      EKG

         normal or minimal RVH

      catheterization

         pul. a. diastolic pr. : 감소

      isolated pul.valvular incompetence

         well tolerable하며 수술이 필요없다

      absence of pul.valve

        <1>동반질환

           1> VSD

           2> TOF

        <2> neonate or infant에서 pul. a.는 widely dilated and compress the bronchi하여

           1> recurrent episodes of wheezing

           2> pul. collapse

           3> pneumonitis를 잘 발생시킨다

        <3> 사망원인

           1> bronchial compression

           2> hypoxemia

           3> heart failure

        <4> 치료

              plication of massive pul.arteries along with intracardiac correction

386. 23 Congenital MI

      Associated anomaly

         PDA, COA, VSD, corrected TOGV

         anomalous origin of the LT coronary a from the pul. a

         endocardial fibroelastosis

         Marfan syndrome

         atrioventricular septal defect

      auscultation

         high pitched apical holosystolic m

         apical low pitched mid-diastolic rumbling m

            increased diastolic flow

      EKG

         bifid p wave, LVH or RVH

      X-ray

         LV is prominent

         pul. vascularity is normal or prominent

      Echo

         enlarge of LA and LV

         motion of MV is excessive with a steep E-F slope on M mode

      Cath

         elevated LA pres

         pul. a hypertension

      Tx

         mitral valvuloplasty

         instillation of prosthetic valve 

386. 24 Mitral Valve Prolapse

원인

       - 하나 혹은 양쪽의 mitral leaflets의 billowing(소용돌이) 특히, post. cusp이 end systole

         시 left atrium으로 향하게 되는 abnormal mitral valve mechnism에 의해 발생한다

      항상 congenital ( autosomal dominant ) adolescence or adulthood때 까지는 인지되지           못함

      girls에 많다

      잘 발견되는 경우

        ① Marfan syndrome

        ② straight back syndrome

        ③ pectus excavatum

        ④ scoliosis

        ⑤ congenital rheumatic

        ⑥ viral myocarditis

        ⑦ secundum ASD

      청진소견 : 특징적

        <1> apical murmur

           1) late systolic in timing

           2) click이 선행됨

           3) standing or sitting position에서 click은 early systole때 murmur은 late systole때 

               현저함

        <2> arrhythmia

        <3> primarily unifocal or multifocal premature ventricular contraction

      EKG

        <1> usually normal

        <2> diphasic T wave (lead II, III, AVF, V6)

      Chest X-ray : normal

      Echocardiogram

        <1> mid or late systole때 post. mitral leaflet의 post. movement

        <2> ant. and post. mitral leaflets의 pansystolic prolapse

        <3> Two-dimensional real time echocardiography

               mitral leaflet의 free edge and body가 systole   LA를 향해 post.로 move

      infective endocarditis의 위험성이 높으므로 surgery or dental procedure antibiotic

      prophylaxis가 필요

      thickened and redundant MV leaflets의 존재하에서 MV prolapse를 가진 성인(남자에서

      흔함)에서

      발생할수 있는 cardiovascular complication

         <1> sudden death

         <2> arrhythmia

         <3> CVA

         <4> progressive valve dilatation

         <5> heart failure

         <6> endocarditis

386.25 TR

      Associated with Ebstein anomaly of TV

      Older children : acyanotic form

      newborn : severe cyanosis

      accompanies RV dysfunction

      encountered in newborn with perinatal asphyxia

ADDITIONAL CONGENITAL HEART LESIONS

386. 26 Anomalous Of The Aortic Arch

Right Aortic Arch

1) aorta는 right로 curve

   만일 aorta가 vertebral column의 right side로 descending하면 other cardiac

   malformation과 관계

2) 발생되는 경우

   (1) TOF의 20%에서 발생

   (2) TA

3) trachea는 midline의 left side로 deviated

4) barium filled esophagus의 Rt.border가 idented

Vascular Rings

1) vascular ring을 나타내는 common anomaly

  (1) double aortic arch (Fig. 386-7 and 386-8)

  (2) right aortic arch with left ligamentum arteriosum

  (3) anomalous innominate a. arising further to the left on the arch than usual

  (4) anomalous left carotid a.arising further to the right than usual and passing

     ant. to the trachea

  (5) anomalous left pul.a. (vascular sling)

       abnormal vessel은 elongated main pul. a. or right pul. a.에서 arise

2) associated congenital heart disease

    5-20%

3) vascular ring이 infancy때 trachea와 esophagus를 압박하면 symptom

  (1)wheezing respiration : crying, feeding, neck flexion 시 심해짐

                          neck extension시 경감

  (2) vomiting

  (3) brassy cough and pneumonia

  (4) sudden death from aspiation

4) X-ray (Fig.386-8)

5) aberrant right subclavian a.가 common 하게 보이지만 trachea를 compression하지않는다

6) Tx. and Px.

  (1) surgery

      trachea compression의 X-ray 소견이 보이는 증상이 있는 환아에 대해서 실시

  (2) severe tracheomalacia가 나타나면 poor Px.

7) Dx.

  (1) 2-D echocardiography

  (2) MRI

  (3) digital subtraction angiography

  (4) angiography during cardiac cath.

386.27 Anomalous Origin Of Coronary Arteries

Anomalous Origin Of The Left Coronary A. From The Pul. A.

# decreased pul. a. pr. after birth

    --> inadequate left coronary artery perfusion

    --> *reversed left coronary artery flow

    --> myocardial infarction and fibrosis

# occasionally interarterial collateral anastomosis

# Myocardial steal syndrome

3) 합병증

  (1) mitral incompetence

     papillary m.의 infarction에 의해 발생

  (2) localized aneurysm in LV

Clinical Manifestation

; *CHF within 1st few months

; often precipitated by respiratory infection

; recurrent attacks of discomfort, restlessness, irritability, sweating, dyspnea, pallor with or without cyanosis

; 청진소견

  ① murmur : ejection type, non-specific

  ② Gallop rhythm

  ③ continuous murmur : intercoronary anastomosis 있는 old pt.에 발생

Diagnosis

; X-ray

  - cardiomegaly(contour and pulsation은 nonspecific)

; EKG

   ① QR pattern followed by inverted T wave (lead I and aVL)

 ② deep Q wave and elevated ST segments and inverted T wave (V5 and V6)

  ③ old pt.에서는 exercise study가 도음이 된다

     ST-T wave changes or symptom이 발생

; aortography

  ① diagnostic

  ② immediate opacification of right coronary a.

Treatment and Prognosis

  ① 첫 6개월내에 heart failure로 사망

  ② medical Tx.

     a. for heart failure

        diuretics, digoxin, captopril

     b. for controlling ischemia

        nitrates, calcium channel blochers, beta blocking agent

  ③ surgical Tx.

     a. pul. a.에서 anomalous coronary a.를 분리

     b. anomalous coronary a.를 aorta에 anastomosis

Anomalous Origin Of The Right Coronary A. From The Pul. A.

1) infancy와 early childhood에서는 증상을 나타내지 않는다

2) left coronary a. : enlarged

     right coronary a. : thin-walled and midly enlarged

3) right coronary a.의 early infancy perfusion : pul. a.에서 origin

   right coronary a.의 later perfusion : collaterals of the left coronary vessel에서origin

4) angina & sudden death

      adolescence or adult에서 발생

5) Tx.

      right coronary a.를 aorta에 reanastomosis

Ectopic Origin of Coronary A. from The Aorta with Aberrant Proximal Course

1) aberrant a. : left, right, or major branch coronary a.

2) origin site

   ① wrong sinus of Valsalva

   ② proximal coronary a.

3) ostium

       hypoplastic, slit-like, normal caliber

4) ostia hypoplasia에 의한 obstruction은 aorta & RV outflow tract or

   interventricular septum 사이에 tunnel을 형성하고 acute angulation은

   myocardial fibrosis or myocardial infarction을 유발함

5) 증상

   ① myocardial infarction

   ② ventricular arrhythmia

   ③ sudden death

   ④ angina pectoris

   ⑤ syncope                    

386.28 Pul. Vascular Disease (=Eisenmenger Syndrome)

     # reversed or bidirectional shunt through a VSD as a result of pul. vascular obstructive            disease

     # assiciated with

         1) ASD

         2) A-V canal

         3) PDA

         4) other communication between the aorta and pul.a

     # pul.vascular resistance는 early infancy때 정상적으로 감소되고 난 이후에  high or rise            된다

       -> 이러한 현상은 이차적으로 prolonged elevated pul.pr.의 결과로 발생하여 vessel에

           severe obliterative intimal lesion을 야기시킨다.

     # pul. vascular disease  의 factor

        1) pul, a pres의 증가

        2) pul. blood flow의 증가

        3) hypoxia

        4) hypercarbia

     # pul hypertension due to pul, blood flow의 증가

     # Eisenmenger에서 pul. hypertension은  pul. vascular disease 때문

Pathology And Pathophysiology

# occurs in small pul. arterioles and muscular arteries (*< 300um)

# ★Heath-Edwards Classification

    ; type I - medial thickening

  ; type II - medial and intimal thickening

    ; type III - type I, II plus plexiform lesions secondary to hypoplasia of medial layer of small muscular arteries

# ♥Physiologic Definitions

    ; absolute elevation of pul. artery resistance > 12 wood units/m2

    ; Rp/Rs > 1.0

# pul. vascular hypertension

         <1> trisomy 21환자에서는 출생 후 즉시 발생가능

         <2> 합병증

              1) pul. vascular pr.가 높은 환자의 natural history를 complicated

              2) transmission of systemic pr. to the pul.circulation

              3) exposure to low PO2 (high altitude)

              4) high pul. blood flow from birth

Clinical Manifestation

2nd or 3rd decade까지는 증상이 나타나지 않는다.

irreversible pul. vascular obstruction은 high pul. vascular resistance을 유발함

pul. resistance가 systemic resistance보다 클 때는 정상적인 left to right

 shunt가 right to left shunt로 변할 수 있다.

cyanosis, dyspnea, fatogue, dysrhythmia

late stage : heart failure, chest pain, syncope, hemoptysis

physical examination : right ventricular loud, narrowly split S2, soft ejection

 pul. a. pulsation이 left upper sternal border에서 palpable

  Graham Steel murmur (pul. valve의 functional incompetence에 의해 left sternal

              border에서 들리는 blowing diastolic murmur)

Diagnosis

 cyanosis with polycythemia

 X-ray

 1) heart size : normal to enlarged

 2) main pul. a. : prominent

 3) hilar area의 pul. vessels : enlarged

    pul. vessel의 peripheral branch의 caliber : diminished

 4) RV and RA : prominent

 EKG

 1) RVH

 2) tall, spiked P wave

 echocardiography

 1) thick walled RV

 2) communication between the systemic and pul. circulation

 3) right sided systolic time interval : 증가

 catheterization

 1) defect site에서 bidirectional rhunt

 2) systemic and pul.circulation의 systolic pr.는 equal

 3) pul. capillary wedge pr. : normal

 4) arterial oxygen saturation : 감소

   #cf) vasodilator theraphy에 반응을 보이면 reversible pul. hypertension을 의미

Treatment

㉿Table 386-2

Chapter 387. Cyanotic Congenital Heart Disease

387.1 Evaluation Of The Critically Ill Neonate With Cyanosis And Respiratory Distress

      Cardiac disease : CHD is responsible for cyanosis

                         obstruction to RV outflow ( RT to LT shunt )

                         anatomic defect

                         pul. edema

                         persistence of fetal pathway

      CNS disease : CNS depression→ irregular shallow breathing

                     → alveolar ventilation↓

                     → lower alveolar oxygen tension

                     → PCO2↓

                        ex) ICH

      pulmonary disease

         HMD, atelectasis, pneumonitis

         → inflammation, collapse, fluid accumulation in alveoli

         → incomplete oxygenation

      hemoglobinopathy

         methemoglobinemia

      DDx

         careful observation of infant breathing pattern : CNS

         hyperoxia test : pul. disease

         murmur : cardiac disease

         2-D echo : cardiac disease    

CYANOTIC LESIONS ASSOCIATED WITH DECREASED PUL. BLOOD FLOW

387.2 TOF

# ★Consist Of

    1) obstruction to right ventricular outflow(PS)

    2) VSD

    3) dextroposition of the aorta

    4) RVH

Pathophysiology

1) pul. valve : small ring (bicuspid), site of stenosis

2) crista supraventricularis의 hypertrophy는 infundibular stenosis를 유발하여 

   variable size and contour의 infundibular chamber를 형성하게 된다.

3) PS가 있는 경우 pul. blood flow는 aorta의 collateral vessel로부터 공급받음

4) VSD ; non-restrictive, large, aortic valve 바로 밑에 존재, related to the post.

         and  right aortic cusps

5) mitral and aortic valve의 normal continuity는 유지

6) aorta arches to the right(20%)

   aortic root : large, overrides VSD

 

(1) RA and RV로의 systemic venous return은 정상

         (2) PS가 있는 경우 RV가 contraction하면 blood는 VSD를 통해 aorta로 가게 된다.

            이 결과로 persistent arterial desaturation and cyanosis가 발생한다.

         (3) PS에 의해 pul. blood flow가 심하게 restiction되면, pul. blood flow는 bronchial  

             collateral circulation and PDA에 의해 공급받게 된다.

         (4) RV outflow obstrction의 정도를 결정하는 요소

             1) severity of cyanosis

             2) presence of RVH

             3) acyanotic or pink TOF : RV flow obsruction이 moderate할 경우 VSD를

               통해 balanced shunt가 있으면 환아는 청색증을 보이지 않는다.

Clinical Manifestation

Cyanosis

1) 가장 현저한 증상

2) 출생시에는 보통 나타나지 않음. infant는 RV outflow obstrction이 심하지 않고 large left to right shunt and CHF를 나타내개 된다.

3) 보통 1세말 경 나타남 : lips and mouth의 mucous membrane, fingernails, toenails에 현저함

Dyspnea

          - occur on exercise

            physical effort에 의한 dyspnea해소를 위해 squatting position을 취함.

Paroxysmal Hypercyanotic Attacks(Hypoxic Or Blue Spells)

    ; particular problem *during 1st 2yr of life

    ; 증상

           - hyperpneic and restless, cyanosis increase, gasping respirations ensure, syncope

           - if severe spells

                   / unconsciousness, convulsion, hemiparesis

    ; *▲frequently in the morning upon first awakening or following episodes of vigorous crying

    ; disappearance or decrease in systolic murmur due to decreased RVOT blood flow

    ; last a few minutes to a few hours

    ; *rarely fatal

    ; only mildly cyanotic infant --> more prone to develop hypoxic spell

    ; ㉿치료

         - knee chest position with no constricting clothing

         - administration of oxygen

         - morphine sc

                   / *not in excess of 0.2mg/kg

         - correction of metabolic acidosis

             / *PaO2 below 40mmHg→ immediately bivon iv

         - *beta-adrenergic blockade(propranolol)

                   / *0.1-0.2mg/kg iv

                   / in severe spell, esp. with tachycardia

           - *methoxamine, phenylephrine

                 / increase of systemic vascular resistance

Delayed Growth And Development

Physical Examination

    ; pULSE : normal

    ; SYSTOLIC THRILL ; 3rd and 4th parasternal space의 left sternal border를

                          따라 잘 느껴짐

    ; 청진소견

systolic murmur

 (1) left sternal border에서 잘 들림

 (2) RV outflow tract을 통한 turbulence에 의해 발생

 (3) severe obsruction and large right to left shunts시 less prominent

S2 ; single, aortic valve closure에 의해 발생

continuous murmur

 발생원인 (1) enlarged bronchial collateral vessels

         (2) persistent PDA(rarely)

# X-ray

A-P view

 (1) narrow base

 (2) pul. a. area의 left border가 concavity

 (3) normal heart size

   (4) RVH에 의해 diaphragm의 보다 위쪽에 위치한 rounded apical shadow

 (5) coeur en sabot;cardiac silhouette이 wooden shoe모양을 나타내는 것

Lat.view

 (1) hilar areas and lung fields가 relatively clear

      원인 1) pul.blood flow의 감소

           2) small size of pul. a.

        3) aorta : large, 20%에서 right side로 arches

                  -> indentation of the leftward positioned air-filled

                     tracheobronchial shadow가 발생함

        4) poststenotic dilatation of pul. a.

             valvular pul.stenosis를 의미함

2> EKG

     1) RAD

     2) RVH

     3) P wave;tall and peaked,sometimes bifid

3> Two-dimensional echocardiography

4> cardiac cath.

     1) RV=LV(systolic Pr.)

     2) mean pul. a. Pr. : 5-10mmHg

     3) RA Pr. : normal

     4) VSD를 통해 우심실에서 쉽게 대동백으로 들어갈 수 있다.

     5) a.oxygen saturation은 right to left shunt의 양에 따라 결정된다; moderate                     cyanosis 환아가 휴식시 PAO2는 75-85%(정상은 95%)]

        <5> selective right ventriculography

             TOF의 anatomy를 잘 볼 수 있다.

        <6> Left ventriculography

             ventricle size, VSD 위치, overriding aorta

        <7> Aortography or coronary arteriography

Complications

Cerebral Thrombosis

   1) site ; 보통 cerebral veins or dural sinuses, 때로는 cerebral arteries

   2) extreme polycythemia, dehydration시에 잘 발생

cerebral ishemia

   1) 2세 이하에서 흔히 발생

   2) Hb.and Hct.는 정상범위이나 때  로 iron deficiency anemia를 동반함

   3) 치료

     (1) adequate hydration

     (2) supportive measures

     (3) phlebotomy and volume replacement with FFP : extremely polycythemia에

                                                       적용

     (4) heparin은 거의 효과가 없다.

     (5) physical theraphy : affected extremity에 가능한 빨리 실시

Brain Abscess

    ; less common than cerebral vascular events

    ; over 2yr

    ; insidious with low-grade fever and/or behavior change

    ; some acute onset of headache, nausea, vomiting

    ; epileptiform seizures

    ; elevated  ESR & WBC

    ; 치료

           - massive antibiotic theraphy

    - *surgical drainge

                   / *almost necessary

Bacterial Endocarditis

 1) 수술하지 않은 환아의 pul., aortic, or rarely tricuspid valve의 infundibulum에서 발생

   2) dental or surgical procedures 전후에 항생제 예방요법이 필수적.

CHF

   1) pul. atresia and large collateral blood flow를 가진 young infant에서 발생

       -> 생후 첫 1달내에 사라짐

       -> 환아는 pul.blood flow 감소와 함께 cyanosis를 나타냄  

   2) TOF의 흔한 증상은 아니다.

Associated Cardiovascular Anomalies

    ; PDA

    ; ASD

    ; absence of the pul.valve

   1) mild cyanosis

   2) heart;large and hyperdynamic

   3) loud to-and-fro murmur들림

   4) neonatal period에 치명적이나, 때로 spontaneous regression하는 경우도 있다.

    ; absence of a pul.a.

   1) left pul.a.가 없어 right lung은 more vascularized

     -> X-ray상 양쪽 pul.vasculature가 다를 때 반드시 의심해야함

   2) affected lung의 hypoplasia를 동반함

    ; right aortic arch     

    ; multiple VSD

    ; atrioventricular canal;Down syndrome시 잘 동반됨

    ; absence of branch pul. a

Treatment

         depends on the severity the RV outflow

1>목적 ; 즉각적으로 pul. blood flow를 증가시켜 severe hypoxia의 sequelea        

         ( shock, resiratory failure, intractable acidosis )를 방지함

2> medical treatment

  1) PEG1 (0.05-0.20ug/kg/min)

    (1) ductal smooth m.의 potent and specific relaxant로서 ductus arteriosus를

       확장시킴->adeqate pul. blood flow

    (2) long-term theraphy로는 사용치않음

  2) dehydration의 예방 및 즉각치료

      hemoconcentration and possible thrombotic episodes를 방지함

  3) iron deficiency anemia를 치료

        Hct.는 55-65%로 유지

  4) oral propranolol(1mg/kg every 6hrs)

      dyspneic spells의 frequency and severity를 감소시킴 

3> surgical treatment

    1 ) 1st month of life내에 severe cyanosis가 있을 때 systemic-pul. a .shunt로서

        pul. a. blood flow를 증가시켜 hypoxia를 해소시키고 small pul. vessel의 성장 을 도운다

  (1) modified Blalock-Taussing shunt

      most common aorto-pul shunt procedure

      side to side anastomosis subclavian a. to branch of pul. a

  (2) Blalock-Taussing shunt

      subclavian a.-pul. a.의 homolat.branch의 direct anastomosis

  (3) Waterson shunt

      1.ascending aorta.-right pul. a.

  (4) Potts shnt

      1. upper descending aorta-left pul. a.

      2. rarely done

      3. complication (CHF, late-onset pul. hypertension)이 잘 발생

  (5)성공적으로 shunt op.되었을 경우

      1. cyanosis감소

      2. machinery type murmur발생

  (6) systemic to pul.a.shunt시 infective endocarditis가 치명적이므로 예방요법이 필요하다

  (7) thoracotomy후의 합병증

      1. chylothorax

      2. diaphragmatic paralysis

      3. Horner syndrome

      4. cardiac failure

 2 ) corrective surgery

     (1) 방법

         1. RV outflow tract obstruction을 해소

         2. closure of VSD

     (2) risk는 5%이하

     (3) 수술의 성공률을 높이는 요소

         1. optimal total body perfusion

         2. adequate myocardial protection during bypass

         3. RV outflow obstruction해소

         4. air embolism 방지

         5. meticulous postoperative care

     (4) 합병증

         1. pul. valve insufficiency

         2. working capacity, maximal heart rate, cardiac output 감소

         3. conduction disturbance

Prognosis

immediate postoperative problems

1) RV failure

2) transient heart block

3) residual VSD with left to right shunting

4) myocardial infarction from manipulation of an aberrant coronary a.

5) residual collateals에 의해 불균형적으로 LA Pr.증가

TOF repair후에는 premature ventricualr beats가 발생할 수 있는데 이것은

 benign and nonprogressive하다

 휴식시 나타나지 않는 부정맥은 exercise study로서 찾아낼 수 있다

ventricular ectopy치료

  quinidine, propranolol, dilantin, or combinations of these agents

387.3 Pulmonary Atresia With Ventricular Septal Defect

; extreme form of TOF

; pul. valve

    - atretic, rudimentary, or absent

; pul. trunk

    - atretic or hypoplastic

Clinical Manifestation

; cyanosis within 1st few hr or days

    - *mostly severe cyanotic

           --> *require urgent PGE1 infusion and palliative surgicl intervention

# 청진소견

    ; *absent systolic murmur

    ; S1

           - followed by an ejection click by enlarged aortic root

    ; S2

           - *moderately loud and single

    ; continuous murmur

           - audible over entire precordium

# X-Ray

    ; small or enlarged heart

       - depending on pul. blood flow

    ; concavity at the position of the pul.arterial segment

  ; often reticular pattern of bronchial collateral flow

# Electrocardiogram

    ; *RVH

# Echocardiogram

    ; aortic override

  ; thick right ventricular wall

  ; pul. atresia

# Cardiac Catheterization

    ; right ventriculography

    ; large aorta opacified immediately by passage of the contrast medium through the septal defect

    ; no dye entering the lungs through the right ventricular out flow tract

    ; pul. blood flow from aorta to lungs

Treatment

; surgical procedure depend on there is adequate main pul. a segment and on the size of the branch pul. a

; option

    - aortopulmonary shunt

    - connection from the RV to pul. a

387. 4 Pulmonary Atresia With Intact Ventricualr Septum

; pul. valve leaflets

    - completely fused to form a membrane

; pul. blood flow

    - only supply via PDA

; Rt. ventricle

    - *usually hypoplastic

    - small right ventricular cavity

           / small TV annulus

           / sinusoid channel within Rt. ventricular wall

                   --> coronary arterial circulation communication

    - intermediate size or large ventricle cavity

           / tricuspid insufficiency

Clinical Manifestation

; markedly cyanosis

    - ductus arteriosus가 닫힐 때

# 청진 소견

    ; *loud single S2

    ; *no murmurs

    ; *sometimes systolic or continuous murmur due to ductal blood flow

# electrocardiogram

  ; *QRS axis - 0 ~ +90 degree

    ; tall, spiked P waves due to right atrial enlargement

    ; *left ventricular dominance or hypertrophy

# X-Ray Findings

    ; variable heart size with *marked decreased pul.vascularity

# Cardiac Catheterization

    ; right atrial and right ventricular hypertension

  ; ventriculography

           - size of the ventricular cavity

        - atretic right ventricular outflow tract

         - the degree of ticuspid regurgitation

           - intramyocardial sinusoid filling the coronary vessel

Treatment

1> urgent medical and surgical management하여 예후는 개선되었다.

2> PGE1;intervention전에 투여하면 ductus opening을 유지하여 수술 전에

    hypoxemia and acidemia를 감소시킨다

3> pul. valvotomy

    outflow obstruction을 해소

    이 시술기간동안에 adequate pul.blood flow를 유지하기 위해 systemic

        - pul. arterial anastomosis를 시행한다.

4> unroofing the outflow tract and patch grafting

     수술 목적은 right ventricular chamber 성장을 도와 forward flow를 좋게하는것이다.

5> more extensive valvutomy & shunt op.

     나중에 실시함

6> Fontan procedure

    - right ventricular chamber가 작은 경우에 실시하여 right atrium에서 pul. a.로 직접 blood flow를 흐르게 하는 방법

7> myocardial sinusoids를 통해 right ventricle을 거쳐 coronary perfusion이 있으면 예후는 좋지 않다.

387.5 Tricuspid Atresia

Pathophysiology

; entire systemic venous return

    --> foramen ovale or ass. with ASD

    --> left heart

; pul.blood flow

    - *depend on size of VSD and presence & severity of PS

    - *maybe depend on PDA

; *if intact ventricular septum, right ventricle is completely hypoplastic with pul. atresia

; decreased pul. blood flow, cyanosis in early months

; less often *VSD in absence of right ventricular outflow obstruction

    - *high pul. flow

    - mild cyanosis & congestive heart failure

; one variant

    - *ass. with TGA(30%)

Clinical Manifestations

    ; cyanosis at birth

    ; polycythemia, easy fatigability, exertional dyspnea, occasional hypoxic episodes

    ; 85% diagnosis before 2mo

    ; spontaneous VSD closure

           --> cyanosis를 악화시킴

# 청진소견

  ; *holosystolic murmurs along audible the left sternal border

  ; *single S2

# X-ray finding

    ; pul. undercirculation - normal related great vessels

    ; overcirculation - TGA

# Electrocardiogram

    ; ㉿LAD, LVH

    ; prominent R wave --> rS complex in right precordial leads

    ; qR complex followed by normal flat diphasic or inverted T wave in left precordial leads

    ; RV6 is normal or tall

    ; SV1 is generally deep

    ; P wave

           - biphasic with the initial component tall and spiked in lead II

# ★Cyanosis with LAD

    --> *highly suggestion of TA

# Two-dimensional echocardiogram

① absence of the tricuspid valve

② the small right ventricle

③ large left ventricle and aorta

# Cardiac Catheterization

 right atrial pr.는 정상 혹은 약간 증가되어 있으면서 prominent "a" wave를 보임

Treatment

# PGE1

# Surgical Aorto-Pulmonary Shunt Procedure

    ; *Blalock-Taussing procedure(or its variations)

         - preferred

    ; Rashkind balloon atrial septostomy(BAS)

# Palliative Surgery

    ; Glenn anastomosis(right sup.vena cava to right pul. a.)

           - *▲often performed after outgrowing sign of previous aorto-pulmonary shunt

           - *4-12mo

           - benefits

                   / reduces volume work on the Lt ventricle

                   / lessen chances of developing LV dysfunction

           - disadvantages

                   / SVC syndrome

                   / spontaneous closure of shunt

# Later surgical management

    ; modified Fontan operation

           - *1.5-3yr

           - caval-pulmonary isolation procedure

           - advantages

                   / decrease possibility of RA dilatation

                   / reduce incidence of postoperative pleural effusion

           - ♥Contraindication

                   / very young infants

           / elevated pul. vascular resistance( >4 wood units/m²)

           / pul. a. hypoplasia

           / LV dysfunction

           - *patients는 반드시 sinus rhythm, no MI이어야 한다.

    ; ★Postoperative Problem After Fontan Procedure

    - marked elevated systemic venous pr.

    - fluid retention

           - pleural or pericardial effusion

         / pleural effusion persist more than 3wks 30-40% of patients

    ; modified procedure을 사용하면, 5%로 감소된다.

    ; ♥Late Complication Of Fontan Procedure

       - residual obstruction

            / sup. or inf. vena caval syndrome

           - vena caval or pul. a. thromboembolism

           - protein-loosing enteropathy

    - supraventricular arrhythmias

            / atrial flutter, paroxysmal atrial tachycardia

           - sudden death

387. 6 DORV With PS

     특징 : aorta and pul.a.가 right ventricle에서 origin

             left ventricle의 outlet는 VSD

       aortic and mitral valve는 연결되어 있지 않다.

       VSD는 crista supraventricularis 밑에 위치함

       physiology는 TOF와 유사함

       two-dimensional echocardiography : anatomy, double outlet right ventricle,                              mitral-aortic valve discontinuity를 나타냄

       operation : intraventricular channel을 만들어 left ventricle에서 VSD를 통해 aorta를

                  통해 blood를 aorta로 ejection하게함.

                  pul. obstruction은 pul. or aortic homograft로써 해소시킬 수 있다.

                  aortic pul. shunt로써 증상을 개선시킬 수 있다.

387. 7 TGA With VSD And PS

      TOF와 유사

      obstruction site

         <1> valvular

         <2> subvalvular

                successful atrial septostomy or pul.arterial banding후에 잘 발생

      clinical manifestation

          cyanosis, decreased exercise tolerance, poor physecal develpment

 

      enlarged heart

      pul. vascularity : normal

      EKG

         <1> RAD 

         <2> RVH and LVH

         <3> tall spiked P waves

      cardiac catheterization

         <1>low pul. a. pr.

         <2> oxygenation saturation : pul. a.> aorta

      selective right and left ventriculography

         <1> aorta는 RV