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
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♥ 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
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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 RV에서 origin
<2> pul. a.