congenital heart disease 先天性心臟病

2006/4/4 兒童心臟科張文王醫師1

Congenital Heart Disease

先天性心臟病行政院衛生署彰化醫院兒童心臟科張文王醫師



Acyanotic congenital heart diseaseThe Left-to-Right shunt lesions

ASD, PAPVR, ECD, VSD, PDA, AP window defect, Coronary A-V fistula…

The Obstructive lesionsPS with IVS, DCRV with PS, PPS, AS, CoA, Conge

nital MS,

Regurgitant lesionsPV insufficiency, MR, TR



Cyanotic Congenital Heart Disease– Lesions associated with decreased pulmonary blood

flowTOF, PA with/without VSD, TA, DORV with PS, TGA with

VSD, Ebstein anomaly…

– Lesions associated with increased pulmonary blood flow

d-TGA, l-TGA, DORV without PS, TAPVR, Truncus Arteriosus, single ventricle, Hypoplastic Left Hear syndrome, asplenia/polysplenia syndrome…


Congenital Heart Disease---VSD

VSD is the most common cardiac malformation and accounts for 25% of congenital heart disease.– Membranous type : most , anterior to the septal leaf

let of the TV– Supracristal type : superior to the crista supraventri

cularis , less common but may impinge on an aortic sinus caused AR

– Subpulmonary type : between the crista and papillary muscle of the conus , associated with PS

– Muscular type : in the midportion or apical region of septum, single or multiple (Swiss cheese septum)



Pathophysiology – Determine the L-to-R shunt magnitude Qp/Qs:

• The size of the VSD : restrictive VSD (< 0.5 cm2), nonrestrictive VSD (>1.0 cm2) – RV and LV pressure equlized

• The level of the ratio of pul to systemic vascular resistance : after birth, PVR remain higher. Because of normal involution of the media of small pulmonary arterioles, the size of shunt increases. Qp/Qs < 1.75:1 shunt is small, but > 2:1 left side volume overload occurs, as dose RV and PAH


Congenital Heart Disea

se---VSD



Clinical Manifestations – Vary according to the size of the defect and pulmon

ary blood flow and pressure• Small : asymptomatic, PE revealed a loud harsh or blowin

g holosystolic murmur over LLSB accompanied by a thrill.• Large: dyspnea, feeding difficulties, poor growth, profuse

perspiraion, recurrent pul infections, and cardiac failure in early infancy. Duskiness (+), PE revealed systolic thrill and palpable parasternal lift. Holosystolic murmur less harsh, P2 heart sound increased indicated pul hypertension. A mid-diastolic, low-pitshed rumble at the apexis caused by increased blood flow across MV and indicated Qp/Qs > 2



Diagnosis (1)– CxR :

• Small – normal or minimal cardiomegaly and a borderline increase in pul vasculature

• Large – gross cardiomegaly with prominence of both ventricles, LA and PA. Increased pul vascular marking. Pul edema, pleural effusion.

– ECG :• Small – normal but may suggest LV hypertrophy

• Large – RV hypertrophy, biventricular hypertrophy, P wave notched or peaked



Diagnosis (2)– 2-D echocardiogram and color Doppler

• Show the position and size• Estimating shunt size by examining the degree of volume o

verload of LA• Pulsed Doppler calculated the pressure gradient and RV, P

A pressure

– Cardiac catheterization• Performed only when the size of shunt is uncertain, when L

ab data do not fit well with clinical findings, when pul vascular disease is suspected.

• Pre-op Qp:Qs ratio



Prognosis and Complications (1)– Natural course of a VSD depends to a large d

egree on the size of the defect.• 30-50% of small defects close spontaneously duri

ng the first 2 yr if life• Small muscular type are more likely to close (up

to 80%) than membranous type (up to 35%)• Septal aneurysms limit the magnitude of the shu

nt.• Long-term risk is infective endocarditis.



Prognosis and Complications (2)– Less common for mod. or large VSDs to clos

e spontaneously• Repeated episodes of URI and heart failure ( infa

nt-failure to thrive)• At risk for pul. Vascular disease with time as a re

sult of high pul blood flow• Development of aortic valve regurgitation – the g

reatest risk occurring in p’ts with supracristal VSD



Treatment (1)– Small VSD

• Encouraged to live a normal life,

• Surgical repair is not recommended, but protection against infective endocarditis

• Spontaneous closure – echocardiogram F/U



Treatment (2)– Large VSD

• Medical management has two aims: control heart failure and prevent the development of the pulmonary vascular disease

• Indications for surgery: 1. any age with large defects, 2. 6-12 m/o with pulmonary hypertension, 3. >2 y/o Qp:Qs ratio > 2, 4. supracristal VSD

• Contraindication : severe pulmonary vascular disease

– Clamshell-type catheter occlusion devices are being tested as a means of closing apical muscular VSDs


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congenital heart disease 先天性心臟病

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