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
2006/4/4
兒童心臟科 張文王醫師

2. Congenital Heart Disease
Acyanotic congenital heart disease
The Left-to-Right shunt lesions
ASD, PAPVR, ECD, VSD, PDA, AP window defect, Coronary A-V fistula…
The Obstructive lesions
PS with IVS, DCRV with PS, PPS, AS, CoA, Congenital MS,
Regurgitant lesions
PV insufficiency, MR, TR
2006/4/4
兒童心臟科 張文王醫師

3. Congenital Heart Disease
Cyanotic Congenital Heart Disease
Lesions associated with decreased pulmonary blood flow
TOF, 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…
2006/4/4
兒童心臟科 張文王醫師

4. 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 leaflet of the TV
Supracristal type : superior to the crista supraventricularis , 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)
2006/4/4
兒童心臟科 張文王醫師

5. Congenital Heart Disease---VSD
2006/4/4
兒童心臟科 張文王醫師

6. Congenital Heart Disease---VSD
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
2006/4/4
兒童心臟科 張文王醫師

7. Congenital Heart Disease---VSD
2006/4/4
兒童心臟科 張文王醫師

8. Congenital Heart Disease---VSD
Clinical Manifestations
Vary according to the size of the defect and pulmonary blood flow and pressure
Small : asymptomatic, PE revealed a loud harsh or blowing 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
2006/4/4
兒童心臟科 張文王醫師

9. Congenital Heart Disease---VSD
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
2006/4/4
兒童心臟科 張文王醫師

10. Congenital Heart Disease---VSD
Diagnosis (2)
2-D echocardiogram and color Doppler
Show the position and size
Estimating shunt size by examining the degree of volume overload of LA
Pulsed Doppler calculated the pressure gradient and RV, PA pressure
Cardiac catheterization
Performed only when the size of shunt is uncertain, when Lab data do not fit well with clinical findings, when pul vascular disease is suspected.
Pre-op Qp:Qs ratio
2006/4/4
兒童心臟科 張文王醫師

11. Congenital Heart Disease---VSD
2006/4/4
兒童心臟科 張文王醫師

12. Congenital Heart Disease---VSD
Prognosis and Complications (1)
Natural course of a VSD depends to a large degree on the size of the defect.
30-50% of small defects close spontaneously during 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 shunt.
Long-term risk is infective endocarditis.
2006/4/4
兒童心臟科 張文王醫師

13. Congenital Heart Disease---VSD
Prognosis and Complications (2)
Less common for mod. or large VSDs to close spontaneously
Repeated episodes of URI and heart failure ( infant-failure to thrive)
At risk for pul. Vascular disease with time as a result of high pul blood flow
Development of aortic valve regurgitation – the greatest risk occurring in p’ts with supracristal VSD
2006/4/4
兒童心臟科 張文王醫師

14. Congenital Heart Disease---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
2006/4/4
兒童心臟科 張文王醫師

15. Congenital Heart Disease---VSD
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, 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
2006/4/4
兒童心臟科 張文王醫師

16. 感謝聆聽 敬請指教
2006/4/4
兒童心臟科 張文王醫師