Congenital Heart Disease 先天性心臟病

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 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 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… 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) 兒童心臟科 張文王醫師

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

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

11. Congenital Heart Disease---VSD 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. 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 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 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, 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 兒童心臟科 張文王醫師

16. 感謝聆聽敬請指教 兒童心臟科 張文王醫師