Congenital Heart Disease 先天性心臟病

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

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

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

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

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

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

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

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

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

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

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

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

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

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Congenital Heart Disease 先天性心臟病