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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
2006/4/4 兒童心臟科 張文王醫師2
Congenital Heart Disease
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
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
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…
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 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)
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 Disea
se---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 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
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 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
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 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.
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 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
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, 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
2006/4/4 兒童心臟科 張文王醫師16
感謝聆聽敬請指教