小儿心脏病的治疗

Approach to child with heart disease Pushpa Raj Sharma Professor of Child Health Institute of Medicine Diseases of heart Pericardium Myocardium Endocardium Blood vessels Prevalence n Congenital n Cyanotic: 22% n Acyanotic: 68% n VSD 25% n ASD 6% n PDA 6% n TOF 5% n PS5% n AS 5% n Acquired n Kawasaki disease n Rheumati c n Tubercul ar n Collagen Ceylon Med J 2001 Sep; 46 (3): 96-8; Indian J Pediatr. 2001 Aug;68 (8):757-7 Nelsons Textbook of pediatrics; 17 ed. Common acyanotic lesions n Ventricular septal defects n Atrial septal defects n Atrio-ventricular septal defects n Patent ductus arteriosus n Truncus arteriosus n Pulmonary stenosis n Aortic stenosis n Mitral stenosis/incompetence n Coarctation of aorta n Tricuspid regurgitation Common Cyanotic Lesions Decreased flow 1. Tetralogy of Fallot 2. Tricuspid Atresia 3. Severe Pulmonic Stenosis 4. Ebsteins anamoly Increased Flow 5. Transposition of great vessles 6. VSD with pulmonary atresia Common Lesions producing cyanosis 7. Truncus Arteriosus 8. Hypoplastic left heart 9. Single ventricle 10. TAPVR with infradiaphragmatic obstruction Presenting complaints/signs n Failure to thrive n Exercise intolerence n Easy fatigability n Chest indrawing n Sweating during feeding n Bluish spells n Fever with rigor n Palpitation n Convulsion n Fast breathing n Oedema n Hepatomegaly, n spleenomegaly n Clubbing n Cyanosis n Focal neurological lesion n Other organ defects n Chromosomal anomalies Cyanosis: is it a cardiac cause or lung cause n Hyperoxia test n Neonates with cyanotic congenital heart disease usually do not have significantly raised arterial Pao2 during administration of 100% oxygen. Ventricular Defect n Small VSD n Asymptom atic n A loud, harsh, or blowing holosystolic murmur. n Large VSD n dyspnea, feeding difficulties, poor growth, profuse perspiration, recurrent pulmonary infections, and cardiac failure in early infancy. 80% Syndromes associated with this condition VSD: ECG is normal but may show right ventricular hypertrophy, if present indicates defect is large and presence of pulmonary hypertension or pulmonry stenosis Ventricular Septal Defect (VSD) Large VSD: The presence of right ventricular hypertrophy, olegeimic lung fields (pulmonary hypertension or an associated pulmonic stenosis), gross cardiomegaly with prominence of both ventricles, the left atrium. Small VSDs, the chest radiograph is usually normal Ventricular Septal defects n 3050% of small defects close spontaneously, most frequently during the 1st 2 yr of life. n Small muscular VSDs are more likely to close (up to 80%) than membranous VSDs are (up to 35%). n infants with large defects have repeated episodes of respiratory infection and heart failure despite optimal medical management. n Surgical repair prior to development of an irreversible increase in pulmonary vasculalr resistance (usually prior to the patients second birthday). Atrial Septal Defects: secundum n Most common form of ASD (fossa ovalis) n In large defects, a considerable shunt of oxygenated blood flows from the left to the right atrium. n Mostly asymptomatic n The 2nd heart sound is characteristically widely split and fixed. Secundum Atrial Septal Defects:primum n Situated in the lower portion of the atrial septum and overlies the mitral and tricuspid valves. In most instances, a cleft in the anterior leaflet of the mitral valve is also noted. n Combination of a left-to-right shunt across the atrial defect and mitral insufficiency n C/F similar to that of an ostium secundum ASD Atrial Septal Defect n Enlargement of the right ventricle n Enlargement of atrium n Large pulmonary artery n increased pulmonary vascularity is. The electrocardiogram in patients with a complete AV septal defect is distinctive. The principal abnormalities are (1) superior orientation of the mean frontal QRS axis with left axis deviation to the left upper or right upper quadrant, (2) counterclockwise inscription of the superiorly oriented QRS vector loop, (3) signs of biventricular hypertrophy or isolated right ventricular hypertrophy, (4) right ventricular conduction delay (RSR pattern in leads V3 R and V1 ), (5) normal or tall P waves, and (6) occasional prolongation of the P-R interval Atrial Septal Defects n Secundum ASDs are well tolerated during childhood. n Antibiotic prophylaxis for isolated secundum ASDs is not recommended. n Surgery or transcatheter device closure is advised for all symptomatic patients and also for asymptomatic patients with a Qp:Qs ratio of at least 2:1. n Ostium primum defects are approached surgically Patent Ductus Arteriosus n Small defect no symptoms. n Large defect: n Wide pulse pressure n Enlarged heart n Thrill in L second IS n Continuo us murmur n X-ray: prominent pulmonary artery with increased vascular markings. Primary Pulmonary Hypertension n Prominent pulmonary artery. n Prominent right ventricle n Prominent vascularity in the hilar areas n Decreased vascualr marking in the periphery. n No treatment Mitral insufficiency: Rheumatic High volume load Inflammatory process Enlarged left ventricles Dilatation of the left atrium Pulmonary congestion Symptoms of left sided failure Repeated insult Spontaneous improvement Chronic mitral insufficiency Raised Pulmonary AP Enlarged right ventricle and atriumSymptoms of right heart failure Mitral insufficiency: Rheumatic n Signs of heart failure n Heaving apical impulse n Apical systolic thrill n Accentuated 2nd sound n Holosystolic murmur radiating to axilla n ECG: bifid P waves and left ventricular hyertrophy n X-ray: prominent left atrium and ventricle (straight left border)Prophylaxis against recurrence of rheumatic fever Rheumatic valvular disease: Mitral stenosis n Takes 10 years to develop n Symptoms proportionate to severity n Left ventricular failure right ventricular failure n Loud first heart sound with opening snap. n Diastolic murmur n Absent murmur if heart failure. n Surgical intervention if symptomatic Mitral Stenosis n Loud 1st sound n Diastolic murmur n left atrial enlargement n prominence of the pulmonary artery n enlarged right -sided heart chambers; n ECG: prominent notched P wave. Pericardial Effusion n Presenting complaint n Precordial pain n Cough n Dyspnoea n Abdominal pain n Vomiting n Fever n Other organs involvement n Signs: n Position: leaning forward. n Puffy face n Friction rub n Absent apical impulse n Muffled heart sounds n Pulsus paradoxus n Distended neck veins n Low QRS complex, T inversion Pericardial Effusion n A relatively large pericardial effusion must be present to cause an enlarged cardiac shadow with the usual “water bottle” configuration on a chest roentgenogram The test that differentiates The cardiac seize and the vascularity in the chest X