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CONGENITAL HEART DISEASE.

CONGENITAL HEART DISEASE. Anatomy of the Heart. Figure 12.2. Epidemiology. Prevalence:0.5-0.8% of live births (8/1000).Leading cause ofdeath in children with CHD. Etiology:Unknown,multifactorial inheritance,genetic factors implicated,high incidence in first degree relatives.

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CONGENITAL HEART DISEASE.

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  1. CONGENITAL HEART DISEASE.

  2. Anatomy of the Heart Figure 12.2

  3. Epidemiology • Prevalence:0.5-0.8% of live births (8/1000).Leading cause ofdeath in children with CHD. • Etiology:Unknown,multifactorial inheritance,genetic factors implicated,high incidence in first degree relatives. • 3% have a single gene defect,13% have associated chromosomal abnormalities. • 2-4% are associated with environmental or maternal conditions & teratogenic influences. • Gender differences:ASD,VSD,PDA & Pulmonic stenosis more common in girls,left sided lesions in boys.

  4. Recurrence of CHD Congenital Heart Disease: Etiologies Most cases (70-80%) are “multifactorial” The Recurrence Risk with: - 1 sib with CHD: 2-4% - 2 sibs with CHD: 6-12% - Mother with CHD:6-12% - Father with CHD: 2-4% In 1/2 of these families the same defect recurs.

  5. Common acyanotic lesions *Ventricular septal defects *Atrial septal defects *Atrio-ventricular septal defects *Patent ductus arteriosus *Truncus arteriosus *Pulmonary stenosis *Aortic stenosis *Mitral stenosis/incompetence *Coarctation of aorta *Tricuspid regurgitation

  6. Common Cyanotic Lesions Decreased flow 1. Tetralogy of Fallot 2. Tricuspid Atresia 3. Severe Pulmonic Stenosis 4. Ebstein’s anamoly Increased Flow 5. Transposition of great vessles 6. VSD with pulmonary atresia

  7. Common Lesions producing cyanosis 7. Truncus Arteriosus 8. Hypoplastic left heart 9. Single ventricle 10. TAPVR with infradiaphragmatic obstruction

  8. Etiology • Congenital Heart Disease: Etiologies • 6-12% have gross chromosomal anomalies • - Trisomy 21 (40% have CHD): AV canal • - Trisomy 18 (100% have CHD): VSD, PS • - Trisomy 13- 15: VSD, ASD, TGV • - XO (Turner): Coarc, AS, VSD • - XXY (Klinefelter): Ebstein, Tetralogy

  9. Congenital Cyanotic: 22% Acyanotic: 68% VSD 25% ASD 6% PDA 6% TOF 5% PS 5% AS 5% Acquired Kawasaki disease Rheumatic Tubercular Collagen Prevalence Ceylon Med J 2001 Sep; 46 (3): 96-8; Indian J Pediatr. 2001 Aug;68 (8):757-7 Nelson’s Textbook of pediatrics; 17 ed.

  10. Fetal Physiology Right-to-left shunting at atrial level (PFO) and at arterial level (ductus arteriosus) High pulmonary vascular resistance Little pulmonary blood flow Ventricles work in parallel

  11. Transition From the Fetal Circulation Pulmonary vascular resistance falls Ductus venosus and ductus arteriosus close Right-to-left shunting through foramen ovale ceases Timing of these events determines the timing of presentation of congenital heart defects

  12. Cyanosis: is it a cardiac cause or lung cause • Hyperoxia test • Neonates with cyanotic congenital heart disease usually do not have significantly raised arterial Pao2 during administration of 100% oxygen.

  13. Small VSD Asymptomatic A loud, harsh, or blowing holosystolic murmur. Large VSD dyspnea, feeding difficulties, poor growth, profuse perspiration, recurrent pulmonary infections, and cardiac failure in early infancy. Ventricular Defect 80% Syndromes associated with this condition

  14. VSD: ECG is normal but may show right ventricular hypertrophy, if present indicates defect is large and presence of pulmonary hypertension or pulmonry stenosis

  15. Ventricular Septal Defect (VSD) Small VSDs, the chest radiograph is usually normal 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.

  16. Ventricular Septal defects • 30–50% of small defects close spontaneously, most frequently during the 1st 2 yr of life. • Small muscular VSDs are more likely to close (up to 80%) than membranous VSDs are (up to 35%). • infants with large defects have repeated episodes of respiratory infection and heart failure despite optimal medical management. • Surgical repair prior to development of an irreversible increase in pulmonary vasculalr resistance (usually prior to the patient's second birthday).

  17. Investigations • CXR:cardiomegaly,enlarged LA&LV. • ECG:extreme lt axis is charecteristic,biventricular hypertrophy. • ECHO:chamber size & pressures. • Cardiac catheter:O2 content,PA pressure,size & no of defects. • Treatment:Endocarditis prophylaxis,digoxin,diuretics. • Surgical closure before pulmonary vascular changes become irreversible.

  18. ATRIAL SEPTAL DEFECT. • Sinus venosus defect:high in the septum. • Ostium secundum defect:midseptum. • Ostium primum defect:low in the septum. • Pathophysiology:L-R shunt-increased flow across Rt heart-RV & PA enlargement. • Clinical features:asymptomatic,slow wt gain,frequent LRTI. • Diagnosis:Rt ventricular heave,systolic murmur,fixed wide split S2.

  19. Most common form of ASD (fossa ovalis) In large defects, a considerable shunt of oxygenated blood flows from the left to the right atrium. Mostly asymptomatic The 2nd heart sound is characteristically widely split and fixed. Atrial Septal Defects: secundum Secundum

  20. 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. Combination of a left-to-right shunt across the atrial defect and mitral insufficiency C/F similar to that of an ostium secundum ASD Atrial Septal Defects:primum

  21. Enlargement of the right ventricle Enlargement of atrium Large pulmonary artery increased pulmonary vascularity is. Atrial Septal Defect

  22. ASD primum&secondum

  23. Investigations: • CXR:enlarged heart & PA,increased vascularity. • ECG:Rt axis in secundum defect,hallmark of primum defect is extreme Lt axis,RVH. • ECHO:RVH,valve anatomy,flow direction. • Treatment:closure during cardiac cathetrization,surgical closure.

  24. PATENT DUCTUS ARTERIOSUS. • Connection between PA & descending aorta • 10% of CHD. • Pathophysiology:Lt-Rt shunt,reverses if pulmonary resistance increases-RV enlargement.If PDA is large Eissenmenger syndrome can develop. • Clinical features:depend on size & direction of flow,slow growth,LRTI,SOB,cyanosis. • Diagnosis:bounding pulse,continous murmur,loud S2.

  25. Patent Ductus Arteriosus • Small defect no symptoms. • Large defect: • Wide pulse pressure • Enlarged heart • Thrill in L second IS • Continuous murmur • X-ray: prominent pulmonary artery with increased vascular markings.

  26. Investigations • CXR:cardiomegaly,increased pul vascularity. • ECG:Lt or biventricular hypertrophy. • ECHO:2D visualises PDA,doppler shows turbulance. • Cardiac catheter:PA pressures & O2 sats. • Treatment:Endocardial prophylaxis as long as patent,Indomethacin. • Surgical:ligation is curative.

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