11. Congenital Heart Disease1.pptxvvvvhg

Congenital heart diseases

1. CARDIOLOGY Cardiology lecture 11 :- Congenital Heart Disease part 1 Date:- 20/12/2020 Prepared by Dr Guled Mohamud Nur (GMN@N ) MBBS

2. Congenital heart disease refers to a defect in the structure of the heart and great vessels , which is present at birth .

3. Epidemiology Approximately 0.8% of the population is born with congenital heart disease. Up to 40% of them are cured spontaneously ( mainly small VSDs ) and, with current surgical and interventional techniques, 85% survive into adulthood (grown-up congenital heart disease— GUCH ) According to data of the European Surveillance of Congenital Abnormalities, the live birth prevalence of congenital heart disease is 7/1000 births .

4. Congenital Heart Disease In most cases, diagnosis usually made by age 1 month. Murmurs may not be heard in early life because of increased pulmonary vascular resistance (from fetal to neonatal transition physiology). Etiology Most are unknown Associated with teratogens , such as alcohol and rubella Genetic predisposition trisomies ; Marfan, Noonan, DiGeorge syndromes.

5. LEFT TO RIGHT SHUNTS Ventricular Septal Defect (VSD)

6. Ventricular Septal Defect (VSD) Most common congenital heart lesion Most are membranous Shunt determined by ratio of PVR to SVR As PVR falls in first few weeks of life, shunt increases When PVR>SVR, Eisenmenger syndrome (must not be allowed to happen).

7. Eisenmenger Syndrome Transformation of any untreated left-to-right shunt into a bidirectional or right-to-left shunt Characterized by cyanosis Results from high pulmonary blood flow, causing medial hypertrophy of pulmonary vessels and increased pulmonary vascular resistance.

8. Clinical findings Asymptomatic if small defect with normal pulmonary artery pressure (most); large defect dyspnea, feeding difficulties, poor growth, sweating, pulmonary infection, heart failure Harsh holosystolic murmur over lower left sternal border ± thrill; S2 widely split With hemodynamically significant lesions, also a low-pitched diastolic rumble across the mitral valve heard best at the apex.

9. Diagnosis Chest-ray (large heart, pulmonary edema ), ECG (LVH), Echocardiogram is definitive

10. Treatment Small muscular VSD more likely to close in first 1–2 years than membranous Less common for moderate to large to close → medical treatment for heart failure (control failure and prevent pulmonary vascular disease) Surgery in first year ; indications: Failure to thrive or unable to be corrected medically Infants at 6–12 months with large defects and pulmonary artery hypertension

11. Complications Large defects lead to heart failure , failure to thrive Endocarditis Pulmonary hypertension

12. Atrial Septal Defect (ASD) Ostium secundum defect most common (in region of fossa ovalis) Clinical Few symptoms early in life because of structure of low-flow, left-to-right shunt In older children, often with large defects; varying degrees of exercise intolerance With hemodynamically significant lesions, also a low-pitched diastolic rumble across the tricuspid valve heard best at the lower sternum.

13. Physical examination Wide fixed splitting of S2 Systolic ejection murmur along left mid to upper sternal border (from increased pulmonary flow)

14. Diagnosis Chest x-ray varying heart enlargement (right ventricular and right atrial); increased pulmonary vessel markings, edema ECG right-axis deviation and RVH Echocardiogram Definitive

15. Treatment Most in term infants close spontaneously; symptoms often do not appear until third decade Surgery or transcatheter device closure for all symptomatic patients or 2:1 shunt Complications Dysrhythmia

16. Patent Ductus Arteriosus (PDA) Results when the ductus arteriosus fails to close; this leads to blood flow from the aorta to the pulmonary artery Risk factors More common in girls by 2:1 Associated with maternal rubella infection Common in premature infants (developmental, not heart disease).

17. Presentation If small possibly no symptoms If large heart failure, a wide pulse pressure, bounding arterial pulses, characteristic sound of “machinery,” decreased blood pressure (primarily diastolic).

18. Diagnostic tests Chest x-ray increased pulmonary artery with increased pulmonary markings and edema; moderate-to-large heart size ECG left ventricular hypertrophy Echocardiogram increased left atrium to aortic root; ductal flow, especially in diastole

19. Treatment May close spontaneously Indomethacin (preterm infants) Surgical closure Complications Congestive heart failure Infective endocarditis

20. Reference By Clinical Cardiology Current Practice Guidelines And Short textbook of medical diagnosis & treatment. 11 th Edition