CONGENITAL HEART DEFECTS DR. HANA OMER
CONGENITAL HEART DEFECTS
D. HANA OMER
• The major development of the fetal heart occurs between the fourth and seventh weeks of gestation, and most congenital heart defects arise during this time.
• resulting from an interaction between a genetic predisposition toward development of a heart defect and environmental influences.
• Approximately 13% of children with congenital heart disease have an associated chromosomal abnormality.
• maternal conditions and teratogenic influences, including maternal diabetes, congenital rubella, maternal alcohol
ingestion, and treatment with anticonvulsant drugs.
Acyanotic and Cyanotic Disorders
• It is devided into cyanotic and acyanotic disorders .• Left-to-right shunts commonly are categorized as acyanotic
disorders and right-to left shunts are cyanotic disorders.• Shunting of blood refers to the diverting of blood flow from one system to the other from the arterial to the venous system (i.e., left-to-right shunt) or from the venous to the
arterial system (i.e., right-to-left shunt).
CYANOTIC HEART DISEASE
1. Tetralogy of fallots.2. Transposition of great arteries (TGA).3. Tricuspid atresia.4. Truncus arteriosus.5. Eisenmenger’s syndrome.
Acyanotic Disorders
• With left to right shunt :-1. Atrial septal defect (ASD).2. Ventricular septal defect (VSD).3. Patent ductus arteriosis.
• With no shunt :-1. Coarctation of aorta.2. Congenital aortic stenosis.3. Pulmonary stenosis, tricuspid stenosis. 4. Ebstein’s anamoly.5. Dextrocardia.
MITRAL STENOSIS
• Almost all mitral stenosis is due to rheumatic heart disease .• Rheumatic mitral stenosis is much more common in women
(about 1/3 case) .• Rare causes of mitral stenosis may be congenital, or because
calcification and fibrosis of the valve in elderly .
PATHOPHYSIOLOGY
• The commisures of mitral valve become adherent and the chordae tendinae are short and deformed
• The normal mitral valve orifice is about 4-6 cm² in diastole, it is reduced to about 1 cm² in severe mitral stenosis.
• left atrial , pulmonary venous, pulmonary capillary pressure.
• Also result in atrial fibrillation pulmonary edema pulmonary hypertension .
• All cases may develop pulmonary hypertension and right ventricular hypertrophy .
• All patients with mitral stenosis are at risk of left atrial thrombosis and systemic thromboembolism .
• Mitral stenosis is frequently associated with mitral regurgitation or disease of the aortic or tricuspid valve .
MITRAL STENOSIS
CLINICAL FEATURES
SYMPTOMS
1. DYSPNEA .2. COUGH .3. PALPITATION . 4. FEATURES OF CHRONIC RIGHT HEART FAILURE .
ON EXAMINATION
• INSPECTION : left parasternal pulsation due to right ventricular hypertrophy .
• PALPATION :1. Apex beat not displaced .2. Tapping apex beat .3. Left parasternal heave .4. Palpable P₂ .• AUSCULTATION : HEART SOUNDS :1. Loud first heart sound .2. Loud P₂. 3. Opening snap .
• MURMURS :1. Mid-diastolic rumbling murmur .2. Pre-systolic accentuation of murmur .
COMPLICATION
COMPLICATION
1. ATRIAL FIBRILLATION .2. SYSTEMIC EMBOLIZATION .3. PULMONARY HYPERTENSION .4. PULMONARY INFARCTION .5. INFECTIVE ENDOCARDITIS .6. TRICUSPID REGURGITATION .7. RIGHT VENTRICULAR FAILURE .
INVESTIGATION
• X-RAY CHEST :1. Double shadow behind right heart :enlarged left atrium .2. Kerley’s B lines : which are small 1-2 cm horizontal lines
present in the costohrenic angle appearing due to raised pulmonary venous pressure .
• ECG .• ECO .
Kerley’s B lines
TREATMENT
MEDICAL TREATMENT
• MILD DYSPNEA : salt restriction, low doses of diuretics .• SINUS RHYTHM : beta blockers .• ATRIAL FIBRILLATION : beta blockers, calaium channel blocker • Prphylactic antibiotics to prevent infective endocarditis .
SURGICAL TREATMENT
• PERCUTANEOUS TRANSLUMINAL MITRAL COMMISSUROTOMY (PTMC) :
• INDICATIONS:1. Uncontrolled pulmonary edema .2. Symptoms of pulmonary congestion persist despite therapy .3. Recurrent systemic emboli despite anticoagulation .
THANK YOU .