congenital heart defects dr. hana omer. congenital heart defects d. hana omer

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CONGENITAL HEART DEFECTS DR. 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 .

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