Rapitful Shqip

What are the constituents of Fallot's tetralogy ?   • VSD with a right-to-left shunt. • Pulmonary stenosis (infundibular or valvular). • Right ventricular hypertrophy. • Dextroposition of the aorta with it overriding the ventricular septal defect.

What are the fundal findings in coarctation of aorta ?  Hypertension due to coarctation of aorta causes retinal arteries to be tortuous with frequent 'U' turns; curiously, the classical signs of hypertensive retinopathy are rarely seen.

What are the complications of aortic coarctation ? •Severe hypertension and resulting complications: • Stroke. • -Premature coronary artery disease. • Left ventricular failure (two thirds of patients over the age of 40 years who have • uncorrected aortic coarctatioh have symptoms of heart failure). • Rupture of aorta. • Infective endocarditis endarteritis (at the site of the coarctation or on a con-genitally bicuspid aortic valve). • Intracranial haemorrhage (combination of hypertension and ruptured berry aneurysm). • Three quarters die by the age of 50, and 90% by the age of 60 (Bt Heart J 1970: 32: 63340).

What conditions are associated with coarctation of aorta ?  It may occur in conjunction with gonadal dysgenesis (e.g. Turner's syndrome), bicuspid aortic valve, ventricular septal defect, patent ductus arteriosus, mitral stenosis or regurgitation, or aneurysms of the circle of Willis.

Is aortic coarctation more common in men or women ?  This condition is two to five times as frequent in men and boys as in women and girls.

What is Erb's point ?  The third left intercostal space adjacent to the sternum is Erb's point. The murmur of infundibular pulmonary stenosis is best heard in this space and in the left lourth intercostal space.

What are the types of pulmonary stenosis ? • Valvular. • Subvalvular: infundibular and subinfundibular. • Supravalvular.

How is the severity of pulmonary valve stenosis determined ?  • Mild: it' the valve area is larger than 1.0 cm2 per square metre, the transvalvular gradient is less than 50 mmHg, or the peak right ventricular systolic pressure is less than 75 mmHg. • Moderate: if the valve area is 0.5-1.0 cm2 per square metre, the transvalvular gradient is 50-80 mmHg, or the right ventricular systolic pressure is 75-100 mmHg. • Severe: if the valve area is less than 0.5 cm2 per square metre, the transvalvular gradient is more than 80 mmHg, or the right ventricular systolic pressure is more than 100 mmHg.

What is the normal area of the pulmonary valve ?  The area of the pulmonary valve orifice in a normal adult is about 2.0 cm2 per square metre of body surface area, and there is no systolic pressure gradient across the valve.

What is the underlying cause of pulmonary stenosis ? • Congenital (commonest cause). • Carcinoid tumour of the small bowel.

Mention a few causes of continuous murmurs.   • Venous hum. • Mitral regurgitation murmur with aortic regurgitant murmur. • VSD with aortic regurgitation. • Pulmonary arteriovenous fistula. • Rupture of the sinus of Valsalva. • Coronary arteriovenous fistula. • Arteriovenous anastomosis of intercostal vessels following a fractured rib.

Mention a few causes of a collapsing pulse. Hyperdynamic circulation due to: • Aortic regurgitation. • Thyrotoxicosis. • Severe anaemia. • Paget's disease. • Complete heart block.

What do you know about the genetics of hypertrophic cardiomyopathy ?  • Hypertrophic cardiomyopathy is an autosomal dominant heart muscle disorder. • Mutations in the gene encoding contractile proteins cause disease in 50-60'7c of patients. • There are mutations in the gene encoding for myofibrillary proteins: at least 9 individual genes have been identified. Beta heavy chain myosin gene mutations are associated with left ventricular outflow obstruction, whereas troponin T mutations are associated with rather modest left ventricular wall thickening, and mutations in myosin binding protein C are associated with onset in late adult life. Arginine gene mutations have a worse prognosis than leucine gene mutations.

Which condition has the most common association with hypertrophic cardiomyopathy ?  Friedreich's ataxia.

What is the most characteristic pathophysiological abnormality in hypertrophic cardiomyopathy ?  Diastolic dysfunction.

What are the complications of hypertrophic cardiomyopathy ? • Sudden death. • Atrial fibrillation. • Infective endocarditis. • Systemic embolization.

Is prophylaxis against infective endocarditis recommended in atrial septal defect ?  Prophylaxis against infective endocarditis is not recommended for patients with atrial septal defects (repaired or unrepaired) unless a concomitant valvular abnor-mality (e.g. mitral valve cleft or prolapse) is present. Leonardo da Vinci's description in 1513 of a 'perforating channel' in the atrial septum is believed to be the first recorded account of a congenital malformation of the human heart. Rene Lutembacher, a French physician, described the Lutembacher syndrome in 1916. Mary Holt, cardiologist, King% Culluge Hu~pltdI, London. Samuel Oram, cardiologist, King's College Hospital, London.

How would you manage an uncomplicated atrial septal defect ?   Early childhood   If the defect is detected in early childhood, surgical closure is recommended between the ages of 5 and 10 years to prevent the late onset of either right ven-tricular failure, atrial arrhythmias or right heart failure.   In adults   • Small ASDs can be left alone, although many believe that all ASDs must be closed. Those operated on before the age of 25 years have an excellent prognosis and one may anticipate normal long-term survival, but older patients require regular supervision. In a recent study, surgical repair of atrial septal defects in middle-aged and elderly patients was found to improve longevity and reduce functional limitation due to heart failure, and is therefore superior to medical treatment. However, the risk of atrial arrhythmias, especially fibrillation and flutter, and the attendant risk of thromboembolic events was not reduced by closure of the defect.   • Left-to-right shunt sat

How is pregnancy tolerated in a woman with atrial septal defect ?  Pregnancy is usually well tolerated in uncomplicated atrial septal defects; however, when the defect is complicated by significant pulmonary hypertension there is increased maternal and fetal morbidity and mortality and hence pregnancy should be avoided in Eisenmenger syndrome. Rapidly progressive pulmonary vascular disease may develop during pregnancy, therefore routine closure of atrial septal defect is recommended before pregnancy.

What are the complications of atrial septal defect ?  • Atrial arrhythmias: atrial fibrillation is most common. Atrial fibrillation is often accompanied by the appearance of tricuspid regurgitation. Patients are usually in normal sinus rhythm in the first three decades of life, alter which atrial arrhythmias including atrial fibrillation and supraventricular tachycardia may appear. • Pulmonary hypertension with the development of right ventricular disease. • Eisenmenger syndrome with reversal of shunt. • Paradoxical embolus. • Infective endocarditis in patients with ostium primum defects only.  • Recurrent pulmonary infections.