A case of double outlet right ventricle, ventricular D-loop, L-malposition of the great arteries, and stenosis of the pulmonary outflow at valvar, supra-, and subvalvar level in a patient with situs solitus is described. Fibrotic structures closely resembling semilunar valves turned upside down, with the three pockets looking upstream were found in the subpulmonary infundibulum. They lay parall...

LEOPARD multiple congenital anomaly syndrome inherited in an autosomal dominant manner. LEOPARD is an acronym for Lentigines, Eletrocardiographic conduction defects, Ocular hypertelorism, Pulmonary valve stenosis, Abnormalities of the genitalia, Retardation of growth, and Deafness. Clinical diagnosis is primarily based on multiple lentigines, typical facial features, and the presence of hypertr...

Severe pulmonary stenosis presents early in life; late presentation is rare. Very late presentation like in our case is rarest of rare. Likewise calcification of pulmonary valve is rare. We describe here, one such case in an eighty-one year old female who astonishingly not only tolerated severe form of pulmonary stenosis but had also gone through three full term pregnancy, successfully without ...

In the assessment of cases of aortic valve disease with a view to operation, the standard methods of investigation are of limited value. Right heart catheterization gives some indirect information by measuring the cardiac output and the pulmonary capillary pressure. Some information can be obtained from a study of the peripheral arterial pulse wave, but this too cannot always supply a definite ...

Four patients with primitive ventricle and normally related great vessels with stenotic subpulmonary outlet chamber (Holmes' heart with pulmonary stenosis) are reported. The history, physical examination, and chest x-ray film are not helpful in distinguishing Holmes' heart with pulmonary stenosis from tetralogy of Fallot. Electrocardiogram often provides the first clue to the presence of Holmes...

Disruption of pulmonary valve integrity after Tetralogy of Fallot repair often results in a cascade of hemodynamic and electrophysiological abnormalities. Here we report an uncommon case of severe pulmonary regurgitation with concomitant rheumatic mitral stenosis diagnosed 25 years after primary Tetralogy of Fallot repair. A 33-year-old man presented with symptomatic palpitation and exercise in...

Four cases are presented to illustrate the echo patterns of pulmonary valve motion in patients with pulmonary regurgitation caused by pulmonary hypertension, idiopathic dilatation of the pulmonary artery, or congenital absence of the pulmonary valve or in association with pulmonary stenosis. Absence of the pulmonary 'a' wave, fluttering of the e-f slope, and midsystolic closure or 'notching' of...

A 61 year old man presented with mitral regurgitation and glomerulonephritis caused by suspected infective endocarditis. His glomerulonephritis remitted on immunosuppression but mitral valve replacement with a Carpentier-Edwards porcine xenograft later proved necessary. The patient became dialysis dependent and cadaveric renal allografting was complicated by delayed graft function and refractor...

Critical pulmonary valve stenosis (CPVS) and atrial septal defect (ASD) is an uncommon form of congenital heart disease. Concurrent transcatheter pulmonary valvuplasty and closure of secundum atrial septal defect appears to be an interesting alternative to surgical correction. We present the simultaneous balloon valvuloplasty of critical pulmonary stenosis (PS) with supra systemic right ventric...

BACKGROUND Pulmonary hypertension complicating severe aortic stenosis increases morbidity and mortality. Causes and mechanisms of this are unclear. METHODS This is a retrospective observational study of 626 patients with severe aortic stenosis who had measurable pulmonary arterial pressure by Doppler echocardiography. Clinical, echocardiographic and pharmacological data were related to the pr...