background total correction of tetralogy of fallot (tof) anomaly in early childhood has been practiced in many centers with good results, but in some of patients after few years sever pulmonary valve insufficiency occurred. materials and methods at a cross- sectional study from january 2015 to january 2016, 10 patients who had history of primary repair of tof with free pulmonary insufficiency (...

Three patients with severe congenital aortic stenosis in whom there was unusual thickening of the aortic valve associated with supra-aortic stenosis and aortic hypoplasia are described. The clinical presentation suggested the diagnosis of organic subaortic stenosis and surgical management presented problems. It is suggested that the aortic stenosis in these patients may be part of a more diffus...

PATIENTS presenting with high-pitched diastolic decrescendo murmurs along the left sternal border may have insufficiency of either the aortic or pulmonary semilunar valve. If the peripheral pulses are those of aortic insufficiency in type, the source of the diastolic murmur is located easily. However, in the absence of both the peripheral physical signs of aortic insufficiency and the electroca...

The calculated systolic, diastolic and mean pulmonary artery pressure, the ratio of sizes right left ventricles heart, eccentricity index ventricle, systolic excursion fibrous ring tricuspid valve, movement myocardium at level valve area atrium are main echocardiographic indicators for assessing idiopathic similar forms hypertension according to current Russian, Eurasian European guidelines in ...

Between 1971 and December 1976, 27 children with congenital heart disease underwent right ventricular outflow tract reconstruction with conduits incorporating a Björk-Shiley valve. The conditions corrected were pulmonary atresia with ventricular septal defect (14 patients), truncus arteriosus (6 patients), and complex disease (7 patients). At operation the children were aged from 12 days to 16 ...

Congenital heart valve defects in humans occur in approximately 2% of live births and are a major source of compromised cardiac function. In this study we demonstrate that normal heart valve development and cardiac function are dependent upon Galnt1, the gene that encodes a member of the family of glycosyltransferases (GalNAc-Ts) responsible for the initiation of mucin-type O-glycosylation. In ...

BACKGROUND Carcinoid tumor with consecutive endocardial fibroelastosis of the right heart, known as carcinoid heart valve disease (CHVD) or Hedinger's syndrome, is accompanied by combined right-sided valvular dysfunction with regurgitation and stenosis of the affected valves. Cardiac surgery with replacement of the tricuspid and/or pulmonary valve is an established therapeutic option for patien...

OBJECTIVE Cardiac manifestations of neurofibromatosis type 1 (NF1) may include hypertension, congenital heart disease, and hypertrophic cardiomyopathy. The aim of this study was to evaluate cardiac abnormalities in patients with NF1. METHODS Sixty-five NF1 patients (mean age: 9±4.48 years) were retrospectively studied. Standard electrocardiography and echocardiography were performed in all pa...

LEOPARD syndrome is an autosomal dominant genetic disorder involving a complex of malformations and other features, including lentiginosis, electrocardiographic conduction abnormalities, ocular hypertelorism, pulmonary valve stenosis, abnormal genitalia, growth retardation, and sensorineural deafness. The most common cardiovascular manifestations of this condition are pulmonary valve stenosis, ...

The autopsy on a 32-year-old man revealed an absence of the pulmonary valve, a ventricular septal defect, and a single coronary artery. Congenital absence of the pulmonary valve has not been previously reported. The diagnosis of the accompanying pulmonary incompetence is discussed in the light of the catheterization studies and clinical findings. The pathologic abnormalities as found at postmor...