Two-chambered right ventricle associated with primary pulmonary hypertension.
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Biomedical subjects
Publications and source records attributed to S G Kinare.
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Twenty eight autopsy specimens of Ebstein's anomaly were studied in order to evaluate the morphologic features of the abnormal tricuspid valve. All cases showed marked dilatation of the original tricuspid annulus, a normally positioned anterior leaflet and variable downward displacement of the posterior and septal leaflets. Sixteen cases showed a very large anterior leaflet. All three leaflets showed dysplastic features and a wide range of anatomic abnormalities in the valve and valve apparatus. A thin walled atrialised right ventricle was present in nine cases. Associated cardiac anomalies were seen in 21 cases, the commonest being an atrial septal defect (17 cases).
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Taussig-Bing Complex, an uncommon anomaly, was found in 8 out of 110 hearts of double outlet right ventricle (7.2%). The mean age at death was 23.5 months with maximum survival upto 10 years. There was only one case of dextrocardia; viscero-atrial and atrio-ventricular concordance was seen in all. Subpulmonary ventricular septal defect (VSD) was restrictive in two cases, in one of whom it was due to spontaneous closing process. Additional VSD was found in 2 cases. The great vessels showed side by side relationship with bilateral conii in 7 cases. In the remaining case there was L malposition with absent subpulmonary conus. Subpulmonary stenosis was noted in 3 cases. Tubular hypoplasia of aorta was seen in 2 cases with subaortic obstruction in one. Mitral valve abnormalities were present in 5 cases of which stenotic lesions were seen in 3. Coronary artery abnormalities were noted in 3 cases.
The morphological characters of 21 cases of single ventricle which constituted 1.63% of cases of congenital heart disease were studied. The single ventricular chamber with left ventricular characters was seen in 11 cases. In 8 of these, the great vessels were transposed with aorta arising from outlet chamber (SLL-7:SDD-1). Except in one case where there was common A-V valve, two A-V valve, two A-V valves entered the main chamber with some abnormality of A-V valves in all the cases. Bulbo-ventricular foramen was obstructive in 6 cases with resultant hypoplasia of aorta. Aortic arch anomalies were present in 5 of these. Valvular pulmonary stenosis was present in two. In 3 cases with normally related great vessels (SDS), bulbo-ventricular foramen was obstructive in two with hypoplastic pulmonary artery. Abnormalities of A-V valves were similar to the previous group. The incidence of single right ventricle was high in this series (47%). In half the cases, there was associated asplenia syndrome. This group in general showed common atrium with exception of one case, common A-V canal, both great vessels arising from same outflow with atrophic conal septum. Anomalies of pulmonary veins were common. The subsets observed were ADD-3, ADL-1, AL single trunk-1. In the remaining cases without asplenia, both A-V valves were present though some abnormalities were present in all. Systemic and pulmonary venous anomalies were rare. The subsets observed were SLL-3, SDL-1, SDD-1.(ABSTRACT TRUNCATED AT 250 WORDS)
Four cases of congenital aneurysm of sinus of Valsalva (CASV) with rupture and dissection into the intraventricular septum are described. Their incidence (26.6%) in 15 consecutive cases of CASV is unusually high. Only one of these showed secondary rupture into the left ventricle. If these aneurysms remain unruptured, their diagnosis during life is difficult as in the case of other unruptured aneurysms of CASV. The occurrence of conduction abnormalities in young patients should be one of the indications, and 2-D echocardiography would help to arrive at a definite diagnosis and aid in the institution of appropriate surgical treatment.
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