Cardiac ultrastructure in tropical endomyocardial fibrosis.
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Biomedical subjects
Publications and source records attributed to M S Valiathan.
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Forty-six patients with endomyocardial fibrosis underwent endocardiectomy and replacement of tricuspid, mitral, or both atrioventricular valves between April, 1981, and October, 1984, at the Sree Chitra Tirunal Institute, Kerala State, India, which has a high incidence of the disease. Six patients were in New York Heart Association Functional Class III and 40 patients in Class IV. The operative mortality within 30 days of the procedure and late mortality during the first two years postoperation were 21.7% and 13%, respectively. Age under 15 years was a significant correlate of operative mortality (p = .05). Non-fatal thromboembolic episodes occurred in 6 patients during the two years of postoperative observation. The life table estimate of survival inclusive of operative mortality at two years was 67%. Despite high operative mortality, endocardiectomy with atrioventricular valve replacement is advisable for functionally disabled patients with endomyocardial fibrosis whose prognosis otherwise is dismal.
In a search for geochemical factors that could play a role in the pathogenesis of tropical endomyocardial fibrosis, endomyocardial tissue samples obtained from patients at necropsy or operation were analysed for major elements present in laterite and monazite, which are important soil constituents of Kerala State of India. Atomic absorption spectrophotometry was used for detecting iron, silicon, aluminium, zinc, magnesium, calcium, sodium, potassium, and manganese and neutron activation analysis for thorium. Compared with control samples from victims of fatal accidents, an excess of thorium, sodium, and calcium and a deficiency of magnesium were present in samples from patients. It has been shown earlier that the staple diets of people in Kerala have high concentrations of thorium, and these data show that thorium can become concentrated in cardiac tissues. It is speculated that thorium excess in conjunction with magnesium deficiency may play a role in the causation of tropical endomyocardial fibrosis.
25 patients with angiographic features suggestive of endomyocardial fibrosis underwent endocardiectomy and atrioventricular valve replacement. There were 8 deaths. 17 survivors were reevaluated after a mean follow-up of 13.5 months. 8 had right ventricular endomyocardial fibrosis (RVEMF) 5 had left ventricular endomyocardial fibrosis (LVEMF) and 4 had biventricular disease (BVEMF). The survivors showed a significant improvement in their clinical features and functional status. There was a significant reduction in atrial size in X-ray. Hemodynamic data showed a significant fall in right heart filling pressure, right ventricular systolic pressure and pulmonary artery mean pressure, and a significant fall in left heart filling pressure in patients with left ventricular disease either alone or as a part of biventricular disease. In ventriculogram, while there was no change in right ventricular shape after endocardiectomy, left ventricular shape was restored to near normal. The significant improvement is mostly due to the correction of the regurgitant lesion and to some extent due to restoration of ventricular compliance.
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Endomyocardial fibrosis is a fatal disease of obscure aetiology which is notably prevalent in Uganda, Brazil, Kerala State in India, and certain other parts of the world. The main disability in the disease results from fibrotic obliteration of ventricular chambers and insufficiency of the mitral and tricuspid valves. The poor response to medical treatment and the bleak prognosis of the patients led to the introduction of endocardiectomy and replacement of the atrioventricular valves as a palliative procedure by Dubost and colleagues. The present report on the early results in 17 patients from Kerala State would support the continued use of this palliative procedure in endomyocardial fibrosis with severe disability.
Right ventricular (RV) angiograms were done in 45 patients with left ventricular (LV) endomyocardial fibrosis (EMF). 34 patients had angiographic features compatible with the diagnosis of RV and LV EMF. 11 patients showed the angiographic features of EMF on the left side, but did not show the classical angiographic features on the right side. The RV angiograms of these 11 patients showed a disturbance of the normal parenchymal pattern along the septal margin, irregular small filling defects near the apex and the septal region and minimal tricuspid regurgitation. Biopsies from the RV taken from 2 of these patients during operation for mitral valve replacement had histological features compatible with the diagnosis of EMF on the right side also. This study demonstrates that the early angiographic features of RV EMF differ from those of well-established cases already described in the literature, and that it is possible to spot RV EMF well before the conventional features appear in RV angiograms.
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The surgical management of a case of right aortic arch with isolation of the left subclavian artery and bilateral patent ductus arterioses is described. A review of the 39 cases of isolation of the left subclavian artery reported in the literature revealed only one single case which on autopsy was found to have associated bilateral ductus arterioses. The case we report is the first in which the diagnosis was made during life and successful surgical correction was carried out.