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Biomedical subjects

S S Adwani

Publications and source records attributed to S S Adwani.

5 recordsLinked to original sources
PubMed1998

Ichthyosiform erythroderma and cardiomyopathy: report of two cases and review of the literature.

We report two children with ichthyosiform erythroderma who at the ages of 9 weeks and 8 years, respectively, developed dilated cardiomyopathy, which was fatal in one and required heart transplantation in the other. A link between these conditions is considered likely, either as a primary genetic syndrome or secondary to micronutrient deficiency and/or infection. Owing to its insidious onset, cardiomyopathy may be overlooked, or symptoms attributed to the other conditions such as severe infections and failure to thrive that are common in these patients. We therefore recommend that children with congenital erythroderma are monitored closely, clinically and with echocardiography, for cardiomyopathy.

Cardiomyopathy, Dilated↗
PubMed1997

Percutaneous transhepatic dual chamber pacing in children with Fontan circulation.

Permanent pacing is often required following the Fontan operation and is usually performed epicardially as there is no direct access to the ventricle from the systemic veins. Dual chamber endocardial pacing was achieved by the transhepatic approach in two children with Fontan circulation. The patients were a 7 year old boy with left atrial isomerism, single ventricle with pulmonary stenosis, interrupted inferior vena caval vein with azygous continuation, and direct drainage of the hepatic veins to the right sided atrium, and a 6 year old girl with tricuspid atresia. This approach to endocardial pacemaker implantation is potentially of considerable value in patients who do not have direct access to the ventricle from the systemic veins.

Cardiac Pacing, Artificial↗
PubMed1995

Heart transplantation for dilated cardiomyopathy.

Between 1988 and 1994, 23 patients underwent heart transplantation for dilated cardiomyopathy. The age of the 13 boys and 10 girls was from 8 months to 16 years (mean 7.1 years). Selection criteria included failure to thrive despite maximal antifailure treatment and/or intravenous inotrope dependence. The aetiology of cardiomyopathy was idiopathic (n = 13), congenital (n = 3), anthracycline induced (n = 4), Barth's syndrome (n = 1), and maternal systemic lupus erythematosus (n = 2). The waiting period of heart transplantation ranged from one day to 147 days (mean 22 days). Maintenance immunosuppression included cyclosporin, azathioprine, and prednisolone. Follow up after transplantation was from one month to 62 months (median 27 months) with a mean actuarial survival of 95% at one year and 87% at three years. Four patients developed coronary artery disease, one of whom died as a consequence 15 months after heart transplantation. Heart transplantation has emerged as an acceptable therapeutic option, at least in the short term, for patients with dilated cardiomyopathy.

Adolescent↗
PubMedDate not supplied

Heart transplantation for Barth syndrome.

Barth syndrome is an X-linked recessive disorder comprising dilated cardiomyopathy, muscular hypotonia, and cyclical neutropenia. Affected children usually die during infancy as a consequence of septicemia, cardiac failure, or both. We report a patient with Barth syndrome who underwent successful heart transplantation.

Cardiomyopathies↗