Bardet-Biedl syndrome a rare cause of cardiomyopathy.

Bardet Biedl syndrome (BBS) is a rare autosomal recessive condition characterized by retinitis pigmentosa, polydactyly, obesity, learning disabilities, hypogonadism and renal anomalies. Cardiomyopathy in association with BBS has previously being reported only twice in literature. We report a case of a patient presenting with features of cardiomyopathy, who was subsequently diagnosed to have BBS.

The Tei index and asymptomatic myocarditis in children with severe dengue.

This study aimed to assess myocardial involvement in infants and children with severe dengue (as per the new World Health Organization [WHO] classification 2009) using the Tei index. This prospective observational study was conducted in the Department of Pediatrics, PGIMER and the associated Dr. RML Hospital, New Delhi from August to December 2010. The study included 67 children (ages 3 months-14 years) who satisfied the WHO criteria for the diagnosis of probable dengue fever with warning signs or severe dengue and tested positive for dengue via immunoglobulin-M (IgM) capture enzyme-linked immunoassay (MAC-ELISA). The patients were subjected to a complete blood count, liver function tests, renal profile, electrocardiography, myocardial band enzymes of creatine phosphokinase (CPK-MB), chest x-ray, abdomen ultrasonography, and two-dimensional echocardiography with color-flow Doppler mapping. Ejection fraction and Tei index measurements were performed. Significantly fewer patients with severe dengue were found to have myocardial involvement at admission by ejection fraction (48 %) and E/E' (37 %), than by the Tei index (70 %). Of the 67 patients with severe dengue, one died, giving a case fatality rate of 1.5 %. At discharge, the Tei index persisted on the high side for patients with myocardial involvement, whereas the ejection fraction improved for the majority of them. Most of the patients with severe dengue had asymptomatic myocarditis, as evident by a deranged Tei index, which improved but did not normalize by the time of discharge, necessitating a longer follow-up period. For the majority of the patients, inotropic support was not required to maintain hemodynamic stability.

Ruptured sinus of Valsalva masquerading as rheumatic heart disease.

Ruptured sinus of Valsalva is a rare lesion in pediatric age group. We are reporting a twelve year old child with hyperdynamic circulation being diagnosed and treated as Rheumatic heart disease.