World Journal of
Pharmaceutical and Life Sciences

CHILDHOOD HYPERTROPHIC CARDIOMYOPATHY: AN UNDERRECOGNIZED ENTITY

Anju Khairwa and Kim Vaiphei*

ABSTRACT

Background: Hypertrophic cardiomyopathy (HCM) is a known cause for sudden death in young adults and adults. It is also been increasingly recognized as an important underlying etiology in neonates and young children. Aim: to analyze the clinical profiles and to correlate with the histopathological features in 45 neonates and pediatric patients at autopsy. Material and methods: Retrospective study in 45 autopsy cases in whom unexplained left or both right and left ventricular hypertrophy were observed during gross examination of the organs. Detail histological studies were carried out in the multiple sections sampled form heart. Results: Clinically only one case was diagnosed as hypertrophic congenital heart disease, remaining cases had various clinical diagnoses including recurrent pneumonia. Grossly the heart weights were increased in all the cases with equal numbers of symmetrical and asymmetric ventricular hypertrophy. One third of the cases had associated cardiac anomaly. Microscopic examination showed classical histological features of HCM. There were myocardial fiber disarray with hypertrophied myocytes, anisonucleosis, streaky myocardial and perivascular fibrosis, patchy endocardial sclerosis and swollen endothelial cells of the perforating vessels. Conclusion- diagnosis of HCM may be difficult clinically. Autopsy may be considered in aa unresponsive and unexplained deaths. Histological features of HCM are characteristic to make the diagnosis.

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