Abstract

Congenital Heart Diseases (CHD) carry significant morbidity and mortality in paediatric patients. Transposition of Great Arteries (TGA) is a common cyanotic CHD. However, onset of Pulmonary Arterial Hypertension (PAH) is potentially severe and rare complication reported in 1-3% of newborns. Hypertrophic Cardiomyopathy (HOCM) is a primary disease of cardiac muscle usually recognised in adulthood. Neonatal HOCM without other congenital anomalies, no family history of HOCM, no history of exposure to corticosteroids or any inborn errors of metabolism is rarely recognised. Authors hereby report two cases of neonatal autopsy showing TGA with PAH (Grade 2) and another with HOCM without any primary cause received in our hospital. With this case report authors wish to stress on the importance of cardiac examination (heart and the associated vessels) in autopsy specimen which in turn requires training of pathologists in cardiac pathology along with routine fetal anomaly scanning in arriving successively at the final diagnosis and detecting the cause of death which helps in management of future pregnancies by the clinicians.

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