Heterotaxy syndrome presenting as severe pulmonary artery hypertension in a young old female: case report

P. Papalkar 1, S. Kumar 1, S. Agrawal 1, N. Raisinghani 1, G. Marfani 2, A. Mishra 1

1 Department of Medicine, Jawahar Lal Nehru Medical College, Datta Meghe Institute of Medical Sciences (DU), Wardha, Maharashtra, India; 2 Department of Radiodiagnostics, Jawahar Lal Nehru Medical College, Datta Meghe Institute of Medical Sciences (DU), Wardha, Maharashtra, India

Heterotaxy syndrome is a rare congenital disorder characterized by situs ambiguus, congenital heart defects and splenic malformations. We describe a case of 65 year young- old female who presented with sudden onset dyspnoea in emergency department. Her chest x-ray, 2 D echo and computerised tomography of chest was suggestive of sever pulmonary hypertension as a rare manifestation of Heterotaxy syndrome. To the best of our knowledge, pulmonary hypertension has not been previously reported as the main clinical feature in young – old patients with Heterotaxy syndrome.

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