Heterotaxy syndrome presenting as severe pulmonary artery hypertension in a young old female: case report
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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© Società Italiana di Gerontologia e Geriatria (SIGG) , 2018
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