DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20210137

Ivemark syndrome with obstructed supracardiac TAPVC- case report

Sravani Vemuri, Chaitanya Challa, Vala Ramgopal Rao, Sudharshan Raj C.

Abstract


Heterotaxy disorder is a disturbance in the usual left and right distribution of the thoracic and abdominal organs. Ivemark syndrome is one such heterotaxy disorder. It is a rare disorder which affects males more than females with majority of cases presenting in the neonatal period mainly due to complex congenital cardiac disease. Here is a case report of rare disorder of the neonate with Ivemark syndrome with obstructed supracardiac total anomalous pulmonary venous connection (TAPVC). Management of obstructed supracardiac TAPVC is complicated as PGE1 infusion is contraindicated and immediate surgical correction is advised.


Keywords


heterotaxy, Obstructed supracardiac TAPVC, PGE1

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