Ivemark syndrome with obstructed supracardiac TAPVC- case report

Authors

  • Sravani Vemuri Department of Pediatrics, Mediciti Institute of Medical Sciences, Telangana, India
  • Chaitanya Challa Department of Pediatrics, Mediciti Institute of Medical Sciences, Telangana, India
  • Vala Ramgopal Rao Department of Pediatrics, Mediciti Institute of Medical Sciences, Telangana, India
  • Sudharshan Raj C. Department of Pediatrics, Mediciti Institute of Medical Sciences, Telangana, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20210137

Keywords:

heterotaxy, Obstructed supracardiac TAPVC, PGE1

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.

Author Biography

Sravani Vemuri, Department of Pediatrics, Mediciti Institute of Medical Sciences, Telangana, India

Paediatrics,

post graduate

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Published

2021-01-22

Issue

Section

Case Reports