Pulmonary atresia and ventricular septal defect with MAPCAs associated with right sided endocarditis and paradoxical embolic event

European Journal of Echocardiography : the Journal of the Working Group on Echocardiography of the European Society of Cardiology
Kivilcim OzdenYelda Basaran

Abstract

Pulmonary atresia and ventricular septal defect (PA-VSD) with major aortopulmonary collaterals (MAPCAs) is a complex and extremely heterogeneous anomaly. PA-VSD with both pulmonary arteries originating from systemic arterial circulation without MAPCAs and patent ductus arteriosus (PDA) is a very rare disease and according to our knowledge a case without cyanosis and symptoms of congestive heart failure after the first decade of life has not been reported. The majority of untreated patients die in their first decade of life as a result of intractable congestive heart failure or respiratory distress. This report informs about a 21-year-old PA-VSD patient who presented without cyanosis with both pulmonary arteries arising from aorta associated with right sided endocarditis and a paradoxical embolic event.

Citations

Jan 20, 2021·International Reviews of Immunology·Maryam MohtashamiReza Yazdani

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