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European Journal of Echocardiography 2007 8(4):292-295; doi:10.1016/j.euje.2006.03.004
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Copyright © 2006, The European Society of Cardiology

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

Kivilcim Özden*, Bülent Mutlu, Gökhan Kahveci, Fatih Bayrak, Levent Saltik, Salih Güran and Yelda Basaran

Kartal Kosuyolu Heart Education and Research Hospital, Sonomed Imaging Center, Cerrahpasa Faculty of Istanbul University, Istanbul, Turkey

Received 16 September 2005; received in revised form 16 February 2006; accepted after revision 2 March 2006.

* Corresponding author. Bahar Sitesi B4 blok No: 9 Barbaros Mah.Kosuyolu, Uskudar, Istanbul, Turkey. Tel.: +905054549888. drkivilcim{at}yahoo.com


  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.

Keywords: Pulmonary atresia; Ventricular septal defect; Tricuspid endocarditis; Paradoxical embolia; Abnormal origin of both pulmonary arteries


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