Pulmonary atresia and ventricular septal defect with MAPCAs associated with right sided endocarditis and paradoxical embolic event.
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.
Ozden
Kivilcim
K
Kartal Kosuyolu Heart Education and Research Hospital, Sonomed Imaging Center, Cerrahpasa Faculty of Istanbul University, Istanbul, Turkey. drkivilcim@yahoo.com
Mutlu
Bülent
B
Kahveci
Gökhan
G
Bayrak
Fatih
F
Saltik
Levent
L
Güran
Salih
S
Basaran
Yelda
Y
D000328:Adult; D003097:Collateral Circulation / Q000502:physiology*; D003330:Coronary Vessel Anomalies; D019320:Embolism, Paradoxical / Q000000981:diagnostic imaging* / Q000503:physiopathology; D004697:Endocarditis, Bacterial / Q000000981:diagnostic imaging* / Q000503:physiopathology; D006345:Heart Septal Defects, Ventricular / Q000000981:diagnostic imaging*; D006801:Humans; D008297:Male; D011651:Pulmonary Artery / Q000473:pathology* / Q000601:surgery; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging* / Q000503:physiopathology; D011664:Pulmonary Valve / Q000473:pathology*; D014463:Ultrasonography