Santoro Giuseppe, Palma Gaetano, Merlino Ettore, Bigazzi Maurizio Cappelli, Palladino Maria Teresa, Calabrò Raffaele, Vosa Carlo
Department of Pediatric Cardiology, Second University of Naples, Naples, Italy.
Ital Heart J. 2003 Aug;4(8):559-61.
A 3-month-old infant with tetralogy of Fallot presented with progressive severe cyanosis and intractable acidosis about 2 months after a successful modified right-sided Blalock-Taussig shunt. At cardiac catheterization, the suspected shunt malfunction was confirmed. It was due to a bulky, pear-like mass arising from the right subclavian artery and compressing the polytetrafluoroethylene conduit. Any attempt to recanalize the shunt by percutaneous techniques proved unsuccessful. At surgery, a huge dilation of the anterior wall of the right subclavian artery, that sharply bent the prosthetic conduit, was found. Pathologic examination revealed that the compressing mass was pseudo-aneurysmal in nature. Despite a second successful shunt operation with a dramatic clinical improvement, the patient died due to multiorgan failure 72 hours following surgery. Extrinsic compression by a false aneurysm is a rare cause of shunt occlusion that should always be suspected in patients presenting with a rapidly progressive shunt malfunction late after a successful shunt procedure.