Intravascular hemolysis may follow the insertion of prosthetic valves1 and patches at cardiac surgery2,3 and has recently been described after a classical Blalock-Taussig shunt.4 It has also occurred following transcatheter occlusion of a patent ductus arteriosus (PDA)5 and in acquired valvular heart disease.6 We believe this to be the first case of clinically important intravascular hemolysis occurring in unoperated congenital heart disease (ventricular septal defect [VSD] and PDA) that showed complete resolution after surgical correction.
Patient Report. A 5-week-old male infant presented with anemia associated with lethargy, poor feeding, and failure to thrive. There was no history of jaundice, and the family history was unremarkable. On examination he was clinically anemic, and there was a pansystolic murmur at the lower-left sternal edge associated with a thrill but with no evidence of heart failure. There was no hepatosplenomegaly.
Echocardiography demonstrated a perimembranous VSD almost completely occluded