Abstract
We present a very rare case of pulmonary atresia and ventricular septal defect with staged definitive repair more than 40 years after palliative surgery. The patient, a 43-year-old male, had undergone a Waterston operation at the age of one and had been untreated since then. Two years ago, he underwent an urgent surgery for impending rupture of a huge pulmonary artery aneurysm. Then, after evaluation of cardiac and pulmonary functions, a definitive repair was performed concomitantly with aortic root replacement for the dilated aortic root. He was discharged uneventfully and received ambulatory care 5 years after surgery.