Ten patients, each with two or more risk factors for morbidity and death, underwent a fenestrated Fontan procedure in which a 4 to 6 mm circular fenestration was left between the systemic and pulmonary venous chambers. None died; a similar group of high-risk patients without fenestration had a mortality rate of 2 of 8. Patients with fenestration had significantly less drainage from the chest tube, less need for inotropic support, and shorter intensive care and hospital stays than did patients without fenestration. Comparison with a group of low-risk patients undergoing the Fontan operation showed no statistical difference in these postoperative parameters. Fenestrations were closed in all 10 patients at from 9 days to 6 months after operation by means of the transcatheter clamshell occluder device. Two patients had left pulmonary artery balloon angioplasty and three patients had other atrial communications closed with additional clamshell devices. During short-term follow-up periods averaging 18 months, all patients were clinically well; however, one patient with mitral atresia required reoperation for obstruction between the left atrium and the tricuspid valve, not related to the clamshell device. These data indicate that fenestration may be one method of achieving lower morbidity and mortality rates among high-risk patients undergoing the Fontan procedure.