Modified Fontan procedure for Ebstein's anomaly of the tricuspid valve--an alternative surgical approach preserving Ebstein's anatomy.

In young patients with Ebstein's anomaly and symptoms of congestive heart failure or severe cyanosis tricuspid valve repair may be unsuccessful and prosthetic valve replacement is needed, which is associated with a high operative mortality. This report describes the implantation of a valved aortic homograft as a conduit between right atrium and pulmonary artery to at least postpone valve surgery in three symptomatic children (4.1, 8.3, and 10 years of age) with an arterial oxygen saturation of 75 to 82%. The intracardiac anatomy was left untouched except for closure of the associated secundum atrial septal defect. One patient with stable postoperative hemodynamics died of severe endobronchial bleeding on the 2nd postoperative day. Due to the increase of antegrade pulmonary blood flow via the conduit the arterial oxygen saturation rose to 92% in the two surviving patients. Postoperative hemodynamic evaluation revealed low right atrial and mean pulmonary artery pressures (12 mmHg). Pulsed Doppler ultrasound sampling of flow in the conduit demonstrated antegrade flow during atrial and ventricular systole. In symptomatic patients with severe malformation of the tricuspid valve a partial modified Fontan procedure offers a promising palliation avoiding tricuspid valve surgery in early childhood.