MODIFIED FONTAN PROCEDURE FOR EBSTEINS-ANOMALY OF THE TRICUSPID-VALVE- AN ALTERNATIVE SURGICAL APPROACH PRESERVING EBSTEINS ANATOMY

In young patients with Ebstein's anomaly and symptoms of congestive he art failure or severe cyanosis tricuspid valve repair may be unsuccess ful and prosthetic valve replacement is needed, which is associated wi th a high operative mortality. This report describes the implantation of a valved aortic homograft as a conduit between right atrium and pul monary artery to at least postpone valve surgery in three symptomatic children (4.1, 8.3, and 10 years of age) with an arterial oxygen satur ation of 75 to 82 %. The intracardiac anatomy was left untouched excep t for closure of the associated secundum atrial septal defect. One pat ient with stable postoperative hemodynamics died of severe endobronchi al bleeding on the 2(nd) postoperative day. Due to the increase of ant egrade pulmonary blood flow via the conduit the arterial oxygen satura tion rose to 92 % in the two surviving patients. Postoperative hemodyn amic evaluation revealed low right atrial and mean pulmonary artery pr essures (12 mmHg). Pulsed Doppler ultrasound sampling of flow in the c onduit demonstrated antegrade flow during atrial and ventricular systo le. In symptomatic patients with severe malformation of the tricuspid valve a partial modified Fontan procedure offers a promising palliatio n avoiding tricuspid valve surgery in early childhood.