From Glenn to Fontan. A continuing evolution.

Abstract

BACKGROUND: Aristotle's and Galen's erroneous understanding of the circulation eventually was rectified, mostly by Colombo and Harvey, who in the 16th and 17th centuries, respectively, provided the foundation to our present knowledge of the pulmonary and systemic circulation. The "indispensability" of the right ventricle was not questioned until the 20th century. Rodbard, in 1948, for the first time successfully bypassed the canine right ventricle by anastomosing the right atrial appendage to the proximally ligated main pulmonary artery. This important experiment triggered a number of other studies that eventually led to the clinical introduction of the Glenn operation in 1959 and the Fontan operation in 1968. METHODS AND RESULTS: Clinical experience with modifications of the original Fontan operation at The Children's Hospital in Boston included 1) direct atrial-to-pulmonary artery connection (without valves), 2) cavocaval baffle-to-pulmonary artery connection, 3) bidirectional cavopulmonary artery anastomosis, and 4) fenestrated Fontan approach. The original surgical mortality of 20% has been reduced in 1990-1991 to 4%, including patients with complex pathology other than tricuspid atresia. The incidence of prolonged pleural effusions has also decreased from an earlier 40% to < 5% after bidirectional cavopulmonary artery anastomosis and to 11% after the fenestrated Fontan operation. CONCLUSIONS: Much progress has been made since Dr. Glenn's and Dr. Fontan's epic contributions. Ideally, a more appropriate selection of patients and some of the modifications of the original Fontan operation will have a more favorable impact on early and long-term results.