CLINICAL IMPROVEMENT AFTER REVISION IN FONTAN PATIENTS

Background. Arrhythmias, decreased exercise tolerance, or malabsorptio n will develop in a significant number of Fontan patients. Fontan revi sion consisting of creation of lateral atrial tunnel, reconnection of the Glenn shunt when present, or both appears to improve these patient s. Methods. Over a 34-month period, 9 patients underwent Fontan revisi on. The mean age was 11 +/- 5 years and the mean interval from Fontan operation to revision was 3 +/- 2 years. The reason for revision inclu ded marked impairment in exercise capacity, inability to go to school consistently, and chronic fatigue in 6 patients, 3 of whom also had se rious atrial arrhythmias. Five of the 6 patients had a classic Glenn s hunt. The mean right atrial pressure was greater than the pressure of the Glenn shunt (20 +/- 1.6 versus 17 +/- 0.8 mm Hg). Three of the 6 p atients also showed a significant gradient between the right or left p ulmonary artery wedge and ventricular end-diastolic pressure, indicati ng pulmonary vein obstruction from the bulging atrial septum or partit ioning patch (13 +/- 3 versus 6.8 +/- 1 mm Hg). The remaining 3 patien ts had revision because of malabsorption (1), hepatomegaly and obstruc ted right pulmonary veins from bulging atrial septum (1), and tricuspi d insufficiency (1). Fontan revision was accomplished with creation of a lateral atrial tunnel and Glenn reconnection in 6 patients, Glenn r econnection in 2, and creation of a lateral atrial tunnel in 1. Four p atients had additional procedures. Results. One patient died of Pseudo monas pneumonia. Early extubation, chest tube removal, and postoperati ve hospital discharge were accomplished in 8 patients (mean = 1.4 +/- 1, 2.8 +/- 1, and 8 +/- 3 days, respectively). One patient died 8 mont hs postoperatively of brain damage after ventricular fibrillation from attempted cardioversion for atrial flutter. The remaining patients ha d marked improvement in exercise capacity with ability to consistently go to school, improvement in duration and tolerance to arrhythmias on less medication, and resolution of malabsorption up to 37 months post operatively (mean, 20 +/- 12 months). Conclusions. We conclude that cr eation of lateral atrial tunnel with excision of a bulging atrial sept um or atrial partitioning patch that causes pulmonary venous obstructi on, reconnection of the Glenn shunt, which allows better distribution of now based on the pulmonary vascular bed and resistance of each lung , or a combination of these procedures will improve Fontan patients.