Abstract
The working hypothesis for a one and a half ventricle repair has been that the benefits of a pulsatile pulmonary circulation may negate some of the late complications of the Fontan procedure. Those benefits are thought to outweigh the downside risk of having retrograde pulsatility in the superior vena cava. We sought to define the long-term fate of this strategy.
One hundred fourteen patients who underwent a superior vena cava to pulmonary artery anastomosis as an adjunct to biventricular repair were identified for the years 1965 to 2003. Median follow-up was 92.3 months (range, 1 month to 38 years).
The long-term outcome for operative survivors was 83.4%, 80.1%, and 69.3% at 5, 10, and 20 years, respectively. The survival in the most recent 10 years is 91.8% (
p
= 0.063). Of the late deaths, 69.6% (16 of 23) were known cardiac deaths or sudden. Patients with chronic right ventricular dysfunction demonstrated the best 10-year survival (91.6%). Of the late survivors, 98.8% of patients are in New York Heart Association class I or II. Arterial O
2 saturation increased significantly from before to late after repair. (83.5% to 94.5%,
p
< 0.001; n = 82). Freedom from new atrial arrhythmia was 92.2% at 20 years. The superior vena cava to pulmonary artery anastomosis was taken down in 3. There was no patient with clinically evident protein-losing enteropathy.
The most common cause of late mortality is cardiac. Atrial and ventricular arrhythmias occur, but no protein-losing enteropathy was identified. The serious complication risk related to pulsatility in the superior vena cava was 2.6%.