Objective;
We have experienced 894 patients (pts) with functionally univentricular hearts except hypoplastic left heart syndrome from September 1980 through October 2008. The overall rate of Fontan procedure is 56% in our institution. This study evaluated the clinical outcome in adult pts (20 year-old and more) with Fontan physiology.
Methods;
Eighty-one adult pts with Fontan physiology were enrolled. We analyzed post-operative morbidity, hemodynamics, and medications retrospectively.
Results;
The table shows a summary of the characteristics of the pts enrolled. The median age at Fontan operation was 10 years and the median age at the examination was 23 years. Twenty-six pts had diagnosed as single right or left ventricle heart, 24 as heterotaxy and 20 as tricuspid atresia. Two late-deaths were observed and 11 drop-outs. The New York Heart Association functional classification I was in 68 pts whose medications were warfarin potassium in 66 pts (66/68=97%), aspirin in 49 pts (72%), angiotensin converting enzyme inhibitors in 35 pts (51%), carvedilol in 24 pts (35%), angiotensin II receptor blockers in 15 pts (22%) and others in 37 pts (54%). Ten pts had been treated for dysrhythmias, in which 3 pts had undergone catheter ablation, 2 pts concomitant surgical therapy at fontan operation and 2 pts pacemaker implantation.
Conclusions;
In our institute, warfarin potassium, aspirin and ACE-I/ARB/carvedilol are basic medications for cardiovascular protection in patients with Fontan physiology. The present data show the encouraging survival rate in adult pts with Fontan physiology, but that the morbidity of dysrhythmias remains suboptimal.