794-5 Long–term Survival in Unoperated Single Ventricle

Abstract

Survival into adulthood is rare in patients with single ventricle without palliative surgery or radical repair. The Fontan operation is commonly utilized in those patients with ideal hemodynamics in the hope of conferring improved survival. From 1984–1994,12 patients (7 males, 5 females) aged 23-66 years (mean 41) with a single ventricle and no prior surgery were evaluated. Eleven had double-inlet left ventricle (DILV), one had tricuspid atresia, and of these 12, 8 had moderate to severe pulmonary stenosis with a peak gradient from 71-112 mmHg (mean 94). The other four had moderate to severe pulmonary hypertension (PH) with a pulmonary artery systolic pressure ranging from 78–122 mm Hg (mean 102). Six of these 12 patients were followed from 124 years (mean 14 yrs). Eleven patients had an ability index of 2, and one had an ability index of 3. Plasma hemoglobin ranged from 15.3 - 21.8 gm/dL and was higher in those with PH. Only 4 of 12 patients had had phlebotomy. Ventricular function by 2–D echocardiogram or left ventriculography was normal in 10 patients and mildly depressed in 2 patients. Two of the 12 patients were on diuretics. Rhythm was sinus in 10 patients; two others had a history of atrial fibrillation controlled on medication. Two patients had a permanent pacemaker. one for chronic atrial fibrillation with slow ventricular response and the other for complete heart block. One 60-year-old patient with a history of stroke died from a second stroke. While this group is biased by referral and natural selection, patients demonstrated excellent functional capacity and preserved ventricular function. Adult patients with DILV and perfectly balanced pulmonary circulation may do as well as, if not better than, those with a Fontan operation who have a 60% 10-year survival. This should be considered before sending an adult patient with single ventricle for a Fontan repair.