1002-87 The Results of Spontaneous and Clamshell Umbrella Closure of the Fenestrated Fontan

The fenestrated Fontan (FF) procedure was performed in 24 consecutive patients (pts) — aged 1 to 13 years (mean 5.4) — with one or more high risk factors in order to reduce operative mortality and morbidity. This study evaluated their outcome. The fenestration was 2.7mm in 4 pts, 4mm in 12, and 4.5mm in 8. One pt (4.2%) died in hospital. One pt died 7 months after FF at cardiac transplantation. Survivors have been followed for 16 to 48 months (mean 34). Early O2 saturations (sats) were 88–92% (mean 90) in 2.7mm and 69–89% (mean 79.4) in larger FF. All four 2.7mm, one 4mm and four 4.5mm fenestrations closed spontaneously but of these one 2.7mm (at 2 weeks) and one 4mm (at 3 years) required recreation of a hole by trans-baffle puncture and balloon dilation because of congestive failure. Nine 4mm and two 4.5mm fenestrations were closed with a clamshell umbrella (U). Following U closure, RA pressure increased from 12±1.8 to 14.7±2.4mmHg (p<0.01), O2 sats increased from 80.2±6.6 to 93.7±4.6% (p<0.001), but the artriovenous 02 saturation difference increased from 18.8±4.1 to 25.8±6.7% (p<0.02). Unusual systemic venous collaterals resulted in R to L shunts in 3 pts and recurrent pericardial effusions in 1; all were successfully coil occluded. Atrial baffle leaks resulted in large R to L shunts in 3 pts after effective fenestration closure with U. One was successfully coil occluded; the other 2 have O2 sats of 84 and 88% late post U. Clinical follow-up has shown a high incidence of dysrhythmias (atrial tachycardia in 5, 4 requiring amiodarone; sick sinus syndrome in 3; seven of these required a pacemaker). Three pts have had severe congestive failure, one immediately post U closure. Two pts have protein losing enteropathy. Two pts have had strokes: 1 early and minor; the other 4 months after U closure and major. Eighteen of 22 survivors are in NYHA Class 1.ConclusionsThe fenestrated Fontan resulted in a low operative and intermediate mortality. Most fenestrations either closed spontaneously or were closed with a clamshell umbrella. Although most patients have a good functional result, dysrhythmias and other late complications are frequent