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(Circulation. 2000;102:2094.)
© 2000 American Heart Association, Inc.

Clinical Investigation and Reports

Clinical Outcome of Fenestrated Fontan Patients After Closure

The First 10 Years

Donna A. Goff, MS; Elizabeth D. Blume, MD; Kimberlee Gauvreau, ScD; John E. Mayer, MD; James E. Lock, MD; Kathy J. Jenkins, MD, MPH

From the Departments of Cardiology (D.A.G., E.D.B., K.G., J.E.L., K.J.J.) and Cardiac Surgery (J.E.M.), Children’s Hospital, and the Departments of Pediatrics (D.A.G., E.D.B., K.G., J.E.L., K.J.J.) and Surgery (J.E.M.), Harvard Medical School, Boston, Mass.

Background—The late clinical status of Fontan patients after fenestration closure is unknown. Data are now available on all patients who underwent closure from 1989 to 1999.

Methods and Results—All patients who underwent catheter closure of a Fontan fenestration were enrolled in either the Clamshell (1989 to 1994) or CardioSEAL (1996 to 1999) regulatory trials. Physiological values obtained at catheterization helped assess the hemodynamic effects of fenestration occlusion. In addition to survival, outcomes assessed included O₂ saturations, medication use, significant clinical findings (eg, heart failure, protein-losing enteropathy, or new arrhythmias), and somatic growth. Of 181 patients who underwent closure, 27 had additional significant leaks. The remaining 154 patients constituted the study group. Median time from closure to latest follow-up was 3.4 years (range 0.4 to 10.3 years). Fenestration closure increased O₂ saturation 9.4% on average (P<0.001). The numbers of patients receiving digoxin or diuretics decreased at the most recent follow-up compared with baseline (P<0.001), but use of antiarrhythmic agents increased marginally (P=0.05). Height and weight percentiles rose (medians of 2 and 4, respectively; P<0.001). Clinical decompensation during follow-up of 154 patients was rare (4.5%), with 2 deaths, 3 Fontan revisions, and 1 patient each with protein-losing enteropathy and ascites. No other patient developed chronic congestive symptoms; 21 patients developed new arrhythmias, and 2 had a stroke or transient ischemic attack.

Conclusions—Fenestration closure in Fontan patients was followed by improved oxygenation, reduced need for anticongestive medication, and improved somatic growth at latest follow-up. Death (1.3%) or chronic decompensation (3.2%) was rare.

Key Words: Fontan procedure • outcome • fenestration

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