Author + information
- Received February 10, 2014
- Revision received April 21, 2014
- Accepted April 21, 2014
- Published online July 8, 2014.
- Anitha S. John, MD, PhD∗,†∗ (, )
- Jennifer A. Johnson, MD‡,§,
- Munziba Khan, MPH∗,
- David J. Driscoll, MD†,‡,
- Carole A. Warnes, MD†,‡ and
- Frank Cetta, MD†,‡∗∗ ()
- ∗Division of Cardiology, Children's National Medical Center, George Washington University School of Medicine, Washington, DC
- †Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota
- ‡Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota
- §Division of Pediatric Cardiology, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania
Background Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis.
Objectives The aim of this study was to review outcomes in patients with PLE following the Fontan operation.
Methods From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively.
Results Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m2 vs. 2.7 ± 0.7 l/min/m2; p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]).
Conclusions PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received February 10, 2014.
- Revision received April 21, 2014.
- Accepted April 21, 2014.
- American College of Cardiology Foundation