Format

Send to

Choose Destination
J Am Coll Cardiol. 2014 Jul 8;64(1):54-62. doi: 10.1016/j.jacc.2014.04.025.

Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation.

Author information

1
Division of Cardiology, Children's National Medical Center, George Washington University School of Medicine, Washington, DC; Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota. Electronic address: anjohn@cnmc.org.
2
Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota; Division of Pediatric Cardiology, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.
3
Division of Cardiology, Children's National Medical Center, George Washington University School of Medicine, Washington, DC.
4
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota; Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
5
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota; Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota. Electronic address: cetta.frank@mayo.edu.

Abstract

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/m(2) vs. 2.7 ± 0.7 l/min/m(2); 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.

KEYWORDS:

Fontan procedure; protein-losing enteropathy

PMID:
24998129
DOI:
10.1016/j.jacc.2014.04.025
[Indexed for MEDLINE]
Free full text

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center