Abstract | BACKGROUND: 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.
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Authors | Anitha S John, Jennifer A Johnson, Munziba Khan, David J Driscoll, Carole A Warnes, Frank Cetta |
Journal | Journal of the American College of Cardiology
(J Am Coll Cardiol)
Vol. 64
Issue 1
Pg. 54-62
(Jul 08 2014)
ISSN: 1558-3597 [Electronic] United States |
PMID | 24998129
(Publication Type: Journal Article)
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Copyright | Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved. |
Topics |
- Adolescent
- Adult
- Child
- Child, Preschool
- Cohort Studies
- Female
- Fontan Procedure
(mortality, trends)
- Humans
- Infant
- Male
- Middle Aged
- Protein-Losing Enteropathies
(mortality, surgery)
- Retrospective Studies
- Survival Rate
(trends)
- Treatment Outcome
- Young Adult
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