Low pulmonary vascular compliance predisposes post-Fontan patients to protein-losing enteropathy.

Abstract

BACKGROUND:

Protein-losing enteropathy (PLE) is a life-threatening and poorly understood complication after the Fontan operation. We sought to determine the pre-operative risk factors for PLE which developed after the extracardiac conduit Fontan operation.

METHODS:

Two hundred thirty-five patients who underwent the extracardiac conduit Fontan operation as an initial Fontan type procedure (median age at operation: 3.5 years) were enrolled in this cross-sectional retrospective study. Pre-operative and peri-operative variables were surveyed through a review of medical records.

RESULTS:

Within the median follow-up duration of 5 years, 12 patients developed PLE (12/234, 5.1%) at a median interval of 2.2 years after the Fontan procedure, and 4 died of PLE at a median interval of 1.2 years (range 0.21-7.62) after diagnosis. Factors found to be related to the time to the development of PLE on univariate analysis were pulmonary vascular compliance (Cpv) (p=0.0019), central venous pressure at postoperative 12 hours (p=0.0026), days of ICU stay (P=0.0449), days of hospitalization (p=0.0135), and days of chest tube indwelling (p=0.0493). Multivariate analysis, however, showed that only Cpv (p=0.0367) remained significant. The range of Cpv was 8.8-26.1 mm(2)/m(2)/mmHg (median 17.9) in patients with PLE, and 6.6-122.3 mm(2)/m(2)/mmHg (median 26.8) in patients without PLE.

CONCLUSIONS:

Low pulmonary vascular compliance is associated with the development of PLE after the extracardiac conduit Fontan operation.

• Authors: Jeong Jin Yu, Tae-Jin Yun, Sung-Cheol Yun, Yu Mi Im, Seung Chul Lee, Hong Ju Shin, Hong Ki Ko, Jeong-Jun Park, Dong-Man Seo, Young-Hwue Kim, Jae-Kon Ko, In-Sook Park
• Journal: International journal of cardiology (Int J Cardiol) Vol. 165 Issue 3 Pg. 454-7 (May 25 2013) ISSN: 1874-1754 [Electronic] Netherlands
• PMID: 21944467 (Publication Type: Journal Article)
• Copyright: Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Topics

• Adolescent
• Adult
• Child
• Child, Preschool
• Cross-Sectional Studies
• Female
• Follow-Up Studies
• Fontan Procedure (adverse effects)
• Humans
• Infant
• Male
• Postoperative Complications (diagnosis, epidemiology)
• Protein-Losing Enteropathies (diagnosis, epidemiology)
• Retrospective Studies
• Risk Factors
• Treatment Outcome
• Vascular Resistance (physiology)
• Young Adult