Protein-Losing Enteropathy and Plastic Bronchitis Following the Total Cavopulmonary Connections.

Abstract

BACKGROUND:

We aimed to evaluate incidence, outcomes, and predictors of protein-losing enteropathy (PLE) and plastic bronchitis (PB) in a cohort of total cavopulmonary connection (TCPC).

METHODS:

We included 620 consecutive patients undergoing TCPC between 1994 and 2021. Prevalence and predictors for onset of PLE/PB were evaluated. Death and heart transplantation after onset of PLE/PB were examined.

RESULTS:

A total of 41 patients presented with PLE/PB (31 with PLE, 15 with PB, and 5 developed both PLE and PB). Their median age at TCPC was 2.2 (interquartile ranges [IQRs], 1.7-3.7) years, and time period to onset for PLE was 2.6 (IQR: 1.0-6.6) years and for PB was 1.1 (IQR: 0.3-4.1) years after TCPC. Independent factors for developing PLE/PB were dominant right ventricle (RV, hazard ratio [HR], 2.243; 95% confidence interval [CI], 1.129-4.458, P = .021) and prolonged pleural effusion after TCPC (HR, 2.101; 95% CI, 1.090-4.049, P = .027). In PLE/PB population, freedom from death or transplantation after PLE/PB diagnosis at 5 and 10 years were 88.7% and 76.4%, respectively. Eleven surgical interventions were performed in 10 patients, comprising atrioventricular valve repairs (n = 4), Fontan pathway revisions (n = 2), pacemaker implantation (n = 2), secondary fenestration (n = 1), diaphragm plication (n = 1), and ventricular assist device implantation (n = 1). In nine patients, a recovery from PLE with the resolution of PLE symptoms and normal protein levels was achieved. Eight patients died and the remaining continued to have challenging protein loss.

CONCLUSIONS:

Protein-losing enteropathy and PB remain severe complications in the cohort of TCPC. Patients with dominant RV, and prolonged pleural effusions, were at risk for PLE/PB.

• Authors: Veronika Hammer, Thibault Schaeffer, Helena Staehler, Paul Philipp Heinisch, Melchior Burri, Nicole Piber, Julia Lemmer, Alfred Hager, Peter Ewert, Jürgen Hörer, Masamichi Ono
• Journal: World journal for pediatric & congenital heart surgery (World J Pediatr Congenit Heart Surg) Vol. 14 Issue 6 Pg. 691-698 (Nov 2023) ISSN: 2150-136X [Electronic] United States
• PMID: 37551120 (Publication Type: Journal Article)

Topics

• Humans
• Fontan Procedure (adverse effects)
• Protein-Losing Enteropathies (complications)
• Retrospective Studies
• Pulmonary Artery (surgery)
• Bronchitis (etiology)
• Heart Defects, Congenital (surgery)
• Treatment Outcome