Commentary: Lymphatic fluid flows uphill in both hemispheres—and leaks in the same places

Abstract

Sharma and colleagues1 provide a comprehensive description of protein-losing enteropathy (PLE) and plastic bronchitis (PB) in patients with Fontan circulation—yet another testament to the utility of their Fontan registry. In a cohort of 1561 patients spanning 4 decades, the authors report 48 patients with PLE only, 4 with PB only, and 3 with both. Right ventricular morphology with hypoplastic left heart syndrome (hazard ratio [HR] of 2.3), prolonged effusion after the Fontan (HR of 2.4), and older age at Fontan (2-year increment, HR of 1.13) were associated with development of PLE/PB, whereas once PLE/PB was diagnosed, only older age at Fontan was associated with death or transplant (HR of 1.23).