Impact of fenestration creation on managing patients with protein losing enteropathy complicating Fontan procedure

Abstract

Protein losing enteropathy (PLE) is a well known complication following Fontan procedure and one way of managing such complication is to create fenestration if it is not present or enlarging it if it is small to reduce the Fontan pressure and reduce venous congestion which end up by intestinal protein loss. To evaluate the effect of such procedure in our population. From February 2006 through October 2011, 9 patients who underwent fenestration creation due to development of PLE were assessed in regard to clinical, laboratory results and hemodynamic effect prior and post-procedure. Median age is 7 years (4–21), median weight is 23 kg (15–52), male:female ratio is 3.5:1 (male and 2 female), median saturation pre- and post-procedure was 93% and 82%, respectively, median albumin pre- and post-procedure was 18 g/dl and 31 g/dl during the first 2 weeks and 36 g/dl thereafter, median pulmonary artery pressure before and after was 25 mmHg (17–32 mmHg) and 16 mmHg (14–19 mmHg), transpulmonary pressure gradient reduced from a median of 11 mmHg to 5 mmHg, no immediate deaths, 2 patients need re-dilatation, there was 2 (22%) late deaths (one has stent thrombosis 1 month after followed up by fulminant pulmonary embolism, though all patients were on anticoagulant, other one with sudden arrest came to the emergency could not be resuscitated) 3 (33%) patients have persistent low albumin though the fenestration is present in patient. Transcatheter fenestration creation as a management of PLE following Fontan procedure is a feasible procedure, can be done in the cath. lab with little morbidity and mortality and with beneficial effect, however late complication and complete resolving of PLE is of concern especially if done late.