Long term outcome of protein-losing enteropathy in cardiac disease

Abstract

Introduction: Protein-losing enteropathy (PLE) is a devastating complication of cardiac disease, especially after Fontan operation. The purpose of this study was to investigate the clinical characteristics, the responsiveness to the treatment options, and outcome of PLE patients at a single institution. Also we also tried to determine the successful treatment modality and the factors associated with the adverse outcome. Methods: We reviewed medical records of 34 patients (12 female, 22 male) with PLE from cardiac disease from 1992 to 2016. Results: Median age at PLE diagnosis was 11.4 years (range 0.8-28.3). The follow-up duration was 7.7±5.8 years. The underlying cardiac disease was functional single ventricle in 26 patients (76%), constrictive pericarditis in 3 patients (9%), valvular heart disease in 1 patient (3%), and restrictive cardiomyopathy in 1 patient (3%). Most patients (73%) underwent Fontan operation and 5 patients (14%) did not receive any surgery. PLE occurred in 4.5% of patients after Fontan operation. The survival rate was 80.7% at 5 years and 73.9% at 10 years. Twelve patients died during follow-up in 6.9±5.9 years after PLE onset. Aortic oxygen saturation<90% (HR=10.755 P=0.042), hemoglobin level<12g/dl (HR=6.520, P=0.023), decreased ventricular function (HR=5.094, P=0.024), NYHA functional class III or IV (HR 5.522, P=0.017) were predictors of mortality in PLE patients after Fontan operation. For the management of PLE, medical treatments were more frequently used including diuretics, ACEI/ARB, diet modification, subcutaneous heparin injection, oral corticosteroids. Interventional and surgical therapies such as Fontan pathway fenestration creation (4[16%]), Fontan conversion (4[16%]), and Fontan takedown surgery (2[8%]) were applied in selected patients. One third of PLE patients after Fontan operation showed resolution of PLE. In Fontan patients, resolution of PLE was achieved by heparin in 4 patients, surgical Fontan fenestration in 2 patients, Aorto-pulmonary collaterals surgical ligation and transplantation in 1 patient each. Pulmonary vasodilator alone could not achieve resolution of PLE. Higher Fontan pathway pressure (16.8± 4.5mmHg vs. 13.0 ±1.9mmHg, P=0.02) was associated with intractable PLE. Conclusions: The survival of PLE with cardiac disease has improved with the advancement of the conservative care. Although there is no definitive method, heparin and surgical/medical hemodynamic treatment led the resolution of PLE in one third of patient. Further investigation is also needed to determine the individual susceptibility of PLE as well as to develop new method of prevention and therapy.