Echocardiographic and Ultrasonographic Parameters Associated with Protein-losing Enteropathy in Patients with Fontan Physiology: a Systematic Review with Meta-analysis

Abstract

Fontan circulation was described as the final stage of palliation for congenital heart disease with univentricular physiology. 1 In this circulation, blood from the superior and inferior vena cava is bypassed to the pulmonary circulation, so that the dominant ventricle is responsible for systemic circulation. Since its description, this technique has increased the survival and quality of life of patients with univentricular physiology. 2,3 However, this type of circulation may be associated with significant morbidity. 4 That is because the patient is predisposed to a chronic low output, with increased central venous pressure and congestive heart failure. 5 These conditions can lead to pleural and pericardial effusion, gastrointestinal dysfunction, and proteinlosing enteropathy (PLE). 6 PLE is a severe condition derived from chronic venous hypertension leading to inflammation, tumor necrosis factor release, and consequent changes in the enterocyte membrane's composition. Related complications from this condition are hypoalbuminemia, hypocalcemia, hypogammaglobulinemia, and hypercoagulable states. 6 PLE affects up to 10% of patients after Fontan