Usefulness of Heparin Therapy in Protein-Losing Enteropathy Associated With Single Ventricle Palliation

• Published in: The American journal of cardiology 2008-01, Vol.101 (2), p.248-251
• Main Authors: Ryerson, Lindsay, MD, Goldberg, Caren, MD, Rosenthal, Amnon, MD, Armstrong, Aimee, MD
• Format: Article
• Language: English
• Subjects: Anticoagulants - administration & dosage; Anticoagulants - adverse effects; Anticoagulants - therapeutic use; Biological and medical sciences; Cardiology. Vascular system; Cardiovascular; Child; Cohort Studies; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Female; Gastroenterology. Liver. Pancreas. Abdomen; Heart; Heart Ventricles - pathology; Heparin - administration & dosage; Heparin - adverse effects; Heparin - therapeutic use; Humans; Male; Medical Records; Medical sciences; Michigan - epidemiology; Other diseases. Semiology; Protein-Losing Enteropathies - blood; Protein-Losing Enteropathies - drug therapy; Protein-Losing Enteropathies - mortality; Retrospective Studies; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; Survival Analysis
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/j.amjcard.2007.08.029

This retrospective study was designed to evaluate the effectiveness of subcutaneous heparin therapy for the treatment of protein-losing enteropathy (PLE) associated with single-ventricle palliation and to evaluate the side effects of long-term heparin use. PLE affects 4% to 13% of Fontan operative survivors. Five-year survival after onset of PLE is only 46% to 59%. We studied a cohort of patients with single-ventricle palliation who developed PLE and were treated with subcutaneous heparin. Seventeen patients were included in the study. Symptoms of PLE appeared on average 43 months after surgical palliation. At diagnosis of PLE, mean albumin level was 2.0 ± 0.4 g/dl. At cardiac catheterization, mean systemic venous pressure was 11.6 mm Hg. Subjective symptomatic improvement on heparin therapy occurred in 13 patients (76%). Three patients (18%) went into clinical remission. Compared with the period before initiation of heparin, there was no significant difference in the number of hospital admissions (p = 0.99) or albumin infusions (p = 0.88) during the first year of heparin therapy. Five patients had x-rays of their thoracolumbar spine, and 9 patients had bone mineral analyses; all scans were grossly abnormal. In conclusion, subcutaneous heparin therapy leads to subjective improvement of PLE symptoms in most patients; however, it does not change the need for frequent albumin infusions and does not increase the rate of remission above that for standard medical therapy.