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Background: Single ventricle terminal palliation with a completed cavopulmonary anastomosis is well understood to case myriad comorbidities including Fontan associated liver disease, systemic systolic ventricular failure, and protein losing enteropathy with accompanying ascites. The presence of elevated pressures within the cavopulmonary circuit logically precipitates sequelae. The first sign of which may be a reduction in exercise tolerance.
Methods: We evaluated 6 patients in a single-center with elevated pressures (defined as >15 mmHg) who had previously been on a stable dose of sildenafil 20mg by mouth three times daily with new initiation of macitentan 10mg daily. They were followed for a minimum of 6 months following initiation of therapy.
Results: 4 patients improved their baseline 6 minute walk duration by 20% (>80m) over the course of 6 month follow up. 2 patients had decreased frequency of the need of abdominal paracentesis to > 1 month intervals, and albumin levels increased in each of these patients. No patients reported significant side effects.
Conclusions: This small pilot study is suggestive that serial addition of macitentan to sildenafil in patients with Fontan physiology and elevated Fontan pressures is safe, and in some cases helpful. More study is needed to determine longer term benefits including delay and reversal of hepatic fibrosis and congestive hepatic hepatopathy as well as improvement in exercise tolerance.
Poster Hall, Hall C
Saturday, March 18, 2017, 9:45 a.m.-10:30 a.m.
Session Title: Fontan in the Modern World
Abstract Category: 9. Congenital Heart Disease: Adult
Presentation Number: 1183-022
- 2017 American College of Cardiology Foundation