Percutaneous tricuspid valve implantation in a Fontan patient with congestive heart failure and protein-losing enteropathy.

A 47-year-old patient visited our outpatient clinic. She was born with tricuspid atresia with normally connected great arteries without pulmonary stenosis (type Ic); a ventricular septal defect connected the pulmonary artery and hypoplastic right ventricle (RV) with the left ventricle. When she was 14 years old, a Fontan-Bjork procedure was performed.1 The atrial and the ventricular septal defects were closed and a valved conduit (Hancock prosthesis) was placed between the right atrium (RA) and the hypoplastic RV, thus providing pulsatile flow to the pulmonary arteries. Ten years later, the conduit was severely obstructed and replaced by a 23-mm aorta homograft. At the age of 38 years, she had atrioventricular block for which a dual-chamber pacemaker system was implanted with placement of the ventricular lead through the homograft in the RV apex. In the last 2 years, she had progressive heart failure and diarrhea. She was clinically diagnosed with protein-losing enteropathy (PLE), based on hypalbuminemia, edema, and diarrhea. Other causes of protein losses or decreased protein production were excluded. Within a few months, serum albumin levels decreased from 29 to 19 g/L. She was hospitalized, and despite increasing doses of diuretics and dietary protein adjustments, her clinical condition deteriorated (New York Heart Association functional class III/IV) with decrease of renal function. Echocardiography and angiography demonstrated significant regurgitation of the RA-RV homograft and severe dilation of the RA. The originally hypoplastic RV had a remarkably good size and systolic function. The homograft appeared to be heavily calcified and attached to the dorsal side of the sternum. The risk of surgical conversion to a total cavopulmonary connection was considered too high because of her poor clinical condition. It was decided to restore “tricuspid” valve function by placement of a Melody transcatheter valve2 (Medtronic, Minneapolis, MN) in the RA-RV homograft, as recently described by Eicken et al.3 Coronary angiography was performed during sizing of the conduit with a 22-mm balloon to exclude the potential risk of right coronary artery compression. The Melody valved stent, mounted on a 22-mm Ensemble delivery system (Medtronic), was positioned in the Figure 1. A, Before implantation: Direction of flow from the RA via the RV to the pulmonary artery (PA). Note the size of the hypoplastic RV. B, Before implantation: Regurgitant flow in the aortic homograft in tricuspid valve position (arrow). RVOT indicates right ventricular outflow tract; RPA, right pulmonary artery. C, Placement of a 22-mm Melody valved stent in the aortic homograft with entrapment of the ventricular pacing lead. D, Melody valve functioning as competent tricuspid valve between RA and RV (arrow).