Surgical repair of isolated congenital tricuspid regurgitation with artificial chordae--a case of two year-follow up

Isolated congenital tricuspid regurgitation derived from primary dysplasia of the valvular apparatus is a rare cardiac abnormality. A 23-year-old woman was first diagnosed to have an isolated tricuspid regurgitation during infancy and was followed up at our hospital. She developed progressive cardiomegaly at the age of 22 years. The chest roentgenogram at the time of admission revealed marked cardiomegaly with a cardiothoracic ratio of 64%. Despite the severe regurgitation of the tricuspid valve, the catheter examination disclosed v and mean pressures of the right atrium of 9 mmHg and 5 mmHg, respectively. The operative findings revealed a markedly dilated tricuspid annulus of 45 mm in diameter (157% of normal) and fragile and redundant valve leaflets, anterior leaflet in particular. Anterior papillary muscle was absent without any vestige thereof. Chordae tendinae of the anterior and posterior leaflets were absent and those of the septal leaflet were attached to the ventricular septum. Each commissure was tethered to the ventricular wall by thick and short chordal tissue. The chordal abnormalities were repaired by four artificial chordae of 4-0 ePTFE suture and an annuloplasty with Carpentier-Edwards ring (36 mm) was added. She recovered uneventfully and was discharged on postoperative day 30. A follow-up echocardiography at 2 years after surgery showed excellent function and trivial regurgitation of the valve. No evidences of thrombus or calcification of the artificial chordae were detected. This experience draws us to conclude that the artificial chordal replacement is one of the useful surgical options for the repair of isolated congenital tricuspid regurgitation.