Cor Vasa 2009, 51(7-8):500-505 | DOI: 10.33678/cor.2009.125

Outcomes of surgical management of Ebstein's anomaly of the tricuspid valve

Sylvia Krupičková^1,*, Václav Chaloupecký¹, Tomáš Tláskal¹, Bohumil Hučín¹, Roman Gebauer¹, Gabriela Hrádková¹, Jana Popelová², Štěpán Černý², Tomáš Brychta³, Tomáš Minařík⁴, Jan Škovránek¹

¹ Dětské kardiocentrum a Centrum výzkumu chorob srdce a cév, Fakultní nemocnice v Motole

² Nemocnice Na Homolce, Praha

³ Fakultní nemocnice Brno, Brno

⁴ Fakultní nemocnice Ostrava, Ostrava-Poruba, Česká republika

Aim: A retrospective study to analyze the outcome of surgery for Ebstein's anomaly with respect to the surgical technique used and the development of tricuspid valve function.

Patients and methods: Over the years 1982-2005, tricuspid valvuloplasty or tricuspid valve replacement was performed in a total of 25 patients with Ebstein's anomaly, aged 2-58 years (median, 11 years) in Kardiocentrum, Motol University Hospital. Indications for the surgery was either severe tricuspid valve regurgitation or right-to-left atrial shunt. Tricuspid regurgitation was assessed using Doppler echocardiography performed prior to the defect repair, immediately after the repair, and on all the subsequent examinations.

Results: Morphologically, type A Ebstein's anomaly was seen in two patients (8%), type B in 14 (56%), type C in seven (28 %), and type D in two patients (8%). The preoperative severity of tricuspid regurgitation was not related to the morphological type of Ebstein's anomaly. Tricuspid annuloplasty was performed in 17 cases (68%). The tricuspid valve was replaced in eight patients (32%) using biological and mechanical prostheses in five and three patients, respectively. Bidirectional cavopulmonary anastomosis was additionally performed in seven patients. Two patients (8%) died within 30 postoperative days, with another two patients dying at three and six years postoperatively. The actuarial survival probability for the whole series at 5, 10, and 15 years after the surgery was 87%, 81%, and 81%, respectively. In the subgroup of 17 patients undergoing tricuspid annuloplasty, valve function early postoperatively improved by at least one grade in five cases (29%). Seven patients (41%) required redo surgery for a significant residual tricuspid regurgitation. Among the eight patients receiving a prosthetic valve, four (50%) required its subsequent replacement. The actuarial probability of survival without a redo surgery in the whole series at 5, 10, and 15 years after the Ebstein's anomaly repair was 68%, 62%, and 35%, respectively. At the latest outpatient follow-up of 18 patients aged 7-44 years (median 23 years), at six months to 25 years (median 10 years) after the repair, 16 (89%) individuals were in NYHA Class I or II. One patient awaiting heart transplantation experienced major problems while another patient with neurological impairment was not classified. Among the 11 patients having tricuspid annuloplasty, six (55%) had moderate (grade 2), four (36%) significant (grade 3), and one massive residual tricuspid regurgitation. Four and three patients have biological and mechanical prostheses in the tricuspid position, respectively.

Conclusion: The outcome of surgical management of Ebstein's anomaly is determined by the severity of tricuspid valve and right ventricle impairment. In pediatric age, surgery is indicated only in symptomatic individuals with either significant tricuspid regurgitation or significant right-to-left shunt.

Keywords: Ebstein's anomaly; Tricuspid regurgitation

Published: July 1, 2009  Show citation