Double-chambered right ventricle in 73 patients: Spectrum of the disease and surgical results of transatrial repair

OBJECTIVE: To review the spectrum of double-chambered right ventricle (DCRV ) and the outcome of surgical repair in patients diagnosed between February 1988 and March 1999. DESIGN: The charts of patients with DCRV were studied. SETTING: Tertiary care hospital. PATIENTS AND METHODS: A total of 73 patients were identified. Sixty-nine un derwent surgical repair, while four are awaiting surgery. The repair was th rough a transatrial approach in 61 patients, while in eight an additional v entriculotomy was performed. MAIN RESULTS: An associated ventricular septal defect (VSD) was present in 56 of 73 patients (77%). These patients were significantly younger (P<0.05) than the 17 patients without a VSD. Among patients with a VSD, the 31 requ iring patch closure were significantly younger than the 25 patients having direct closure. Five older patients among those with intact septum had impa ired right ventricular (RV) function as well as higher intraventricular gra dients. At surgery the intraventricular obstruction was relieved by myomect omy. There was no hospital or late mortality. Following surgery, at a mean follow-up of 13.6 months, no increase in the intraventricular gradient was detected by Doppler echocardiography. CONCLUSIONS: The development of DCRV is associated with VSD in early life. The probability of the presence of a VSD decreases with age. The disease is progressive, resulting in increased intracavitary gradient within the RV a nd in RV impairment if it is not treated in a timely fashion. Transatrial r epair is safe with excellent midterm results. In the presence of high gradi ents within the RV, a ventriculotomy may be necessary to obtain acceptable results.