Mid-term Results of Double Chamber Right Ventricle in Association with Genetic Syndromes

Abstract

Double chamber right ventricle (DCRV) is a congenital disease in which a hypertrophied muscle band divides the right ventricle chamber into two. The early-mid-term follow-up of 6 patients with DCRV and distant genetic syndromes is reported in this paper. A retrospective analysis was performed of 6 DRCV patients with a mean age of 3.9±1.4 years. Concomitant cardiac anomalies were perimembranous ventricular septal defect (n=3), atrial septal defect (n=1), mild aortic regurgitation (n=1), discrete subaortic membrane (n=1). Associated genetic syndromes were Costello (n=1), Seckel (n=1) and Down syndromes (n=4). The mean follow-up period was 4.86±4.6 years. Mean systolic pressure gradient in the right ventricle in the postoperative was 18.5±11.5 mmHg. No mortality occurred and there was no requirement for reintervention. To the best of our knowledge, this is the first report in literature of concomitant DCRV with Costello and Seckel syndromes.