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

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Muğla Sıtkı Koçman Üniversitesi Tıp Dergisi
Volume:11 Issue:1
Mid-term Results of Double Chamber Right Ventricle in Association with Genetic Syndromes

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

Authors : Hande İştar, Buğra Harmandar

Pages : 9-11

Doi:10.47572/muskutd.1292204

View : 81 | Download : 59

Publication Date : 2024-04-30

Article Type : Research Paper

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.
Keywords : Costello Sendromu, Double Chambered Right Ventricle, Down Sendromu, Seckel Sendromu

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