Genetik Sendromların Eşlik Ettiği Çift Odacıklı Sağ Ventrikülde Orta Dönem Sonuçlarımız

Year 2024, Volume: 11 Issue: 1, 9 - 11, 30.04.2024

Hande İştar , Buğra Harmandar

https://doi.org/10.47572/muskutd.1292204

Abstract

Çift odacıklı sağ ventrikül (DCRV), hipertrofik kas bantlarının sağ ventrikülü ikiye böldüğü konjenital bir hastalıktır. Genetik sendromun da eşlik ettiği 6 hastamızın erken-orta dönem cerrahi sonuçlarını sunmaktayız. DCRV tanılı, genetik sendromun eşlik ettiği 6 hasta ile ilgili bulguları derlendi. Ortalama yaş 3.9±1.4 yıldı. Eşlik eden ek kalp anomalileri perimembranöz ventriküler septal defekt (n=3), atrial septal defekt (n=1), orta aort yetmezliği (n=1), diskret subaortik membran (n=1) idi. Eşlik eden genetik sendromlar Costello (n=1), Seckel (n=1), Down sendromuydu (n=4). Ortalama takip süresi 4.86±4.6 yıldı. Sağ ventriküldeki ortalama sistolik basınç gradienti 18.5 ± 11.5 mmHg idi. Takipte mortalite ya da tekrar operasyon gerekliliği olmadı. Çift odacıklı sağ ventrikül, Costello ve Seckel Sendrom birlikteliğinin literatürde ilk kez yayımlandığı düşüncesindeyiz.

Keywords

Costello Sendromu, Double Chambered Right Ventricle, Down Sendromu, Seckel Sendromu

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.

Keywords

Costello Syndrome, Double Chambered Right Ventricle, Down Syndrome, Seckel Syndrome

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