Öz
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.
Anahtar Kelimeler
Costello Sendromu Double Chambered Right Ventricle Down Sendromu Seckel Sendromu
Kaynakça
- 1. Loukas M, Housman B, Blaak C, et al. Double-chambered right ventricle: A review. Cardiovasc Pathol. 2013;22:417-23.
- 2. Khoueiry G, Bhat T, Tantray M, et al. A rare case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve. Clin Med Insights Circ Respir Pulm Med. 2014;8:1-3.
- 3. Choh N, Amreen S, Bashir A, et al. Two cases of six-chambered hearts. Ann Saudi Med. 2020;40:496-500.
- 4. Park JG, Ryu HJ, Jung YS, et al. Isolated double-chambered right ventricle in a young adult. Korean Circ J. 2011;41:272-5.
- 5. Penkoske PA, Duncan N, Collins-Nakai RL. Surgical repair of double-chambered right ventricle with or without ventriculotomy. J Thorac Cardiovasc Surg. 1987;93:385-93.
- 6. Hachiro Y, Takagi N, Koyanagi T, et al. Repair of double-chambered right ventricle: Surgical results and long-term follow-up. Ann Thorac Surg. 2001;72:1520-1.
- 7. Mądry W, Karolczak MA, Zacharska-Kokot E. Double-chambered right ventricle in a 16-year-old patient with Williams syndrome. J Ultrason. 2017;17:289-93.
- 8. Yamamoto M, Takashio S, Nakashima N, et al. Double-chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome. ESC Heart Fail. 2020;7:721-6.
- 9. Ahmed Eltohami E, Ahmed Hajar H, Folger GM, et al. Double-chambered right ventricle and Down’s syndrome: A proposed new association. Angiology. 1994;45(2):119-23.
- 10. Hennekam RCM. Costello syndrome: An overview. Am J Med Genet C Semin Med Genet. 2003;117(1):42-8.
- 11. Khojah O, Alamoudi S, Aldawsari N, et al. Central nervous system vasculopathy and Seckel syndrome: Case illustration and systematic review. Child’s Nerv Syst. 2021;37:3847-60.
- 12. Ucar B, Beyir Kilic Z, Dinleyici C, et al. Seckel syndrome associated with atrioventricular canal defect: A case report. Clin Dysmorphol. 2004;13(1):53-5.
- 13. Can E, Bulbul A, Uslu S, et al. A case of Seckel syndrome with Tetralogy of Fallot. Genet Couns. 2010;21(1):49-51.
- 14. Arslan D, Çimen D, Guvenc O, et al. A case of Seckel syndrome with tricuspit atresia. Genet Couns. 2014;25(2):171-5.
Öz
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.
Anahtar Kelimeler
Costello Syndrome Double Chambered Right Ventricle Down Syndrome Seckel Syndrome
Kaynakça
- 1. Loukas M, Housman B, Blaak C, et al. Double-chambered right ventricle: A review. Cardiovasc Pathol. 2013;22:417-23.
- 2. Khoueiry G, Bhat T, Tantray M, et al. A rare case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve. Clin Med Insights Circ Respir Pulm Med. 2014;8:1-3.
- 3. Choh N, Amreen S, Bashir A, et al. Two cases of six-chambered hearts. Ann Saudi Med. 2020;40:496-500.
- 4. Park JG, Ryu HJ, Jung YS, et al. Isolated double-chambered right ventricle in a young adult. Korean Circ J. 2011;41:272-5.
- 5. Penkoske PA, Duncan N, Collins-Nakai RL. Surgical repair of double-chambered right ventricle with or without ventriculotomy. J Thorac Cardiovasc Surg. 1987;93:385-93.
- 6. Hachiro Y, Takagi N, Koyanagi T, et al. Repair of double-chambered right ventricle: Surgical results and long-term follow-up. Ann Thorac Surg. 2001;72:1520-1.
- 7. Mądry W, Karolczak MA, Zacharska-Kokot E. Double-chambered right ventricle in a 16-year-old patient with Williams syndrome. J Ultrason. 2017;17:289-93.
- 8. Yamamoto M, Takashio S, Nakashima N, et al. Double-chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome. ESC Heart Fail. 2020;7:721-6.
- 9. Ahmed Eltohami E, Ahmed Hajar H, Folger GM, et al. Double-chambered right ventricle and Down’s syndrome: A proposed new association. Angiology. 1994;45(2):119-23.
- 10. Hennekam RCM. Costello syndrome: An overview. Am J Med Genet C Semin Med Genet. 2003;117(1):42-8.
- 11. Khojah O, Alamoudi S, Aldawsari N, et al. Central nervous system vasculopathy and Seckel syndrome: Case illustration and systematic review. Child’s Nerv Syst. 2021;37:3847-60.
- 12. Ucar B, Beyir Kilic Z, Dinleyici C, et al. Seckel syndrome associated with atrioventricular canal defect: A case report. Clin Dysmorphol. 2004;13(1):53-5.
- 13. Can E, Bulbul A, Uslu S, et al. A case of Seckel syndrome with Tetralogy of Fallot. Genet Couns. 2010;21(1):49-51.
- 14. Arslan D, Çimen D, Guvenc O, et al. A case of Seckel syndrome with tricuspit atresia. Genet Couns. 2014;25(2):171-5.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Cerrahi |
| Bölüm | Araştırma Makalesi |
| Yazarlar | |
| Yayımlanma Tarihi | 30 Nisan 2024 |
| Gönderilme Tarihi | 4 Mayıs 2023 |
| Yayımlandığı Sayı | Yıl 2024 Cilt: 11 Sayı: 1 |
