Biküspid aort kapağı tanısı alan pediatrik hastalarda aort kapak morfolojisi ile aort kapak disfonksiyonu arasındaki ilişki

Year 2023, Volume: 13 Issue: 6, 1095 - 1102, 30.11.2023

Hüseyin Süslü , Ahmet Sert

Abstract

Amaç: Biküspit aort kapağı olarak bilinen konjenital kalp malformasyonu sık görülen bir durumdur. Bu çalışmanın temel amacı biküspid aort kapak rahatsızlığı olan çocuk ve ergenlerin demografik, klinik ve ekokardiyografik özelliklerini analiz etmektir. Ek olarak, incelenen gruplar arasında aort kökü ve çıkan aort boyutlarını değerlendirmeyi ve karşılaştırmanın yanı sıra aort kapak fonksiyon bozukluğunun düzeyini aort kapak morfolojisi ile ilişkili olarak değerlendirmeyi amaçladık.
Gereç ve Yöntem: Şimdiki çalışma, Çocuk Kardiyoloji Bölümü'nde ekokardiyografi kullanılarak biküspit aort kapağı tanısı konulan 18 yaş altı pediatrik hastalarda gerçekleştirildi.
Bulgular: Ekokardiyografi yapılan hastaların %1,42'sinde (10.691 hastanın 152'sinde) biküspit aort kapağı tespit edildi. Bu hastaların %72,4'ü (110) erkekti. Biküspit aort kapağı olan hastalarda en sık gözlenen aort kapak morfolojisi, sol ve sağ koroner kapakçıkların füzyonuydu.
Sonuç: Çalışmamızın sonuçları, sağ ve non-koroner kasp füzyon kohortunun aortik annulus ve sinotubüler bileşke çapının diğer gruplara göre belirgin şekilde daha büyük olduğunu ortaya koydu. Ayrıca biküspid aort kapağı tanısı alan çocuklarda yaş, kilo ve boyun aort kökü ve çıkan aort çapı üzerinde bağımsız prediktif etkiler oluşturduğu gözlendi.

Keywords

ergen, aort kökü, aort kapak disfonksiyonu, çıkan aort, biküspid aort kapağı, çocuklar

The association between the morphology of the aortic valve and the dysfunction of the aortic valve in pediatric patients with a diagnosis of bicuspid aortic valve

Abstract

Aim: The congenital heart malformation known as the bicuspid aortic valve is a prevalent condition. The main objective of this study was to analyze the demographic, clinical, and echocardiographic characteristics of subjects who were children and adolescents with the bicuspid aortic valve condition. Additionally, we aimed to evaluate and compare the dimensions of the aortic root and ascending aorta as well as evaluate the level of aortic valve dysfunction in relation to the morphology of the aortic valve amongst the groups studied.
Materials and Methods: The present investigation was conducted among pediatric patients below the age of 18 years, who had been identified with bicuspid aortic valve through the use of echocardiography, at the Department of Paediatric Cardiology.
Results: Bicuspid aortic valve was detected in 1.42% (152 out of 10,691) of patients who underwent echocardiography. Among these patients, 72.4% (110) were male. The most frequent aortic valve morphology observed in patients with bicuspid aortic valve was the fusion of the left and right coronary cusps.
Conclusion: The results of our investigation have evinced that the diameter of the aortic annulus and sinotubular junction in the fusion of the right and non-coronary cusps cohort was markedly greater than that of the other groups. Moreover, it was observed that age, weight, and height exerted independent predictive effects on the diameters of the aortic root and ascending aorta in children diagnosed with bicuspid aortic valve.

Keywords

adolescent, aortic root, aortic valve dysfunction, ascending aorta, bicuspid aortic valve, children

References

• 1. Siurana JM, Sabaté-Rotés A, Ayerza A, et al. Adolescents with bicuspid aortic valve: Which criteria should we use for aortic dilatation? Int J Cardiol 2021; 333: 90-3.
• 2. Merkx R, Duijnhouwer AL, Vink E, Roos-Hesselink JW, Schokking M. Aortic diameter growth in children with a bicuspid aortic valve. Am J Cardiol 2017; 120(1): 131-6.
• 3. Pees C, Michel-Behnke I. Morphology of the bicuspid aortic valve and elasticity of the adjacent aorta in children. Am J Cardiol 2012; 110(9): 1354-60.
• 4. Ciotti GR, Vlahos AP, Silverman NH. Morphology and function of the bicuspid aortic valve with and without coarctation of the aorta in the young. Am J Cardiol 2006; 98(8): 1096-1102.
• 5. Nadorlik H, Bowman JL, Fitzgerald-Butt S, et al. Abnormal longitudinal growth of the aorta in children with familial bicuspid aortic valve. Pediatr Cardiol 2017; 38(8): 1709-15.
• 6. Cantinotti M, Giordano R, Scalese M, et al. Nomograms for two-dimensional echocardiography derived valvular and arterial dimensions in Caucasian children. J Cardiol 2017; 69(1): 208-15.
• 7. Sievers H-H, Schmidtke C. A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc 2007; 133(5): 1226-33.
• 8. Yener N, Oktar GL, Erer D, Yardimci MM, Yener A. Bicuspid aortic valve. Ann Thorac Cardiovasc Surg 2002; 8(5): 264-7.
• 9. Nistri S, Basso C, Marzari C, Mormino P, Thiene G. Frequency of bicuspid aortic valve in young male conscripts by echocardiogram. Am J Cardiol 2005; 96(5): 718-21.
• 10. Basso C, Boschello M, Perrone C, et al. An echocardiographic survey of primary school children for bicuspid aortic valve. Am J Cardiol 2004; 93(5): 661-3.
• 11. Massardier C, Desroches F, Singbo N, et al. First-degree relatives screening of patients with bicuspid aortic valve: effectiveness and feasibility in pediatric cardiology daily practice. Pediatr Cardiol 2020; 41(8): 1645-50.
• 12. Cozijnsen L, Braam R, Bakker-de Boo M, et al. Family screening in patients with isolated bicuspid aortic valve. Neth Heart J 2022; 30(4): 207-11.
• 13. Nabulsi MM, Tamim H, Sabbagh M, Obeid MY, Yunis KA, Bitar FF. Parental consanguinity and congenital heart malformations in a developing country. Am J Med Genet A 2003;116(4):342-47.
• 14. Reisli I, Keles S, Calıskaner Z. Past and Future of J Project Meetings in Turkey: Evaluatıon of Last 10 Years. Immunology and Genetics Journal 2020: 8-11.
• 15. Erolu E, Akalın F, Çetiner N, Şaylan BÇ. Aortic elasticity and the influence of valve morphology in children with bicuspid aortic valve. Cardiol Young 2018; 28(11): 1338-44.
• 16. Niaz T, Fernandes SM, Sanders SP, Michelena H, Hagler DJ. Clinical history and management of bicuspid aortic valve in children and adolescents. Prog Cardiovasc Dis 2020; 63(4): 425-33.
• 17. Evangelista A, Gallego P, Calvo-Iglesias F, et al. Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease. Heart 2018; 104(7): 566-73.
• 18. Miśkowiec D, Lipiec P, Szymczyk E, et al. Bicuspid aortic valve morphology and its impact on aortic diameters—A systematic review with meta‐analysis and meta‐regression. Echocardiography 2018; 35(5): 667-77.
• 19. Gurvitz M, Chang R-K, Drant S, Allada V. Frequency of aortic root dilation in children with a bicuspid aortic valve. Am J Cardiol 2004; 94(10): 1337-40.
• 20. Schäfers H-J, Schmied W, Marom G, Aicher D. Cusp height in aortic valves. J Thorac Cardiovasc 2013; 146(2): 269-74.
• 21. Blais S, Meloche-Dumas L, Fournier A, Dallaire F, Dahdah N. Long-Term Risk Factors for Dilatation of the Proximal Aorta in a Large Cohort of Children With Bicuspid Aortic Valve. Circ Cardiovasc Imaging 2020; 13(3):e009675.
• 22. Mai Z, Guan L, Mu Y. Association between bicuspid aortic valve phenotype and patterns of valvular dysfunction: A meta‐analysis. Clin Cardiol 2021; 44(12): 1683-91.
• 23. Ward RM, Marsh JM, Gossett JM, Rettiganti MR, Collins RT. Impact of bicuspid aortic valve morphology on aortic valve disease and aortic dilation in pediatric patients. Pediatr Cardiol 2018; 39(3): 509-17.