Long term outcomes of congenital aortic stenosis in adults: preliminary results of a multicentre study. (3rd October 2022)

• Record Type: Journal Article
• Authors: Keuning, Z; Meccanici, F; Sneep, F M; Hirsch, A; Takkenberg, J J M; Van Den Bosch, A E; Roos-Hesselink, J W
• Is Part Of: European heart journal. Volume 43(2022)Supplement 2
• Publication Date: 2022-10-03
• Subjects: Cardiology -- Periodicals; Heart -- Diseases -- Periodicals; 616.12005
• DOI: 10.1093/eurheartj/ehac544.1809
• Languages: English
• ISSNs: 0195-668X

Abstract

Abstract: Background: Congenital aortic stenosis (ConAoS) accounts for 4–8% of all congenital cardiac diagnosis and is associated with important morbidity and mortality. However, little is known about the natural history of ConAoS in adult patients. We aim to evaluate disease progression of ConAoS in young adults, describing both ventricular and valvular characteristics. Methods: For this retrospective multicentre cohort study, data of one centre is collected up till now. We included ConAoS patients with a valvular stenosis (Vmax ≥2.5 m/s) aged 18–55 years from the Dutch CONCOR national registry from 2001 until 2019, excluding patients with prior aortic valve replacement (AVR) or severe aortic regurgitation. Echocardiographic data was used to determine presence of left ventricular (LV) hypertrophy (LV mass index >115 g/m 2 in males and >95 g/m 2 in females) and measure aortic jet velocity. Results: A total of 92 patients (64% male, median age 24 years) were included with a median follow-up duration of 6.3 years (IQR 2.9–13.3). The median aortic jet velocity was 3.3 m/s (IQR 2.7–3.9) at baseline and significantly increased over time to 3.6 m/s (IQR 2.8–4.3) (p<0.001). LV hypertrophy was present in 33% of the patients and 17% of the patients showed signs of concentric remodelling at baseline. AVR was performed in 32 patients (35%, mean age 36±11 years) and one patient died during follow-up. Figure 1 shows significant differences in intervention-free survival for different AS severity groups (p<0.001) and presence of LV hypertrophy (p=0.02). Conclusion: Although the progression rate of ConAoS seems to be slow, it is often necessary to perform AVR at a relatively young age. After data collection is completed, this study will enable us to identify risk factors for rapid disease progression and further improve current treatment protocols. Funding Acknowledgement: Type of funding sources: Foundation. Main funding source(s): Dutch Heart Foundation, Hartekind