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(un)natural History Of Congenital Valvular Aortic Stenosis In Adults: Set-up And Preliminary Results Of A Multicenter Study
Frederike Meccanici, ZoŽ Keuning, Famke Sneep, Annemien van den Bosch, Johanna Takkenberg, Jolien Roos-Hesselink.
Erasmus University Medical Centre, Rotterdam, Netherlands.

OBJECTIVE:
Congenital aortic valvular stenosis (AS) is the most common left heart obstructive lesion in young adults. Scarce data are available on the natural history of congenital AS in adults. We aim to describe long term clinical outcomes, the progression of AS and aortic dilatation in adults with AS.
METHODS:
In this multicenter hospital-based cohort study, data of six tertiary centers will be collected retrospectively of prospectively registered patients in a national database from 2001 until 2019. AS patients (≥2.5 m/s) aged 18-55 were identified, excluding patients with severe aortic regurgitation or prior aortic valve replacement (AVR).
RESULTS:
A total of 92 patients (age 24 [19-34] years, 36% female) were thus far included in one center. The median follow-up time was 6.3 [2.9-13] years. At baseline the aortic jet velocity was 3.3[2.7-3.9] m/s and at follow-up 3.7[2.8-4.3](p<0.001). At the level of the ascending aorta, significant aortic growth was observed (baseline diameter (mm): 38[33-42], follow-up: 41[34-46],p<0.001). During follow-up one patient died (0.2% per patient-year) and 32 patients underwent AVR (5.7% per patient-year). Figure 1 shows significant difference in disease severity groups (p<0.001) for intervention-free survival, however not for prior aortic valve intervention (p=0.40) or sex (p=0.70). Secondary events included heart failure (n=3), (re)intervention on the aortic valve or ascending aorta (n=4), arrhythmia (n=4), thromboembolic events (n=2) and endocarditis (n=2).
CONCLUSIONS:
Progression of AS seems slow, however intervention on the aortic valve and aortic dilatation are common during follow-up in adult patients with congenital AS. In a large dataset with repeated measurements we plan to identify predictors of disease progression, in order to obtain patient-specific risk profiles. Early identification of congenital AS patients at high risk of clinical outcomes or disease progression will help to provide timely (non)-surgical treatment and improve clinical management.


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