Incidence of cardiovascular events and risk markers in a prospective study of children diagnosed with Marfan syndrome

Summary Background Little is known about the incidence of cardiovascular events (CVEs) and their associated risk markers in children with Marfan syndrome (MFS). Aims To assess the incidence of CVEs and determine risk markers in a cohort diagnosed with Marfan syndrome during childhood and followed for several years. Methods From a French multicentre nationwide database, 462 patients with MFS diagnosed during childhood were included prospectively. Patients’ files were screened for a period of 20 years (1993–2013). CVEs (e.g. death, aortic dissection, cardiac valve or aortic root surgery) were assessed during the prospective follow-up. Results Median (interquartile range) age at the end of follow-up was 17.2 (11.1–21.3) years. CVEs were reported for 35 participants (7.6%; 95% confidence interval [CI] 5.3–10.4%). First CVEs were prophylactic aortic root surgery (n = 29), aortic dissection (n = 4; two aged Conclusions CVEs in children with MFS are mainly related to prophylactic aortic root surgery. Aortic dissections are rarely observed in children. The Valsalva sinus Z-score is a strong indicator of subsequent CVEs in children with MFS. Attention to follow-up and beta-blocker observance may be warranted in high-risk children.