Aortic root dilation has been reported commonly after repair of tetralogy of Fallot. However, the rate and risk factors of progression of the dilation are not fully understood. This is a single-centre, retrospective study to assess the rate and factors associated with progressive dilatation of the aortic root in repaired tetralogy of Fallot patients using cardiac MRI. The presence of the significant aortic dilation and the progression of dilation between initial and follow-up cardiac MRI were examined. The study cohort comprised 72 patients with repaired tetralogy of Fallot. The median age at the initial cardiac MRI scan was 19.6 (interquartile range: 14.6–31) years, and the median follow-up interval was 4.3 (2.9–5.7) years. Median dimension of ascending aorta at initial and follow-up cardiac MRI was 27.0 (22.3–31.0) mm and 29.2 (25.0–32.1) mm, respectively. Significant aortic dilation (the percentage predicted ascending aorta ≥150%) was observed in 11 (15.2%) patients at the initial cardiac MRI and 24 (33.3%) at the follow-up cardiac MRI. The significant aortic dilation at follow-up cardiac MRI was associated with increased indexed left ventricular stroke volume (odds ratio 1.062, p = 0.023). Thirteen patients demonstrated the significant progressive dilation of aorta between initial and follow-up cardiac MRI. The progressive dilation was associated with left ventricular ejection fraction at initial cardiac MRI (odds ratio 1.135, p = 0.048). In patients with repaired tetralogy of Fallot, aortic dilation is common and progresses over time. Cardiac MRI is a valuable tool for identifying individuals at risk for progressive aortic dilation.

Background: Women with Turner's syndrome have an increased risk of congenital cardiac malformations, ischaemic heart disease, hypertension and stroke. Aortic dissection seems to occur with increased frequency. Aim: To describe in more detail aortic dissection as encountered in Turner's syndrome, giving attention to clinical, histological and epidemiological aspects. Materials and methods: Based on a retrospective study, we describe the clinical, karyotypic, and epidemiological aspects of aortic dissection as encountered in cases of Turner's syndrome seen in Denmark and Sweden. Results: The median age at onset of aortic dissection in 18 women was 35 years, ranging from 18 to 61 years. Fourteen of 18 women had a 45,X karyotype, while 2 patients had 45,X/45,XY, and 2 had the 45,X/46,X+r(X) complement, respectively. Echocardiography was performed in 10 of 18 patients before their acute illness, and showed signs of congenital cardiac disease, with either bifoliate aortic valves, dilation of the aortic root, or previous aortic coarctation evident in most patients. In 5 patients evidence of a bifoliate aortic valve was conclusive. Hypertension was present in 5 of 18 patients, while 10 of the patients died from aortic dissection, of so-called type A in 6, type B in 3, while in the final case the origin of dissection could not be determined. Biochemical analysis showed altered ratio between type I and type III collagen. Histology showed cystic medial necrosis in 3 of 7 cases. We estimated an incidence of dissection of 36 per 100,000 Turner's syndrome years, compared with an incidence of 6 per 100,000 in the general population, and a cumulated rate of incidence of 14, 73, 78, and 50 per 100,000 among 0–19, 20–29, 30–39, and 40+ year olds, respectively. Conclusion: Aortic dissection is extremely common in the setting of Turner's syndrome, and occurs early in life. Patients with Turner's syndrome should be offered a protocol for clinical follow-up similar to that provided for patients with Marfan syndrome, and each clinic should embrace a programme for follow-up.