Increased risk for ascending aortic dilatation in patients with complex compared to simple aortic coarctation

Abstract Aims Aortic coarctation (CoA) occurs as a “simple” isolated disorder, and in a more “complex” form, combined with associated congenital cardiac abnormalities. Long term outcome of all CoA patients may be complicated by dilatation of the thoracic aorta. The aim of this study was to quantify progressive aortic dilatation, and identify determinants for progressive aortic dilatation. Methods and Results Cardiovascular Magnetic Resonance Imaging (CMR) and echocardiographic data of 93 CoA patients were analyzed retrospectively on the progression, and determinants, of progressive thoracic aortic dilatation. Outcome of simple- versus complex CoA patients were compared. 93 CoA patients (mean age 39 ± 12 years, male 59%) were followed with CMR (follow-up 5.3 ± 1.8 years). Twenty-eight patients were classified as simple- and 68 as complex CoA. The mean progression rate of thoracic aortic dilatation was highest in the ascending aorta with 2.2 ± 2.0 mm/5 years (range 0–7.2 mm/5 years). History of VSD (β = 1.77, P = 0.004) and an increased left ventricular mass index (β = 0.02, P = 0.04) were associated with progressive ascending aortic dilatation. Complex CoA patients show an increased progression rate compared to simple CoA patients with 2.4 mm/5 years versus 1.5 mm/5 years respectively. (P = 0.03). Conclusion Adult post-coarctectomy patients show an increased mean progression rate of ascending aortic dilatation with 2.2 mm/5 years. The progression rate of ascending aortic dilatation is increased in complex CoA patients, as compared to simple CoA patients. These findings point towards a more comprehensive genetic subset of patients with an increased risk for progressive ascending aortic dilatation.