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Surgery on a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents

  • Jae Gun Kwak (a1), Woong-Han Kim (a1), Eung Re Kim (a2), Yoon Jin Kang (a1), Jooncheol Min (a1), Jae Hong Lim (a1) and Yong Jin Kim (a2)...



This research investigated patients who underwent surgery for a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents.

Materials and Methods:

The medical records of 11 patients who underwent aortic surgery for dilatation resulting from a connective tissue disease or inflammatory vasculitis between 2000 and 2017 were retrospectively reviewed.


The median age and body weight of the patients were 9.6 years (range 5.4 months–15.5 years) and 25.8 kg (range 6.8–81.5), respectively. The associated diseases were Marfan syndrome (n = 3), Loeys-Dietz syndrome (n = 3), Kawasaki disease (n = 1), Takayasu arteritis (n = 1), PHACE syndrome (n = 1), tuberous sclerosis (n = 1), and unknown (n = 1). The most common initially affected area was the ascending aorta. During the 66.4 ± 35.9 months of follow-up, two Marfan syndrome patients died, and four patients (one Marfan syndrome and three Loeys-Dietz syndrome) had repeated aortic operation. Except for one patient, the functional class was well maintained in all patients who were followed up.


Cases of surgical treatment for a dilated aorta associated with a connective tissue disease and inflammatory vasculitis are rare in children and adolescents at our institution. Most of the patients in this study showed a tolerable postoperative course. However, the aorta showed progressive dilation over time even after surgical treatment, especially in patients with Loeys-Dietz syndrome. In these patients, close and more frequent regular follow-up is required.


Corresponding author

Author for correspondence: W.-H. Kim, MD, PhD, Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Daehak-Rho 101, Yeongeon-Dong 28, Jongrho-Gu, Seoul, Republic of Korea. Tel: 82-2-2072-363782-2-764-3664; E-mail:


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This topic was presented in the 7th World Congress of Pediatric Cardiology and Cardiac Surgery, Barcelona, Spain, 2017.



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