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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)...

Abstract

Introduction:

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.

Results:

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.

Conclusion:

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.

Copyright

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: woonghan@snu.ac.kr

Footnotes

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

Footnotes

References

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