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

Published online by Cambridge University Press:  21 May 2019

Jae Gun Kwak Open the ORCID record for Jae Gun Kwak [Opens in a new window] ,
Woong-Han Kim ,
Eung Re Kim ,
Yoon Jin Kang ,
Jooncheol Min ,
Jae Hong Lim Open the ORCID record for Jae Hong Lim [Opens in a new window]  and
Yong Jin Kim
Jae Gun Kwak
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
Woong-Han Kim*
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
Eung Re Kim
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Bucheon, Korea
Yoon Jin Kang
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
Jooncheol Min
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
Jae Hong Lim
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
Yong Jin Kim
Affiliation:
Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Bucheon, Korea
*
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: [email protected]
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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.

Keywords

Aortaaortic surgeryconnective tissue diseasevascular diseasechildren
Type
Original Article
Information
Cardiology in the Young , Volume 29 , Issue 5 , May 2019 , pp. 564 - 569
Copyright
© Cambridge University Press 2019 

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Footnotes

*

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

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