Hostname: page-component-76fb5796d-22dnz Total loading time: 0 Render date: 2024-04-26T17:58:32.925Z Has data issue: false hasContentIssue false
  • English
  • Français

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: woonghan@snu.ac.kr
Get access Rights & Permissions [Opens in a new window]

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 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

*

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

References

Turtle, EJ, Sule, AA, Webb, DJ. Bath LE Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations. Arch Dis Child 2015; 100: 662–666.CrossRefGoogle ScholarPubMed
Karthekeyan, BR, Vakamudi, M. Thangavel, P. Repair of idiopathic ascending aortic aneurysm in a 7-year-old child. Cardiol Young 2015; 25: 154–157.CrossRefGoogle Scholar
Bolin, E, Moodie, DS, Fraser, CD Jr., Guirola, R, Warren, R, Eldin, KW Takayasu arteritis presenting as severe ascending aortic arch dilation and aortic regurgitation in a 10-year-old female. Congenit Heart Dis 2011; 6: 630–633.CrossRefGoogle Scholar
Cattaneo, SM, Bethea, BT, Alejo, DE, et al. Surgery for aortic root aneurysm in children: a 21-year experience in 50 patients. Ann Thorac Surg 2004; 77: 168–176.CrossRefGoogle ScholarPubMed
Williams, JA, Loeys, BL, Nwakanma, LU, et al. Early surgical experience with Loeys-Dietz: a new syndrome of aggressive thoracic aortic aneurysm disease. Ann Thorac Surg 2007; 83: S757–S763; discussion S85–S90.CrossRefGoogle ScholarPubMed
Patel, ND, Alejo, D, Crawford, T, et al. Aortic root replacement for children with Loeys-Dietz syndrome. Ann Thorac Surg 2017; 103: 1513–1518.CrossRefGoogle ScholarPubMed
Sim, HT, Seo, DJ, Yu, JJ, Baek, JS, Goo, HW, Park, JJ Valve sparing aortic root replacement in children with Loeys-Dietz syndrome. Korean J Thorac Cardiovasc Surg 2015; 48: 272–276.CrossRefGoogle ScholarPubMed
Rodriguez-Gonzalez, M, Matamala-Morillo, MA, Segado-Arenas, A, Marin-Iglesias Mdel, R. Lechuga-Sancho AM chest pain in children with suspected Type I Fibrillinopathy: a case report. Pediatrics 2015; 136: e1035–e1038.CrossRefGoogle ScholarPubMed
Rached-d’Astous, S, Dahdah, N, Brochu, P, Saint-Cyr, C. Rapidly progressive aortic aneurysmal dilation in a child with systemic lupus erythematosus: too early too severe. BMJ case rep, 2014; 2014: bcr2013–201014.CrossRefGoogle Scholar
Judge, DP. Dietz HC Marfan’s syndrome. Lancet 2005; 366: 1965–1976.CrossRefGoogle ScholarPubMed
Neptune, ER, Frischmeyer, PA, Arking, DE, et al. Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome. Nat Genet 2003; 33: 407–411.CrossRefGoogle ScholarPubMed
Etchevers, HC, Vincent, C, Le Douarin, NM, Couly, GF. The cephalic neural crest provides pericytes and smooth muscle cells to all blood vessels of the face and forebrain. Development 2001; 128: 1059–1068.Google ScholarPubMed
Frieden, IJ, Reese, V, Cohen, D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 1996; 132: 307–311.CrossRefGoogle ScholarPubMed
Drolet, BA, Dohil, M, Golomb, MR, et al. Early stroke and cerebral vasculopathy in children with facial hemangiomas and PHACE association. Pediatrics 2006; 117: 959–964.CrossRefGoogle ScholarPubMed
Bayer, ML, Frommelt, PC, Blei, F, et al. Congenital cardiac, aortic arch, and vascular bed anomalies in PHACE syndrome (from the International PHACE Syndrome Registry). Am J Cardiol 2013; 112: 1948–1952.CrossRefGoogle Scholar
Metry, D, Heyer, G, Hess, C, et al. Consensus statement on diagnostic criteria for PHACE syndrome. Pediatrics 2009; 124: 1447–1456.CrossRefGoogle ScholarPubMed
Salerno, AE, Marsenic, O, Meyers, KE, Kaplan, BS, Hellinger, JC. Vascular involvement in tuberous sclerosis. Pediatr Nephrol 2010; 25: 1555–1561.CrossRefGoogle ScholarPubMed
Wang, Y, Tao, Y. Diagnosis and treatment of congenital abdominal aortic aneurysm: a systematic review of reported cases. Orphanet J Rare Dis 2015; 10: 4.CrossRefGoogle ScholarPubMed
Kato, H, Koike, S, Yokoyama, T. Kawasaki disease: effect of treatment on coronary artery involvement. Pediatrics 1979; 63: 175–179.Google ScholarPubMed
Faivre, L, Collod-Beroud, G, Ades, L, et al. The new Ghent criteria for Marfan syndrome: what do they change? Clin Genet 2012; 81: 433–442.CrossRefGoogle ScholarPubMed
Liang, P, Hoffman, GS. Advances in the medical and surgical treatment of Takayasu arteritis. Curr Opin Rheumatol 2005; 17: 16–24.CrossRefGoogle ScholarPubMed