Aortic dissection and prophylactic surgery in congenital heart disease
Kuijpers, Joey M.; Koolbergen, Dave R.; Groenink, Maarten; Matthijs Boekholdt, S.; Meijboom, Folkert J.; Jongbloed, Monique R.M.; Hoendermis, Elke S.; Duijnhouwer, Anthonie L.; Mulder, Barbara J.M.; Bouma, Berto J.
(2019) International Journal of Cardiology, volume 274, pp. 113 - 116
(Article)
Abstract
Background: Proximal aortic dilatation in certain congenital heart diseases (CHD) prompts concerns about dissection and consideration of prophylactic surgery. To evaluate contemporary prophylactic practice, we determined incidence of aortic dissection and prophylactic surgery in aortopathy-associated CHD, compared to Marfan syndrom (MFS) and controls. Methods and results: We followed patients from
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the CONCOR adult CHD registry (2002–2015), with a native proximal aorta and aortopathy-associated CHD, comprising bicuspid aortic valve/aortic stenosis (‘BAV/AS’; n = 2239) and aortic coarctation/conotruncal defects/univentricular heart/ventricular septal defect (‘At-risk CHD’; n = 5439). As reference, we selected MFS (n = 356) and ‘Control’ (atrial septal defect, pulmonary stenosis; n = 2940) patients. Cumulative incidences of dissection and prophylactic proximal aortic replacement – considered competing events – were determined, and compared corrected for age and sex. Median follow-up was 6.7 years. Ten-year dissection-incidence was 0.3% (95%CI: 0.0–0.7) in BAV/AS and 0.2% (0.0–0.3) in At-risk CHD, both significantly lower than in MFS (4.1%; 1.8–6.4) and similar to Controls (0.1%; 0.0–0.3). Ten-year prophylactic-surgery incidence was 9.3% (7.6–11.0) in BAV/AS and 0.7% (0.5–1.0) in At-risk CHD, both significantly lower than in MFS (21.3%; 16.3–26.3) and higher than in Controls (0.1%; 0.0–0.3). Conclusions: In contemporary practice, aortic-dissection incidence is low in adults with aortopathy-associated CHDs, while prophylactic-surgery incidence is high in BAV/AS. To reduce surgical burden, BAV/AS patients could benefit from more individualised prophylactic-surgery algorithms.
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Keywords: Adult, Aortic dissection, Congenital heart disease, Marfan syndrome, Prophylactic aortic surgery, Aortic Aneurysm, Thoracic/etiology, Humans, Male, Treatment Outcome, Blood Vessel Prosthesis, Young Adult, Heart Defects, Congenital/complications, Female, Aneurysm, Dissecting/etiology, Vascular Surgical Procedures/methods, Aorta, Thoracic/surgery, Cardiology and Cardiovascular Medicine, Journal Article
ISSN: 0167-5273
Publisher: Elsevier Ireland Ltd
Note: Funding Information: This work was supported by the Netherlands Heart Institute (Utrecht, the Netherlands). The work described in this study was carried out in the context of the Parelsnoer Institute. The Parelsnoer Institute is part of and funded by the Dutch Federation of University Medical Centers. The authors thank Lia Engelfriet, Sylvia Mantels and Odilia Woudstra for their efforts in retrieving clinical data concerning incident aortic dissections and replacements. Publisher Copyright: © 2018 The Authors
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