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. 2020 Jul 17;6(1):173.
doi: 10.1186/s40792-020-00935-5.

Significant delayed aortic dilatation after tetralogy of Fallot repair: a case report

Yosuke Nishimura  1 , Toru Yasutsune  2 , Shohei Shimajiri  3 , Yuki Jinzai  2 , Eigo Ikushima  2 , Takehiro Kishigami  2 , Tomoya Takigawa  2
Affiliations

Significant delayed aortic dilatation after tetralogy of Fallot repair: a case report

Yosuke Nishimura et al. Surg Case Rep. .

Abstract

Background: Aortic dilatation may occur in some patients even after complete repair of tetralogy of Fallot (TOF). The progression rate of the aortic diameter is so slow, and the incidence of aortic dissection is so low that it is suspected that frequent imaging of the aorta may not be necessary.

Case presentation: We describe an asymptomatic 41-year-old man with hypertension in whom aortic dilatation was accidentally discovered 39 years after TOF repair. He underwent ambulatory follow-up without any difficulty for 21 years after the repair. Contrast-enhanced computed tomography revealed significant aortic dilatation (maximum diameter of 88 mm at the sinus of Valsalva), and echocardiography revealed severe aortic regurgitation, which seemed to progress during the last 18 years without any evaluation or follow-up. The Bentall procedure was successfully performed using a valved graft, under deep hypothermic circulatory arrest with antegrade cerebral perfusion, and his postoperative course was uneventful. Histopathological examination of ascending aorta specimens revealed severe cystic medial degeneration.

Conclusions: Keeping in mind that a patient with rapid progression of the aortic dilatation after TOF repair exist, periodic follow-up for evaluation of the aorta is essential in patients with TOF.

Keywords: Aneurysm; Aortic dilatation; Tetralogy of Fallot.

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Conflict of interest statement

All authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Contrast-enhanced computed tomography scan showing a significantly dilatated aortic root and ascending aorta. Aortic aneurysm was located in the ascending aorta (a), and the maximum diameter of the aortic aneurysm is 88 mm at the sinus of Valsalva (b)
Fig. 2
Fig. 2
Photomicrographs of the ascending aorta (a-c) and aortic valve (d) in the patients described in this report. Degeneration of the aorta with significant medial destruction and changes in smooth muscle orientation are observed in addition to accumulation of mucopolysaccharides and collagen deposition (a × 50 and b × 200, hematoxylin and eosin stain). Fragmentation and loss of elastic lamellae are observed across large areas of the media (c, elastica van Gieson stain × 200). Mucoid degeneration of the aortic valve is clearly visualized (d, hematoxylin and eosin stain × 200)
See this image and copyright information in PMC

References

    1. Shimizu H, Endo S, Natsugoe S, Doki Y, Hirata Y, Kobayashi, et al. Thoracic and cardiovascular surgery in Japan in 2016: annual report by the Japanese Association for Thoracic Surgery. Committee for Scientific Affairs, The Japanese Association for Thoracic Surgery. Gen Thorac Cardiovasc Surg. 2019;67(4):377–411. doi: 10.1007/s11748-019-01068-9. - DOI - PubMed
    1. Cuypers JA, Menting ME, Konings EE, Opić P, Utens EM, Helbing WA, et al. Unnatural history of tetralogy of Fallot: prospective follow-up of 40 years after surgical correction. Circulation. 2014;130(22):1944–1953. doi: 10.1161/CIRCULATIONAHA.114.009454. - DOI - PubMed
    1. Niwa K. Aortic dilatation in complex congenital heart disease. Cardiovasc Diagn Ther. 2018;8(6):725–738. doi: 10.21037/cdt.2018.12.05. - DOI - PMC - PubMed
    1. Egbe AC, Miranda WR, Ammash NM, Anavekar NS, Missula VR, Kothapalli S, et al. Aortic disease and interventions in adults with tetralogy of Fallot. Heart. 2019;105(12):926–931. - PubMed
    1. Kay WA, Cook SC, Daniels CJ. Evaluation by MRA of aortic dilation late after repair of tetralogy of Fallot. Int J Cardiol. 2013;167(6):2922–2927. doi: 10.1016/j.ijcard.2012.07.015. - DOI - PubMed

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