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Tetralogy of Fallot and Pulmonary Atresia with Ventricular Septal Defect

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Aortopathy

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Abstract

Aortic dilatation and regurgitation in unrepaired tetralogy of Fallot imposes volume overload on both ventricles. A significant subset of adults late after repair of tetralogy of Fallot exhibits progressive aortic root dilatation, which may lead to aortic regurgitation and predispose to dissection and rupture that can be fatal, and necessitating aortic valve and aortic root surgery. The aortic dilatation relates medial abnormalities coupled with previous long-standing volume overload of the ascending aorta. Risk factors for aortic dilatation and regurgitation in tetralogy of Fallot relate to specific hemodynamic abnormalities, such as pulmonary atresia, right aortic arch, and a history of an aortopulmonary shunt, and patient demographics such as male sex and the association of chromosome 22q11 deletion. There is no current consensus on beta-blocker or angiotensin II receptor blocker (ARB) administration for limiting progressive dilatation of the aortic root in patients with repaired tetralogy of Fallot. Aortic root surgery should be considered for these patients and address aortic regurgitation and/or prevent the risk of aortic dissection. Thus, in tetralogy of Fallot meticulous follow-up of the aortic root after repair is recommended.

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Authors and Affiliations

  1. Cardiovascular Center, St Luke’s International Hospital, 9-1 Akashi-cho, Tokyo, 104-8560, Japan

    Koichiro Niwa M.D., Ph.D., FACC, FAHA, FJCC

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  1. Koichiro Niwa M.D., Ph.D., FACC, FAHA, FJCC
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Correspondence to Koichiro Niwa M.D., Ph.D., FACC, FAHA, FJCC .

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Editors and Affiliations

  1. Cardiovascular Center, St Luke’s International Hospital, Chuo-ku, Tokyo, Japan

    Koichiro Niwa

  2. ACHD Unit, German Heart Centre Munich, Munich, Bayern, Germany

    Harald Kaemmerer

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Niwa, K. (2017). Tetralogy of Fallot and Pulmonary Atresia with Ventricular Septal Defect. In: Niwa, K., Kaemmerer, H. (eds) Aortopathy. Springer, Tokyo. https://doi.org/10.1007/978-4-431-56071-5_17

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  • DOI: https://doi.org/10.1007/978-4-431-56071-5_17

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  • Publisher Name: Springer, Tokyo

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  • Online ISBN: 978-4-431-56071-5

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