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Aortopathy Including Hereditary Disease (Marfan Syndrome, Bicuspid Aortic Valve, etc.)

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Adult Congenital Heart Disease

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Abstract

Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are consistently associated with ascending aortic or para-coarctation medial abnormalities. Medial abnormalities in ascending aorta were prevalent in other type of patients with a variety form of congenital heart disease such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range, and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery. These congenital heart diseases exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. These aortic dilatations and increased stiffness can induce aortic aneurysm, rapture of the aorta, and aortic regurgitation but also provoke left ventricular hypertrophy, reduced coronary artery flow, and left ventricular failure. Therefore, this association of aortic pathophysiological abnormality, aortic dilation and aorto-left ventricular interaction is called as a new clinical entity: “aortopathy”.

Aortic dilation is frequently observed in congenital heart diseases (CHD). Aortic dilation predisposes to aortic annular enlargement and then severe aortic regurgitation (AR). Concomitant aortic valve replacement or plasty with replacement of the aortic root and the diseased ascending aorta will be required in those patients (aortic root replacement). The Bentall operation is an aortic root replacement with a composite tube graft that contains a mechanical valve in it. Valve-sparing root replacement, i.e., remodeling and reimplantation procedure, has been developed for the patients whose aortic valves are feasible to be preserved. The ascending aorta and Valsalva sinuses are replaced with a trimmed tube graft in remodeling procedure; on the other hand, the aortic root is included in the tube graft in reimplantation. In contrast, the pulmonary autograft is used to replace the aortic root and aortic valve in the Ross procedure. Enucleated RV outflow tract and the main pulmonary artery are reconstructed usually with homograft or other valved conduit. Some controversy has been provoked to indicate the Ross procedure and expanded application of valve-sparing root replacement.

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Authors and Affiliations

  1. Chiba Cerebral and Cardiovascular Center, 575 Tsurumai, Ichihara, Chiba, 290-0512, Japan

    Kozo Matsuo M.D.

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

    Koichiro Niwa M.D.,Ph.D.,F.A.H.A.,F.A.C.C.

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  1. Kozo Matsuo M.D.
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  2. Koichiro Niwa M.D.,Ph.D.,F.A.H.A.,F.A.C.C.
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Correspondence to Kozo Matsuo M.D. .

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  1. Department of Surgery, Yokohama City Univ. Grad. School of Med., Yokohama, Kanagawa, Japan

    Munetaka Masuda

  2. Cardiovascular Center, St Luke’s International Hospital, Tokyo, Japan

    Koichiro Niwa

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Matsuo, K., Niwa, K. (2017). Aortopathy Including Hereditary Disease (Marfan Syndrome, Bicuspid Aortic Valve, etc.). In: Masuda, M., Niwa, K. (eds) Adult Congenital Heart Disease. Springer, Singapore. https://doi.org/10.1007/978-981-10-4542-4_13

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