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Aortopathies: Clinical Manifestation

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Aortopathy

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Abstract

Many native, surgically or interventionally treated patients with congenital heart anomalies and genetic aortic syndromes (GAS) (e.g., Marfan, Loeys-Dietz, Ehlers-Danlos, Turner syndrome) are at risk for the development of aortic aneurysm, dissection, or rupture, resulting from inborn anomalies of the structure and metabolism of the aortic wall.

Therefore, all caregivers should be familiar with signs and symptoms of aortopathies and imperative diagnostic and therapeutic measures.

The diagnosis of aortopathies largely depends on recognition of aortic dilatation prior to the development of acute complications like dissection or rupture, as well as organ involvement from complications. Clinical suspicion is crucial, as usually the growth of aortic aneurysm is asymptomatic until aortic complications occur. The diagnostic approach to the diagnosis of aortopathies incorporates primarily the clinical impression, the medical history, and the results of the physical examination, supplemented by sophisticated modern laboratory and imaging techniques, particularly advanced echo, MRI, and CT techniques, which have improved the clinical diagnosis substantially.

Only timely discovery provides the chance to induce prophylactic or therapeutic measures.

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Author information

Authors and Affiliations

  1. Klinik für Kinderkardiologie und angeborene Herzfehler, Deutsches Herzzentrum München, German Heart Center Munich, Technische Universität München, Technical University Munich, Lazarettstr. 36, D-80636, Munich, Germany

    Harald Kaemmerer

  2. Centre of Cardiology and Cardiovascular Surgery, German Aortic Center Hamburg, University Heart Center, University Medical Centre, Hamburg, Germany

    Yskert von Kodolitsch

Authors
  1. Harald Kaemmerer
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  2. Yskert von Kodolitsch
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Correspondence to Harald Kaemmerer .

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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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Kaemmerer, H., von Kodolitsch, Y. (2017). Aortopathies: Clinical Manifestation. In: Niwa, K., Kaemmerer, H. (eds) Aortopathy. Springer, Tokyo. https://doi.org/10.1007/978-4-431-56071-5_4

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  • DOI: https://doi.org/10.1007/978-4-431-56071-5_4

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  • Print ISBN: 978-4-431-56069-2

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