Universität zu Köln

Huntgeburth, Michael, Neidenbach, Rhoia Clara, von Kodolitsch, Yskert and Kaemmerer, Harald (2018). Genetic Aortic Syndromes - Recommendations for Cardiologists in Practice and Clinic. Aktuelle Kardiol., 7 (3). S. 220 - 231. STUTTGART: GEORG THIEME VERLAG KG. ISSN 2193-5211

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Abstract

Dilatation of the aorta is a condition. Knowledge of the underlying causes and mechanisms allows adequate diagnosis and guidance of patients. Genetically determined aortic diseases are associated with increased risk for aortic complication (dissection/rupture). The underlying conditions include syndromic (e.g. Marfan-, Loeys-Dietz-, vascular Ehlers-Danlos-Syndrome) and non-syndromic conditions (e.g. bicuspid aortic valve - BAV, aortic coarctation, complex congenital heart diseases, familial thoracic aortic aneurysm - FTAA, dissections FTAAD). Family history, clinical investigation, imaging and genetic testing are used for diagnosis. A precise diagnosis and risk stratifying for aortic complications are crucial for the prognosis of these patients. The management includes modifications in lifestyle (e.g. physical stress in sport and work), a medical prophylaxis of aortic growth (reduction of wall stress), an elective aortic replacement and regular follow-up examinations. Due to the complexity of aortopathies, a multi-disciplinary care is required. Additionally, a family screening of all first-degree family members is necessary to identify others at risk.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Huntgeburth, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Neidenbach, Rhoia Clara UNSPECIFIED UNSPECIFIED UNSPECIFIED von Kodolitsch, Yskert UNSPECIFIED UNSPECIFIED UNSPECIFIED Kaemmerer, Harald UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-184283
DOI:	10.1055/a-0626-6961
Journal or Publication Title:	Aktuelle Kardiol.
Volume:	7
Number:	3
Page Range:	S. 220 - 231
Date:	2018
Publisher:	GEORG THIEME VERLAG KG
Place of Publication:	STUTTGART
ISSN:	2193-5211
Language:	German
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CONGENITAL HEART-DISEASE; MARFAN-SYNDROME; MANAGEMENT; ADULTS; ASSOCIATION; GUIDELINES; DISSECTION; SCORE Multiple languages Cardiac & Cardiovascular Systems Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/18428