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Konservative Therapieoptionen bei genetischen Aortensyndromen (GAS)

Conservative therapy options for heritable thoracic aortic disorders (H-TAD)

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Zusammenfassung

Hintergrund

Genetische Aortensyndrome (GAS) wie das Marfan-Syndrom führen unbehandelt zu einem frühen Tod aufgrund von Aortendissektion und -ruptur. Des Weiteren besteht eine hohe kardiovaskuläre Morbidität und Mortalität durch Arrhythmien, Herzinsuffizienz und Herzklappenerkrankungen mit einem Risiko für den plötzlichen Herztod von bis zu 4 %.

Ziel der Arbeit

Diese Arbeit stellt die konservativen Therapieoptionen genetischer Aortensyndrome zusammenfassend vor, wobei der Schwerpunkt auf das Marfan-Syndrom gelegt wurde.

Material und Methoden

Es erfolgte eine selektive Literaturrecherche und Zusammenfassung der Daten unter Einbeziehung persönlicher Erfahrung.

Ergebnisse und Schlussfolgerungen

Durch konservative Therapiemaßnahmen und prophylaktische aortenchirurgische Eingriffe konnte die Lebenserwartung in den letzten Jahren deutlich verbessert werden. Schwerpunkte in der Therapie sind regelmäßige Kontrollen der Aortendiameter, frühzeitige medikamentöse Therapie und Reduktion von Risikofaktoren wie Schlafapnoe und arteriellem Hypertonus.

Die kardialen Manifestationen wie Klappenerkrankungen und Kardiomyopathie mit Risiko für Herzinsuffizienz und Arrhythmien sollten gleichrangig mit der Aortenerkrankung in den Fokus gestellt werden, um lebensbedrohliche Komplikationen zu vermeiden.

Abstract

Background

Untreated patients with heritable thoracic aortic disorders (H-TAD), such as Marfan syndrome die prematurely due to dissection and rupture of the aorta. There is also a high cardiovascular morbidity and mortality because of arrhythmias, heart failure and heart valve disease with a risk for sudden cardiac death up to 4 %.

Objective

This article summarizes the current standard of conservative treatment of H‑TAD with a focus on Marfan syndrome.

Material and methods

A selective review of the literature was performed and summarized including experience gained at our department.

Results and conclusion

Life expectancy has significantly increased in recent years due to conservative therapy and prophylactic aortic surgery. The main topics in therapy of H‑TAD are regular controls of the aortic diameter, early initiation of medicinal treatment, and reduction of risk factors, such as sleep apnea and hypertension. Cardiac manifestations, such as heart valve disease and cardiomyopathy with a risk for heart failure and arrhythmias should be taken into account in equal measure with the aortic disease to avoid life-threatening complications.

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Authors and Affiliations

  1. Klinik für Kardiologie mit Schwerpunkt Elektrophysiologie, Universitäres Herzzentrum Hamburg, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland

    N. Gessler, B. A. Hoffmann & S. Willems

  2. Klinik für Allgemeine und Interventionelle Kardiologie, Universitäres Herzzentrum Hamburg, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland

    H. Schueler, Y. von Kodolitsch & M. Rybczynski

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  1. N. Gessler
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  2. H. Schueler
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  3. Y. von Kodolitsch
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  4. B. A. Hoffmann
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  5. M. Rybczynski
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  6. S. Willems
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Corresponding author

Correspondence to N. Gessler.

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Interessenkonflikt

N. Gessler, H. Schueler, Y. von Kodolitsch, B.A. Hoffmann, M. Rybczynski und S. Willems geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Gessler, N., Schueler, H., von Kodolitsch, Y. et al. Konservative Therapieoptionen bei genetischen Aortensyndromen (GAS). Gefässchirurgie 21, 403–410 (2016). https://doi.org/10.1007/s00772-016-0175-1

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  • DOI: https://doi.org/10.1007/s00772-016-0175-1

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