Konservative Therapieoptionen bei genetischen Aortensyndromen (GAS)

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.