Summary
Marfan syndrome is a hereditary disease of the connective tissue with increased mortality mostly due to changes of the cardiovascular system. We describe our experience with the surgical treatment of 243 patients with Marfan syndrome and cardiovascular complications. We report the results of treatment of annulo-aortal ectasia using the classical surgical methods of Bentall DeBono and Cabrol and the method for preservation of the native aortic valve and concomitant surgery of the mitral valve. Reconstruction or replacement of the mitral valve is mainly based on the classical indications. Preservation of the native aortic valve and reconstruction of the mitral valve in patients with Marfan syndrome is possible but the long-term results are still unknown.
Zusammenfassung
Das Marfan-Syndrom ist eine erbliche Bindegewebserkrankung, die mit einer erhöhten Inzidenz von kardiovaskulären Komplikationen verbunden ist. Wir beschreiben unsere Erfahrungen bei der chirurgischen Behandlung von 243 Patienten mit Marfan-Syndrom. Bezüglich der Behandlung der anuloaortalen Ektasie zeigen wir unsere Ergebnisse bei der Verwendung der klassischen Operationsmethoden nach BentallDeBono und Cabrol. Neure Operationsmethoden wie die Verfahren nach David und Yacoub werden kritisch gewertet und eine eigene Modifikation zum Klappen erhaltenden Ersatz der Aorta ascendens wird vorgestellt.
Bei einer großen Zahl von Patienten waren Operationen wegen der Manifestetion des Marfan-Syndroms im Bereich der distal der Aorta ascendens gelegenen Aortensegmente, der Mitralklappe und der Trikuspidalklappe erforderlich. Die Indikation zur Rekonstruktion bzw. zum Ersatz der Mitralklappe wird weitgehend entsprechend den klassischen Kriterien gestellt. Die Langzeitresultate des Erhalts der nativen Aortenklappe sowie der Mitralklappenrekonstruktion sind noch nicht vorhanden.
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Pasic, M., Bauer, M. & Hetzer, R. Marfan-Syndrom und Herzklappenerkrankungen. Z Kardiol 90 (Suppl 6), 105–111 (2001). https://doi.org/10.1007/s003920170017
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DOI: https://doi.org/10.1007/s003920170017