Jason Claus, Lauritz Schoof, Thomas S Mir, Anna Lena Kammal, Gerhard Schön, Kerstin Kutsche, Christian-Alexander Behrendt, Klaus Kallenbach, Tilo Kölbel, Christian Kubisch, Till Joscha Demal, Johannes Petersen, Jens Brickwedel, Michael Hübler, Christian Detter, Paulus Kirchhof, Eike Sebastian Debus, Meike Rybczynski, Yskert von Kodolitsch
BACKGROUND: Marfan syndrome (MFS) guidelines recommend optimal pharmacological therapy (OPT) and replacement of the ascending aorta (RAA) at 5.0cm diameters to prevent acute type A aortic dissection (ATAAD) and death. The effect of early MFS diagnosis and initiation of therapy on outcomes is not known. OBJECTIVE: To evaluate the effect of age at MFS diagnosis and therapy initiation on delayed RAA and death. METHODS: This retrospective observational cohort study with long-term follow-up included consecutive patients with MFS, pathogenic FBN1 variant, and regular visits to a European Reference Network Center...
September 19, 2024: Journal of Thoracic and Cardiovascular Surgery