Results of Cardiovascular Surgery in the Marfan Syndrome:A Retrospective Study of 49 Patients

Abstract

To evaluate the outcome of cardiovascular surgery in the Marfan syndrome, the records of 49 patients (median age 35 years) who underwent 60 operations were reviewed. Primary surgery was elective in 39 patients and emergency in ten. Non-dissecting aneurysm with diameter 4-19 cm was present in 34 cases and distal, isolated aneurysm in four. In eight cases there was type A acute aortic dissection with median diameter 5.0 cm. One patient was operated on for mitral valve insufficiency, one for ventricular septal defect and one (acute) for endocarditis. Composite grafts were used for aortic root reconstruction. Operative complications occurred in 24% of the patients. The 30-day survival was 92%. There were five (10%) late deaths. Survival after a median of 8 years postoperatively was 82%. The early and late results of cardiovascular surgery in the Marfan syndrome thus are concluded to be generally favourable. As late reoperation frequently is needed, however, close monitoring is advocated even after successful primary surgery.

Perioperative Aortic Dissection: Early Recognition Improves Outcome

“…Interestingly, there were no patients with an enlarged ascending aorta at the initial operation, the largest diameter being 4.3 cm. In the literature, other predisposing factors have been observed, such as thin aorta, cystic medial necrosis or connective tissue disease [13,14,17,20,21], but iatrogenic injury still appears to be the main trigger. In off-pump coronary bypass surgery, all manipulations of the ascending aorta are performed with the heart beating.…”

Perioperative Aortic Dissection: Early Recognition Improves Outcome

“…Interestingly, there were no patients with an enlarged ascending aorta at the initial operation, the largest diameter being 4.3 cm. In the literature, other predisposing factors have been observed, such as thin aorta, cystic medial necrosis or connective tissue disease [13,14,17,20,21], but iatrogenic injury still appears to be the main trigger. In off-pump coronary bypass surgery, all manipulations of the ascending aorta are performed with the heart beating.…”

Das Marfan-Syndrom: Therapie bei kardiovaskulären Manifestationen

“…1). Die elektive Implantation einer klappentragenden Aortenprothese in klassischer Bentall-Technik (5) ist unterhalb eines Durchmessers von 50 mm aufgrund einer fehlenden natürlichen Mobilisierung der Koronararterien durch die dilatierten Sinus und ihrer daher schwierigen Anastomosierung an die Aortenprothese problematisch und wird daher überwiegend abgelehnt (58,69).…”

“…Operation nach Bentall: Die häufigste Operationsindikation bei Marfan-Syndrom ist die Aortenwurzelektasie mit Verlust der Geometrie der Taschenklappen und dadurch gleichzeitig bestehender Aortenklappeninsuffizienz. Die Standardtherapie besteht derzeit aus dem kombinierten Ersatz von Aortenklappe und Aorta ascendens durch eine klappentragende Gefäßprothese (5,38,55,69,74,77); bei 54 % bis 95 % aller Eingriffe im Bereich der Aortenwurzel wird diese Technik eingesetzt (Tab. 2).…”