Early and Long-term Outcomes after Aortic Surgery in Patients with Marfan Syndrome

Ali El-Sayed Ahmad; Nestoras Papadopoulos; Anton Moritz; Andreas Zierer

Volume : VI, Issue : XI, November - 2017

Early and Long-term Outcomes after Aortic Surgery in Patients with Marfan Syndrome

Ali El Sayed Ahmad, Nestoras Papadopoulos, Anton Moritz, Andreas Zierer

Abstract :

Background:The aim of this study is to determine the early- and long-term results after aortic surgery in Marfan syndrome patients.

Methods:Between January 2000 and December 2015, 36 patients affected by Marfan syndrome underwent open surgical aortic and root repair. Isolated ascending replacement, hemiarch replacement and total arch replacement was performed in 5 patients (14%), 14 patients (39%) and 17 patients (47%), respectively. Elephant trunk technique and frozen elephant trunk technique was performed in 10 patients (28%) and 3 patients (8%), respectively. The surgery was performed urgently in 16 patients (44%) and electively in 20 patients (56%). Hundred twenty-seven imaging studies were analyzed for segmental aortic diameter at our institution. Clinical data were prospectively entered into our institutional database. Mean late follow up for late survival was 5 � 3 years and was 98 % complete.

Results:Thirty-day mortality was 6% (n=2). One patient died in the hospital. Intensive care unit stay was 3 � 4 days. One patient (3%) experienced permanent neurologic deficits and 4 patients (11%) transient neurologic deficits. 5 Patients (14%) underwent redo surgery. Late survival and freedom from re-operation/re-intervention were 89 � 2 % and 81 � 3% at 6 years.

Conclusions:Marfan syndrome patients seem to benefit the most from total arch replacement with elephant trunk. We recommend rigorously adjust the systolic blood pressure less than 120 mmHg and a postoperatively treatment with �-blockers and ACE inhibitors. The time of re-operation and the increase of the aortic size still unpredictably therefore a yearly follow-up lifelong is recommended.