Nazan Puluca1,2, Melchior Burri1,2, Julie Cleuziou1,2, Markus Krane1,2,3, Rüdiger Lange1,2,3. 1. Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany. 2. Department of Cardiovascular Surgery, Institute for Translational Cardiac Surgery (INSURE), German Heart Center Munich, Technische Universität München, Munich, Germany. 3. German Heart Center Munich-DZHK Partner Site Munich Heart Alliance, Munich, Germany.
Abstract
OBJECTIVES: Most patients (75%) with Marfan syndrome present with aortic root dilatation that may require surgical intervention. However, associated cardiovascular disorders are not limited to the aortic root. These patients frequently require consecutive operations on the remaining thoracic aorta or the heart valves. Our intent was to characterize the spectrum of such procedures. METHODS: Data from all patients with Marfan syndrome undergoing aortic root surgery at our centre between 1988 and 2016 were analysed retrospectively. RESULTS: Overall, 73 patients (26 women) were selected for the study. The median age at 1st operation was 30 years (3-68 years). Indications for aortic root surgery were aneurysm (78%) and dissection (22%). Initially, 33 Bentall procedures and 40 valve-sparing root replacement procedures were performed, with a 97% rate of follow-up completion. The median follow up was 8 years (0-28 years). Survival at 1, 10 and 15 years was 100%, 85% and 82%, respectively. During follow-up monitoring, 48 subsequent procedures were performed in 33 patients (aorta, 23; aortic valve, 11; mitral valve, 7 and combined procedures, 7). The 30-day mortality rate after subsequent procedures was 4.2%. Freedom from subsequent operation and death (combined end-point) after 5, 10 and 15 years was 70%, 53% and 34%, respectively. CONCLUSIONS: After the initial surgery, subsequent procedures required in the setting of Marfan syndrome most often involve the remaining native aorta, followed by the aortic and mitral valves. The continued need for additional operative procedures remains high, even decades after the initial surgeries are undertaken. Therefore, long-term patient monitoring at specialized centres is imperative.
OBJECTIVES: Most patients (75%) with Marfan syndrome present with aortic root dilatation that may require surgical intervention. However, associated cardiovascular disorders are not limited to the aortic root. These patients frequently require consecutive operations on the remaining thoracic aorta or the heart valves. Our intent was to characterize the spectrum of such procedures. METHODS: Data from all patients with Marfan syndrome undergoing aortic root surgery at our centre between 1988 and 2016 were analysed retrospectively. RESULTS: Overall, 73 patients (26 women) were selected for the study. The median age at 1st operation was 30 years (3-68 years). Indications for aortic root surgery were aneurysm (78%) and dissection (22%). Initially, 33 Bentall procedures and 40 valve-sparing root replacement procedures were performed, with a 97% rate of follow-up completion. The median follow up was 8 years (0-28 years). Survival at 1, 10 and 15 years was 100%, 85% and 82%, respectively. During follow-up monitoring, 48 subsequent procedures were performed in 33 patients (aorta, 23; aortic valve, 11; mitral valve, 7 and combined procedures, 7). The 30-day mortality rate after subsequent procedures was 4.2%. Freedom from subsequent operation and death (combined end-point) after 5, 10 and 15 years was 70%, 53% and 34%, respectively. CONCLUSIONS: After the initial surgery, subsequent procedures required in the setting of Marfan syndrome most often involve the remaining native aorta, followed by the aortic and mitral valves. The continued need for additional operative procedures remains high, even decades after the initial surgeries are undertaken. Therefore, long-term patient monitoring at specialized centres is imperative.