Mid-term outcomes of cardiovascular surgery for patients with Marfan syndrome

• Published in: General thoracic and cardiovascular surgery 2015-05, Vol.63 (5), p.267-272
• Main Authors: Tochii, Masato, Hattori, Koji, Ishikawa, Hiroshi, Ishida, Michiko, Higuchi, Yoshiro, Amano, Kentaro, Sakurai, Yusuke, Noda, Mika, Watanabe, Takashi, Takagi, Yasushi
• Format: Article
• Language: English
• Subjects: Abdomen; Adult; Aneurysm, Dissecting - complications; Aneurysm, Dissecting - mortality; Aneurysm, Dissecting - surgery; Aortic Aneurysm - complications; Aortic Aneurysm - mortality; Aortic Aneurysm - surgery; Aortic aneurysms; Aortic dissection; Aortic Valve Insufficiency - complications; Aortic Valve Insufficiency - mortality; Aortic Valve Insufficiency - surgery; Cardiac Surgery; Cardiology; Coronary vessels; Doppler effect; Emergency Treatment - mortality; Emergency Treatment - statistics & numerical data; Female; Females; Heart surgery; Hematoma; Humans; Male; Marfan syndrome; Marfan Syndrome - complications; Medicine; Medicine & Public Health; Middle Aged; Original Article; Ostomy; Outpatient care facilities; Patients; Reoperation - mortality; Reoperation - statistics & numerical data; Retrospective Studies; Statistical analysis; Surgical Oncology; Survival Rate; Thoracic Surgery; Tomography; Treatment Outcome; Veins & arteries; Young Adult
• ISSN: 1863-6705; 1863-6713
• DOI: 10.1007/s11748-014-0507-6

Objectives Cardiovascular manifestations determine the prognosis and survival of patients with Marfan syndrome (MFS). We assessed the early and mid-term outcomes of cardiovascular surgery for this patient population. Patients and methods We conducted a retrospective evaluation of patients with MFS who underwent surgery in our department. The endpoint was the requirement for a second cardiovascular surgery, and death from any cause. Results From February 2002 to March 2013, we performed 24 surgeries on 13 patients with MFS. At the time of initial surgery, the patients’ aged ranged from 19 to 64 years of age (mean 34.7 ± 11.3 years) and included 5 men (38.5 %). Five of 13 initial surgeries (38.5 %) were emergencies. Seven patients (53.8 %) presented with aortic dissection at initial surgery, and one of the remaining six patients suffered from new aortic dissection during follow-up. Eight patients (61.5 %) underwent a second surgery, and a second surgery was not required for 100, 75.0, and 53.6 % of patients at 1, 3, and 5 years, respectively. The mean time interval between the first and the second surgeries was 52.8 ± 28.2 months. No patient died while hospitalized, and three subsequently died from unknown causes. Survival rates were 100, 90.9 and 64.9 % at 3, 5, and 7 years, respectively. Conclusion Although we obtained satisfactory early outcomes for patients with MFS, there were three late deaths from unknown causes. Therefore, we recommend that patients with MFS should remain under close surveillance.