Consecutive surgical sequelae in children and adolescents with Marfan syndrome after primary cardiovascular surgical interventions

Abstract

OBJECTIVES: This study focuses on the consecutive sequelae of children and adolescents with Marfan syndrome after primary cardiovascular surgical interventions and the long-term outcomes. METHODS: A retrospective review of children with Marfan syndrome who underwent primary surgical intervention in childhood (<18 years) and sequential cardiovascular operations between July 1986 and September 2014 was undertaken. RESULTS: Thirty-six children (mean age 12.38 ± 4.4, range 0.62-17.14 years, at the first operation; 12 females) underwent a total of 97 sequential cardiovascular operations. Primary pathologies were annuloaortic ectasia, aortic root and ascending aorta aneurysm, aortic valve insufficiency, floppy mitral valve and midaortic syndrome. Initial operations were emergency in 2 and urgent and elective in 10 and 24 patients, respectively. Associated skeletal abnormalities were seen in 8. All underwent a second operation; 17 underwent a third operation; 6 had a fourth; and 2 had a fifth. The survival rate of children <13 years of age at the first operation compared to those who were older and male, was not statistically significant (P = 0.244). The cumulative survival rate based on age at first operation was 63.24 ± 0.16% at a mean follow-up period of 13.9 ± 6.8 (median 11.8, range 2-32.6) years. CONCLUSIONS: Patients with Marfan syndrome undergoing surgery during childhood needed sequential reinterventions. However, even with multiple consecutive surgeries, long-term outcome is deemed satisfactory.