Impact of Severe Uncorrected Pectus Excavatum on Outcomes After Aortic Surgery in Marfan Syndrome

The prognostic impact of uncorrected severe pectus excavatum (PE) in patients with Marfan syndrome undergoing aortic surgery remains unclear. This study evaluated whether severe PE influences clinical outcomes after aortic surgery.

We retrospectively analyzed 253 patients with Marfan syndrome who underwent aortic surgery between 2005 and 2020. Patients were stratified into a nonsevere PE group (n = 191; including no PE and nonsevere PE) and a severe PE group (n = 62; Haller index >3.5). The primary end point was 10-year mortality. Secondary outcomes included in-hospital morbidity and long-term event-free survival. Multivariable Cox regression and propensity score matching were performed to adjust for confounding variables.

Patients with severe PE were younger and demonstrated lower pulmonary function and smaller left ventricular dimensions preoperatively. Operative characteristics were similar between groups. Early mortality did not occur in either group. Prolonged mechanical ventilation was more frequent in the severe PE group (8.0% vs 1.0%; P = .013), although this association was attenuated after adjustment. At 10 years, overall survival (98.3% vs 96.8%; P = .69) and event-free survival (82.0% vs 77.2%; P = .66) were comparable. Rates of heart failure readmission, respiratory readmission, major bleeding, stroke, and cardiovascular reoperation did not differ significantly between groups. Long-term echocardiographic follow-up revealed no significant intergroup differences in right ventricular systolic pressure or left ventricular end-diastolic diameter.

Severe uncorrected PE does not adversely affect long-term survival or cardiopulmonary outcomes in patients with Marfan syndrome undergoing aortic surgery. Careful perioperative respiratory management is warranted because of an increased risk of prolonged ventilation.