Surgical management of children and young adults with marfan syndrome and pectus excavatum

Significant chest wall deformities occur in two thirds of children with Marfan syndrome (MS). The symptoms, physical findings, and surgical outcome of 11 patients with MS and severe pectus excavatum who required operative repair were reviewed. The diagnosis of MS was made before the pectus repair in six patients, at the time of evaluation of pectus in two patients, and after the repair in three patients. Symptoms included dyspnea upon exertion, tachypnea, and chest pain. Physical findings included aortic root enlargement or valvular disease, mitral valve disease, ligamentous disease, congestive heart failure, and ocular disease. All patients had severe pectus deformities with a narrow anteroposterior diameter in the midline, as well as a broad chest defect that extended bilaterally to the midclavicular line. The heart was shifted into the left side of the chest in all patients. A Ravitch-type pectus repair with a stainless steel substernal strut was used in eight patients, with one patient suffering a late recurrence; in three patients no strut was used, and all three had recurrence. There were no postoperative complications. In the postoperative follow-up of seven patients, symptomatic improvement of cardiopulmonary performance was noted. Four of the patients required subsequent open heart surgery, including replacement of the aortic valve in one patient, and composite grafts of the ascending aorta in three patients. Postoperative cardiac arrest was the only major open heart complication. All four patients recovered and did well after surgery, showing significant cardiac and pulmonary functional improvement. Since a majority of patients with MS have pectus excavatum, it is important to evaluate the body habitus of children with pectus excavatum and screen for MS when indicated. In addition, substernal bar support is important in a successful repair due to the connective tissue disorder. Our experience indicates that repair of pectus excavatum in patients with MS and serious cardiac involvement can be accomplished safely. Indeed, the pectus repair may be obligatory before open heart surgery, in order to permit a median sternotomy and to allow space for standard cannulation of the displaced heart. The additional improvement in pulmonary function and chest wall dynamics greatly facilitates postoperative intensive care management of these complex patients.