Emergent Pectus Excavatum Repair After Aortic Root Replacement in Marfan Patient

Abstract

Chest wall deformities, including pectus excavatum, can complicate cardiac operations by impeding sternal entry and cardiac exposure and by affecting cardiac hemodynamic performance. We describe a patient with Marfan syndrome who underwent elective replacement of an aortic root aneurysm. The patient required a simultaneous, unplanned, emergent repair of her severe pectus excavatum because of hemodynamic instability after sternal closure. The success of this procedure suggests that emergent single-stage repair of pectus excavatum should be considered when median sternotomy closure precipitates hemodynamic instability in such patients.

Aortic surgery in Marfan patients with severe pectus excavatum

“…A separate aortic valve and supracoronary graft replacement and aortic root replacement with xenograft were reported in one case respectively. 1,22 Emergency Type-A AAD repair was reported in five cases, 2,3,12,15,17 two of which left an uncorrected pectus excavatum. Isolated median sternotomy was the most commonly employed access (21 of 39 procedures), followed by transverse sternotomy (3 of 39 procedures), left anterior thoracotomy (3 of 39 procedures) and partial sternotomy (2 of 39 procedures).…”

“…Cardiopulmonary bypass time (mean 149 AE 35 min) and aortic cross clamp time (mean 112 AE 39 min) were reported for six patients. 15,17,22,27 The mean total operative time was 266 AE 118 min in five patients. 7,13,17,18 Complications after a staged or a combined approach were uncommon (a case of severe deep sternal wound infection and bleeding, and two cases of hemodynamic impairment after sternal closure without correction of pectus excavatum).…”

“…The same authors advocate the combined pectus excavatum and aortic root repair through sternotomy as the preferable surgical strategy in patients who have not had prior cardiac surgery. 11,22 Limitations: The small sample of patients and the heterogeneity of the treatments are the major limitations of this study. Data were sometimes poorly and incompletely reported and the study period is covering more than 60 years.…”

Concomitant Tirone David and Ravitch Procedure in Marfan Syndrome: A Case Report

“…Most authors propose the single-time approach as the best, as it allows an early cosmetic result and satisfaction. Besides, at time of sternal closure, a non-repaired pectus may compress the right ventricle with hemodynamic deterioration immediately after surgery [4]. The simultaneous correction allows a better cardiac and pulmonary function after surgery and allows a better surgical exposition, as well [5].…”

Manifestations respiratoires du syndrome de Marfan

“…L'indication chirurgicale dépend du retentissement sur la tolérance à l'exercice mais surtout de la tolérance psychologique de la déformation. L'indication d'une chirurgie cardiaque pour remplacement valvulaire ou aortique doit également être prise en compte, car quelques observations de suites opératoires compliquées après chirurgie aortique en cas de déformation importante de la cage thoracique ont été rapportées [19]. Les résultats de la chirurgie de réparation sont identiques, que le pectus excavatum soit isolé ou s'intègre dans une maladie du tissu conjonctif [20][21][22].…”