Single-stage open repair of severe asymmetric pectus excavatum and mitral valve replacement in connective tissue disease

Abstract

We present a 14-year-old boy with Loey-Dietz syndrome with severe mitral regurgitation, pectus excavatum and scoliosis. The Haller index was 25. The heart was displaced into the left hemithorax. The right inferior pulmonary vein was very close to the sternum and vertebral body. Single-stage surgery was performed. An osseo-myo-cutaneous pedicled flap was created by sterno-manubrial junction dislocation and rib resection with bilateral internal mammary arteries supplying the flap. Cardiopulmonary bypass and mitral valve replacement was performed. The defect was bridged with three straight plates. The flap was laid on top and anchored. Early outcome at three months was good.