Palliative repair of transposition of the great arteries with criss-cross heart: Ventricular septal defect and hypoplastic right (systemic) ventricle

Abstract

Four cases of palliative Mustard or Senning repair for transposition of the great arteries (TGA) with ventricular septal defect (VSD), hypoplastic right ventricle, and superior-inferior ventricular configuration are presented. The palliative Mustard procedure-a Mustard repair without VSD closure-is usually reserved for patients with pulmonary vascular obstructive disease (PVOD). In such cases, VSD closure would result in left ventricular failure. Each of our four patients had normal or only slightly elevated pulmonary resistance (1.0 to 5.2 Wood units). However, in each case a hypoplastic right ventricle precluded VSD closure. All four patients had transposition-like hemodynamics with unfavorable streaming to the great arteries, despite the fact that two patients had a levo arterial configuration. In each case, the ventricular relationship included a hypoplastic, superior right ventricle with a horizontal ventricular septum-the so-called "upstairs-downstairs" or "superior-inferior" heart. All patients had previous balloon atrial septostomy or open septectomy to improve atrial mixing. Two patients had previous pulmonary artery banding because of increased pulmonary flow. All four patients remain survivors of the palliative Mustard or Senning repair, which was performed at 10 months, 5 1/2, 12, and 16 years. In each case, there was a marked improvement of symptomatology with a decrease of hemoglobin (mean 21.1 gm/dl preoperatively to 15.3 gm/dl postoperatively) and an increase of arterial oxygen saturation (mean 78 vol % preoperatively to 93 vol % postoperatively). This is the first palliative Mustard or Senning repairs in patients with TGA, VSD, and hypoplastic right ventricle without PVOD. The procedure produces gratifying palliation for these patients.