Complete transposition of the great arteries: Surgical concepts for patients with systemic right ventricular failure following intraatrial repair

One of the most serious late complications of the intraatrial baffle proced ure (Mustard or Senning correction) in patients suffering from transpositio n of the great arteries, (TGA) is the late systemic right ventricular failu re. Nearly all patients presenting with right ventricular dysfunction have severe associated tricuspid regurgitation. The surgical options for these p atients include tricuspid valve reconstruction or replacement, staged conve rsion to the arterial switch operation and orthotopic heart transplantation . Review of 189 operative survivors who underwent the Mustard or Senning op eration for TGA between 1970 and 1993 in our institution revealed 12 patien ts (6.3 %) who died from severe systemic right ventricular dysfunction (mea n follow-up 16+/-3.5 years), which was the most common cause of late death. All of them had concomitant severe tricuspid regurgitation. 7 patients (3. 7 %) died from sudden cardiac death. The actuarial survival at 10 years is 93 % for simple TGA and 85 % for TGA associated with ventricular septum def ect or coarctation. At our institution, 4 adolescent or adult patients unde rwent tricuspid valve surgery; tricuspid valve replacement was performed in 2 patients and valve repair in 2 patients. In the mid-term follow-up, 2 of these patients died. Two additional patients underwent orthotopic heart tr ansplantation, and one died on the waiting list. Staged conversion from the Senning/Mustard atrial repair to the arterial switch operation was initial ly reported by Mee. The procedure for pulmonary artery banding starts with inducing left ventricular reconditioning with subsequent arterial switch. T he mortality of this two-staged procedure was as high as 20 % to 30 % in ou r early experience, and some of the candidates underwent heart transplantat ion. Tricuspid valve repair or replacement do not improve right ventricular function in patients with a failing right ventricle following the Mustard/ Senning operation. Staged conversion to arterial switch may improve right v entricular function by decreasing the work load of the right ventricle and provides anatomic repair with left ventricle-to-aorta continuity. Orthotopi c heart transplantation is the only alternative if the left ventricle does not respond to pulmonary artery banding.