T. Carrel et Jp. Pfammatter, Complete transposition of the great arteries: Surgical concepts for patients with systemic right ventricular failure following intraatrial repair, THOR CARD S, 48(4), 2000, pp. 224-227
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.