Left ventricular reconditioning and anatomical correction for systemic right ventricular dysfunction.

The morphologically right ventricle (mRV) fails after managing systemic workload for a variable period of time in some patients with transposition of the great arteries (TGA) who have undergone an atrial switch operation and in patients with congenitally corrected transposition of the great arteries (cc-TGA). Conventional therapy for progressive mRV failure, including tricuspid valve replacement and cardiac transplantation, has been disappointing. Anatomical correction, reincorporating the morphologically left ventricle (mLV) into systemic circulation, was performed in a total of 84 patients (39 TGA, 45 cc-TGA) in 2 institutions (Royal Children Hospital 1981-1993; Cleveland Clinic Foundation 1993-1999). The mVL was retrained to generate systemic pressure by means of pulmonary artery band in 43 patients. The overall mortality for patients entering this program is 15.4% (8 early and 5 late operative deaths). All of the operative deaths were patients with TGA and a prior atrial switch operation. Four patients (5%) have been transplanted or are on a transplantation waiting list. In patients with TGA and previous atrial switch operation, older age and abnormal coronary anatomy was associated with a higher operative mortality. None of the measured parameters of the mLV and mRV were predictors of failure of mLV reconditioning. Follow-up echocardiographic evaluations of the 46 survivors of anatomical correction showed normal mRV function in 41 (89%) and normal mLV function in 42 patients (91%). mLV retraining and anatomical correction produces good results in prepubescent patients. The response of older patients is less predictable and associated with a higher early and late mortality. Copyright 2000 by W.B. Saunders Company