Out Comes of Univentricular Repair for Complete Transposition of the Great Arteries or Double Outlet Right Ventricle with Left Ventricular Outflow Tract Obstruction

Abstract

Objective: We performed the Fontan operation to prevent postoperative left ventricular outflow tract obstruction (LVOTO) in patients with complete transposition of the great arteries (TGA) or TGA type double outlet right ventricle (DORV) with LVOTO. This study compared patient outcomes after Fontan operation and Rastelli operation.
Methods: Twenty-one patients with either TGA or DORV with LVOTO underwent the Fontan (n=16) or Rastelli (n=5) operations, respectively. Surgical outcomes and cardiac function were compared between the two patient populations.
Results: Maximum follow-up duration after definitive repair using the Fontan and Rastelli operation was 16 (median, 4) years and 19 (median, 15) years, respectively. Early death did not occur in either patient population, but one late death occurred after the Fontan operation. Reoperation was not required after the Fontan operation, but three patients required four reoperations after the Rastelli operation. Ratios of freedom from cardiac events were 94% and 40% at 15 years after the Fontan and Rastelli operations, respectively. Cardiac catheterization revealed higher left ventricular end-diastolic pressure and LVOTO in some patients at 1 year after the Rastelli operation. Cardiac function was good for all patients who survived for> 5 years after either surgery, but hANP and BNP values were abnormally high in three patients after the Rastelli operation. One patient required exercise limitation, and another patient required catheter ablation of atrial fibrillation after the Rastelli operation.
Conclusions: These mid-term results suggest that the Fontan operation is the method of choice for definitive repair in patients with TGA or DORV with LVOTO.