CONGENITAL HEART DISEASE Changing Results and Concepts in the Surgical Treatment of Double-OutletRight Ventricle: Analysis of 137 Operationsin 126 Patients GIAN PICCOLI, MD, ALBERT D. PACIFICO, MD, JOHN W. KIRKLIN, MD, EUGENE H. BLACKSTONE, MD, JAMES K. KIRKLIN, MD, and LIONEL M. BARGERON, Jr., MD One hundred thirty-seven operations (98 repairs and 39 preliminary or palliative operations) were per- formed for double-outlet right ventricle (DOW) with concordant atrioventricular connection from January 1987 through July 1982, with 38 hospital deaths (28 % ). Sixteen ( 12 % of the total of 137 operations) of the nonrepair operations were palliative for un- correctable malformations. “Classic” DOW was palliated rather than re- paired only when there was severe left ventricular or mitral hypoplasia or both (n = 4). Incremental risk factors for hospital death after repair of thls subset for the entire period (n = 42) included young age at operation, type of repair and the era. In the current era (1978 through July 1982), 1 death (5%; 70% confidence limits 0.7 to 17%) occurred among 19 patients; the incremental risk for young age was evident only in patients <3 months old. Eleven deaths (39%) occurred among 28 patients who underwent repair of the Taussig-Bing type of DORV, wlth no decrease in mortality rate in the current era. Inclusion of a venous switch In the repair was the only incremental risk factor. Twenty-four patients with more-or-less normally positioned great arteries or with D-malposition but with noncommitted VSDs or complete atrioventrlcular canal defects under- went repair, with 7 hospital deaths; only 1 death occurred in the 11 operated upon in the current era. Four patients with DORV and L-malposition under- went repair, with no deaths. Three patients with superior-inferior ventricles and inlet VSD were considered uncorrectable and received successful palliative procedures. Thus, results of repair In the current era are good in all subsets, even in infants, except for the Taussig-Bing heart. For this condition, a repair that does not include a venous switch is recom- mended. (Am J Cardiol 1983;52:549-554) Many reports concerning the repair of double-outlet right ventricle (DORV) have failed to classify accurately the morphology of the malformation in the patients treated and the relation of this to the risks of the oper- ation. Little cognizance has been taken in the surgical literature of the fact that some kinds of DORV are not amenable to repair iand must be managed by other techniques. The results of repairs done by current techniques, including the use of cold cardioplegia, have not been separated from those of earlier periods. From the Departments of Surgery and Pediatrics, School of Medlcine and the Medical Center, The University of Alabama in Birmingham, and the Alabama Congenital Heart Disease Diagnosis and Treatment Center, Birmingham, Alabama. Manuscript received January 31, 1983; revised manuscript received May 12, 1983, accepted May 18, 1983. Address for reprints: John W. Kirklin, M.D., Department of Surgery, University Station, Birmingham, Alabama 35294. To define the current risks and residual problems of repair, we reviewed and analyzed our surgical experi- ence with DORV with concordant atrioventricular connection to provide this kind of information. Methods Definition: For this study, we define DORV as a condition in which both great arteries arise wholly or nearly so from the morphologic right ventricle. The study includes only patients with atrioventricular concordant connection. Patients with situs ambiguus were tabulated but excluded from the anal- ysis. A few special situations require comment: (1) Tetralogy of Fallot is a morphologic spectrum that includes a variable amount of dextroposition of the aorta. In some cases the aorta arises >50% from the right ventricle. In this study, the mal- formation was considered DORV only when the aorta arose almost entirely (290%) from the right, ventricle. (2) The 549