' (VSD). This group of patients is seldom severely hypoxemic in infancy, in contrast to babies with an intact ventricular septum, but is at high risk to develop congestive heart failure, pulmonary arterial hypertension (PAH), and pulmonary vascular obstructive disease early in life.6"' Palliation with a pulmonary artery band carries a high mortality,'0 11 and until recently4 11 complete repair in infancy was not possible. In the past most of these patients who were not successfully banded died in congestive heart failure in infancy or early childhood. A small number, however, developed pulmonary vascular obstructive disease (PVO) and survived into later childhood and adolescence.This report presents the surgical experience at the Children's Hospital Medical Center, Boston, Massachusetts, in eight consecutive patients with either d-transposi- tion of the great arteries and ventricular septal defect,3 or double outlet right ventricle (DORV) with a subpulmonary ventricular septal defect and no pulmonary stenosis' (Taussig-Bing malformation or partial transposition).'2 All eight patients had PAH and PVO. Four patients had a patent ductus arteriosus (PDA), and two, coarctation of the aorta. The clinical, anatomic, and pathophysiologic features of these patients are described and the surgical management and results using the Mustard procedure are presented. Materials and MethodsFollowing Lindesmith's reports'3 " of successful palliative Mustard operation in patients with TGA, VSD, and PAH, 19 patients with marked cyanosis and exercise intolerance underwent detailed cardiac catheterization and angiographic studies in our institution to identify individuals suitable for the proposed surgical intervention. In some patients the cardiac anatomy was unknown or incompletely diagnosed; others were thought, on clinical grounds, to have transposition with a ventricular septal defect and pulmonary vascular obstruction. The major hemodynamic criteria sought were 1) markedly elevated pulmonary arterial pressure; 2) markedly elevated pulmonary arteriolar resistance; 3) pulmonary arterial oxygen saturation appreciably greater than ascending aortic saturation. The important anatomic prerequisites were 1) complete or partial transposition of the great arteries; 2) ventricular septal defect; and 3) two well-developed ventricular sinuses with two atrioventricular valves. Eight patients from the initial group of 19 fulfilled the criteria and have undergone surgical treatment (table 1). One 23-month-old patient (CHMC #83-71-12) with TGA, VSD, and PAH is excluded from this

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