Total Anomalous Pulmonary Venous Return: Correlation of Hemodynamic Observations and Surgical Mortality in 58 Cases

Abstract

One hundred twenty-five patients undergoing surgical correction of total anomalous pulmonary venous return were studied. The overall mortality was 37% and was related to age at the time operation was required. Mortality was 57% during the first year of life, 29% in patients between 13 and 24 months, and 15% in those between 2 and 10 years; no deaths occurred in those over 10 years. Mortality was highest in patients with infracardiac lesions (62%), and lowest in those with cardiac defects (30%). The major cause of death was pulmonary edema, and survival was closely related to the degree of increased pulmonary vascular resistance. Surgical treatment should be delayed until at least 6 months of age, but the development of congestive heart failure may necessitate earlier operation. mong cyanotic congenital cardiac defects that require operation during the first year of life, total anomalous pulmonary venous return A (TAPVR) ranks fourth in frequency at the Texas Heart Institute, following transposition of the great vessels, tetralogy of Fallot, and tricuspid atresia. The anomaly continues to represent a challenging problem to both the cardiologist and the cardiovascular surgeon. Despite recent advances in treatment, this severe malformation in its various anatomical forms has a high surgical mortality during early infancy. Because of the high natural mortality in the untreated infant, and the surgical risk in the first year of life, timing of the operation remains important for optimal results. The clinical findings in patients with TAPVR have been well described [7,9, 10, 12, 17, 19,20,23,24], with the major hemodynamic abnormalities consisting of volume overload of the right ventricle, leading to cardiac failure, and severe arterial desaturation. Muller [21] in 1950 first undertook a palliative procedure by performing a closed side-to-side anastomosis between the common pulmonary venous trunk and the left atrium. The first successful correction of TAPVR, however, was reported from this surgical unit in 1957 [6]. Prior and subsequent to that time, various techniques for repair have been described [ l , 3, 1416,25,26]. In 1.962 From the Division of Surgery of the Texas Heart Institute, St. Luke’s Episcopal and Texas Presented at the Twenty-first Annual Meeting of the Southern Thoracic Surgical Association, Address reprint requests to Dr. Wukasch, Texas Heart Institute, P.O. Box 20345, Houston, Children’s Hospitals, Houston, Tex. Williamsburg, Va., Nov. 7-9, 1974.