Observations on changing hemodynamics in tricuspid atresia without associated transposition of the great vessels

Three patients are discussed who, in early infancy, presented with mild cyanosis, cardiac decompensation, failure to thrive, and pulmonary congestion. These manifestations were associated with tricuspid atresia without transposition of the great vessels. The clinical course of these children, who in varying periods of time became progressively more cyanotic, with hearts becoming smaller, and with decreasing pulmonary blood flow, is unique. Cardiac catheterization and angiocardiographic studies were obtained at each end of the clinical spectrum in all of the children. Although a closing ventricular septal defect is thought to be the cause of the decreasing pulmonary flow, the studies were not conclusive. In all three patients, palliative systemic venous-to-pulmonary arterial anastomosis (Glenn procedure) relieved the symptoms of hypoxia.