The pulmonary circulation in transposition of the great arteries
In the days before a Rashkind septostomy the average life-expectancy was 1.3 months in babies with transposition of the great arteries (TGA) and an intact ventricular septum (IVS), and 3.4 months in those with a TGA and ventricular septal defect (VSD) . Those with a VSD survived longer because mixing of the pulmonary and systemic circulations persisted after closure of the foramen ovale and ductus arteriosus. A high effective pulmonary blood flow ensures a greater systemic arterial oxygen saturation. The presence of a large VSD however, leads to the rapid development of severe pulmonary vascular disease [6, 17]. In the presence of an intact ventricular septum a patent ductus arteriosus also leads to the early development of pulmonary vascular disease and should be closed surgically at 3 months of age if it has not already closed spontaneously. In transposition as a whole, patient outcome correlates with the pulmonary arterial pressure . The outlook for patients with an IVS was transformed by the introduction of the Senning operation in 1959 and the Mustard repair in 1964, but not until the revival of the arterial switch procedure in 1976 did the long-term outlook improve for those with a VSD [16,18,20,21,22]. This procedure enables an early repair to be carried out with an acceptable risk, before severe pulmonary vascular disease has developed.
KeywordsVentricular Septal Defect Ventricular Septal Defect Pulmonary Vascular Disease Intimal Proliferation Intact Ventricular Septum
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