ABSTRACT
Patients with tetralogy of Fallot with pulmonary atresia may fall anywhere within an extremely wide spectrum of severity ranging from simple valvar atresia to complete absence of the true pulmonary arteries. In the past, this anomaly was thought of as being part of a spectrum with tetralogy with pulmonary stenosis and would have been described in the same chapter. Although there is no question that the two entities share a common embryological background and are part of a spectrum anatomically, nevertheless the management of these patients and the results of management are so different that tetralogy with pulmonary atresia will be considered here as a separate chapter. Many centers prefer the terminology “pulmonary atresia with VSD” to recognize the differences between the two anomalies. There is probably no entity which better illustrates the collaborative role that can be played by the interventional cardiologist and the congenital cardiac surgeon than tetralogy with pulmonary atresia. The child at the severe end of the spectrum with hypoplastic true pulmonary arteries and multiple aortopulmonary collaterals requires a carefully coordinated team approach in order to achieve optimal outcome. Even with optimal management, these children require so many interventions that there is a significant impact on their quality of life. Indeed many would consider that the quality of life for children at the severe end of the spectrum including ultimate exercise capacity may be more limited than for children with hypoplastic left heart syndrome and heterotaxy. Certainly the total financial cost of treatment of these children is at the very far end of the entire spectrum of congenital heart disease.
