Pulmonary Atresia and Pulmonary Stenosis

Miroslav Elek
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Treatment and Prognosis
If there is a suspected „ductus dependent“ pulmonary circulation first step can be infusion of prostaglandine (PGE1) for keeping ductus arteriosus patent. Next step is surgical or transcatheter perforation of pulmonary valve, stent placement in ductus arteriosus or creation of modified Blalock Taussig shunt. If possible complete surgical correction is performed. What makes surgical correction difficult is existence of major aorto pulmonary collateral arteries (MAPCAs), also small and very hypertrophic right ventricle and distal pulmonary stenosis. Newborns with cyanosis and developing heart failure without palliative interventions have very high mortality rate.
Treatment for children with moderate and severe pulmonary stenosis is tansluminal balloon dilatation and stent placement, and if necessary and possible surgical reparation.
Authors report that a mild pulmonary stenosis pressure gradient in children above 1 year of age will not increase with time, and that there is even a possibility to decrease, while in other groups of patients with severe stenosis pressure gradient actually increases. The most important is adequate follow-up and well-timed clinical menagement of this patients, in the individual estemated time intervals based mostly on pressure gradient and clinical manifestatons.


References
Pulmonary Atresia and Pulmonary Stenosis Pulmonary Atresia and Pulmonary Stenosis 05/14/2016
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