[Pulmonary arterial hypertension in congenital heart defects with shunt: a heterogeneous and complex constellation].

It will be explained how pulmonary vascular disease can develop in congenital heart defects with primary left-to-right shunt across the different ages and stages of development, with and without surgical, interventional or medical treatment. Furthermore it will be discussed, in which specific stages of pulmonary vascular disease "advanced therapy" can be used.