General management of pulmonary arterial hypertension associated with adult congenital heart disease

Over the past 15 years there have been significant improvements in the treatment of pulmonary arterial hypertension due to congenital heart disease. Patients now live for several decades, but morbidity and mortality remain high. This article describes the holistic management of this patient group with an emphasis on both the physical and psychosocial aspects of care, taking into account the consequences of chronic cyanosis, avoiding...