Pulmonary arterial hypertension related to congenital heart disease

InhaltsverzeichnisTopic Lead author Foreword Beghetti Corresponding authors Abbreviations and glossary Section 1: Pathophysiology, epidemiology and classification PAH-CHD: classification Galiè Current understanding and unanswered questions (epidemiology, natural history, clinical presentation of PAH-CHD) Beghetti Pathophysiology of PAH associated with CHD (shear stress, angiopoeitin, elastase, genetics…) Naeije Endothelial dysfunction in PAH associated with CHD (Endothelin, NO, PGI2) Fineman The special setting of cavopulmonary anastomosis Atz Pathophysiology of postoperative pulmonary hypertension Adatia Section 2: Clinical management of patients with PAH-CHD PAH assessment in pediatric cardiology (Echo, Cath, MRI etc…) Ivy Challenges & therapeutic strategies in the clinical management of PAH associated with CHD in adults Gatzoulis Specific aspects in the management of paediatric PAH-CHD patients Haworth Treatment options in paediatric patients with PAH Barst Treatment of postoperative pulmonary hypertension Wessel Future directions in the clinical research and treatment of PAH-CHD (including a discussion on clinical endpoints for assessing PAH in CHD patients) Rubin