title: Pulmonary hypertension related to congenital heart disease: a call for action.
abstract: Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) is a common type of pulmonary arterial hypertension (PAH). Despite this, little emphasis has been given to this group of patients until recently, when compared with idiopathic PAH. This is largely because of the complexity and the wide range of underlying cardiac anatomy and physiology, with a multitude of adaptive mechanisms not fully understood. Pulmonary arterial hypertension related to congenital heart disease is, therefore, best diagnosed and managed in centres specializing in both CHD and PAH, to avoid common pitfalls and old practices and to provide state-of-the-art care. We discuss the optimal management of PAH-CHD patients in a series of actions to be taken in order to optimize short- and long-term outcome, based on current knowledge of the condition and the advent of targeted advanced therapies.
authors.affiliation: Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, NIHR Cardiovascular BRU, Royal Brompton Hospital and the National Heart & Lung Institute, Imperial College, Sydney Street, London SW3 6NP, UK.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.forename: Stephen John
authors.initials: SJ
authors.lastname: Wort
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
mesh_terms: D000328:Adult; D035843:Biomedical Research / Q000458:organization & administration; D002309:Cardiology / Q000193:education; D003906:Developing Countries; D003937:Diagnosis, Differential; D042241:Early Diagnosis; D004502:Education, Medical, Continuing / Q000379:methods; D004541:Eisenmenger Complex / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D065627:Familial Primary Pulmonary Hypertension; D005838:Genotype; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D007391:International Cooperation; D007400:Interprofessional Relations; D018810:Magnetic Resonance Angiography; D011379:Prognosis; D011788:Quality of Life
keywords: Advanced therapies; Congenital heart disease; Eisenmenger syndrome; Pulmonary hypertension; Pulmonary vascular disease; Targeted therapies; Treat and repair