title: Pulmonary hypertension due to left heart diseases.
abstract: Pulmonary hypertension (PH), a common complication of left heart diseases (LHD), negatively impacts symptoms, exercise capacity, and outcome. Although the true prevalence of PH-LHD is unknown, a subset of patients might present significant PH that cannot be explained by a passive increase in left-sided filling pressures. The term "out-of-proportion" PH has been used to identify that population without a clear definition, which has been found less than ideal and created confusion. We propose a change in terminology and a new definition of PH due to LHD. We suggest to abandon "out-of-proportion" PH and to distinguish "isolated post-capillary PH" from "post-capillary PH with a pre-capillary component" on the basis of the pressure difference between diastolic pulmonary artery pressure and pulmonary artery wedge pressure. Although there is no validated treatment for PH-LHD, we provide insights into management and discuss completed and randomized trials in this condition. Finally, we provide recommendations for future clinical trials to establish safety and efficacy of novel compounds to target this area of unmet medical need.
authors.affiliation: Department of Cardiology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium. Electronic address: jvachier@ulb.ac.be.
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Vachiéry
authors.affiliation: Pulmonary Division, Lady Davis Carmel Medical Center, Faculty of Medicine, The Technion, Institute of Technology, Haifa, Israel.
authors.forename: Yochai
authors.initials: Y
authors.lastname: Adir
authors.affiliation: Hospital Clínic-IDIBAPS, University of Barcelona, Network Center of Biomedical Research on Respiratory Diseases (CIBERES), Barcelona, Spain.
authors.forename: Joan Albert
authors.initials: JA
authors.lastname: Barberà
authors.affiliation: University of Pittsburgh Medical Center Montefiore Hospital, Pittsburgh, Pennsylvania.
authors.forename: Hunter
authors.initials: H
authors.lastname: Champion
authors.affiliation: Department of Cardiology Royal Free Hospital, London, United Kingdom.
authors.forename: John Gerard
authors.initials: JG
authors.lastname: Coghlan
authors.affiliation: Hospices Civils de Lyon, Louis Pradel Hospital, Department of Respiratory Diseases, National Reference Center for Rare Pulmonary Diseases, Regional Competence Center for Severe Pulmonary Arterial Hypertension; Claude Bernard University Lyon 1, Lyon, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.affiliation: University of California, San Francisco, San Francisco, California.
authors.forename: Teresa
authors.initials: T
authors.lastname: De Marco
authors.affiliation: Department of Experimental, Diagnostic and Specialty Medicine-DIMES, Bologna University Hospital, Bologna, Italy.
authors.forename: Nazzareno
authors.initials: N
authors.lastname: Galiè
authors.affiliation: Divisione di Cardiologia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Ghio
authors.affiliation: National Heart and Lung Institute, Imperial College London and Department of Cardiology, National Pulmonary Hypertension Service, Hammersmith Hospital, London, United Kingdom.
authors.forename: J Simon R
authors.initials: JS
authors.lastname: Gibbs
authors.affiliation: Division of Pulmonary and Critical Care, University of Michigan Medical Center, Ann Arbor, Michigan.
authors.forename: Fernando
authors.initials: F
authors.lastname: Martinez
authors.affiliation: MGH Heart Failure and Cardiac Transplantation Center, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Marc
authors.initials: M
authors.lastname: Semigran
authors.affiliation: Service de Pneumologie, Hôpital Universitaire de Bicêtre, APHP, Université Paris-Sud, Laboratoire d'excellence en recherche sur le médicament et innovation thérapeutique, and INSERM, Le Kremlin Bicêtre, France.
authors.forename: Gerald
authors.initials: G
authors.lastname: Simonneau
authors.affiliation: Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom.
authors.forename: Athol
authors.initials: A
authors.lastname: Wells
authors.affiliation: University of Giessen and Marburg Lung Center (UGMLC), member of the German Center for Lung Research (DZL), Max-Planck Institute for Heart and Lung Research, Giessen/Bad Nauheim, Germany.
authors.forename: Werner
authors.initials: W
authors.lastname: Seeger
mesh_terms: D000818:Animals; D002986:Clinical Trials as Topic / Q000379:methods; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D011669:Pulmonary Wedge Pressure / Q000502:physiology; D014655:Vascular Resistance / Q000502:physiology; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy
keywords: CO; Cpc-PH; DPD; EF; HF; HF-pEF; LHD; LV; NO; PAH; PAWP; PCWP; PDE5; PH; PVD; PVR; RCT; RHC; RV; SV; TPG; VO(2); cardiac output; clinical trials; combined post-capillary and pre-capillary PH; diastolic pressure difference; ejection fraction; heart failure; heart failure with preserved ejection fraction; left heart disease; left ventricle/ventricular; mPAP; mean pulmonary artery pressure; nitric oxide; oxygen consumption; phosphodiesterase type 5; pulmonary arterial hypertension; pulmonary artery wedge pressure; pulmonary capillary wedge pressure; pulmonary hypertension; pulmonary vascular disease; pulmonary vascular resistance; randomized controlled trial; right heart catheterization; right ventricle; sGC; soluble guanylate cyclase; stroke volume; transpulmonary gradient