State-of-the-Art Paper
Right Heart Adaptation to Pulmonary Arterial Hypertension: Physiology and Pathobiology

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Survival in patients with pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Although pulmonary load is an important determinant of RV systolic function in PAH, there remains a significant variability in RV adaptation to pulmonary hypertension. In this report, the authors discuss the emerging concepts of right heart pathobiology in PAH. More specifically, the discussion focuses on the following questions. 1) How is right heart failure syndrome best defined? 2) What are the underlying molecular mechanisms of the failing right ventricle in PAH? 3) How are RV contractility and function and their prognostic implications best assessed? 4) What is the role of targeted RV therapy? Throughout the report, the authors highlight differences between right and left heart failure and outline key areas of future investigation.

Key Words

echocardiography
heart failure
MRI
myocardium
pulmonary artery hypertension
right ventricle

Abbreviations and Acronyms

BNP
B type natriuretic peptide
CO
cardiac output
Ea
arterial elastance
Ees
ventricular elastance
LV
left ventricular
MHC
myosin heavy chain
MPAP
mean pulmonary arterial pressure
PAC
pulmonary arterial compliance
PAH
pulmonary arterial hypertension
PH
pulmonary hypertension
PVR
pulmonary vascular resistance
RAP
right atrialpressure
RHF
right heart failure
RNA
ribonucleic acid
RV
right ventricular
RVEF
right ventricular ejection fraction

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Dr. Vonk-Noordegraaf has received lecture fees from Actelion, Bayer, GlaxoSmithKline, United Therapeutics, Lilly, Pfizer, and Novartis; is on the industry advisory board from Actelion and Bayer; and serves on the steering committee for Actelion, Bayer, and Pfizer. Dr. Chin has received research grants from Actelion, Bayer, Gilead, GlaxoSmithKline, Novartis, United Therapeutics, GeNO, and the National Institutes of Health; and honoraria for service on advisory boards for Actelion, Bayer, and Gilead. Dr. Forfia is a consultant for Actelion and United Therapeutics. Dr. Lumens has received a research grant within the framework of the Dr. E. Dekker Program of the Dutch Heart Foundation (NHS-2012T010). Dr. Oudiz has received grants and research funding, consulting and speaking fees, and honoraria from Actelion, AIRES, Bayer, Gilead, Ikaria, Lung LLC, Pfizer, United Therapeutics. Dr. Provencher has received consulting fees from Actelion, GlaxoSmithKline, Pfizer, and Unither Biotech; research grants from Actelion, Bayer, and GlaxoSmithKline; and speaker fees from Actelion. Dr. Torbicki has received research grants and speaker’s honoraria from Actelion, Bayer, AOP, United Therapeutics, and GlaxoSmithKline; and has served on advisory boards and scientific committees for Actelion, Bayer, AOP, and GlaxoSmithKline. Dr. Voelkel has received grant support for pre-clinical studies from Actelion and United Therapeutics. Dr. Hassoun has participated on advisory boards for Gilead, Merck, Bayer, and Novartis; has received research funding from United Therapeutics for the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management; and his research was supported by grants P50 HL084946 and R01 HL114910 from the National Heart, Lung, and Blood Institute. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Drs. Vonk-Noordegraaf and Haddad contributed equally to this manuscript.