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Review
. 2019 Jan 24;53(1):1801897.
doi: 10.1183/13993003.01897-2018. Print 2019 Jan.

Pulmonary hypertension due to left heart disease

Jean-Luc Vachiéry  1 , Ryan J Tedford  2 , Stephan Rosenkranz  3 , Massimiliano Palazzini  4 , Irene Lang  5 , Marco Guazzi  6 , Gerry Coghlan  7 , Irina Chazova  8 , Teresa De Marco  9
Affiliations
Review

Pulmonary hypertension due to left heart disease

Jean-Luc Vachiéry et al. Eur Respir J. .

Abstract

Pulmonary hypertension (PH) is frequent in left heart disease (LHD), as a consequence of the underlying condition. Significant advances have occurred over the past 5 years since the 5th World Symposium on Pulmonary Hypertension in 2013, leading to a better understanding of PH-LHD, challenges and gaps in evidence. PH in heart failure with preserved ejection fraction represents the most complex situation, as it may be misdiagnosed with group 1 PH. Based on the latest evidence, we propose a new haemodynamic definition for PH due to LHD and a three-step pragmatic approach to differential diagnosis. This includes the identification of a specific "left heart" phenotype and a non-invasive probability of PH-LHD. Invasive confirmation of PH-LHD is based on the accurate measurement of pulmonary arterial wedge pressure and, in patients with high probability, provocative testing to clarify the diagnosis. Finally, recent clinical trials did not demonstrate a benefit in treating PH due to LHD with pulmonary arterial hypertension-approved therapies.

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Conflict of interest statement

Conflict of interest: J-L. Vachiéry reports consultancy and speaker fees paid to institution, and is an investigator in clinical trials for Actelion Pharmaceuticals and Bayer, consultancy fees paid to institution from Novartis, and consultancy fees paid to institution, and is an investigator in clinical trials for Sonivie and Pfizer, during the conduct of the study; consultancy fees paid to institution, and is an investigator in clinical trials for Arena Pharmaceuticals, Bial Portela and Sonivie, consultancy and speaker fees paid to institution, and is an investigator in clinical trials for GSK and Pfizer, consultancy fees and travel grants paid to institution from MSD, and is an investigator in clinical trials for Reata, outside the submitted work. Conflict of interest: R.J. Tedford reports personal fees (Hemodynamic Core Lab) from Actelion, J&J and Merck, and personal fees for steering committee membership from Abbott, outside the submitted work. Conflict of interest: S. Rosenkranz reports personal fees for lectures and/or consultancy from Abbott, Actelion, Arena, Bayer, BMS, MSD, Novartis, Pfizer and United Therapeutics, and institutional research grants from Actelion, Bayer, Novartis, Pfizer and United Therapeutics, outside the submitted work; and serves as chair of the Working Group “Pulmonary circulation and right ventricular function” of the European Society of Cardiology. Conflict of interest: M. Palazzini has nothing to disclose. Conflict of interest: I. Lang reports grants and personal fees from Actelion and AOP Orphan Pharma, and personal fees from Sanofi and Novartis, outside the submitted work. Conflict of interest: M. Guazzi has nothing to disclose. Conflict of interest: G. Coghlan has nothing to disclose. Conflict of interest: I. Chazova has nothing to disclose. Conflict of interest: T. De Marco reports grants from Actelion Pharmaceuticals, Pfizer, United Therapeutics, Gilead, Boston Scientific, Bellerophon, Respirex, Arena Pharmaceutical and Novartis, outside the submitted work.

Figures

FIGURE 1
FIGURE 1
Haemodynamic assessment of pulmonary hypertension (PH) due to heart failure with preserved ejection fraction (HFpEF). RV: right ventricular; RHC: right heart catheterisation; LHD: left heart disease; PAWP: pulmonary arterial wedge pressure; LVEDP: left ventricular end-diastolic pressure; CTEPH: chronic thromboembolic PH. a) Pre-test probability of PH-LHD is based on the features presented in table 1. RHC is recommended in intermediate probability when risk factors of pulmonary arterial hypertension/CTEPH are present and/or if there is evidence of right ventricle abnormality. If the probability is high, patients should be managed according to recommendations for LHD. b) For the assessment of PH, RHC should be performed at expert centres. In patients with intermediate/high probability (table 1) and PAWP between 13 and 15 mmHg, PH-HFpEF is not excluded; provocative testing (tables 2 and 3) should be considered. #: for patients with systemic sclerosis, risk factors for CTEPH and/or unexplained dyspnea; : after [2]; +: if PAWP >15 mmHg, LVEDP validation should be considered.
See this image and copyright information in PMC

Comment in

References

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