Diagnosis of pulmonary hypertension

Adaani Frost, David Badesch, J Simon R Gibbs, Deepa Gopalan, Dinesh Khanna, Alessandra Manes, Ronald Oudiz, Toru Satoh, Fernando Torres, Adam Torbicki, Adaani Frost, David Badesch, J Simon R Gibbs, Deepa Gopalan, Dinesh Khanna, Alessandra Manes, Ronald Oudiz, Toru Satoh, Fernando Torres, Adam Torbicki

Abstract

A revised diagnostic algorithm provides guidelines for the diagnosis of patients with suspected pulmonary hypertension, both prior to and following referral to expert centres, and includes recommendations for expedited referral of high-risk or complicated patients and patients with confounding comorbidities. New recommendations for screening high-risk groups are given, and current diagnostic tools and emerging diagnostic technologies are reviewed.

Conflict of interest statement

Conflict of interest: A. Frost reports personal fees and non-financial support (travel and lodging for attendance and participation in the 6th WSPH) from Actelion, Gilead, United Therapeutics and Bayer, honoraria for presentations from Gilead, and honoraria for participation in an end-point adjudication committee for an FDA-approved study from United Therapeutics, during the conduct of the study; and personal fees (honoraria and travel and lodging for presentations at meetings) from Actelion Pharmaceuticals, outside the submitted work. Conflict of interest: D. Badesch reports grants and personal fees (as steering committee member and site investigator) from Acceleron, Complexa, Bellerophon and Liquidia, grants, personal fees and advisory board work from Actelion, is a long-term stock holder of Johnson and Johnson, grants and personal fees (as advisory board member and site investigator) from Arena, Gilead and United Therapeutics/Lung LLC, personal fees for consultancy from Respira, grants and personal fees (as site investigator, advisory board member and consultant) from Bayer, outside the submitted work. Conflict of interest: J.S.R. Gibbs reports grants and personal fees from Actelion, GSK, MSD and Pfizer, personal fees from Arena, Bayer, Bellerophon, Complexa and Acceleron, and grants from United Therapeutics, during the conduct of the study. Conflict of interest: D. Gopalan has nothing to disclose. Conflict of interest: D. Khanna reports personal fees from Actelion, Bayer, Boehringer Ingelheim, Chemomab, Corbus, Covis, Cytori, EMD Sereno, Genentech/Roche, Gilead, GSK, Sanofi-Aventis and UCB Pharma; grants from Bayer, Boehringer Ingelheim, Genentech/Roche, Pfizer and Sanofi-Aventis; and has stock options with Eicos Sciences, Inc. He was also supported by the NIH/NIAMS (K24 AR063120). Conflict of interest: A. Manes reports grants and personal fees from Actelion, and grants from Bayer and Pfizer, outside the submitted work. Conflict of interest: R. Oudiz reports grants and consulting and speaker fees from Actelion, Gilead and United Therapeutics, grants from Aadi and GSK, consulting fees from Complexa, Acceleron and Medtronic, and grants and consulting fees from Arena and Reata, outside the submitted work. Conflict of interest: T. Satoh has nothing to disclose. Conflict of interest: F. Torres reports personal fees from Actelion, Bayer, Reata and Arena, and grants from Gilead, United Therapeutics, Medtronic, Eiger and Bellerophon, during the conduct of the study. Conflict of interest: A. Torbicki reports personal fees from Actelion, AOP Orphan Pharmaceutics, Bayer and MSD, and non-financial support from Pfizer, outside the submitted work; and is also a chairperson of the Foundation for Pulmonary Hypertension, which receives donations from outside parties to support its activities. The chair receives no financial compensation for this function.

Copyright ©ERS 2019.

Figures

FIGURE 1
FIGURE 1
Algorithm for the diagnosis of pulmonary hypertension (PH) and its causes: triage of urgent cases and diagnosis of common conditions (for more details, see the accompanying text). V/Q: ventilation/perfusion; CTEPH: chronic thromboembolic PH. #: described in the 2015 European Society of Cardiology/European Respiratory Society PH guidelines [24]; ¶: these include chronic thromboembolic disease without PH, which should be considered in patients with risk factors and/or previous venous thromboembolism; +: single photon emission computed tomography or planar V/Q scan is acceptable (interpretation is binary: normal or abnormal); §: see algorithms for left heart disease and lung disease/hypoxia-related PH [–3], which provide details of further management of these patients; ƒ: referral of a patient to be seen in person or for a teleconsultation.
FIGURE 2
FIGURE 2
Algorithm for the diagnosis of pulmonary hypertension (PH) and its causes: role of the PH expert centre. CTEPH: chronic thromboembolic PH; V/Q: ventilation/perfusion; RHC: right heart catheterisation. #: single photon emission computed tomography or planar V/Q scan is acceptable; ¶: these include patients with chronic thromboembolic disease without PH; +: the composition of the multidisciplinary team may differ depending on the type of clinical problem; §: according to clinical classification of PH; ƒ: only in expert centres and only with a reassessment plan in place.

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