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Review
. 2024 Dec 10;14(4):e70005.
doi: 10.1002/pul2.70005. eCollection 2024 Oct.

Screening and diagnosis of pulmonary hypertension associated with chronic lung disease (PH-CLD): A consensus statement from the pulmonary vascular research institute's innovative drug development initiative-group 3 pulmonary hypertension

P Vitulo  1 , L Piccari  2 , S J Wort  3 , O A Shlobin  4 , G Kovacs  5 , C D Vizza  6 , P M Hassoun  7 , H Olschewski  8 , R E Girgis  9 , S M Nikkho  10 , S D Nathan  4
Affiliations
Review

Screening and diagnosis of pulmonary hypertension associated with chronic lung disease (PH-CLD): A consensus statement from the pulmonary vascular research institute's innovative drug development initiative-group 3 pulmonary hypertension

P Vitulo et al. Pulm Circ. .

Abstract

Pulmonary hypertension (PH) is a frequent complication of chronic lung disease (CLD). However, PH is difficult to diagnose early since accompanying symptoms overlap and are similar to those of the underlying CLD. In most cases the PH is mild to moderate and therefore physical signs may be absent or subtle. This consensus paper provides insight into the clues that might suggest the presence of occult PH in patients with CLD. An overview of current diagnostic tools and emerging diagnostic technologies is provided as well as guidance for the work-up and diagnosis of PH in patients with CLD.

Keywords: chronic lung disease; diagnosis; imaging; pulmonary hypertension; screening.

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Conflict of interest statement

Dr. Vitulo has consulted for MSD and AOP and has received support for attending congresses from MSD, Janssen all of which not related to this manuscript. Dr. Piccari has received research funding from and served as a speaker for Janssen and Ferrer, advised Janssen, Ferrer and United Therapeutics as well as received support for attending congresses from Janssen, MSD and Ferrer, all of which not related to this manuscript. Dr. S. John Wort received honoraria from Janssen, MSD, Bayer, Ferrer and Acceleron for advisory boards, received honoraria from Janssen for educational activity, received unrestricted research grants from Janssen, Ferrer and Bayer, and travel grants, conference registration and accommodation from Janssen and Ferrer. Dr. Shlobin has consulted for UT, Bayer, ALtavant, Aerovate, Jenssen&Jenssen Jenssen& Jenssen and Merck, and is on the speaker bureau for UT, Bayer, and J&J; Dr. Stockbridge has no conflict of interest. Dr. Kovacs reports personal fees and nonfinancial support from Actelion, Janssen, Bayer, GSK, MSD, Boehringer Ingelheim, Novartis, Chiesi, Vitalaire, Ferrer, and AOP outside the submitted work. Dr. Vizza has no conflict of interest regarding this topic. Dr. Hassoun serves on a scientific advisory board for Merck, an activity unrelated to the current work. Dr. Olschewski received funding from Actelion, AOP, Astra Zeneca, Bayer, Boehringer, Chiesi, GSK, Iqvia, Janssen, Menarini, MSD, Novartis, Ludwig Boltzmann Society, Ferrer, MedUpdate, and Mondial not related to this work. Dr. Girgis received honoraria from Boehringer Ingelheim and research funding from UT. Dr. Nikkho is an employee of Bayer AG.Dr. Nathan is a consultant for United Therapeutics, Bellerophon, Third Pole, Roche, Boehringer‐Ingelheim, Insmed, Roivant, Merck and Daewoong. He is on the speakers bureau for United Therapeutics and Boehringer‐Ingelheim. He is also on the Gossamer Bio Board of Directors.

Figures

Figure 1
Figure 1
A case of ILD with severe PH demonstrating enlargement of the pulmonary artery (a) in the context of severe fibrotic disease (b).
Figure 2
Figure 2
Highlights the cumulative contribution of clinical features and test parameters in risk stratifying the probability of PH detection, and thereby to confirm the diagnosis. While this is a suggested algorithm, the final decision to proceed with RHC should be based on the individual patient characteristics and the likelihood that the results will change management. Legend: 6MWT = 6‐min walk test; BNP, brain natriuretic peptide; DLco, single breath diffusing capacity for carbon monoxide; FVC, forced vital capacity; JVD, jugular venous distension; NT‐proBNP, N terminal proBNP; O2, oxygen; P2‐second pulmonic heart sound; RV, right ventricle; VO2, maximal oxygen consumption; WHO FC, World Health Organization functional class.
See this image and copyright information in PMC

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