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Review
. 2024 Oct 31;64(4):2401180.
doi: 10.1183/13993003.01180-2024. Print 2024 Oct.

Management of pulmonary hypertension in special conditions

Ioana R Preston  1 , Luke S Howard  2 , David Langleben  3 , Mona Lichtblau  4 , Tomas Pulido  5 , Rogerio Souza  6 , Karen M Olsson  7
Affiliations
Review

Management of pulmonary hypertension in special conditions

Ioana R Preston et al. Eur Respir J. .

Abstract

Care of pulmonary hypertension (PH) patients in special situations requires insightful knowledge of the pathophysiology of the cardiopulmonary system and close interaction with different specialists, depending on the situation. The role of this task force was to gather knowledge about five conditions that PH patients may be faced with. These conditions are 1) perioperative care; 2) management of pregnancy; 3) medication adherence; 4) palliative care; and 5) the influence of climate on PH. Many of these aspects have not been covered by previous World Symposia on Pulmonary Hypertension. All of the above conditions are highly affected by psychological, geographical and socioeconomic factors, and share the need for adequate healthcare provision. The task force identified significant gaps in information and research in these areas. The current recommendations are based on detailed literature search and expert opinion. The task force calls for further studies and research to better understand and address the special circumstances that PH patients may encounter.

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Conflict of interest statement

Conflict of interest: I.R. Preston reports grants from Janssen, Merck, United Therapeutics and Respira, consultancy fees from Janssen, Merck, United Therapeutics, Liquidia, Gossamer and Aerovate, payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen and United Therapeutics, and support for attending meetings from Janssen and United Therapeutics. L.S. Howard reports grants from MSD, consultancy fees from Ferrer, Janssen and Morphic, payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen, MSD, Aerovate and Ferrer, payment for expert testimony from Janssen, support for attending meetings from Janssen and Gossamer Bio, participation on a data safety monitoring board or advisory board with MSD, Janssen, Gossamer Bio, Apollo Therapeutics and Altavant, and stock (or stock options) with Circular, ATXA Therapeutics, iOWNA, Calibre Biometrics and OneWelbeck Clinic. D. Langleben reports grants from Acceleron/Merck, Aerovate and Janssen, consultancy fees from Merck, Janssen, Actelion, Enzyvant, PhaseBio, Gossamer Bio and Bayer, payment or honoraria for lectures, presentations, manuscript writing or educational events from Merck, Janssen and Bayer, support for attending meetings from Acceleron/Merck, Janssen, Enzyvant and Gossamer Bio, and participation on a data safety monitoring board or advisory board with Phase Bio, Merck, Janssen, Actelion, Enzyvant and Gossamer Bio. M. Lichtblau reports payment or honoraria for lectures, presentations, manuscript writing or educational events from, and participation on a data safety monitoring board or advisory board with MSD, and support for attending meetings from MSD and Janssen. T. Pulido reports grants from Aerovate, Bayer, Janssen, MSD, Pfizer and United Therapeutics, consultancy fees from Bayer, MSD, Gossamer Bio, Aerovate and Ferrer, payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer, Janssen, MSN Labs, Tuteur and Ferrer, support for attending meetings from Bayer, Janssen and Ferrer, participation on a data safety monitoring board or advisory board with Janssen, MSD, Bayer and Gossamer Bio, and is consultant and part of the advisory board for the Mexican Society of Pulmonary Arterial Hypertension. R. Souza reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer and Janssen, and participation on a data safety monitoring board or advisory board with Janssen, Bayer and MSD. K.M. Olsson reports grants from Acceleron, MSD and Janssen, consultancy fees from Acceleron, Actelion, Janssen, Ferrer, Merck, AOP Health, Gossamer and Bayer, payment or honoraria for lectures, presentations, manuscript writing or educational events from Acceleron, Actelion, AOP Health, Janssen, Bayer, MSD, Ferrer and Gossamer, and support for attending meetings from Acceleron/MSD and Janssen/Actelion.

Figures

FIGURE 1
FIGURE 1
Perioperative care in patients with pulmonary hypertension (PH). The algorithm emphasises the need to optimise volume status. PAH: pulmonary arterial hypertension; Hb: haemoglobin; ICU: intensive care unit; PAC: pulmonary artery catheter; ECMO: extracorporeal membrane oxygenation; RV: right ventricle; PVR: pulmonary vascular resistance; PEEP: positive end-expiratory pressure; NO: nitric oxide.
FIGURE 2
FIGURE 2
Practical guidance algorithm for pregnancy management in pulmonary arterial hypertension (PAH). PH: pulmonary hypertension; ERA: endothelin receptor antagonist; sGCs: soluble guanylate cyclase stimulators; PRA: prostacyclin analogues; NT-proBNP: N-terminal pro-brain natriuretic peptide; C-section: caesarean section; CVP: central venous pressure; SvO2: mixed venous oxygen saturation; ECMO: extracorporeal membrane oxygenation; ICU: intensive care unit.
FIGURE 3
FIGURE 3
Five interacting dimensions which affect adherence. Information from [31].
FIGURE 4
FIGURE 4
Clinical touchpoints to discuss palliative care. QoL: quality of life; PROMs: patient-reported outcome measures; ED: emergency department; PH: pulmonary hypertension.
See this image and copyright information in PMC

Comment in

References

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