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Pulmonary hypertension is raised blood pressure in the arteries that carry blood from the heart to the lungs. The higher pressure makes the right side of the heart work harder, causing breathlessness on exertion, tiredness, dizziness and swollen ankles. It has several causes — including lung and heart disease and old blood clots — so finding the type is important and usually needs specialist tests. Treatment depends on the cause and may include specific medicines, oxygen and carefully guided exercise. This page explains pulmonary hypertension and how it is managed.
Pulmonary hypertension (PH) is an umbrella term for a heterogeneous group of disorders defined haemodynamically, with management dictated by the underlying clinical group. The 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension are the current international reference.
Definition
Pulmonary hypertension is defined haemodynamically as a mean pulmonary artery pressure (mPAP) >20 mmHg at rest, measured by right heart catheterisation (the threshold was lowered from 25 to 20 mmHg in the 2022 ESC/ERS guideline). Pre-capillary PH (pulmonary arterial hypertension and most PH groups) requires PVR ≥2 Wood units in addition.
Pathophysiology
PH results from progressive remodelling of the pulmonary vasculature with vasoconstriction, smooth muscle hypertrophy, intimal fibrosis, and in PAH, plexiform lesions. The end result is increased pulmonary vascular resistance, right ventricular pressure overload, and ultimately right ventricular failure.
Clinical classification (WHO/ESC groups)
Group 1: Pulmonary arterial hypertension (PAH) — idiopathic, heritable, drug-induced, associated with connective tissue disease, HIV, portal hypertension, congenital heart disease.
Group 2: PH due to left heart disease (most common cause in Australia) — HFrEF, HFpEF, valvular disease.
Group 3: PH due to lung disease and/or hypoxia — COPD, ILD, sleep-disordered breathing, alveolar hypoventilation.
Group 4: PH associated with pulmonary artery obstructions — CTEPH and other pulmonary artery obstructive diseases.
Group 5: PH with unclear or multifactorial mechanisms.
Co-morbidities
Connective tissue disease (particularly systemic sclerosis), congenital heart disease, HIV, portal hypertension, schistosomiasis (globally), chronic thromboembolism, sleep-disordered breathing, anaemia, and iron deficiency.
Prevalence
PAH is rare (15–50 per million); PH from left heart disease and lung disease is far more common but often underdiagnosed. Australian patients with suspected PAH or CTEPH should be referred to one of the designated PH centres for confirmation and management.
Symptoms
Progressive exertional dyspnoea (almost universal), fatigue, exertional pre-syncope or syncope, chest pain, palpitations, peripheral oedema, abdominal distension and early satiety from hepatic congestion in advanced disease. Symptoms are non-specific and diagnostic delay of 2 years from symptom onset remains common.
Diagnosis
Why diagnosis matters
Accurate clinical group classification is essential because treatments differ profoundly between groups — PAH-specific therapies are indicated in Group 1 and selected Group 4 patients but are potentially harmful in Group 2 and Group 3 PH.1
Initial assessment
Echocardiography is the initial non-invasive screening test, estimating right ventricular systolic pressure from tricuspid regurgitation velocity and assessing RV size and function. Probability of PH is graded as low, intermediate or high.
Confirmatory and group-defining investigations
Right heart catheterisation is required to confirm PH and characterise haemodynamics. Group-defining investigations include lung function with DLCO, HRCT chest, ventilation-perfusion scan (essential to exclude CTEPH), connective tissue serology, HIV testing, liver ultrasound, polysomnography, and 6-minute walk testing.
Risk stratification
Risk stratification at diagnosis and at every follow-up uses a multi-parameter approach (functional class, 6MWD, BNP/NT-proBNP, RV function on imaging, haemodynamics). The ESC/ERS four-strata model and the REVEAL Lite 2 risk score are commonly used.2
Management
General measures
Avoidance of pregnancy (PAH carries unacceptably high maternal mortality), supervised exercise rehabilitation, immunisation (influenza, pneumococcal, COVID-19), psychological support, contraception advice, supplemental oxygen for hypoxaemia, and treatment of co-morbidities including iron deficiency.
PAH-specific therapy (Group 1)
Initial dual oral combination therapy (endothelin receptor antagonist plus PDE5 inhibitor) for low- and intermediate-risk patients. Triple therapy with addition of a prostacyclin analogue for high-risk patients. Sotatercept (newly approved in some jurisdictions) is an emerging activin signalling inhibitor.
Group 2 and 3 PH
Treatment is directed at the underlying disease (heart failure, valvular disease, COPD, ILD, sleep-disordered breathing). PAH-specific therapies are generally not indicated and may cause harm.3
CTEPH (Group 4)
Lifelong anticoagulation. Pulmonary endarterectomy is the treatment of choice for operable disease, offered in Australia at a small number of specialised centres. Balloon pulmonary angioplasty and medical therapy (riociguat) are used for inoperable or residual disease.
Advanced therapies
Atrial septostomy and lung transplantation are considered for selected patients with progressive disease despite optimal medical therapy.4,5,6,7
Medications
PAH-specific therapy
Endothelin receptor antagonists (macitentan, ambrisentan, bosentan), phosphodiesterase-5 inhibitors (sildenafil, tadalafil), soluble guanylate cyclase stimulators (riociguat — also used in CTEPH), prostacyclin pathway agents (epoprostenol, treprostinil, selexipag, iloprost), and activin signalling inhibitors (sotatercept).
Supportive medications
Diuretics for right heart failure, anticoagulation in CTEPH, supplemental oxygen for hypoxaemia, iron supplementation, and treatment of co-morbidities.
Multi-system manifestations
Right heart failure
Peripheral oedema, ascites, hepatic congestion, gut oedema with reduced appetite and malabsorption, exertional pre-syncope.
Bleeding and thrombotic complications
Haemoptysis from bronchial arterial bleeding is uncommon but can be life-threatening. Anticoagulation in PAH is no longer routinely recommended outside CTEPH and selected idiopathic PAH.
Sleep-disordered breathing
A high proportion of PH patients have co-existing sleep-disordered breathing, which warrants formal evaluation and treatment.
Iron deficiency and anaemia
Iron deficiency is highly prevalent in PAH and is associated with worse functional capacity and outcomes. Intravenous iron is often preferred to oral replacement.
Warning signs
Living with pulmonary hypertension
Exercise and rehabilitation
Supervised exercise training improves exercise capacity, quality of life, and possibly haemodynamics in stable PAH patients on optimal medical therapy. Programmes should be delivered in centres with experience in PH and should specifically avoid Valsalva manoeuvres, heavy resistance lifting, and unsupervised high-intensity exercise that may precipitate syncope. Low-load high-repetition resistance training is preferred.8,9,10
Pregnancy and contraception
Pregnancy is strongly contraindicated in PAH (maternal mortality 17–33%). Effective contraception (long-acting reversible methods preferred; combined oral contraceptive avoided in most patients) is essential. All women of childbearing age require structured contraception discussion at diagnosis.
Travel
Air travel may require supplemental oxygen assessment. Travel to altitude (>1500 m) carries the risk of hypoxia-induced vasoconstriction and clinical deterioration and warrants pre-travel discussion.
Psychological care
Anxiety and depression are common in PAH given the prognosis, treatment burden, and lifestyle restrictions. Active psychological support and patient organisations (PHA Australia, the global PH community) are valuable.
Prognosis
Prognosis depends on aetiology, severity, response to therapy, and access to advanced treatments. Modern combination therapy and early diagnosis have substantially improved outcomes in PAH; CTEPH is potentially curable with pulmonary endarterectomy in eligible patients.
Idiopathic Pulmonary Fibrosis (IPF)
Role of the physiotherapist
Exercise in pulmonary hypertension must be supervised and carefully titrated. Within a specialist pathway the physiotherapist delivers low-to-moderate, symptom-limited rehabilitation, monitors oxygen saturation and symptoms closely, teaches breathing and pacing techniques, and educates on energy conservation and avoiding unsafe exertion (such as heavy straining).
Part 1 · References
- Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022;43(38):3618–3731.
- Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J 2017;50(2):1700889.
- Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease (INCREASE). N Engl J Med 2021;384(4):325–334.
- Galiè N, Barberà JA, Frost AE, et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension (AMBITION). N Engl J Med 2015;373(9):834–844.
- Hoeper MM, Badesch DB, Ghofrani HA, et al. Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension (STELLAR). N Engl J Med 2023;388(16):1478–1490.
- Delcroix M, Torbicki A, Gopalan D, et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J 2021;57(6):2002828.
- Ghofrani HA, D'Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension (CHEST-1). N Engl J Med 2013;369(4):319–329.
- Grünig E, MacKenzie A, Peacock AJ, et al. Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial. Eur Heart J 2021;42(23):2284–2295.
- Mereles D, Ehlken N, Kreuscher S, et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation 2006;114(14):1482–1489.
- Morris NR, Kermeen FD, Jones AW, Lee JYT, Holland AE. Exercise-based rehabilitation programmes for pulmonary hypertension. Cochrane Database Syst Rev 2023;(3):CD011285.
References are numbered in citation order (Vancouver/BMJ style) and were current at the time of writing. Guidelines are living documents — verify against the latest version before clinical use.
Supervised exercise, breathing technique and self-management education are the mainstay of cardiorespiratory physiotherapy for this condition.
Clinical evidence
Part 1 covers the same condition without the technical detail. What follows is the evidence base behind it, written for clinicians — the literature, the reasoning and the gaps.
For clinicians: this summary supports clinical reasoning and is not a protocol. Check current guidelines and local policy before applying it, and read it alongside the key references and guidelines directory.
Framing. The 2022 ESC/ERS guideline lowered the haemodynamic definition to a mean pulmonary artery pressure above 20 mmHg with pulmonary vascular resistance above 2 Wood units, and organises management around the five groups — only group 1 (pulmonary arterial hypertension) and group 4 (chronic thromboembolic disease) receive targeted vasoactive therapy, while groups 2 and 3 are treated by treating the left heart or the lung disease.1 Exercise training, once considered dangerous here, is now a guideline-recommended adjunct — but only in expert-centre-supervised programmes in patients on stable optimised therapy.1,2
Exercise training evidence
- The first randomised trial of exercise and respiratory training in severe PH found significant improvement in six-minute walk distance, peak oxygen uptake and quality of life, overturning the previous blanket restriction.3
- A large European multicentre randomised trial confirmed that standardised low-intensity supervised training in PAH and CTEPH is feasible, safe and effective, improving walk distance, peak VO2 and quality of life on top of optimised medical therapy.2
- The Cochrane review concludes rehabilitation improves exercise capacity and quality of life, with programmes typically starting at low intensity under close supervision and monitoring.4
- The safety framework is explicit: stable on optimised therapy, no recent syncope or decompensation, supervised initiation in a centre familiar with PH, low starting intensity, and close symptom and saturation monitoring.1,2
Medical therapy that changes the caseload
- Upfront combination therapy (ambrisentan plus tadalafil) reduced clinical failure events compared with monotherapy in AMBITION, establishing early dual therapy as standard in most PAH.5
- Sotatercept improved six-minute walk distance and multiple secondary outcomes when added to background therapy in STELLAR, the first agent targeting the underlying proliferative pathway rather than vasoconstriction.6
- CTEPH is potentially curable by pulmonary endarterectomy, with balloon pulmonary angioplasty and riociguat for inoperable or residual disease — so any suspicion warrants referral to an expert centre.7,8
- PH complicating interstitial lung disease responded to inhaled treprostinil in INCREASE, changing the previous nihilism about group 3 disease.9
- Risk stratification is dynamic, using functional class, walk distance, biomarkers and haemodynamics to escalate therapy — and physiotherapy walk-test data feed directly into it.1,10
Physiotherapy implications
- Do not start an independent exercise programme in group 1 or 4 disease. Coordinate with the PH service, obtain written parameters, and begin at low intensity with supervision.1,2
- Avoid Valsalva, heavy isometric loading and breath-holding, which acutely raise pulmonary pressures and reduce venous return; use low-load, high-repetition resistance work with continuous exhalation.
- Expect exertional desaturation and use supplemental oxygen where prescribed; interval formats with genuine recovery periods are better tolerated than continuous work.
- Syncope, pre-syncope and exertional chest pain are absolute stop signals in this population and require same-day medical review — they signal inadequate cardiac output, not deconditioning.1
- Recognise right heart failure: rising weight, ankle and abdominal swelling, hepatic congestion and worsening fatigue mean decompensation, and training should pause pending review.
- Give the practical safety advice: avoid altitude and unpressurised flight without advice, avoid decongestants and NSAIDs where directed, avoid saunas and hot tubs, and know that pregnancy is high-risk and specialist-managed.1
- Use objective measures deliberately — serial six-minute walk distance with desaturation profile and Borg scores — and report them, because they feed the risk-stratification decision.10
Clinical reasoning
- Establish which group you are dealing with before prescribing: the rules for PAH differ from PH secondary to left heart disease or lung disease.1
- Breathlessness disproportionate to spirometry and imaging, with exertional desaturation or syncope, is a PH question — the average diagnostic delay is years, and physiotherapists are well placed to shorten it.
- A patient whose walk distance falls between visits is deteriorating regardless of how they describe it; escalate rather than adjust the programme.
- Unexplained persistent breathlessness after pulmonary embolism should be screened for CTEPH, which is potentially operable.7
Evidence gaps
- Optimal exercise modality, intensity, progression and duration in PH remain undefined, and trials deliberately used conservative low-intensity protocols.2,4
- Safety of unsupervised home or community programmes outside expert centres is unestablished.
- No trial has tested rehabilitation in group 2 or group 3 PH specifically.9
- Whether training affects hard outcomes — hospitalisation, transplantation, survival — is unknown.
References for the clinical evidence summary
- Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022;43(38):3618–3731.
- Grünig E, MacKenzie A, Peacock AJ, et al. Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial. Eur Heart J 2021;42(23):2284–2295.
- Mereles D, Ehlken N, Kreuscher S, et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation 2006;114(14):1482–1489.
- Morris NR, Kermeen FD, Jones AW, Lee JYT, Holland AE. Exercise-based rehabilitation programmes for pulmonary hypertension. Cochrane Database Syst Rev 2023;(3):CD011285.
- Galiè N, Barberà JA, Frost AE, et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension (AMBITION). N Engl J Med 2015;373(9):834–844.
- Hoeper MM, Badesch DB, Ghofrani HA, et al. Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension (STELLAR). N Engl J Med 2023;388(16):1478–1490.
- Delcroix M, Torbicki A, Gopalan D, et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J 2021;57(6):2002828.
- Ghofrani HA, D'Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension (CHEST-1). N Engl J Med 2013;369(4):319–329.
- Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease (INCREASE). N Engl J Med 2021;384(4):325–334.
- Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J 2017;50(2):1700889.
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