Conditions Treated
Pulmonary Hypertension
Pulmonary hypertension is a serious condition affecting the arteries of the lungs and the right side of the heart. Early specialist diagnosis and modern targeted therapies have significantly improved outcomes.
Overview
What is Pulmonary Hypertension?
Pulmonary hypertension (PH) is defined as an elevation in mean pulmonary arterial pressure, causing increased resistance in the pulmonary circulation and, over time, right heart failure. It is not a single disease but a haemodynamic state with many possible underlying causes, classified by the World Health Organisation into five groups based on aetiology and treatment approach.
Group 1 — pulmonary arterial hypertension (PAH) — includes idiopathic PAH and PAH associated with connective tissue diseases such as systemic sclerosis (scleroderma), lupus, and mixed connective tissue disease. Connective tissue disease is the most common identifiable cause of PAH in the UK, and scleroderma-associated PAH carries a particularly significant prognosis, making early detection critical.
Other groups include PH due to left heart disease (Group 2), lung disease or hypoxia (Group 3), chronic thromboembolic disease (CTEPH, Group 4), and a heterogeneous group with unclear or multifactorial mechanisms (Group 5). Accurate classification requires specialist investigation, as management differs substantially between groups.
Recognition
Symptoms
- Progressive breathlessness on exertion — often the first and most prominent symptom
- Fatigue and reduced exercise tolerance
- Chest pain or pressure, particularly on exertion
- Palpitations or awareness of the heartbeat
- Pre-syncope (near-fainting) or syncope, particularly on exertion
- Ankle swelling and leg oedema — a sign of right heart strain
- Dizziness and light-headedness
- In CTD-associated PH: symptoms may overlap with the underlying condition
Assessment
Diagnosis & Investigations
- Echocardiogram — the key screening investigation, estimating pulmonary pressures and right heart function
- Right heart catheterisation — the gold standard for definitive diagnosis and haemodynamic characterisation
- CT pulmonary angiography — to exclude chronic thromboembolic disease (CTEPH)
- Ventilation-perfusion (V/Q) scan — sensitive screening for CTEPH
- Lung function tests including DLCO (transfer factor) — often reduced, particularly in CTD
- NT-proBNP or BNP — cardiac biomarker reflecting right heart stress
- Six-minute walk test — functional assessment and monitoring
- Autoantibody screen — to identify or characterise underlying connective tissue disease
Management
Treatment Options
Treatment depends on the WHO group and underlying cause. For pulmonary arterial hypertension (Group 1), a range of targeted therapies are now available that act on pathways involved in pulmonary vascular remodelling. These include endothelin receptor antagonists (such as ambrisentan and macitentan), phosphodiesterase-5 inhibitors (sildenafil and tadalafil), and prostacyclin pathway agents (including oral selexipag and inhaled or intravenous prostacyclin analogues). Current guidelines recommend combination therapy as the standard of care for most patients with PAH.
For chronic thromboembolic PH (CTEPH), pulmonary endarterectomy — surgical removal of obstructing material from the pulmonary arteries — can be curative in operable cases. For inoperable CTEPH, balloon pulmonary angioplasty and the soluble guanylate cyclase stimulator riociguat are options. General supportive measures include supplemental oxygen where indicated, diuretics for fluid overload, and anticoagulation in selected patients.
In CTD-associated PH, management of the underlying connective tissue disease runs in parallel. Immunosuppression may be beneficial particularly in inflammatory-predominant PAH, such as that associated with lupus or MCTD. Close collaboration between rheumatology and respiratory/pulmonary hypertension specialists is essential.
Specialist Input
Dr. Schreiber's expertise in pulmonary hypertension
Pulmonary hypertension — particularly when it occurs in the context of connective tissue disease — is one of Dr. Schreiber's core research and clinical interests. His research doctorate (MD, UCL) focused specifically on pulmonary hypertension in connective tissue disease, and he has published in this area in journals including Circulation Research and the European Heart Journal. He was awarded the American College of Rheumatology Hench Award in 2012 for this work.
CTD-associated PH requires dual expertise in rheumatology and pulmonary vascular disease. An early and accurate diagnosis — distinguishing PAH from PH due to interstitial lung disease, left heart disease, or other causes — is critical, as the treatment implications differ substantially. Systematic screening for PH in patients with scleroderma, lupus and other CTDs is part of Dr. Schreiber's routine clinical practice.
Patient Education
Watch: Pulmonary Hypertension Explained
Dr. Schreiber has produced two short patient education videos on pulmonary hypertension, explaining what the condition is and how it is managed.
Common Questions
FAQ
Is pulmonary hypertension the same as high blood pressure?
No. Pulmonary hypertension refers specifically to elevated pressure in the arteries supplying the lungs — not the systemic blood pressure measured in the arm. The two conditions have different causes, mechanisms and treatments. Someone can have normal systemic blood pressure and still have pulmonary hypertension.
I have scleroderma — should I be screened for pulmonary hypertension?
Yes. Systematic annual screening for pulmonary hypertension is recommended for all patients with systemic sclerosis, as CTD-associated PAH is more common in scleroderma than in any other connective tissue disease. Screening typically involves echocardiography and lung function testing (including DLCO). Early detection significantly improves treatment outcomes.
Can pulmonary hypertension be cured?
In most forms, PH is not curable but is manageable with modern targeted therapies. The exception is chronic thromboembolic PH (CTEPH), where pulmonary endarterectomy surgery can be curative in selected patients. For other forms, the goal of treatment is to improve symptoms, exercise capacity and quality of life, and to slow disease progression.
What is the prognosis for pulmonary hypertension?
Prognosis varies considerably depending on the underlying cause, how early it is diagnosed, and the response to treatment. Outcomes have improved substantially with modern combination PAH therapy. Scleroderma-associated PAH tends to carry a more guarded prognosis than idiopathic PAH, highlighting the importance of early diagnosis and expert management in this group.
Concerned about pulmonary hypertension?
Dr. Schreiber offers expert assessment of suspected or confirmed pulmonary hypertension, with particular expertise in CTD-associated disease.