Pulmonary Hypertension

Pulmonary hypertension is raised blood pressure in the arteries of the lungs, defined as a mean pressure above 20 mmHg measured by right heart catheterisation. It is unrelated to ordinary high blood pressure. The right side of the heart must pump against the extra resistance and eventually enlarges and fails.

It is grouped by cause. Most cases result from left heart disease (group 2) or lung disease (group 3). Pulmonary arterial hypertension (PAH, group 1) is rare and is treated with specific drugs. Chronic thromboembolic pulmonary hypertension (CTEPH, group 4) is caused by old clots and matters because it can be cured by surgery.

Symptome

• Breathlessness on exertion, gradually progressive
• Fatigue and weakness
• Dizziness or fainting during exercise
• Chest pressure on effort
• Swollen ankles, legs and abdomen
• Palpitations
• Bluish lips, and a hoarse voice or coughing blood in advanced disease

Ursachen und Risikofaktoren

Group 1 PAH may be idiopathic, heritable (notably BMPR2 variants), or associated with connective tissue disease such as scleroderma, congenital heart defects with shunts, HIV, portal hypertension, and drugs including certain appetite suppressants and methamphetamine. Group 2 follows left-sided heart failure or mitral and aortic valve disease. Group 3 accompanies COPD, interstitial lung disease and sleep apnoea. Group 4 follows pulmonary emboli that fail to dissolve, in about 2 to 4 in 100 survivors. Group 5 includes sarcoidosis, blood disorders and kidney failure.

Wie es diagnostiziert wird

• Echocardiography: The screening test: estimates lung artery pressure and shows right heart size and function.
• ECG, chest X-ray, lung function tests and sleep study: Look for underlying heart and lung causes.
• Ventilation-perfusion (V/Q) scan: Best test to exclude chronic clots; more sensitive than CT for CTEPH.
• CT of the chest and CT pulmonary angiography: Lung tissue disease and clot anatomy.
• Blood tests: BNP, autoimmune screen, HIV and liver function.
• Right heart catheterisation: Mandatory to confirm the diagnosis, classify it and test vasoreactivity before PAH drugs are started.
• Six-minute walk or cardiopulmonary exercise test: Baseline capacity and risk category.

Behandlungsmöglichkeiten

• Treating the cause: Optimising heart failure or valve disease, oxygen and lung disease therapy, CPAP for sleep apnoea. PAH drugs are generally not used for groups 2 and 3.
• PAH-specific medicines: Endothelin receptor antagonists (ambrisentan, macitentan, bosentan), PDE5 inhibitors (sildenafil, tadalafil), riociguat, prostacyclin pathway drugs (selexipag, inhaled iloprost, infused epoprostenol or treprostinil) and sotatercept, usually in combination.
• Calcium channel blockers: Only for the small minority with a positive vasoreactivity test.
• Pulmonary endarterectomy: Surgical removal of chronic clot in CTEPH; potentially curative in expert centres.
• Balloon pulmonary angioplasty and riociguat: For CTEPH that is not operable.
• Supportive care: Diuretics, oxygen, supervised exercise training, vaccination, anticoagulation in CTEPH, and reliable contraception since pregnancy carries high risk.
• Lung transplantation: For severe disease that progresses despite maximal therapy.

Wenn es dringend ist

Fainting, chest pain, rapidly worsening breathlessness, coughing up blood, or a fast irregular heartbeat need emergency care locally. People on continuous prostacyclin infusions must treat any pump or line failure as an emergency. Anyone with significant pulmonary hypertension should ask their specialist before flying, as cabin oxygen levels are lower and in-flight oxygen may be needed.

Reisen nach Türkiye zur Behandlung

PAH is a chronic disease managed for life by a specialist centre, with costly medicines that must be supplied and monitored at home, so ongoing treatment abroad is not realistic. A visit can be worthwhile for a complete diagnostic work-up including right heart catheterisation (3 to 5 days), a second opinion on classification, or assessment for pulmonary endarterectomy or balloon angioplasty in CTEPH. Confirm fitness to fly beforehand.

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