Pulmonary Hypertension

Pulmonary hypertension (PH) is high blood pressure in the arteries that carry blood from the heart to the lungs. Unlike regular high blood pressure (which affects the systemic circulation), PH puts strain on the right side of the heart, which can eventually lead to right-sided heart failure. PH is less common than systemic hypertension but often under-diagnosed and serious. Early diagnosis and treatment substantially improve outcomes.

Types

PH is classified by underlying cause:

• Group 1 — pulmonary arterial hypertension (PAH) — includes idiopathic PAH, hereditary PAH, PH from connective-tissue diseases, HIV, congenital heart defects, schistosomiasis, some drugs
• Group 2 — PH due to left-sided heart disease
• Group 3 — PH due to lung disease (COPD, interstitial lung disease, sleep apnoea, high-altitude exposure)
• Group 4 — chronic thromboembolic PH (from recurrent blood clots)
• Group 5 — miscellaneous causes (blood disorders, systemic diseases)

In India, congenital heart disease, rheumatic heart disease, and infections (including HIV, schistosomiasis-affected areas elsewhere) are relevant causes along with the usual global list.

Symptoms

Symptoms develop gradually and are often attributed to ageing, being out of shape, or anxiety:

• Breathlessness — especially on exertion, later at rest
• Fatigue, weakness
• Chest pain or pressure
• Palpitations
• Dizziness, fainting (especially with exertion)
• Swelling of feet, ankles, or abdomen (later stage)
• Blue lips or skin in severe disease

Diagnosis

• Echocardiogram — estimates pulmonary artery pressure; first screening test
• Right-heart catheterisation — the gold standard for diagnosis
• ECG, chest X-ray, lung function tests
• Blood tests, HIV test, connective-tissue screening
• CT pulmonary angiography — for thromboembolic disease
• Sleep study if sleep apnoea suspected

Treatment

Treatment depends on the underlying cause:

• Treating the underlying cause — left heart disease, lung disease, sleep apnoea, chronic clots
• Specific medicines for PAH — multiple classes are available; substantially improve symptoms and survival
• Oxygen therapy — for low oxygen levels
• Diuretics — for fluid overload
• Blood thinners — for specific types
• Balloon pulmonary angioplasty or pulmonary thromboendarterectomy — for chronic thromboembolic PH
• Lung or heart-lung transplant — for selected severe cases not responding to other treatment
• Avoid pregnancy — pregnancy is very high-risk in PH and requires specialist pre-pregnancy counselling

If you have persistent unexplained breathlessness — particularly with fainting, swelling, or fatigue — ask your doctor about PH. Early referral to a pulmonologist or cardiologist who sees PH regularly matters because the condition is nuanced and specific treatments are highly effective.