AKT · Cardiovascular/Vessels, aorta & venouslow yield

Pulmonary hypertension

Elevated pulmonary arterial pressure → right ventricular pressure overload

Overview

A raised mean pulmonary arterial pressure from one of five WHO groups: pulmonary arterial hypertension (group 1), left heart disease (group 2, commonest), lung disease/hypoxia (group 3), chronic thromboembolic (group 4, CTEPH — potentially curable surgically), and miscellaneous (group 5). It causes progressive right heart failure; identifying the group determines whether specific vasodilator therapy or treatment of the underlying cause is appropriate.

Recognise

Progressive exertional dyspnoea, fatigue, exertional syncope, chest pain; signs of right heart failure (raised JVP, ascites, oedema)
Loud P2, right ventricular heave, tricuspid regurgitation murmur, right-sided 3rd/4th heart sound
Underlying disease clues: connective tissue disease, COPD/ILD, prior PE, left heart disease

Red flags

Right heart failure/syncope → specialist pulmonary hypertension centre
Group 4 (CTEPH) is potentially curable by pulmonary endarterectomy — do not miss it

Differentials & how to tell them apart

Left heart failure (group 2)the commonest cause — raised left-sided filling pressures on catheter

Chronic lung disease (group 3)COPD/ILD on PFTs/CT

Chronic thromboembolic disease (group 4)mismatched defects on V/Q — surgically curable

Investigations

Echo (estimated pulmonary pressures, RV size/function — the screening test); right heart catheterisation (the diagnostic gold standard); V/Q scan (exclude CTEPH); PFTs/CT chest (lung disease); connective tissue and HIV screen; identify the WHO group.

Management

Identify the WHO group; treat the cause (groups 2/3); group 1 → specialist vasodilators; group 4 → endarterectomy

1Screen with echo, then confirm and classify with right heart catheterisation; use V/Q, PFTs/CT and autoimmune screen to assign the WHO group.Gate: Group 4 chronic thromboembolic PH is potentially curable by pulmonary endarterectomy — V/Q scan to find it; and pulmonary vasodilators are NOT appropriate for group 2 (left heart) or most group 3 (lung) disease.
2Treat group 2/3 by optimising the underlying disease; group 1 PAH → specialist vasodilator therapy; group 4 → endarterectomy + anticoagulation; supportive diuretics/oxygen throughout.

Treat the underlying cause (groups 2 & 3) — optimise left heart failure / lung disease and hypoxia (long-term oxygen) — pulmonary vasodilators are NOT for these groups

Group 1 PAH: specific vasodilators (PDE5 inhibitors, endothelin receptor antagonists, prostacyclin analogues) — started in specialist centres after vasoreactivity testing

Group 4 (CTEPH): pulmonary endarterectomy + lifelong anticoagulation — potentially curative surgery

Diuretics for right heart failure, oxygen — supportive

Key points

Progressive exertional dyspnoea + right heart failure + loud P2 = pulmonary hypertension → echo then right heart catheter, and assign the WHO group. The group decides treatment: treat the cause in left-heart/lung disease, specific vasodilators only in group 1, and don't miss surgically curable CTEPH (group 4).

Monitor & prognosis

Functional class/6-minute walk, echo/RV function, specialist follow-up.

Group 1 PAH is progressive but improved by modern therapy; right heart failure marks advanced disease.

Source: ESC/ERS pulmonary hypertension; specialist centres