Pulmonary Hypertension

• Elevated pulmonary arterial pressure (> 25 mmHg) at rest

Classification

• Group 1: Pulmonary arterial hypertension (PAH)
• Group 2: PH associated with left heart disease
• Group 3: PH associated with lung disease
  • Cor pulmonale is the remodeling of the RV in response to lung disease (which causes PH) → may lead to right-sided heart failure
• Group 4: PH associated with pulmonary artery obstructions (e.g. recurrent pulmonary emboli)
• Group 5: PH with unclear and/or multifactorial mechanisms

Signs and Symptoms

• Symptoms: fatigue, lethargy, exertional dyspnoea, syncope, chest pain
• Signs: peripheral oedema, abdominal distention/hepatomegaly, raised JVP (signs of right-sided heart failure), parasternal heaves (RV hypertrophy), may also hear a loud S2, flow murmur and S4 gallop on auscultation
• Often associated with history of COPD, interstial lung disease, heart disease, sickle cell anaemia and pulmonary emboli

Investigation

• Echocardiogram (assessing RV function and pulmonary artery pressure)
• CXR: may show enlarged central pulmonary arteries
• ECG: may show RVH
• Diagnostic: right heart catheterization → measuring mean pulmonary artery pressure > 25 mmHg (normal 8-20 mmHg)

Management

• Treat underlying cause
• Supplemental O₂, diuretics, anticoagulation, digoxin and lifestyle advice may be considered in all groups
• Group 1: consider endothelin receptor antagonist e.g. bosentan, PDE inhibitor and calcium channel blocker
• Group 2: surgical thrombectomy or thrombolytics

Reference: ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension 2022