Overview
Definition
Elevated blood pressure within the pulmonary circulation (Mean Pulmonary Arterial Pressure > 20 mmHg at rest).
Epidemiology
Group 2 (due to left heart disease) and Group 3 (due to lung disease) are the most common.
Etiology & Risk Factors
- Group 1: Pulmonary Arterial Hypertension (Idiopathic, Heritable, Connective Tissue Disease)
- Group 2: Left heart disease (Heart Failure, Valvular disease)
- Group 3: Chronic lung disease/hypoxia (COPD, ILD, OSA)
- Group 4: Chronic Thromboembolic PH (CTEPH)
- Group 5: Unclear/Multifactorial
Clinical Symptoms
- Exertional dyspnea
- Fatigue
- Lethargy
- Syncope
- Right heart failure symptoms (edema, ascites)
Clinical Approach
Diagnosis
- Echocardiogram (screening tool - estimates pulmonary artery systolic pressure)
- Right Heart Catheterization (Gold Standard - measures mPAP > 20 mmHg, PCWP, and PVR)
Management
- Group 1: Advanced therapies targeting endothelial dysfunction (Endothelin receptor antagonists like Bosentan, PDE-5 inhibitors like Sildenafil, Prostacyclin analogs)
- Group 2 & 3: Treat the underlying heart or lung disease
- Group 4: Surgical pulmonary thromboendarterectomy (PTE) or Riociguat
- All groups: Diuretics for volume overload, Oxygen therapy
Complications
- Cor Pulmonale (Right Ventricular Failure)
- Arrhythmias
- Death