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Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension (Group 4) caused by organized, non-resolving pulmonary emboli that obstruct pulmonary arteries, leading to persistent elevation in pulmonary artery pressure, vascular remodeling, and ultimately right heart failure.

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Hemodynamic Criteria (Right Heart Catheterization)

• Mean pulmonary artery pressure (mPAP) ≥ 20 mmHg
• Pulmonary vascular resistance (PVR) > 2 Wood units
• Pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg
• Persistent perfusion defects on imaging ≥3 months after acute PE treatment

Epidemiology

• Occurs in ~3–4% of patients with acute pulmonary embolism (PE)
• Can develop after symptomatic or silent PE
• Frequently underdiagnosed

Pathophysiology

• Incomplete resolution of thromboemboli → organized fibrotic obstructions in pulmonary arteries
• Leads to:
  • Elevated PVR
  • Compensatory hypertrophy of the right ventricle
  • Secondary arteriopathy in non-obstructed vessels (vascular remodeling)
  • Right heart failure if untreated

Clinical Presentation

• Progressive exertional dyspnea (most common)