Chronic thromboembolic pulmonary hypertension

This code describes a severe and persistent form of pulmonary hypertension caused by organized blood clots in the pulmonary arteries that do not resolve over time. These chronic blockages lead to increased pressure in the pulmonary circulation, straining the right side of the heart.

Use this code when documentation clearly indicates a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). This diagnosis is typically confirmed through imaging studies (e.g., CT pulmonary angiography, ventilation-perfusion scan) and right heart catheterization showing elevated pulmonary artery pressures in the presence of chronic thrombi.

• Ensure documentation specifies "chronic thromboembolic" to differentiate from acute or other forms of pulmonary hypertension.
• Do not confuse with acute pulmonary embolism (I26.-) or other types of pulmonary hypertension (e.g., I27.0, I27.20, I27.21, I27.22, I27.23, I27.29).
• Code any associated right heart failure (I50.81) or other complications separately.