Secondary pulmonary arterial hypertension

This code signifies elevated blood pressure within the pulmonary arteries that is a consequence of another underlying medical condition. It indicates that the pulmonary hypertension is not primary (idiopathic) but rather secondary to a distinct etiology affecting the pulmonary vasculature.

Assign this code when documentation clearly states "secondary pulmonary arterial hypertension" and identifies an underlying cause, such as connective tissue disease, congenital heart disease, or chronic thromboembolic disease. This code is appropriate when the pulmonary hypertension is a complication or manifestation of another disease process.

• Always code the underlying cause of the secondary pulmonary arterial hypertension first, followed by this code.
• Distinguish carefully between pulmonary arterial hypertension (PAH) and pulmonary hypertension (PH) due to other causes (e.g., left heart disease, lung disease), as these have different codes.
• Ensure the documentation specifies "arterial" to differentiate from other types of secondary pulmonary hypertension.