Pulmonary arterial hypertension associated with another disease

Pulmonary arterial hypertension associated with another disease (also called associated pulmonary arterial hypertension or PAH associated with underlying conditions) refers to a form of pulmonary arterial hypertension (PAH) that develops in the context of another medical condition. These associated conditions include connective tissue diseases (most commonly systemic sclerosis/scleroderma), congenital heart disease, portal hypertension (portopulmonary hypertension), HIV infection, and schistosomiasis. PAH is characterized by progressive narrowing and remodeling of the small pulmonary arteries, leading to elevated blood pressure in the pulmonary vasculature. This increased resistance places a significant burden on the right side of the heart, ultimately leading to right heart failure if untreated. Key symptoms include progressive exertional dyspnea (shortness of breath during physical activity), fatigue, chest pain, syncope (fainting), peripheral edema (swelling of the legs and ankles), and exercise intolerance. As the disease progresses, patients may develop signs of right heart failure such as jugular venous distension, hepatomegaly, and ascites. The severity and rate of progression can vary depending on the underlying associated condition; for example, PAH associated with systemic sclerosis tends to carry a worse prognosis compared to PAH associated with congenital heart disease. Diagnosis requires right heart catheterization demonstrating a mean pulmonary arterial pressure greater than 20 mmHg with a pulmonary artery wedge pressure of 15 mmHg or less. Treatment involves PAH-specific therapies targeting the endothelin, nitric oxide, and prostacyclin pathways, including endothelin receptor antagonists (e.g., bosentan, ambrisentan), phosphodiesterase-5 inhibitors (e.g., sildenafil, tadalafil), soluble guanylate cyclase stimulators (riociguat), and prostacyclin analogues or receptor agonists (e.g., epoprostenol, selexipag). Combination therapy is increasingly used. Management of the underlying associated condition is also essential. Despite advances in treatment, associated PAH remains a serious and life-limiting condition requiring ongoing specialist care.

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