Pulmonary arterial hypertension associated with HIV infection

Pulmonary arterial hypertension associated with HIV infection (HIV-PAH) is a rare but serious cardiovascular complication that occurs in individuals living with human immunodeficiency virus (HIV). In this condition, the small arteries of the lungs become narrowed, thickened, and stiffened, leading to abnormally elevated blood pressure in the pulmonary vasculature. This increased pressure forces the right side of the heart to work harder to pump blood through the lungs, eventually leading to right heart failure if left untreated. HIV-PAH can occur at any stage of HIV infection and does not appear to correlate directly with the degree of immunosuppression or CD4 cell count. Key symptoms include progressive shortness of breath (dyspnea), particularly during physical exertion, fatigue, chest pain, lightheadedness, syncope (fainting), and peripheral edema (swelling of the legs and ankles). As the disease progresses, patients may experience worsening exercise intolerance and signs of right-sided heart failure, including abdominal distension due to ascites and jugular venous distension. The pathogenesis is not fully understood but is thought to involve endothelial dysfunction, chronic inflammation, and the indirect effects of HIV viral proteins on the pulmonary vasculature rather than direct viral infection of pulmonary endothelial cells. Treatment of HIV-PAH involves a combination of antiretroviral therapy (ART) for the underlying HIV infection and PAH-specific therapies. PAH-targeted treatments include endothelin receptor antagonists (such as bosentan and ambrisentan), phosphodiesterase-5 inhibitors (such as sildenafil and tadalafil), prostacyclin analogues, and soluble guanylate cyclase stimulators. Careful attention must be paid to drug-drug interactions between antiretroviral agents and PAH medications. While ART alone may improve pulmonary hemodynamics in some patients, most require dedicated PAH therapy. Supportive measures include diuretics, oxygen supplementation, and exercise rehabilitation. Prognosis has improved with modern therapies but remains more guarded compared to idiopathic PAH, and early detection through screening in symptomatic HIV-positive patients is important.

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