CH2 Severe pulmonary hypertension in a patient with HIV. (Chest Diseases).

CH2 SEVERE PULMONARY HYPERTENSION IN A PATIENT WITH HIV. Tim Holden, MD, and Mukta Panda, MD. Department of Medicine, University of Tennessee, Chattanooga.

The acquired immunodeficiency syndrome is a worldwide problem with increasing magnitude. However, with improved antiretroviral therapy and effective prophylaxis, HIV-infected individuals have an increased life span. This has led to the recognition of various noninfectious conditions associated with HIV such as HIV cardiomyopathy and HIV-related pulmonary hypertension. We present a case of severe pulmonary hypertension in an HIV-infected patient. A 29-year-old nonsmoking black woman with HIV for 9 years presented to the ER with progressively worsening shortness of breath, exertional dyspnea, and orthopnea for 7 months. She had had a cough for 2 weeks with mild colorless sputum production. She denied any illness since being diagnosed with HIV. Two weeks earlier, her CD4 count was 410 and viral load 1,500. Her medications were proventil and azmacort, given by her PCP for what was believed to be new-onset asthma. Examination revealed a pleasant woman who appeared tachypneic with a BP of 98/65 mm Hg, pulse rate 12 1/min, respiratory rate 21/min, and temperature 98.8[degrees]F. She had no oral lesions, JVD, clubbing, or edema. Lungs were clear to auscultation with decreased air movement. She had a regular rhythm S3 and a localized 2/6 systolic murmur at the lower left sternal border. The remainder of the examination was unremarkable. Pertinent data: ABG: 7.54/24/66/29/95% room air, ECG with sinus tachycardia and RVH. CXR showed mild cardiomegaly. Echo: moderate RVH & LVH, RA severely enlarged, EF >70%, severe tricuspid regurgitation, paradoxical motion of the septum, and a pulmonary artery pressure (PAP) 95 mm Hg. VQ scan: low probability of embolism. Since other potentially reversible causes of pulmonary hypertension were excluded, the patient was diagnosed with HIV-related pulmonary hypertension. Since she did not respond to a trial of short-acting vasodilators at a right heart catheterization, she is currently on continuous prostaglandin infusion therapy with symptomatic improvement. The first case of HIV-related pul monary hypertension (PH) was described in 1987. Since then, only 130 cases have been reported. The development of PH is independent of CD4 count and/or use of antiretroviral medications. Although the exact mechanism is not understood, it appears that alterations in the pulmonary endothelial cell homeostasis are responsible for the development of PH. It has been previously proposed that the endothelial alterations may be a direct result of the virus acting locally, though researchers have been unable to isolate the HIV virus in the pulmonary vasculature. We hypothesize that in individuals with immunogenetic predisposition the immune system is triggered by HIV to react locally in the pulmonary vasculature leading to an excessive localized cytokine production. This results in a plexiform arteriopathy and prothrombotic state similar to that seen in primary pulmonary hypertension. In general HIV-infected patients have responded less favorably to vasodilators (Ca channel blockers). However, with a continuous IV inf usion of prostaglandin (epoprostenol), these patients have shown marked improvement in PAP and symptoms. A high index of suspicion should be present when an HIV-infected patient presents with progressive shortness of breath and/or exercise intolerance with no evidence of infection or underlying cardiopulmonary condition. Initial workup should include a chest x-ray and echocardiogram. If the PA pressure is elevated, other secondary causes for PH must be ruled out before the diagnosis of HIV-related PH is made.