45-year-old male presented with complaints of progressive shortness of breath, dry cough and pleuritic chest pain for the last 3 weeks. He was homeless, HIV-positive since 1992 but noncompliant with HAART. On physical examination patient was afebrile, hypotensive with blood pressure 85/55 mmHg, pulse rate of 98 beats per minute and SaO2 of 90% on room air. He was cachectic with presence of JVD, loud P2, ejection systolic murmur at pulmonic area. On admission, ABG showed ph 7.36, pCO2 24, PO2 74 mmHg on room air. CBC revealed WBC 6.7k/ul, Hg 9.4g/dl and platelets 49k/ul. Troponin was 0.13 ng/ml, lactate 3.4 mmol/L, BNP 2104pg/ml and CD4 104 . EKG showed sinus tachycardia, incomplete RBBB and RAD. Chest x-ray showed mild prominence of cardiomediastinal silhouette. CTA of the chest was negative for pulmonary embolism but showed diffuse enlargement of the pulmonary arteries. Patient’s echocardiogram showed EF of 60%, moderate dilation of RA, moderate to severe dilation of RV, paradoxal septal motion consistent with RV volume overload, severe TR with RVSP of 76 mmHg suggestive severe pulmonary hypertension. V/Q scan showed low probability for pulmonary embolism. Patient initially required bolus intravenous fluids and a pressor to support blood pressure. We were able to exclude alternative etiology that might contribute to pulmonary hypertension i.e WHO group 2,3 & 4 based on history, clinical presentation and work up. Patient was diagnosed with severe pulmonary hypertension with right heart failure likely associated with HIV infection. However, Cardiology did not further work him up with right heart catheterization during this hospitalization as he was thought not to be a good candidate for Pulmonary arterial hypertension PAH therapy with prostanoids or endothelin receptor antagonists given his non compliance. Patients CD4 count came back at 104. Patient was discharged on Bactrim prophylaxis, recommended to follow up with Infectious disease specialist for resuming his HAART.
HIV-PAH is a rare complication of HIV infection, occurring in approximately 0.5% of HIV-infected patients. The development of PAH in HIV-infected individuals is associated with high morbidity and mortality. The cause of HIV-PAH is uncertain. Both viral and host factors play important roles in pathogenesis. Most patients with HIV-PAH infection present with the following symptoms and signs related to PAH: shortness of breath-85%, pedal edema-30%, nonproductive cough-19%, fatigue-13% , syncope or near-syncope-12%, chest pain-7%. Diagnosis requires confirmation of PAH and exclusion of alternative causes of pulmonary hypertension. The gold standard for hemodynamic evaluation remains invasive assessment with Pulmonary artery catherization PAC. If echocardiography supports a possible diagnosis of PAH, PAC is mandatory before initiation of any PAH-specific therapy. The optimal approach to therapy for individuals with HIV-PAH is uncertain, but should include aggressive management of HIV infection and use of PAH-specific therapies.
The development of PAH in HIV-infected individuals is associated with high morbidity and mortality. Physicians treating HIV-infected patients should monitor for symptoms of unexplained or new dyspnea and signs of right heart dysfunction as HIV itself is an independent risk factor for PAH. Both PAH-specific therapies and HAART are important in HIV-related PAH management.
To cite this abstract:Cherukula M. Severe Pulmonary Hypertension in a Patient with Human Immunodeficiency Virus. Abstract published at Hospital Medicine 2016, March 6-9, San Diego, Calif. Abstract 468. Journal of Hospital Medicine. 2016; 11 (suppl 1). https://www.shmabstracts.com/abstract/severe-pulmonary-hypertension-in-a-patient-with-human-immunodeficiency-virus/. Accessed April 5, 2020.