(II-1A.4b) Pulmonary arterial hypertension associated with HIV and HCV infection Michał Furdal, Ewa Mroczek, Jakub Stępniewski

Pulmonary arterial hypertension (PAH) is a rare, but one of the most frequent complications of HIV infection. 3- year survival of patients with HIV related PAH (HIV-PAH) remaining in functional class III or IV by WHO (FC WHO) reaches approximately 20-30%. It is believed that highly active antiretroviral therapy (cART) or dysfunction of immune system and CD4 lymphocytes has less likely influence on the occurrence or the course of PAH. There is still limited data on the utilization of PAH specific agents in the treatment of HIV-PAH. The most promising data refer to the use of endothelin receptor blockers in this group of patients.

Case presentation
32 year-old female patient was referred to our Centre in Wroclaw, Poland, due to exertional dyspnea increasing for the last 8 weeks presenting in FC WHO class III at admission. She had been diagnosed with HIV and HCV co-infection in 2000. She might have been infected in the 90s when she was intravenous drug abuser. In 2010, in her second pregnancy, she was diagnosed with AIDS in stage C3. Since then, she has been effectively treated with cART including raltegrawir, lamiwudynę i tenofowir. Currently, her HIV viremia is undetectable and the CD4 lymphocytes count is normal (786 cells/mm3).
Prior to the admission, she was consulted in a emergancy department, where pulmonary embolism was excluded by the means of computed tomography angiography (angio-CT). During her hospital stay in our Department an echocardiographic evaluation was performed, revealing enlargement of the right ventricle (RV) and atrium (RA), mild tricuspid regurgitation (TR) with the right ventricle systolic pressure (RVSP) of 90 mmHg, reversed flow in the inferior vena cava and assessed mean pulmonary artery pressure (mPAP) of 40 – 45 mmHg. Left ventricle diastolic dysfunction or congenital heart disease were also excluded by the echo study. Right heart catheterization (RHC) confirmed precapillary pulmonary hypertension with the mPAP of 55 mmHg, capillary wedge pressure (PCWP) of 6 mmHg, pulmonary vascular resistance (PVR) of 14,7 Wood units and cardiac output (CO) of 2,06 l/min/m2. Acute pulmonary arteries reactivity test with the use of iloprost was negative.
Ostra próba hemodynamiczna z użyciem iloprostu dała wynik ujemny. Eventaully, she was diagnosed with PAH in the course of HIV infection and was put on sildenafil 75 mg per day. Significant improvement of her clinical status was observed in the follow-up. She was considered to be in FC WHO class II. She returned to work. Unfortunately, after 2 months she begun to deteriorate and now she is in FC WHO class III and requires additional PAH specific agent.

Current guidelines
In the European Society of Cardiology guidelines regarding diagnosing and treatment of PAH (2009) the problem of limited data related to the treatment of HIV-PAH patients was raised. Due to the threat of developing an interaction with cART agents, like HIV protease inhibitors particularly, the use of sildenafil may be limited. BREATHE-4 trial showed improvement in 6-minutes walking test, FC WHO and hemodynamic parameters in HIV-PAH patients treated with bosentan and cART. Additionally, ARIES meta-analysis proves, that treatment with ambrisentan of HIV-PAH patients is well tolerated and provides increase of exercises capacity, relieves dyspnea and improves FC WHO.

1. Sitbon O. HIV-related pulmonary arterial hypertension: clinical presentation and managem ent. AIDS. 2008 Sep;22 Suppl 3:S55-62.
2. Degano B et al. Pulmonary arterial hypertension and HIV infection. Semin Respir Crit Care Med. 2009 Aug;30(4):440-7.
3. Sitbon O et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med. 2008 Jan 1;177(1):108-13.
4. Degano B et al. Long-term effects of bosentan in patients with HIV-associated pulmonary arterial hypertension. Eur Respir J. 2009 Jan;33(1):92-8.
5. Barbaro G et al. Highly active antiretroviral therapy compared with HAART and bosentan in combination in patients with HIV-associated pulmonary hypertension. Heart 2006; 92: 1164–1166.
6. Sitbon O et al. Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2004; 170: 1212–1217.


Expert’s comments:(Written authorization required from each expert)

1. Grzegorz Kopeć MD, PhD
Development of pulmonary arterial hypertension (PAH) in the course of HIV compromises the prognosis for these patients. Although, data on treatment with various PAH specific agents is limited, its initiation and continuing is necessary, in order to improve the outcome. It is recommended to seek for the NFZ Programme for the treatment of PAH funding. Escalation of the sildenafil treatment is not recommended now. There shall not be contraindications for the use of endothelin receptor blockers. Prostacyclins should be left as an alternative option.

2. Prof. Piotr Podolec, MD, PhD
Treatment of pulmonary arterial hypertension is a very important issue in this patient. However, close management of HIV infection together with HCV monitoring is crucial. Combination of these two infections affects the prognosis. Close monitoring of the HCV and HIV viremia is mandatory together with liver function. She should be under constant infectious diseases centre control.

Expert’s conclusions:
This patient should be treated with stage II PAH specific agents similarly to patients with other forms of PAH. Preferable drug should be endothelin receptor blocker. It is advisable to apply to National Health Service (NFZ) for an individual therapy funding, since she does no fulfill the NFZ criteria for the treatment of PAH. HCV antiviral therapy should be considered in this patient.

Michał Furdal1, Ewa Mroczek1, Jakub Stępniewski1

prof. Piotr Podolec, dr Grzegorz Kopeć.

1Department of Cardiology, Regional Hospital in Wroclaw, Poland


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