Severe Pulmonary Hypertension in Sickle Cell Patients with HIV Infection.
Authors: Onyekwere Onyinye C. , Kato Gregory J. , Machado Roberto , Coles Wynoma , Nichols James , Hunter Lori , Gladwin Mark T. , Castro Oswaldo L. 
Published: October 2019

Pulmonary hypertension (PHTN) occurs in 32% of adult patients with sickle cell disease (SCD) and carries a high mortality risk. PHTN is also a complication of HIV infection in non-SCD patients, with a prevalence of 0.5%, which is about 2500 times greater than that of primary PHTN in the general population. Of 201 SCD patients tested at Howard Sickle Cell Center, 15 were HIV positive. The association of PHTN and HIV infection has never been reported in SCD patients. We recently identified two patients with severe PHTN (PAs 90 mm Hg or higher) and HIV infection among the 240 adult SCD patients prospectively screened for pulmonary hypertension. One is a 50-year-old male with HbSS, PHTN, supraventricular tachycardia, leg ulcer, transfusional hemosiderosis, hypertension, and mild renal insufficiency. The patient prior to cardiac catheterization was on chronic blood transfusion program for PHTN and frequent vaso-occlusive episodes. His HIV infection screen prior to cardiac catheterization in July of 2003 was positive. His serology had been negative for HIV infection in 1992. The second patient is a 39-year-old female with HbSC, splenectomy secondary to sequestration/hypersplenism, GERD, and chronic pulmonary disease, who was diagnosed with HIV infection in 2000. She has been on antiretroviral medications. Her echocardiogram in June of 2004 revealed a PAs pressure of 85–90 mm Hg. Both patients had cardiac catheterization and the results are shown in the table.

A review of Howard Sickle Cell Center’s records identified a third patient: A 35 year-old male with HBSS, and sickle retinopathy with retinal detachment. His HIV infection was diagnosed in 1986, an ECHO in 1993 showed a PAs of 64 mm Hg, and he died suddenly while hospitalized for a painful event in 1994.

These observations suggest that HIV infection potentiates the risk for PHTN in patients with SCD. Our published studies have indicated that the risk of PHTN in patients with SCD is associated with high LDH, low hemoglobin and high direct bilirubin levels, and is rare in HbSC disease. The first two SCD patients described above stand in contrast to this risk profile, suggesting that HIV infection may add significant risk for PHTN. We recommend that HIV testing be performed in all patients with SCD diagnosed with PHTN, in order to provide appropriate antiretroviral therapy, which might improve control of PHTN.

Clinical data and pulmonary hemodynamics in 2 SCD patients with HIV infection and PHTN, and in 16 sickle patients with PHTN but without HIV infection

HIV Pt. 1 HIV Pt. 2 No HIV N=16 Mean +/− SD PAs, PAd, and PAm are pulmonary artery systolic, diastolic, and mean pressures. PCWP is pulmonary capillary wedge pressure. C.O. is cardiac output. Hemoglobin concentration (g/dl) 8.2 8.6 8.56+/− 23 PAs (mmHg) 65 66 48.75 +/− 12.41 PAd (mmHg) 35 34 24.13 +/− 6.38 PAm (mm Hg) 46 44 32.19 +/− 6.93 PCWP (mm Hg) 14 10 18.13 +/− 4.47 C.O. (L/min) 6.0 7.9 9.63 +/− 2.46 Lactate dehydrogenase (IU/L) 307 211 456 +/− 213 Total bilirubin (mg/dL) 1.3 0.4 3.3 +/− 3.1 Direct bilirubin (mg/dL) 0.4 0.1 0.9 +/− 1.7