Management and prognosis of HIV-associated pulmonary arterial hypertension: 20 Years of evidence from the REHAP registry.

BACKGROUND: Pulmonary arterial hypertension (PAH) is an independent predictor of death in patients with human immunodeficiency virus (HIV) infection. HIV is the leading cause of PAH (HIV-PAH) worldwide. AIMS: We described the characteristics, treatment patterns, and prognosis of a cohort of HIV-PAH patients and compared them with those of an equivalent cohort of patients with idiopathic/familial PAH (IPAH/FPAH). METHODS: We retrospectively analysed and compared the demographic, clinical, and treatment data from patients with HIV-PAH and those with IPAH/FPAH in the Spanish PAH registry (REHAP) from 1998 to 2018. The HIV-PAH overall survival (OS) rate up to 5 years was compared to the age- and sex-matched IPAH/FPAH population. Changes in treatment patterns in patients with HIV-PAH after 2010 and their effects on OS were also analysed. RESULTS: Compared to those with IPAH/FPAH (n = 739), patients with HIV-PAH (n = 132) were younger, mainly men, and had a better functional status. The clinical presentation, haemodynamics, and respiratory function were similar between the groups. Parenteral drug use was the most common mode of HIV transmission. Approximately 11% of patients with HIV-PAH did not receive PAH-targeted therapy. The age- and sex-adjusted 5-year OS rate from diagnosis was 74.0% for patients with HIV-PAH and 68.7% for those with IPAH (p < 0.159). During/after 2010, 23% of patients with IPAH/FPAH received upfront dual oral combination, while oral monotherapy remained the main first-line treatment in patients with HIV-PAH. The overall OS rate remained stable. CONCLUSIONS: Patients with HIV-PAH were predominantly young men. The short-term prognosis is similar to that of age- and sex-matched patients with IPAH/FPAH, despite a better functional status. Oral monotherapy remains the preferred first-line treatment in the current cohorts.

From Health-Related Quality of Life (HRQoL) of Patients with Pulmonary Hypertension to Patient Experience with the Care Received: Should We Be More Aware of Current Patient Needs?

INTRODUCTION: Self-perceived health-related quality of life (HRQoL) of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) and their experience with the care received are important for improving their management. We conducted a study to assess both patient-reported outcomes (PROs) and how they interrelate. METHODS: This was a cross-sectional, observational study of consecutive patients with PAH and CTEPH attending pulmonary hypertension (PH)-specialized units at 25 hospitals in Spain. PRO measurements used included CAMPHOR/EQ-5D-5L questionnaires (HRQoL) and IEXPAC (healthcare experience). Patient characteristics were collected. Relationships were analysed with Pearson's correlation coefficient and linear regression analyses. RESULTS: A total of 185 patients with PAH and 93 patients with CTEPH aged 54.4 ± 14.4 and 64.8 ± 13.4 years were included: 63.6% and 72% were functional class (FC) I-II; median time from diagnosis was 3 and 2 years, respectively. Most patients with PAH received combination oral therapy. CAMPHOR scores indicated moderate-to-high impairment in the "activity" scale (PAH 21.6 ± 6.8; CTEPH 21.0 ± 6.3). EQ-5D-5L index and visual analogue scale (VAS) score (PAH 0.59 ± 0.15 and 65.55 ± 21.54; CTEPH 0.59 ± 0.13 and 66.95 ± 18.71, respectively) indicated moderate HRQoL impairment. HRQoL was mostly affected by FC. IEXPAC scores (PAH 7.08 ± 1.56 and CTEPH 7.13 ± 1.61) indicated good healthcare experience. In patients with PAH, the CAMPHOR "symptom" and "QoL" domains inversely correlated with the IEXPAC "patient self-management" factor. CONCLUSION: In patients with long-standing PAH and CTEPH with good disease control, functional limitations greatly impact HRQoL while symptoms and generic QoL were less affected. Healthcare received was perceived as good; however, use of information and communication technologies, patient associations and promotion of self-management should improve for enhanced patient experience.

[Noninvasive, indirect measurement of pulmonary artery pressure]. / Determinación indirecta y no invasiva de la presión arterial pulmonar.

OBJECTIVE: The gold standard for measuring pulmonary artery pressure (PAP) is right heart catheterization. However, the usefulness of this technique is limited in patients with lung disease because of a poor acoustic window. The aim of this study was to assess PAP in a group of healthy persons using a noninvasive, indirect measurement procedure derived from the venous return flow velocity wave. MATERIAL AND METHODS: Twenty-three healthy volunteers with a mean (SD) age of 34 (9) years were evaluated. Measurements were made with a Hitachi EUB 5000 ultrasound device (3.5 MHz wave). The subject was placed in supine decubitus with neck hyperextended and head turned to one side. B-mode images were used to locate the point in the internal jugular vein 1 cm higher than its union with the subclavian vein. A color flow image was obtained of the vessel and a pulsed wave was then used to measure velocities. After 5 identical cycles, an estimate of PAP was derived from the ratio of maximum diastolic and systolic flow rates. RESULTS: On the right side, the mean (SD) ratio between flow rates was 0.50 (0.08), corresponding to a mean PAP of 15 (2.4) mm Hg. On the left side, the ratio was 0.55 (0.09), corresponding to a mean PAP of 16 (2.8) mm Hg. The nonsignificant difference between mean PAP values on the right and left was 1.3 (3.1) mm Hg, and higher values were on the left. CONCLUSIONS: Mean PAP values obtained with the described procedure are within the normal range for a healthy population. Transcutaneous venous Doppler ultrasound is useful for estimating PAP in healthy individuals.