Publications

RATIONALE

Single-center studies demonstrated that methamphetamine use is associated with pulmonary arterial hypertension (Meth-APAH). We used the Pulmonary Hypertension Association Registry to evaluate the national distribution of Meth-APAH, and to compare its impact on patient-reported and clinical outcomes relative to idiopathic PAH.

OBJECTIVES

To determine if patients with Meth-APAH differ from those with idiopathic PAH in demographics, regional distribution in the US, hemodynamics, health-related quality of life, PAH-specific treatment, and health care utilization.

METHODS

The Pulmonary Hypertension Association Registry is a US-based prospective cohort of patients new to care at a Pulmonary Hypertension Care Center. The registry collects baseline demographics, clinical parameters, and repeated measures of health-related quality of life, World Health Organization functional class, six-minute walk distance, therapy, and health care utilization. Repeated measures of functional class, health-related quality of life, type of therapy, emergency department visits, and hospitalizations were compared using generalized estimating equations.

RESULTS

Of 541 participants included, 118 had Meth-APAH; 83% of Meth-APAH arose in the Western US. The Meth-APAH group was younger, had a poorer socioeconomic status, and lower cardiac index than the idiopathic PAH group, despite no difference in mean pulmonary artery pressure or pulmonary vascular resistance. The Meth-APAH group had a more advanced functional class in longitudinal models (0.22 points greater, 95%CI: 0.07 to 0.37), and worse PAH-specific (emPHasis-10) health-related quality of life (-5.4, 95%CI: -8.1 to -2.8). There was no difference in dual combination therapy, however, participants with Meth-APAH were less likely to be initiated on triple therapy (OR: 0.43, 95%CI 0.24 to 0.77) or parenteral therapy (OR: 0.10, 95%CI 0.04 to 0.24). Participants with Meth-APAH were more likely to seek care in the emergency department (IRR: 2.30, 95%CI 1.71 to 3.11) and more likely to be hospitalized (IRR: 1.42, 95%CI 1.10 to 1.83).

CONCLUSIONS

Meth-APAH represents a unique clinical phenotype of PAH, most common in the Western US. It accounts for a notable proportion of PAH in expert centers. Assessment for methamphetamine use is necessary in patients with PAH.

The safety and efficacy of tocilizumab for the treatment of severe respiratory symptoms due to COVID-19 remain uncertain, in particular among solid organ transplant (SOT) recipients. Thus, we evaluated the clinical characteristics and outcomes of 29 hospitalized SOT recipients who received tocilizumab for severe COVID-19, compared to a matched control group who did not. Among a total of 117 total SOT recipients hospitalized with COVID-19, 29 (24.8%) received tocilizumab. The 90-day mortality was significantly higher among patients who received tocilizumab (41%) compared to those who did not (20%, P = .03). When compared to control patients matched by age, hypertension, chronic kidney disease, and administration of high dose corticosteroids, there was no significant difference in mortality (41% vs 28%, P = .27), hospital discharge (52% vs 72%, P = .26), or secondary infections (34% vs 24%, P = .55). Among patients who received tocilizumab, there was also no difference in mortality based on the level of oxygen support (intubated vs not intubated) at the time of tocilizumab initiation. In this matched cohort study, tocilizumab appeared to be safe but was not associated with decreased 90-day mortality. Larger randomized studies are needed to identify whether there are subsets of SOT recipients who may benefit from tocilizumab for treatment of COVID-19.