Hospital Variation in Mortality and Ventilator Management among Mechanically Ventilated Patients with ARDS.

RATIONALE: Acute Respiratory Distress Syndrome (ARDS) is associated with significant mortality. Despite the mortality benefits of lung protective ventilation, adherence rates to evidence-based ventilator practice have remained low and ARDS mortality has remained high. OBJECTIVE: Determine variation in ARDS mortality and adherence to low tidal volume ventilation (LTV) across US hospitals. MATERIALS AND METHODS: We identified mechanically ventilated patients with ARDS using data from Philips eICU (2014-2015). We then used multi-variable hierarchical logistic regression models with hospital site as the random effect and patient and hospital level factors as fixed effects to assess the hospital risk adjusted mortality rate and median odds ratio for the association between mortality and hospital site. We then assessed associations between adherence to LTV (defined as 4-8 mL/kg PBW) and hospital risk adjusted mortality rates using Spearman correlation. RESULTS: Among 4441 patients admitted at 110 hospitals with ARDS, the hospital risk-adjusted mortality rate ranged from 19% to 39%, and the MOR for hospital of admission was 1.33 (95% CI 1.25-1.41). Among 3070 patients at 72 hospitals with available ventilator data, 73% of patients had a median set Vt between 4 to 8 mL/kg PBW; hospital adherence rates to LTV ranged from 13% to 95%. There was no association between hospital adherence to LTV and risk-adjusted mortality rate (spearman correlation coefficient -0.01, p = .93). Similarly, among 956 patients who started with a Vt > 8 mL/kg PBW, there was no association between the percent of patients at each hospital whose Vt was decreased to ≤ 8 mL/kg PBW and risk adjusted mortality rate (spearman correlation coefficient .05, p = .73). CONCLUSION: Risk adjusted mortality and use of LTV for patients with ARDS varied widely across hospitals. However, hospital adherence to LTV was not associated with ARDS mortality rates. Further evaluation of hospital practices associated with lower ARDS mortality are warranted.

A novel BMPR2 mutation with widely disparate heritable pulmonary arterial hypertension clinical phenotype.

Mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2) have been associated with heritable pulmonary arterial hypertension (HPAH), whereas mutations in the gene encoding eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) are associated with heritable pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (HPVOD/PCH). We describe two unrelated patients found to carry the same hitherto unreported pathogenic BMPR2 mutation; one of whom presented with typical pulmonary arterial hypertension, whereas the second patient presented with aggressive disease and characteristic clinical features of PVOD/PCH. These two clinically divergent cases representative of the same novel pathogenic mutation exemplify the variable phenotype of HPAH and the variable involvement of venules and capillaries in the pathology of the pulmonary vascular bed in pulmonary arterial hypertension.