Objective To describe temporal trends in the time interval between first and second attempts at defibrillation and the association between this time interval and outcomes in patients with persistent ventricular tachycardia or ventricular fibrillation (VT/VF) arrest in hospital.Design Retrospective cohort studySetting 172 hospitals in the United States participating in the Get With The Guidelines-Resuscitation registry, 2004-12.Participants Adults who received a second defibrillation attempt for persistent VT/VF arrest within three minutes of a first attempt.Interventions Second defibrillation attempts categorized as early (time interval of up to and including one minute between first and second defibrillation attempts) or deferred (time interval of more than one minute between first and second defibrillation attempts).Main outcome measure Survival to hospital discharge.Results Among 2733 patients with persistent VT/VF after the first defibrillation attempt, 1121 (41%) received a deferred second attempt. Deferred second defibrillation for persistent VT/VF increased from 26% in 2004 to 57% in 2012 (P<0.001 for trend). Compared with early second defibrillation, unadjusted patient outcomes were significantly worse with deferred second defibrillation (57.4% v 62.5% for return of spontaneous circulation, 38.4% v 43.6% for survival to 24 hours, and 24.7% v 30.8% for survival to hospital discharge; P<0.01 for all comparisons). After risk adjustment, deferred second defibrillation was not associated with survival to hospital discharge (propensity weighting adjusted risk ratio 0.89, 95% confidence interval 0.78 to 1.01; P=0.08; hierarchical regression adjusted 0.92, 0.83 to 1.02; P=0.1).Conclusions Since 2004, the use of deferred second defibrillation for persistent VT/VF in hospital has doubled. Deferred second defibrillation was not associated with improved survival.
Pulmonary arterial hypertension (PAH) is a known significant complication of connective tissue diseases (CTDs) and patients with PAH associated with systemic sclerosis (SSc-PAH) have the highest mortality rates in PAH. The idiopathic inflammatory myopathies are a heterogenous group of CTDs that have been rarely associated with PAH and are often underappreciated by clinicians. We describe the clinical characteristics, treatment response and survival of myositis-associated PAH (MA-PAH) in the era of pulmonary vasodilators and compared to SSc-PAH.
Rationale Pulmonary arterial hypertension (PAH) is a known, clinically significant complication of connective tissue diseases (CTDs), and patients with PAH linked to systemic sclerosis (SSc-PAH) have been found to have the highest mortality rates in PAH. The idiopathic inflammatory myopathies are a heterogenous group of CTDs that have been rarely associated with PAH and are often underappreciated by clinicians, and the clinical characteristics and overall prognosis of myositis-associated PAH (MA-PAH) has yet to be fully described. Methods We conducted a single-center, retrospective longitudinal multiple cohort study to investigate the clinical features, treatment responses, and survival of a previously under-reported cohort of CTD-PAH patients with myositis-associated autoantibodies compared to more traditional PAH subgroups of idiopathic pulmonary arterial hypertension (IPAH) and SSc-PAH patients. Patients with PAH confirmed by cardiac catheterization were enrolled from August 2012 to December 2018, and patients were classified based on clinical and serologic data (including myositis autoantibody testing). Patient characteristics were compared between groups using the Chi-square test and one-way ANOVA. Survival analysis was performed utilizing the Kaplan-Meier method and the log-rank test. Results 22 MA-PAH patients were compared to 68 IPAH and 42 SSc-PAH patients. The mean age of the overall cohort was 58 years old, and 104 (79%) of patients were female. IPAH patients were less likely to have anemia or chronic kidney disease and had better lung function than MA-PAH and SSc-PAH patients. There were no significant differences in baseline functional capacity or PAH-specific therapies between the three groups. Hemodynamically, IPAH patients had higher mean pulmonary artery pressures (mPAP) relative to MA-PAH patients and SSc-PAH patients, but otherwise had similar hemodynamic profiles. Estimated survival for the MA-PAH cohort was 91%, 71%, and 71% at one year, three years, and five years, respectively, with no statistically significant difference between the three groups. There was a statistically significant difference in survival if only the IPAH and SSc-PAH cohorts were compared (P=0.05). Conclusions Our findings demonstrate that MA-PAH patients clinically and hemodynamically more closely resemble patients with SSc-PAH, but do not have a statistically significant difference in survival. The significant difference in survival between the SSc-PAH and IPAH groups is similar to what has been previously described.
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