Outcomes of Pulmonary Arterial Hypertension Are Improved in a Specialty Care Center.

BACKGROUND: Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressures and is managed by vasodilator therapies. Current guidelines encourage PAH management in specialty care centers (SCCs), but evidence is sparse regarding improvement in clinical outcomes and correlation to vasodilator use with referral. RESEARCH QUESTION: Is PAH management at SCCs associated with improved clinical outcomes? STUDY DESIGNAND METHODS: A single-center, retrospective study was performed at the University of Pittsburgh Medical Center (UPMC; overseeing 40 hospitals). Patients with PAH were identified between 2008 and 2018 and classified into an SCC or non-SCC cohort. Cox proportional hazard modeling was done to compare for all-cause mortality, as was negative binomial regression modeling for hospitalizations. Vasodilator therapy was included to adjust outcomes. RESULTS: Of 580 patients with PAH at UPMC, 455 (78%) were treated at the SCC, comprising a younger (58.8 vs 64.8 years; P < .001) and more often female (68.4% vs 51.2%; P < .001) population with more comorbidities without differences in race or income. SCC patients demonstrated improved survival (hazard ratio, 0.68; P = .012) and fewer hospitalizations (incidence ratio, 0.54; P < .001), and provided more frequent disease monitoring. Early patient referral to SCC (< 6 months from time of diagnosis) was associated with improved outcomes compared with non-SCC patients. SCC patients were more frequently prescribed vasodilators (P < .001) and carried more diagnostic PAH coding (P < .001). Vasodilators were associated with improved outcomes irrespective of location but without statistical significance when comparing between locations (P > .05). INTERPRETATION: The UPMC SCC demonstrated improved outcomes in mortality and hospitalizations. The SCC benefit was multifactorial, with more frequent vasodilator therapy and disease monitoring. These findings provide robust evidence for early and regular referral of patients with PAH to SCCs.

A meta-analysis of outcomes of catheter-directed thrombolysis for high- and intermediate-risk pulmonary embolism.

OBJECTIVE: During the past few years, there has been a surge in the use of catheter-directed thrombolysis (CDT) for acute pulmonary embolism (PE), in the form of either standard CDT or ultrasound-assisted CDT (usCDT). This is a systematic review and meta-analysis of all published series on contemporary CDT for acute PE seeking to determine their clinical efficacy, stratifying by PE severity and CDT modality. METHODS: A comprehensive MEDLINE and Embase search was performed to identify studies that reported outcomes of CDT for acute PE published from 2009 to July 2017. Outcomes included clinical success (in-hospital survival with stabilization of hemodynamics, without decompensation or any major complication), in-hospital mortality, major bleeding, right ventricular/left ventricular ratio, and Miller score changes after CDT. Meta-analyses assumed random effects. RESULTS: Twenty studies with 1168 patients were included in the meta-analysis. Available for subgroup analysis were 210 patients with high-risk PE and 945 patients with intermediate-risk PE; 181 patients received CDT using a standard multiside hole catheter, and 850 received usCDT. The pooled average right ventricular/left ventricular improvement and Miller score drop after CDT were 0.30 (95% confidence interval [CI], 0.22-0.39) and 8.8 (95% CI, 7.1-10.5). For high-risk PE, the pooled estimate for clinical success was 81.3% (95% CI, 72.5%-89.1%), the 30-day mortality estimate was 8.0% (95% CI, 3.2%-14.0%), and major bleeding was 6.7% (95% CI, 1.0%-15.3%). For intermediate-risk PE, the pooled estimate for clinical success was 97.5% (95% CI, 95.3%-99.1%), the 30-day mortality was 0% (95% CI, 0%-0.5%), and major bleeding was 1.4% (95% CI, 0.3%-2.8%). In high-risk PE, clinical success for CDT and usCDT was 70.8% (95% CI, 53.4%-85.8%) and 83.1% (95% CI, 68.5%-94.5%), respectively. In intermediate-risk PE, clinical success for CDT and usCDT was 95.0% (95% CI, 88.5%-99.2%) and 97.5% (95% CI, 95.0%-99.4%), respectively. CONCLUSIONS: Catheter thrombolysis has high clinical success rates in both high- and intermediate-risk PE, but higher mortality and bleeding rates should be anticipated in high-risk PE. Ultrasound-assisted thrombolysis may be more effective than standard CDT in the higher risk population.

Echocardiographic and hemodynamic predictors of mortality in idiopathic pulmonary fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) can lead to the development of pulmonary hypertension, which is associated with an increased risk of death. In pulmonary arterial hypertension, survival is directly related to the capacity of the right ventricle to adapt to elevated pulmonary vascular load. The relative importance of right ventricular function in IPF is not well understood. Our objective was to evaluate right ventricular echocardiographic and hemodynamic predictors of mortality in a cohort of patients with IPF referred for lung transplant evaluation. METHODS: We performed a retrospective cohort study of 135 patients who met 2011 American Thoracic Society/European Respiratory Society criteria for IPF and who were evaluated for lung transplantation at the Hospital of the University of Pennsylvania. RESULTS: Right ventricle:left ventricle diameter ratio (hazard ratio [HR], 4.5; 95% CI, 1.7-11.9), moderate to severe right atrial and right ventricular dilation (HR, 2.9; 95% CI, 1.4-5.9; and HR, 2.7; 95% CI, 1.4-5.4, respectively) and right ventricular dysfunction (HR, 5.5; 95% CI, 2.6-11.5) were associated with an increased risk of death. Higher pulmonary vascular resistance was also associated with increased mortality (HR per 1 Wood unit, 1.3; 95% CI, 1.1-1.5). These risk factors were independent of age, sex, race, height, weight, FVC, and lung transplantation status. Other hemodynamic indices, such as mean pulmonary artery pressure and cardiac index, were not associated with outcome. CONCLUSIONS: Right-sided heart size and right ventricular dysfunction measured by echocardiography and higher pulmonary vascular resistance by invasive hemodynamic assessment predict mortality in patients with IPF evaluated for lung transplantation.