Background: Chronic thromboembolic pulmonary hypertension (CTEPH) results in a progressively worsening course associated with substantial morbidity and mortality. The purpose of this comprehensive study was to determine the clinical efficacy of targeted therapeutic interventions for this disease.
Methods: We searched Medline, Embase, Cochrane databases and Pubmed for relevant clinical studies. Randomized controlled trials comparing the effects of targeted treatments to control in CTEPH population were included. Pooled estimates were calculated using a random effect model. Heterogeneity was determined using the I2 statistic.
Results: This analysis included 6 studies with a total of 565 patients. We found that targeted treatments approved for pulmonary arterial hypertension (PAH) were associated with a larger improvement in exercise capacity, haemodynamic parameters, functional status and clinical symptom. There were no statistically significant differences associated with targeted treatments compared with control in all-cause mortality and safety outcomes.
Conclusions: This is the first systematic review and meta-analysis of randomized controlled trials revealing a positive role of PAH-targeted therapies in CTEPH. Future larger randomized trials with a focus on long-term clinical outcomes are urgently needed.
Keywords: CTEPH; PAH-targeted therapies; pooled analysis; pulmonary.
© 2019 John Wiley & Sons Ltd.