Abstract
Background: Cancer and pulmonary circulation disorders represent increasingly intersecting clinical entities. The prevalence of malignancy in patients with pulmonary hypertension (PH), particularly those with chronic thromboembolic pulmonary hypertension (CTEPH), is higher than in the general population. Moreover, cancer and antineoplastic therapies have been implicated in the development of PH through multiple mechanisms. Methods: We performed a systematic review and meta-analysis of the literature focusing on the prevalence of cancer in patients with PH. Mortality incidence and mortality risk were also evaluated for patients with PH with or without cancer. Specific sub-analyses for patients with CTEPH were also performed. Finally, we evaluated the prevalence of PH and its risk of mortality in patients with cancer. Results: Overall, 12 studies including 4402 patients were selected in the quantitative analysis. All the included studies had an observational design. The prevalence of cancer in patients with any PH group was 13% (95% CI: 11–16%); mortality incidence in patients with any PH group and cancer was 41% (95% CI: 26–58%), compared to 10% (95% CI: 1–48%) in those without cancer. The association was even stronger when considering only patients with CTEPH, with a mortality incidence of 4% (95% CI: 2–9%) in those without cancer compared to 19% (95% CI: 8–37%) in patients with cancer (p for difference: <0.01). Finally, prevalence of any PH group in patients with cancer was 22% (95% CI: 15–31%). Conclusions: We observed a possible correlation between PH and cancer, with a significant impact on mortality in patients with PH, particularly those with CTEPH. This association suggests the need for a close clinical surveillance for early detection of cancer and PH.