Background: Pulmonary angioplasty has been performed in patients with sarcoidosis-associated pulmonary hypertension (SAPH) but most evidence comes from case reports and small case series. Overall outcomes remain unclear. We conducted an individual participant data (IPD) meta-analysis of baseline, procedural, and outcome data of pulmonary angioplasty in patients with SAPH.
Methods: We performed searches and systematically reviewed references from PubMed, Embase, Cochrane, ClinicalTrials.gov, and grey literature. We included IPD of patients who underwent pulmonary angioplasty for SAPH. Those without definitive diagnosis of sarcoidosis or with other causes of pulmonary vascular stenosis or compression were excluded.
Results: Of 1293 screened references, 7 were included. IPD was obtained for 17 patients (mean age 58.6 (±9.1) years; 82.4% female); most of whom were Scadding stages III or IV and had NYHA FC III or IV. All patients with documented changes in 6-minute-walk distance (6MWD) had a significant improvement that ranged from 12.6 to 102.4% (P < 0.01). There were no deaths during a median follow-up of 6 (3-18) months.
Conclusions: Pulmonary angioplasty with or without stenting of focal stenosis or compressions of pulmonary vessels may lead to significant improvement in 6MWD in patients with SAPH. However, this study had a small sample and some methodological limitations, such as analysis mostly of case reports and series. Randomized controlled clinical trials and/or large multicenter registry studies are needed to provide higher evidence in this topic.
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