Hasan B, Hansmann G, Budts W, Heath A, Hoodbhoy Z, Jing ZC, Koestenberger M, Meinel K, Mocumbi AO, Radchenko GD, Sallmon H, Sliwa K, Kumar RK; European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, CSC, PASCAR, PCS, and PCSI.J Am Coll Cardiol. 2020 May 19;75(19):2463-2477. doi: 10.1016/j.jacc.2020.03.047.PMID: 32408981 Free article. Review.
Challenges and special aspects related to the management and prognosis of pulmonary hypertension (PH) in middle- to low-income regions (MLIRs) range from late presentation to comorbidities, lack of resources and expertise, cost, and rare options of lung transplantation. Expert consensus recommendations addressing the specific challenges for prevention and therapy of PH in MLIRs with limited resources have been lacking. To date, 6 MLIR-PH registries containing mostly adult patients with PH exist. Importantly, the global prevalence of PH is much higher in MLIRs compared with high-income regions: group 2 PH (left heart disease), pulmonary arterial hypertension associated with unrepaired congenital heart disease, human immunodeficiency virus, or schistosomiasis are highly prevalent. This consensus statement provides selective, tailored modifications to the current PH guidelines to address the specific challenges faced in MLIRs, resulting in the first pragmatic and cost-effective consensus recommendations for PH care providers, patients, and their families.