PH and PAH in Middle- to Low-Income Regions
CBSMD

1. Challenges and special aspects related to the management and prognosis of pulmonary hypertension (PH) in middle- to low-income regions (MLIRs, gross national product <10,000 US dollars per capita) range from late presentation to comorbidities, lack of resources and expertise, cost, and rare options of lung transplantation.

2. PH in MLIRs is under-recognized; PAH due to CHD/HIV/schistosomiasis and group 2 PH (left heart disease). The overall burden of PH in MLIRs countries is several times higher than that in HIRs.

3. These challenges range from lack of resources to cost of care, limited expertise, unpredictable availability of medications, and the extremely rare option of lung transplant.

4. The disease spectrum is further complicated by late presentation and coexisting comorbidities (i.e., infections, malnutrition, and hypercoagulability).

5. This consensus statement does not replace but must be seen as supplementary to previously published recommendations and guidelines by the European Society of Cardiology (ESC) and European Respiratory Society, the American Heart Association/American Thoracic Society, the publications produced by the World Symposium on Pulmonary Hypertension (WSPH) 2018 and the European Pediatric Pulmonary Vascular Disease Network (EPPVDN).
6. The current PH registries in MLIRs have minimal information on patients <18 years of age; thus, the suggested recommendations on the care of children with PH in this document are an extrapolation from both adults with PH in MLIRs and children with PH in HIRs.

7. The standardized classification of PH according to WHO applies to all the regions of the world.

Figure 1. Algorithm for the Diagnostic Work-Up of Suspected or Confirmed Pulmonary Hypertension in MLIRs Without Access to Cardiac Catheterization

8. PH registries and intensified collaborations between MLIRs are required.

Pre-reading article "Advances in Chronic Thromboembolic Pulmonary Hypertension"

Pre-reading article "The 6th World Symposium on PH: Hemodynamic Definitions and Updated Clinical Classification of PH"