Author + information
- Received February 26, 2020
- Revision received March 19, 2020
- Accepted March 23, 2020
- Published online May 11, 2020.
- Babar Hasan, MBBSa,∗ (, )
- Georg Hansmann, MD, PhDb,∗∗ (, )@pvd_network,
- Werner Budts, MD, PhDc,
- Alexandra Heath, MD, PhDd,
- Zahra Hoodbhoy, MBBSa,
- Zhi-Cheng Jing, MD, PhDe,
- Martin Koestenberger, MDf,
- Katharina Meinel, MDf,
- Ana Olga Mocumbi, MD, PhDg,
- Ganna D. Radchenko, MD, PhDh,
- Hannes Sallmon, MDi,
- Karen Sliwa, MD, PhDj,
- R. Krishna Kumar, MDk,
- on behalf of the European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, CSC, PASCAR, PCS, and PCSI
- aDepartment of Pediatrics and Child Health, The Aga Khan University, Karachi, Pakistan
- bDepartment of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany
- cCongenital and Structural Cardiology, University Hospitals Leuven, Leuven, Belgium
- dDivision of Pediatric Cardiology, Kardiozentrum, La Paz, Bolivia
- eDepartment of Cardiology, Peking Union Medical College Hospital & Key Lab of Pulmonary Vascular Medicine, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- fDivision of Pediatric Cardiology, Department of Pediatrics, Medical University Graz, Graz, Austria
- gInstituto Nacional de Saúde, Vila de Marracuene, Maputo, Mozambique
- hSecondary Hypertension Department with Pulmonary Hypertension Center, State Institute National Scientific Center, MD Strazhesko Institute of Cardiology of Ukrainian National Academy of Medical Science, and Bogomolets National Medical University, Kyiv, Ukraine
- iDepartment of Pediatric Cardiology, Charité University Medical Center, Berlin, Germany
- jHatter Institute for Cardiovascular Research in Africa, Departments of Medicine and Cardiology, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa
- kDepartment of Pediatric Cardiology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
- ↵∗Address for correspondence:
Dr. Babar S. Hasan, Department of Pediatric and Child Health, Stadium Road, P.O. Box 3500, The Aga Khan University Hospital, Karachi, Pakistan.
- ↵∗∗Dr. Georg Hansmann, Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Carl-Neuberg-Str. 1, 30625 Hannover, Germany.
• PH in MLIRs is under-recognized; PAH due to CHD/HIV/schistosomiasis and group 2 PH (left heart disease) are more prevalent in MLIRs than in HIRs.
• Unmet need for specific, feasible recommendations for the diagnosis and treatment of PH in MLIRs.
• Here, an expert consensus panel proposes such recommendations for the global management of PH in MLIRs.
• PH registries and intensified collaborations between MLIRs are required.
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.
This research received no specific grant from any funding agency or industry, except for travel grants from the not-for-profit European Pediatric Pulmonary Vascular Disease Network. Dr. Hansmann was supported by the German Research Foundation (KFO311 HA4348/6-2). Dr. Budts has served on the Speakers Bureau, received honoraria as a consultant, and has served on the Advisory Board for Abbott (moderate) and Occlutech (moderate). Dr. Heath own shares of PFM S.R.L. (none of the products are related to this work). Dr. Koestenberger has served on the Advisory Board for Johnson & Johnson and Actelion (moderate). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Endorsing scientific societies: AEPC = Association for European Paediatric and Congenital Cardiology; CSC = Chinese Society of Cardiology; PASCAR = Pan African Society of Cardiology; PCS = Pakistan Cardiac Society; PCSI = Pediatric Cardiac Society of India.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC author instructions page.
- Received February 26, 2020.
- Revision received March 19, 2020.
- Accepted March 23, 2020.
- 2020 The Authors
- Central Illustration
- Epidemiology (Disease Burden) and Etiology of PH In MLIRs
- Clinical Presentation and Diagnosis of Patients With PH in MLIRs
- Management of PH in the Context of Financial and Infrastructural Constraints in MLIRs
- Special Therapeutic Considerations in MLIRs
- Expert Recommendations on the Diagnosis and Treatment of PH in MLIRs
- Perspectives for PH Patients in MLIRs
- Summary and Global Perspectives