ArticlePulmonary circulation2017
Pulmonary arterial hypertension in the USA: an epidemiological study in a large insured pediatric population.
Article in Pulmonary circulation, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 48 papers, 3 of them syntheses that pooled it.
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Who cites it
48 citing papers in PubMed, 3 syntheses or guidelines pooled it.
- Parenteral treprostinil in paediatric pulmonary arterial hypertension: a systematic review and meta-analysis.European respiratory review : an official journal of the European Respiratory Society · 2026Pooled it
- Interventional Strategies for Children with Progressive Pulmonary Hypertension Despite Optimal Therapy: An Official American Thoracic Society Clinical Practice Guideline.American journal of respiratory and critical care medicine · 2025Guideline
- Efficacy and safety of endothelin receptor antagonists, phosphodiesterase type 5 Inhibitors, and prostaglandins in pediatric pulmonary arterial hypertension: A network meta-analysis.Frontiers in cardiovascular medicine · 2022Pooled it
- Article
- The Role of Genetics in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.Pediatric cardiology · 2026Observational
- Trends and projections of the global burden of pulmonary arterial hypertension (1990-2050): An observational trend study.Medicine · 2025Observational
- Expert consensus on the off-label use of drugs for pediatric rare diseases in China (2025 edition).Translational pediatrics · 2025Review
- Targeting CircNLRP12 attenuates hypoxia-induced pulmonary arterial smooth muscle cell dysfunction by sponging miR-107-5p and suppressing the ITGA2-mediated FAK/PI3K/AKT pathway.European journal of medical research · 2025Article
- Pediatric pulmonary arterial hypertension: global epidemiology and disease burden during the period 1990 to 2021.Frontiers in cardiovascular medicine · 2025Article
- A study on the genetic comorbidity between autoimmune diseases and pulmonary hypertension: an observational study and POST-GWAS analysis.International journal of medical sciences · 2025Observational
- Transcatheter Ductus Arteriosus Stenting for Acute Pediatric Pulmonary Arterial Hypertension is Associated with Improved Right Ventricular Echocardiography Strain.Pediatric cardiology · 2024Article
- Pulmonary Hypertension in Children.Clinics in chest medicine · 2024Review
- Early identification of SOX17 deficiency in infants to guide management of heritable pulmonary arterial hypertension using PDA stent to create reverse Potts shunt physiology.Pulmonary circulation · 2024Article
- Pulmonary Hypertension and Hypothyroidism-Still an Important Clinical Coincidence in Paediatric Population, an Endocrinologist's Point of View.Life (Basel, Switzerland) · 2024Review
- Pulmonary hypertension.Nature reviews. Disease primers · 2024Review
- The novel roles of YULINK in the migration, proliferation and glycolysis of pulmonary arterial smooth muscle cells: implications for pulmonary arterial hypertension.Biological research · 2023Article
- Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects.Journal of cardiovascular development and disease · 2023Review
- Wildfire smoke exposure and early childhood respiratory health: a study of prescription claims data.Environmental health : a global access science source · 2023Article
- Monitoring of Hemodynamics With Right Heart Catheterization in Children With Pulmonary Arterial Hypertension.Journal of the American Heart Association · 2023Article
- An intronic variant in TBX4 in a single family with variable and severe pulmonary manifestations.NPJ genomic medicine · 2023Article
Corrections and comments
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Authors and funding
6 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pulmonary arterial hypertension (PAH) is rare in children and few data are available in a pediatric general population. This study aims to calculate the annual incidence and prevalence of PAH and to describe these children in a large US population of patients aged under 18 years. Using the US MarketScan claims database we identified 695 children with PAH in 2010-2013. We calculated annual incidence rates and prevalence overall, by age and PAH type (idiopathic and non-idiopathic) using Byar's method. We also described characteristics, co-morbidities, treatment patterns, and diagnostic procedures for these children. In 2010-2013, the annual incidence rates of PAH per 1,000,000 children-years was in the range of 4.8-8.1; 0.5-0.9 for idiopathic PAH and 4.3-7.3 for non-idiopathic PAH. The annual prevalence of PAH was in the range of 25.7-32.6 per 1,000,000 children; 4.4-6.0 for idiopathic PAH and 21.3-27.0 for non-idiopathic PAH. Incidence rates and prevalence were highest in children under age 2 years. Around 36% of affected children were born prematurely. Most (75%) had some type of congenital heart defect and 13% had Down's syndrome. Most patients received PAH monotherapy (83%), while 13% received dual therapy. Phosphodiesterase type 5 inhibitors were the most commonly used treatments. Around 92% had at least one echocardiogram and 37% a right heart catheterization. PAH is very rare in children especially in the absence of etiological factors such as congenital heart defects. A large proportion of diagnoses in children seem to be based on echocardiography rather than right heart catheterization.
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