ArticleEuropean heart journal. Quality of care & clinical outcomes2024
Long-term outcome of children with newly diagnosed pulmonary arterial hypertension: results from the global TOPP registry.
Article in European heart journal. Quality of care & clinical outcomes, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 2 of them syntheses that pooled it.
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The trial behind it
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Who cites it
22 citing papers in PubMed, 2 syntheses or guidelines pooled it, 28 citations in OpenAlex.
- 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
- Survival, morbidity, and quality of life in pulmonary arterial hypertension patients: a systematic review of outcomes reported by population-based observational studies.Respiratory research · 2024Pooled it
- Registry-based estimation of cardiac event-free survival in congenital heart disease complicated by pulmonary hypertension: A nationwide registry study from Japan.International journal of cardiology. Congenital heart disease · 2026Article
- Efficacy, Safety, and Pharmacokinetics of Macitentan in Japanese Pediatric Patients With Pulmonary Arterial Hypertension: Prospective, Multicenter, Open-Label Study.Circulation reports · 2026Article
- Long-Term Subcutaneous Treprostinil in Pediatric Pulmonary Arterial Hypertension at a Latin American Referral Center: A Retrospective Cohort Study.Pulmonary circulation · 2026Article
- Early Results of Sotatercept Treatment in Patients With Severe Pulmonary Hypertension and a Potts Shunt.Pulmonary circulation · 2026Article
- Macitentan in Children with Pulmonary Arterial Hypertension: A Multicenter Experience.Pediatric cardiology · 2026Observational
- Current State of RV Multimodality Imaging in Pediatric Pulmonary Hypertension: Current Evidence, Knowledge Gaps, and Future Research Directions.Circulation. Cardiovascular imaging · 2026Review
- The Incidence of Pulmonary Hypertension and the Association with Bronchopulmonary Dysplasia in Preterm Infants of Extremely Low Gestational Age: Single Centre Study at the Maternity Hospital of University Medical Centre Ljubljana, Slovenia.Children (Basel, Switzerland) · 2025Article
- Microvascular preservation and cardiomyocyte hyperplasia underlie adaptive right ventricle development in congenital heart disease-pulmonary arterial hypertension.American journal of physiology. Heart and circulatory physiology · 2025Article
- Challenges in accurately assessing acute vasoreactivity in paediatric pulmonary arterial hypertension: case reports highlighting the impact of sedation on diagnostic accuracy.European heart journal. Case reports · 2025Article
- Global pulmonary arterial hypertension trends and projections to 2046: a multi-method analysis of epidemiologic and demographic drivers using GBD 2021.Journal of thoracic disease · 2025Article
- Enhancing Drug Development for Paediatric Pulmonary Hypertension-An Integrative Perspective.Pulmonary circulation · 2025Review
- Macitentan: Pediatric First Approval.Paediatric drugs · 2025Review
- Mechanism and Treatment of Right Ventricular Failure Due to Pulmonary Hypertension in Children.Children (Basel, Switzerland) · 2025Review
- Magnetic resonance imaging of pulmonary hypertension.Pediatric radiology · 2025Review
- Missed or Delayed Diagnosis of Heart Disease by the General Pediatrician.Children (Basel, Switzerland) · 2025Review
- Embracing the challenges of neonatal and paediatric pulmonary hypertension.The European respiratory journal · 2024Review
- Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension.Pulmonary circulation · 2024Article
- Application of a modified clinical classification for pulmonary arterial hypertension associated with congenital heart disease in children: emphasis on atrial septal defects and transposition of the great arteries. An analysis from the TOPP registry.Frontiers in cardiovascular medicine · 2024Article
Corrections and comments
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Authors and funding
11 authors at 9 institutions in 9 countries.
Funding
Abstract
BACKGROUND AND
aimsThe Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global network established to gain insights into the disease course and long-term outcomes of paediatric pulmonary arterial hypertension (PAH). Previously published cohorts in paediatric PAH are obscured by survival bias due to the inclusion of both prevalent (previously diagnosed) and incident (newly diagnosed) patients. The current study aims to describe long-term outcome and its predictors in paediatric PAH, exclusively of newly diagnosed patients. METHODS AND
resultsFive hundred thirty-one children with confirmed pulmonary hypertension, aged ≥3 months and <18 years, were enrolled in the real-world TOPP registry at 33 centres in 20 countries, from 2008 to 2015. Of these, 242 children with newly diagnosed PAH with at least one follow-up visit were included in the current outcome analyses. During long-term follow-up, 42 (17.4%) children died, 9 (3.7%) underwent lung transplantation, 3 (1.2%) atrial septostomy, and 9 (3.7%) Potts shunt palliation (event rates: 6.2, 1.3, 0.4, and 1.4 events per 100 person-years, respectively). One-, three-, and five-year survival free from adverse outcome was 83.9%, 75.2%, and 71.8%, respectively.Overall, children with open (unrepaired or residual) cardiac shunts had the best survival rates. Younger age, worse World Health Organization functional class, and higher pulmonary vascular resistance index were identified as independent predictors of long-term adverse outcome. Younger age, higher mean right atrial pressure, and lower systemic venous oxygen saturation were specifically identified as independent predictors of early adverse outcome (within 12 months after enrolment).
conclusionThis comprehensive analysis of survival from time of diagnosis in a large exclusive cohort of children newly diagnosed with PAH describes current-era outcome and its predictors.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.