ArticleJournal of the American Heart Association2022
Use of Pulmonary Arterial Hypertension Therapies in Patients with a Fontan Circulation: Current Practice Across the United Kingdom.
Article in Journal of the American Heart Association, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
13 citing papers in PubMed, 23 citations in OpenAlex.
- Changes in pharmacotherapy in adults with Fontan circulation and associations with outcomes.International journal of cardiology. Congenital heart disease · 2026Article
- Article
- New perspectives in the management of adults with congenital heart disease: insights from the 2025 ACHD Congress.European heart journal supplements : journal of the European Society of Cardiology · 2026Article
- Pathophysiology of endothelial dysfunction in Fontan circulation: from bench to bedside and back again.Angiogenesis · 2025Review
- The Use of Vasodilator Therapy in Fontan Patients: A Single-Centre Experience.Children (Basel, Switzerland) · 2025Article
- Endothelin-1 in the failing Fontan: pathobiology, precision therapeutics, and future trial design.Frontiers in pediatrics · 2025Review
- The effects of standardized intravenous treprostinil in pulmonary arterial hypertension patients after total cavo-pulmonary connection procedure.European journal of medical research · 2024Article
- Medical Therapy for Heart Failure in Adult Congenital Heart Disease Patients.Structural heart : the journal of the Heart Team · 2024Review
- Article
- A retrospective review of sildenafil in the Australia and New Zealand Fontan registry: indications and treatment responses.International journal of cardiology. Congenital heart disease · 2024Article
- Pulmonary vascular disease as a complication of pediatric congenital heart diseases.Translational pediatrics · 2023Review
- Cardiac Drugs in ACHD Cardiovascular Medicine.Journal of cardiovascular development and disease · 2023Review
- Long-term Outcome of Fontan-Associated Protein-Losing Enteropathy: Treatment Modality and Predictive Factor of Mortality.Korean circulation journal · 2022Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
16 authors at 11 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background The Fontan circulation is a successful operative strategy for abolishing cyanosis and chronic volume overload in patients with congenital heart disease with single ventricle physiology. "Fontan failure" is a major cause of poor quality of life and mortality in these patients. We assessed the number and clinical characteristics of adult patients with Fontan physiology receiving pulmonary arterial hypertension (PAH) therapies across specialist centers in the United Kingdom. Methods and Results We identified all adult patients with a Fontan-type circulation under active follow-up in 10 specialist congenital heart disease centers in England and Scotland between 2009 and 2019. Patients taking PAH therapies were matched to untreated patients. A survey of experts was also performed. Of 1538 patients with Fontan followed in specialist centers, only 76 (4.9%) received PAH therapies during follow-up. The vast majority (90.8%) were treated with a phosphodiesterase-5 inhibitor. In 33% of patients, PAH therapies were started after surgery or during hospital admission. In the matched cohort, treated patients were more likely to be significantly limited, have ascites, have a history of protein-losing enteropathy, or receive loop diuretics (
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
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 OpenQuestion graph.