ArticlePulmonary circulation2014
Repair of congenital heart disease with associated pulmonary hypertension in children: what are the minimal investigative procedures? Consensus statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI).
Article in Pulmonary circulation, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 23 papers.
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23 citing papers in PubMed, 63 citations in OpenAlex.
- Single-stage surgical repair of congenital aortopulmonary window associated with interrupted aortic arch in two toddlers: a case report.Journal of cardiothoracic surgery · 2026Article
- Congenital Heart Disease and Pulmonary Arterial Hypertension: Current Perspectives.Reviews in cardiovascular medicine · 2026Review
- Late presentation of congenital heart diseases in low- and middle-income countries: impact on outcomes.Indian journal of thoracic and cardiovascular surgery · 2025Review
- Hybrid treat-and-repair strategy for large patent ductus arteriosus: a proof-of-concept case report.European heart journal. Case reports · 2024Article
- Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome.Scientific reports · 2024Article
- Down syndrome and postoperative hemodynamics in patients undergoing surgery for congenital cardiac communications.Scientific reports · 2024Article
- Case report: Cilioretinal artery occlusion combined with central retinal vein occlusion as the initial presentation of pulmonary arterial hypertension.Frontiers in medicine · 2024Article
- Current Management and Future Directions for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease.Journal of personalized medicine · 2023Review
- Pulmonary vascular disease as a complication of pediatric congenital heart diseases.Translational pediatrics · 2023Review
- Pulmonary arterial hypertension in children with congenital heart disease: a deeper look into the role of endothelial progenitor cells and circulating endothelial cells to assess disease severity.Frontiers in pediatrics · 2023Article
- Late presentation of shunt lesions in Down syndrome patients: the importance of multidisciplinary assessment and lifelong follow-up.European heart journal. Case reports · 2021Article
- Pulmonary Hypertension in Children across Africa: The Silent Threat.International journal of pediatrics · 2021Review
- Pulmonary Vascular Resistance Measurement Remains Keystone in Congenital Heart Disease Management.Frontiers in cardiovascular medicine · 2021Review
- Pulmonary vascular disease in Africa: Lessons from registries.Global cardiology science & practice · 2020Review
- EXPRESS: Parameters associated with outcome in pediatric patients with congenital heart disease and pulmonary hypertension subjected to combined vasodilator and surgical treatments.Pulmonary circulation · 2019Article
- When to Operate on Pediatric Patients with Congenital Heart Disease and Pulmonary Hypertension.Arquivos brasileiros de cardiologia · 2017Article
- Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease.Pulmonary circulation · 2016Article
- Cardiac catheterization in children with pulmonary hypertensive vascular disease: consensus statement from the Pulmonary Vascular Research Institute, Pediatric and Congenital Heart Disease Task Forces.Pulmonary circulation · 2016Article
- Case 1/2016--56-Year-Old Male with Atrial Septal Defect, Pulmonary Arterial Hypertension, Hospitalized Due to Eisenmenger Syndrome.Arquivos brasileiros de cardiologia · 2016Article
- Article
Corrections and comments
- Erratum issuedErratum.2014
Authors and funding
31 authors at 20 institutions in 13 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.
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