EPPVDN Expert Consensus Statement on the Diagnosis and Treatment of Paediatric Pulmonary Hypertension

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Published 1 May 2016

Georg Hansmann, Christian Apitz, Hashim Abdul-Khaliq, Tero-Pekka Alastalo, Phillip Beerbaum, Damien Bonnet, Karl-Otto Dubowy, Matthias Gorenflo, Alfred Hager, Anne Hilgendorff, Michael Kaestner, Martin Koestenberger, Juha W Koskenvuo4,5, Rainer Kozlik-Feldmann12, Titus Kuehne, Astrid E Lammers, Heiner Latus, Ina Michel-Behnke, Oliver Miera, Shahin Moledina, Vivek Muthurangu, Joseph Pattathu, Dietmar Schranz, Gregor Warnecke, Peter Zartner

Heart. 2016 May;102(2)

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European Pediatric Pulmonary Vascular Disease Network (EPPVDN)

The European Paediatric Pulmonary Vascular Disease (PVD) Network is a registered, non-profit organisation that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of paediatric pulmonary hypertensive vascular disease, including specific forms such as pulmonary arterial hypertension (PAH)-congenital heart disease, pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, persistent PH of the newborn, and related cardiac dysfunction.

This collection of documents seeks to establish a framework that better defines the course of paediatric PH, identifies current gaps in our knowledge and provides a consensus statement with practical recommendation on how to best manage paediatric PH in diverse settings, acknowledging that most recommendations will have level of evidence (LOE) B or C. A total of 9 original consensus articles with graded recommendations (COR/LOE) were developed, including diagnosis/monitoring, genetics/biomarker, cardiac catheterisation, echocardiography, cardiac magnetic resonance/chest CT, associated forms of PH, intensive care unit/ventricular assist device/lung transplantation, and treatment of paediatric PAH.

This supplement was endorsed by ISHLT in 2016.

Read at Heart

The ten individual documents that make up the full Expert Consensus Statement on the Diagnosis and Treatment of Paediatric Pulmonary Hypertension are listed below.

  1. Diagnostics, monitoring and outpatient care in children with suspected pulmonary hypertension/paediatric pulmonary hypertensive vascular disease
  2.  Transthoracic echocardiography for the evaluation of children and adolescents with suspected or confirmed pulmonary hypertension
  3. Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary vascular disease
  4. Cardiac MR and CT imaging in children with suspected or confirmed pulmonary hypertension/pulmonary hypertensive vascular disease
  5. Genetic testing and blood biomarkers in paediatric pulmonary hypertension
  6. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD)
  7. Pulmonary hypertension associated with acute or chronic lung diseases in the preterm and term neonate and infant
  8. Pulmonary hypertension in the intensive care unit
  9. Treatment of children with pulmonary hypertension
  10. Executive summary

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