Research Projects

Immediate past and future research projects include the multipaper expert consensus statement on pediatric PH, normal reference values for non-invasive diagnostics, a survey and subsequent patient registry on children with both chronic (parenchymal) lung disease and pulmonary hypertension (e.g. bronchopulmonary dysplasia). Additional research projects are already in preparation or can be proposed by PVD network members.

Learn more

Publications

Since its establishement in 2015, the PVD Network published the expert consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension in Heart. In 2017, a clinical study enrolling 810 children with and without PH, entitled „Normal reference values and z scores of the pulmonary artery acceleration time in children and its importance for the assessment of pulmonary hypertension“, was published in Circulation Cardiovascular Imaging.

Learn more

Meetings & Workshops

On April 7, 2016, the PVD Network conducted the worldwide first hands-on workshop on pediatric PH. The workshop included an introductory talk on echocardiography by Prof. Martin Köstenberger, hands-on teaching with live cases in the echo and cath labs, an ECMO circuit and specific ICU issues, including iNO and HFOV. Next day our PH Symposium took place in Hannover. Future PH workshops and scientific PH meetings are in preparation.

Learn more

PVD Network Videos

Lorem ipsum dolor sit amet, consetetur sadipscing elitr, sed diam nonumy eirmod tempor invidunt ut labore et dolore magna aliquyam erat, sed diam voluptua. At vero eos et accusam et justo duo dolores et ea rebum. Stet clita kasd gubergren, no sea takimata sanctus est Lorem ipsum dolor sit amet.

For example: Video summary of the PAH-Genetics abstract by Dr. Juha Koskenvuo – presented at the Pulmonary Hypertension Symposium – Update 2016 in Hannover, Germany.

More Videos
More Videos

Research on PH and Heart Failure

Rapid Increase in the Number of Publications on PAH Therapies (1990-2010)

© Gopinath Sutendra and Evangelos D. Michelakis Sci Transl Med 2013;5:208sr5

In the last 25 years, rapid, accelerated knowledge gain due to an increasing number of publications on pathobiology and clinical trials have led to more tailored (“personalized”), goal oriented treatment modalities for PAH. From 2011-2015, new insights into metabolic dysregulation and previously unknown gene mutations, and high impact, high volume, longterm randomized controlled trials (RCT) using combined outcomes such as “first morbidity and mortality”, have been published, leading to the approval of more PAH drugs acting on previously known pathways. However, prospective studies in children and adolescents with PAH are largely lacking (only one RCT published by January, 2016).

The number of publications addressing experimental therapies in PAH began to increase in 1995 after landmark studies in human PAH tissues and randomized controlled clinical PAH trials had been published.

  1. First observation of increased endothelin-1 expression in lungs of PAH patients
  2. First observation of reduced endothelial NOS expression in lungs of PAH patients
  3. First successful randomized clinical trial in PAH (intravenous epoprostenol)
  4. First demonstration of BMPRII mutations in familial PAH
  5. First successful randomized clinical trial in PAH with an oral agent (bosentan)

Molecular Targets of approved Pulmonary Hypertension Therapies (2016)

By 01/2016, more than 12 drugs have been approved for the treatment of adult PAH, all of which are primarily vasodilators. More than 20 randomized clinical trials (RCT) have shown a moderate effect of these agents – based on short-term improvement in functional capacity or longer time to clinical worsening. Hemodynamically, the drugs tend to increase cardiac output but have only little effect on elevated PA pressure and RV dysfunction. Although recent PAH studies on early combination therapy and new targets in classical signaling pathways (guanylate cyclase, IP receptor) have raised great hope for better composite outcomes, until today, no RCT lasting longer than 16 weeks has shown a decrease in mortality as single primary outcome.

Legend: The molecular targets, signaling pathways, and modes of action of approved pulmonary hypertension (PH) therapies. cAMP, cyclic adenosine monophosphate; cGMP, cyclic guanosine phosphodiesterase type 5; PKA, phosphate kinase A; PKG, cGMP-dependent protein kinase; sGC, soluble guanylate cyclase.

© Humbert M, Ghofrani H-A. Thorax 2016; 71: 73–83

PVD Network – Featured Articles

You can open and download the following four featured articles in pdf format, recently published by members of the PVD Network.

„Executive Summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension.“

The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK
Heart, 2016

„Diagnosis of Paediatric PH. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension.“

The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK
Heart, 2016

„Treatment of Paediatric PH. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension.“

The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK
Heart, 2016

Recent Publications of the PVD Network

Heart, May 2016 – Volume 102 – Suppl 2
Read TOC on heart website
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
Astrid E Lammers, Christian Apitz, Peter Zartner, Alfred Hager, Karl-Otto Dubowy, Georg Hansmann
Heart 2016;102:ii1-ii13 doi:10.1136/heartjnl-2015-307792
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
Martin Koestenberger, Christian Apitz, Hashim Abdul-Khaliq, Georg Hansmann
Heart 2016;102:ii14-ii22 doi:10.1136/heartjnl-2014-307200
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
Christian Apitz, Georg Hansmann, Dietmar Schranz
Heart 2016;102:ii23-ii29 doi:10.1136/heartjnl-2014-307340
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
Heiner Latus, Titus Kuehne, Philipp Beerbaum, Christian Apitz, Georg Hansmann, Vivek Muthurangu, Shahin Moledina
Heart 2016;102:ii30-ii35 doi:10.1136/heartjnl-2015-308246
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
Joseph Pattathu, Matthias Gorenflo, Anne Hilgendorff, Juha W Koskenvuo, Christian Apitz, Georg Hansmann, Tero-Pekka Alastalo
Heart 2016;102:ii36-ii41 doi:10.1136/heartjnl-2014-307238
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
Rainer Kozlik-Feldmann, Georg Hansmann, Damien Bonnet, Dietmar Schranz, Christian Apitz, Ina Michel-Behnke
Heart 2016;102:ii42-ii48 doi:10.1136/heartjnl-2015-308378
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
Anne Hilgendorff, Christian Apitz, Damien Bonnet, Georg Hansmann
Heart 2016;102:ii49-ii56 doi:10.1136/heartjnl-2015-308591
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
Michael Kaestner, Dietmar Schranz, Gregor Warnecke, Christian Apitz, Georg Hansmann, Oliver Miera
Heart 2016;102:ii57-ii66 doi:10.1136/heartjnl-2015-307774
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
Georg Hansmann, Christian Apitz
Heart 2016;102:ii67-ii85 doi:10.1136/heartjnl-2015-309103
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network,
endorsed by ISHLT and DGPK
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 Koskenvuo, Rainer Kozlik-Feldmann, 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;102:ii86-ii100 doi:10.1136/heartjnl-2015-309132
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network
Circulation Cardiovascular Imaging, 2017
Martin Koestenberger, MD; Gernot Grangl, MD; Alexander Avian, PhD; Andreas Gamillscheg, MD; Marlene Grillitsch, MD; Gerhard Cvirn, PhD; Ante Burmas, MD, PhD; Georg Hansmann, MD, PhD
Read article (open access)
The European Paediatric Pulmonary Vascular Disease Network
Echocardiography. 2018 Mar 5. doi: 10.1111/echo.13852. [Epub ahead of print] Koestenberger M, Avian A, Sallmon H, Gamillscheg A, Grangl G, Kurath-Koller S, Schweintzger S, Burmas A, Hansmann G
Read article (open access)