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Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
The Dutch National Network
for Pulmonary Hypertension
in Childhood
Menno Douwes
Coordinator Dutch Network Paediatric PH
University Medical Center Groningen
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Introduction
• Centralization of care for paediatric PH
– Developing experience in a rare disease
– Expertise for a complex disease
– Standardized care
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
The Dutch Paediatric PH Network
9 Network centres
• Amsterdam AMC
• Amsterdam VUMC
• The Hague Haga Ziekenhuis
• Leiden LUMC
• Maastricht MUMC+
• Nijmegen UMC St Radboud
• Rotterdam EMCR
• Utrecht UMCU
• Veldhoven MMC
1 National PH referral centre
• Groningen UMCG
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
The Dutch Paediatric PH Network
Formed in the ’90s
Patient inclusion since ’94
National coverage ’97
2001 Ministry of Health
advised to centralize
2004 Centre of Expertise
Supported by Dutch
Paediatric Society
2006 Standardization of
care in Netherlands
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
A Dutch child with PH
• Local hospital
– Unexplained dyspnoea
– Episodes of syncope
– Loud 2nd heart sound
Suspected to have PH
Patient at first presentation
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
A Dutch child with PH
PH detection
– ECG
– Thorax X-ray
– Echocardiography
Patient referral to a network centre
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
A Dutch child with PH
• Confirmation diagnosis – Heart catheterization
– Acute response tests
• PH Classification
• Multidisciplinary team
• Initiation of therapy
• Standardized follow-up
Referral to paediatric PH expert centre Groningen
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Dutch network paediatric PH
• Centralization of expertise
• Standardized diagnostic procedures
• Standardized treatment and follow-up
• Education for patients and professionals
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
www.KinderPH.nl
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Dutch network paediatric PH
• Centralization of expertise
• Standardized diagnostic procedures
• Standardized treatment and follow-up
• Education for patients and professionals
• National PH patient cohort
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Epidemiologic data
1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005
year of diagnosis
90
80
70
60
50
40
ann
ual
inci
den
t ca
ses
per
mill
ion
5
4
3
2
1
0
Van Loon RL, Circulation 2011 Oct 18;124(16):1755-64
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Clinical characterization
Van Loon RL, J Pediatr. 2009 Aug;155(2):176-82
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Comorbidities - syndromes
Van Loon RL, J Pediatr. 2009 Aug;155(2):176-82
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Identification associated mutations
Kerstjens-Frederikse WS, J Med Genet. 2013 Aug;50(8):500-6.
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Outcome data
Van Loon RL, Am J Cardiol. 2010 Jul 1;106(1):117-24
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Standardized hemodynamic data
Douwes JM, Eur Heart J. 2011 Dec;32(24):3137-46
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Pulsatile hemodynamic data
Douwes JM, Int J Cardiol. 2013 Jan 19. [Epub ahead of print]
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
• Describes collaboration
within network
• Standardization of
diagnosis and treatment
• First edition in 2006
Dutch standard paediatric PH
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
Dutch standard paediatric PH
• Emerging paediatric data
• Recent insights
• Paediatric task force during
world symposium Nice 2013
• Need for an update of the Dutch Standard
Center for Congenital Heart Disease Beatrix Children’s Hospital
UMC Groningen
