Academic Center for Congenital Heart Disease

Last updated: 318 days ago.

The Erasmus MC Centre for Congenital Heart Disease is a clinical and research institute for early diagnosis and optimal medical and invasive treatment of children and adults with congenital heart disease (CHD).

It is one of the 4 nationally acknowledged centers (by VWS) with permission to perform surgical and percutaneous interventions and patients are referred supra-regionally. In this setting we want to achieve the best possible care and treatment for individual patients and their families with a clear focus on research into outcome, quality of life, innovation and new treatment possibilities.

A large clinical program exists, based on strong and focused cooperation, with a clear multidisciplinary decision strategy (Pediatric Cardiology, Cardiology, Cardio-thoracic surgery, Anaesthesiology, Obstetrics & Gynaecology , Clinical genetics). The clinical program includes cardiac surgery, percutaneous interventions and a large outpatient clinic with a yearly increase of 7% per year which also includes advanced imaging (echocardiography, MRI and CT), anesthesiology, clinical genetics consultation, pre-pregnancy counseling and psychosocial care.

Academic Center of Excellence

Research Activities

Our center is one of the leading centers in the world in research in CHD, with great impact on the outcome and quality of life of patients with CHD.

Current projects:

  • Nation-wide project with financial support from the Hartstichting and Stichting Hartekind
  • Prospective worldwide registry on pregnancy and heart disease with financial support from European Society of Cardiology and Stichting Zabawas
  • Rotterdam-Quality of life study: longitudinal study with now 45 years follow up after surgery for CHD with financial support from Thorax Foundation
  • Size Matters: investigating gender differences in aortic pathology with support of ZonMW and Thorax Foundation

Type of

Collaborations

Collaboration with the department of cellular biology for the CellRhytmic study on medication for genetic arrhytmias, extended collaboration with teh department of radiology and neonatology for the study on long term consequences of neonatal pulmonary vascular disease and with the national departments of pediatric cardiology.

Collaboration with dr. Magalie Ladouceur, cardiologist at l'Hopital George Pompidou, Paris and prof. Michael Gatzoulis, cardiologist at The Royal Brompton Hospital in London, with dr. Craig Broberg, cardiologist at Oregon Health and Science University in Portland, USA,

Third project in the worldwide ROPAC collaboration with prof. Mark Johnson and prof. Roger Hall from the United Kingdom.

Epidemiolgical studies concern local and national cohort studies. National databases for children (KinCor) and adults (CONCOR) with congenital heart disease and a large worldwide registry on Pregnancy and cardiac disease (ROPAC) and a Periconception birth cohort on the developmental origin, prediction and prevention of congenital heart defects.

Educational

Contributions

  • The ACE provides education in the Junior med school, Bachelor, Master and PhD phase as well as continuing education for medical specialists
  • A very successful MINOR CHD is organized this year for the 5th time as a shared initiative by the dept. of Pediatric Cardiology, Cardiology and Cardio-thoracic Surgery in collaboration with many other departments. It was scored to be the second best minor of the year.
  • Excellent feedback from SET Q and D-RECT.
  • Specific visitation for surgical and interventional congenital interventions with excellent results. - Echo courses for CHD are organized yearly by the dept. of Cardiology and Pediatric Cardiology.
  • Annual course on Congenital Heart Disease for general pediatricians
  • Annual national multidisciplinary symposium on CHD for Pediatric Cardiologists, Cardiologists and Cardiac Surgeons.
  • Biannual international symposium on aortic pathology, jointly organized by the dept. of cardiology, clinical genetics, cardiothoracic surgery and vascular surgery.
  • Post-doctoral courses for Pediatrics, Cardiologists and Surgeons in this field.
  • International writing committees for clinical guideline development (European Soc of Cardiology)
  • Active international role in education through proctoring
  • Students / fellows from United Kingdom, India, Belgium, Romania, China

New educational activities since the start of the ACE are:

- a new course on congenital echocardiography in collaboration with the Radboud UMC Nijmegen,

- lectures for congresses and symposia from the ESC working groups on echocardiography and congenital cardiology

- public lecture on congenital cardiology for the 50th anniversary of the ThoraxCentrum

- "refereeravond" adult congenital heart disease

Patient

Care Activities

Our ACE provides quaternary care with 100% meeting ROBIJN criteria. The NFU-recognized our ACE as "Center of expertise for rare cardiac conditions" (Centre for congenital heart diseases) Intensive collaboration with LUMC, Leiden (Medical Delta) (dept. of Pediatric Cardiology and Cardio-thoracic surgery and Clinical Genetics) is currently established. Intensive collaboration with Radboud UMC, Nijmegen, (dept. of pediatric cardiology, cardiology and cardiothoracic Surgery) has been established in 2015. Also MUMC is involved in this large collaboration.

This will lead to extending numbers of surgical and interventional therapy for patients with Congenital Heart Disease, thus providing a more solid base to innovative treatment regimens. Further concentration of care is expected, with Erasmus MC as central base. Multidisciplinary clinical pathways ("zorgpaden")) for cardiothoracic surgery and percutaneous interventions are developed and in use. Multidisciplinary meetings ("hartteam") are in place, weekly for adults and for children (cardiologist, external referring cardiologists, cardiothoracic surgeon, radiologist, echocardiography expert etc) and bi-weekly for specialised prepregnancy counselling and pregnant cardiac patients (cardiologist, gynaecologist, anaesthesiologist) and for cardiogenetic pathology (pediatric cardiologist, cardiologist, clinical geneticist, fundamental clinical molecular geneticists, nurse practitioner). Excellent results are available for visitations and audit reports, both for all three departments (Pediatric Cardiology, Cardiology and Cardiothoracic Surgery), and also specifically for audit Congenital heart Disease.

Our unique long-term outcome studies have provided excellent opportunities to improve the care for this specific patient population. Indeed results leading to changes in care management have been implemented.

 

Since the start of the ACE, the collaboration with Radboud UMC Nijmegen has been intensified. Multidisciplinary meetings are held monthly, both for adults and children. Collaboration with MUMC Maastricht is being establised since last 2018.

Societal Relevance to Research, Education and Patient Care

In the Netherlands 1 in 100 children is born with a congenital heart defect. In the 1960s only 15% of the children born with a CHD reached adult age. Nowadays, due to improvements in diagnostic and treatment modalities over 90% of these children reach adult age and many can fully participate in the society. Numbers of these adults are growing from 600 patients in 1990 to over 4000 patients seen regularly in our outpatient clinic nowadays. Our unique long-term outcome studies (longitudinal follow-up after cardiac surgery at young age with in-hospital examination and extensive assessment of quality of life) provide important information on mortality and morbidity but also on quality of life, sports participation and sexual functioning.

This is communicated orally and in written communications to patients, patient organisations and healthcare providers. Innovations in patient care include new minimally invasive surgical treatment and new catheter-based interventions for congenital heart defects (e.g. percutaneous valve implantation).

Substantial improvement have been reached in imaging, both echocardiography, CT and MRI (incl 4D-flow), with considerable reduction in radiation dose. Shared-care information portals and decision aids are being developed and implemented in the care path of patients with CHD or regarding choice of prosthetic heart valve. PIs of this ACE are involved in development of guidelines in the different disciplines.

One PI was the chairperson of the European Society of Cardiology guidelines on Pregnancy and Cardiac disease (2018), participated in the Taskforce to develop the ESC guidelines on endocarditis (2015) and is one of the PIs for the transatlantic guidelines for Turner syndrome (2019). She is also a member of the Taskforce to develop the new guidelines for Adult Congenital Heart Disease (2020).

A specific minor Congenital Heart disease was developed. Specific courses are organised e.g.on echocardiography, hands-on catheterinterventions and on cardiac problems in pregnancy. The Thorax Foundation was established to contribute to the knowledge of (congenital) heart disease in the general population and to involve the community in our research programme and fundraising activities. All these developments are well communicated, often together with the Dutch Heart Foundation, ZonMW and patient organizations.

Viability of Research, Education and Patient Care

We adopted a multidisciplinary approach that greatly facilitates knowledge sharing between the various cardiac and surgical (para)medical disciplines for children and adults. Transition programs are up and running and will continue to provide opportunities for research, specifically but not exclusively long-term follow-up studies of rare conditions from childhood into adulthood.

Many PhD students co-operate with research centers nationally and internationally, and often spend time working abroad as part of their PhD curriculum. Within the different consortia specific talent programs are in place.

At least 2 PhD students started an international career. One PI is currently the president of the working group on Congenital Heart Disease of the European Society of Cardiology (ESC). One PI is chairperson of a worldwide registry on pregnancy and cardiac disease (45 countries participating). Another member is a leading steering-committee member of the AEPC-Psychosocial group, organized the AEPC Psychosocial congress in Rotterdam, 2016, and president of the National dutch organization Pediatric Psychology.PIs are organizing international symposia (aorta pathology, cardiac problems in pregnancy, echocardiography, psychosocial aspects of CHD. Since last year, one PI is member of the organizing committee of EuroGUCH, the international congress of the working group on Congenital Heart Diseaseof the ESC. Another investigator of the ACE is member of the working group on echocardiography of the ESC.

An European Reference Network is currently formed.The staff related to the ACE are leading scientists in their fields, publishing in top ranking journals both in basic and clinical science. Bibliometric network analysis: Bibliometric network analysis is not yet available for this ACE.

Erasmus MC ranks number 1 in the Netherlands with respect to MNCS (1.76) and MNJS (1.53)(2009-2013/14). The normalized impact of the domain Cardiac&Cardiovascular systems in ErasmusMC is 1.64 (scores>1,2 are considered high), representing 7.2% of the ErasmusMC research output.

Key and relevant publications of the last five years

  • Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, Cifkova R, Ferreira R, Foidart J-M, Gibbs JSR, Gohlke-Baerwolf C, Gorenek B, Lung B, Kirby M, Maas AHEM, Morais J, Nihoyannopoulos P, Pieper PG, Presbitero P, Roos-Hesselink JW, Schaufelberger M, Seeland U, Torracca L, Bax J, Auricchio A, Ceconi C, Dean V, Deaton C, Fagard R, Funck-Brentano C, Hasdai D, Hoes A, Knuuti J, Kolh P, McDonagh T, Moulin C, Poldermans D, Popescu BA, Reiner Z, Sechtem U, Sirnes PA, Torbicki A, Vahanian A, Windecker S, Baumgartner H, Aguiar C, Al-Attar N, Garcia AA, Antoniou A, Coman I, Elkayam U, Gomez-Sanchez MA, Gotcheva N, Hilfiker-Kleiner D, Kiss RG, Kitsiou A, Konings KTS, Lip GYH, Manolis A, Mebaaza A, Mintale I, Morice M-C, Mulder BJ, Pasquet A, Price S, Priori SG, Salvador MJ, Shotan A, Silversides CK, Skouby SO, Stein J-I, Tornos P, Vejlstrup N, Walker F, Warnes C. ESC Guidelines on the management of cardiovascular disease during pregnancy: The Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Eur Heart Jl 2011 32:24 (3147-3197)
  • van de Laar MBH, Oldenburg RA, Pals G, Roos-Hesselink JW, de Graaf BM, Verhagen JMA, Hoedemaekers YM, Willemsen R, Severijnen LA, Venselaar H, Vriend G, Pattynama PM, Collée M, Majoor-Krakauer D, Poldermans D, Frohn-Mulder IME, Micha D, Timmermans J, Hilhorst-JHofstee Y, Bierma-Zeinstra SM, Willems PJ, Kros JM, Oei EHG, Oostra BA, Wessels MW, Bertoli-Avella AM. Mutations in SMAD3 in the TGF-β pathway cause a new syndromic form of aortic aneurysms and dissections with early-onset osteoarthritis. Nature Genetics 2011; 43:2, 121-126.
  • Roos-Hesselink JW, Ruys PTE, Stein JI, Thilén U, Webb GD, Niwa K, Kaemmerer H, Baumgartner H, Budts W, Maggioni AP, Tavazzi L, Taha N, Johnson MR. Hall R. Outcome of pregnancy in patients with structural or ischaemic heart disease: Results of a registry of the European Society of Cardiology. Eur H Journal 2013, 34;9: 657-665
  • Van der Linde D, Konings EEM, Slager MA, Witsenburg M, Helbing WA, Takkenberg JJM, Roos-Hesselink JW. Birth prevalence of congenital heart disease worldwide. JACC 2011; 58 (21):2241-2247
  • Eindhoven JA, van den Bosch AE, Jansen PR, Boersma E, Roos-Hesselink JW. The usefulness of brain natriuretic peptide in complex congenital heart disease. J Am Coll Card 2012; 60: 21: 2140-2149
  • van der Zwaan, HB, Helbing WA, McGhie J, Geleijnse ML, Luijnenburg SE, Roos-Hesselink JW, Meijboom FJ. Clinical Value of Real-Time Three-Dimensional Echocardiography for Right Ventricular Quantification in Congenital Heart Disease: Validation With Cardiac Magnetic Resonance Imaging. JASE 2010; 23:134–140
  • Habib G, Lancelotti P, Antunes MJ, Bongiorni MG, Casalta J-P, Del Zotti F, Dulgheru R, El Khoury G, Erba PA, Lung B, Miro JM, Mulder BJM, Plonska-Gosciniak E, Price S, Roos-Hesselink JW, Snygg-Martin U, Thuny F, Tornis Mas P, Vilacosta I, Zamorano JL. 2015 ESC Guidelines for the management of infective endocarditis. The Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC). Eur Heart J 2015; 36(44):3075-3128
  • Van der Bom T, Winter MM, Bouma BJ, Groenink M, Vliegen HW, Pieper PG, VanDijk APJ, Sieswerda GT, Roos-Hesselink JW, Zwinderman AH, Mulder BJM. Effect of valsartan on systemic right ventricular function a double-blind, randomized, placebo-controlled pilot trial. Circulation 2013;22; 123(3): 322-30
  • Eindhoven JA, van den Bosch AE, Ruys TP, Opic P, Cuypers JA, McGhie JS, Witsenburg M, Boersma E, Roos-Hesselink JW. N-terminal proBrain natriuretic peptide and its relation with cardiac function in adult patients with congenital heart disease. J Am Coll Cardiol 2013;24,62 (13):1202-1212
  • Mostafa Mokhles M, Rizopoulos D, Andrinopoulou ER, Bekkers JA, Roos-Hesselink JW, Lesaffre E, Bogers AJJC, Takkenberg JJM. Autograft and pulmonary allograft performance in the second post-operative decade after the Ross procedure: insights from the Rotterdam Prospective Cohort Study. Eur Heart J 2012; 33: 2213-2224
  • Since 2016:
  • Regitz-Zagroshek V, Roos-Hesselink JW, Bauersachs J et al, 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy, European Heart Journal 2018 Sep 7;39(34):3165-3241
  • Gillis E, Kumar AA, Luyckx I, et al, Candidate Gene Resequencing in a Large Bicuspid Aortic Valve-Associated Thoracic Aortic Aneurysm Cohort: SMAD6 as an Important Contributor. Frontiers in Physiology 2017 Jun 13;8:400
  • Baggen VJM, Schut ARW, Cuypers JAAE et al, Prognostic value of left atrial size and function in adults with tetralogy of Fallot. International Journal of Cardiology 2017 Jun 1;236:125-131
  • de Wijs-Meijler DP, Duncker DJ, Tibboel D, et al, Oxidative injury of the pulmonary circulation in the perinatal period: Short- and long-term consequences for the human cardiopulmonary system. Pulmonary Circulation 2017 Jan 1;7(1):55-66
  • Baggen, VJM, van den Bosch AE, Eindhoven JA, et al, Prognostic Value of N-Terminal Pro-B-Type Natriuretic Peptide, Troponin-T, and Growth-Differentiation Factor 15 in Adult Congenital Heart Disease. Circulation 2017 Jan 17;135(3):264-279
  • Kerstjens-Frederikse WS, van de Laar IMBH, Vos YJ, et al, Cardiovascular malformations caused by NOTCH1 mutations do not keep left: data on 428 probands with left-sided CHD and their families. Genetics in Medicine 2016 Sep;18(9):914-23
  • Budts W, Roos-Hesselink JW, Radle-Hurst T et al, Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology. European Heart Journal 2016 May 7;37(18):1419-27
  • Steegers-Theunissen RPM, Verheijden-Paulissen JJFM, van Uitert EM et al, Cohort Profile: The Rotterdam Periconceptional Cohort (Predict Study). International Journal of Epidemiology 2016 Apr;45(2):374-81
  • Etnel JRG, Elmont LV, Ertekin E et al, Outcome after aortic valve replacement in children: A systematic review and meta-analysis. Journal of Thoracic and Cardiovascular Surgery 2016 Jan;151(1):143-52
  • Eindhoven JA, van den Bosch EA, Oemrawsingh RM et al, Release of growth-differentiation factor 15 and associations with cardiac function in adult patients with congenital heart disease. International Journal of Cardiology 2016 Jan 1;202:246-51

PhD theses of the last five years

  • R.W.J. van Grootel. Speckle tracking echocardiography in adult congenital heart disease. 27-10-2020
  • L Swart. Improving the diagnosis of prosthetic heart valve endocarditis. 4-06-2019
  • Judith Verhagen. Genetics of Cardiovascular disorders. 4-06-2019
  • S Boccalini. CT Imaging of the Thoracic Aorta: Size assessment and follow-up after surgical and endovascular intervention. 6-11-2019
  • L. Bons. Thoracic Aortic Disease: Imaging and Clinical Aspects. 7-04-2020
  • M.E. Menting; Adult congenital heart disease: clinical outcome and novel echocardiographic techniques. 10-5-2016
  • J.A.A.E. Cuypers; The unnatural history of congenital heart disease: outcome up to 40 years after surgical repair. 15-12-2015
  • N. Duppen; Exercise training in children and young adults with congenital heart disease. 20-10-2015
  • S. M. Bossers; Outcomes after contemporary Fontan operation: step by step. 20-10-2015
  • J. A. Eindhoven; Cardiac biomarkers in adult congenital heart disease. 16-1-15
  • R.W.J. van Grootel. Speckle tracking echocardiography in adult congenital heart disease. 27-10-2020
  • D. van der Linde; Congenital aortic stenosis and aneurysms. 19-4-2013 "Cum Laude"
  • P. Opic; Impact of congenital heart disease at adulthood. 20-9-2013
  • P.T. Ruys; Adult congenital heart disease with focus on pregnancy. 13-9-2013
  • I.van de Laar; Clinical and genetic studies in inherited cardiovascular malformations. 27-6-2012
  • Vivan J.M. Baggen Risk Prediction in Adult Congenital Heart Disease 18-09-2018
  • Jackie McGhie Simultaneous Multiplane 2D-Echocardiography 27-06-2017
  • Jerome Cornette Hemodynamic Profiling in Complicated Pregnancies 29-12-2016
  • Aart Mookhoek Changing Roots: remodeling after the Ross procedure 16-11-2016

Non-scientific publications related to the ACE

  • "Toch je hele leven hartpatiënt", Trouw 24-08-2018
  • “De lange termijn uitkomsten na operatie voor een aangeboren hartafwijking op jonge leeftijd” Sinus (blad van de vereniging voor patiënten met aangeboren hartafwijkingen, juni 2016,
  • "Hoe safe is het om zwanger te worden". Gezondheid.blog.nl 25-12-2013
  • "Mijn vader, de kinderhartchirurg", Vrij Nederland Kerstnummer 2015
  • Interview prof. Jolien Roos-Hesselink in Erasmus Magazine 2018
  • "Pregnant women with heart disease should give birth at no later than 40 weeks gestation" MedicalXpress 25-8-2018
  • Presentaties prof. W. Helbing bij symposium Stichting Hartekind in januari 2018
  • Interview Aangeboren hartafwijkingen door Prof W.Helbing voor Cyberpoli.nl
  • Gatzoulis MA, Diller GP, Budts W, Roos-Hesselink JW. The ESC Working Group on Adult Congenital Heart Disease. Eur Heart J. 2020 May 21;41(20):1870-1871.

Principal coordinator(s)