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Reply to Piquereau and Perros, and to Pullamsetti and de Jesus Perez.

Kurakula K, Boucherat O, Van der Feen DE, Bogaard HJ, Kulikowski E, Bartelds B, Provencher S, Berger RMF, Bonnet S, Goumans MJ.

Am J Respir Crit Care Med. 2019 Aug 16. doi: 10.1164/rccm.201905-1078LE. [Epub ahead of print] No abstract available.


Thoracotomy versus sternotomy for patent ductus arteriosus closure in preterm neonates.

Verhaegh AJFP, Accord RE, Kooi EMW, Arrigoni SC, Bos AF, Berger RMF, Ebels T.

Ann Thorac Surg. 2019 Aug 10. pii: S0003-4975(19)31168-3. doi: 10.1016/j.athoracsur.2019.06.068. [Epub ahead of print]


CMR feature tracking left ventricular strain-rate predicts ventricular tachyarrhythmia, but not deterioration of ventricular function in patients with repaired tetralogy of Fallot.

Hagdorn QAJ, Vos JDL, Beurskens NEG, Gorter TM, Meyer SL, van Melle JP, Berger RMF, Willems TP.

Int J Cardiol. 2019 Aug 1. pii: S0167-5273(19)30911-8. doi: 10.1016/j.ijcard.2019.07.097. [Epub ahead of print]


A stented bovine pericardial prosthesis in the pulmonary position.

Pragt H, Schoots MH, Accord RE, Arrigoni SC, Berger RM, Mariani MA, Willems TP, Ebels T, Van Melle JP.

J Thorac Cardiovasc Surg. 2019 Jul 10. pii: S0022-5223(19)31355-8. doi: 10.1016/j.jtcvs.2019.05.086. [Epub ahead of print]


The clinical value of proposed risk stratification tools in pediatric pulmonary arterial hypertension.

Haarman MG, Douwes JM, Ploegstra MJ, Roofthooft MTR, Vissia-Kazemier T, Hillege HL, Berger RMF.

Am J Respir Crit Care Med. 2019 Jul 12. doi: 10.1164/rccm.201902-0266LE. [Epub ahead of print] No abstract available.


EXPRESS: Acute Vasoreactivity Testing in Pediatric Idiopathic Pulmonary Arterial Hypertension: an international Survey on Current Practice.

Caicedo L, Hopper RK, Garcia H, Ivy DD, Haag D, Fineman J, Humpi T, Al-Tamimi O, Feinstein J, Berger RM, Berman-Rosenzweig E, Kashour T, Diaz GF, Mendoza A, Bobhate P, Handler S, Lopes AA, Barwad P, Kumar Rahit M, Krishnan U, Adatia ITK, Moledina S, Abman S, Cerro Marin MJD.

Pulm Circ. 2019 May 30:2045894019857533. doi: 10.1177/2045894019857533. [Epub ahead of print] No abstract available.


Right ventricular-vascular coupling ratio in pediatric pulmonary arterial hypertension: A comparison between cardiac magnetic resonance and right heart catheterization measurements.

Breeman KTN, Dufva M, Ploegstra MJ, Kheyfets V, Willems TP, Wigger J, Hunter KS, Ivy DD, Berger RMF, Truong U.

Int J Cardiol. 2019 May 10. pii: S0167-5273(19)31199-4. doi: 10.1016/j.ijcard.2019.05.021. [Epub ahead of print]


New Strategies for the Conduct of Clinical Trials in Pediatric Pulmonary Arterial Hypertension: Outcome of a Multistakeholder Meeting With Patients, Academia, Industry, and Regulators, Held at the European Medicines Agency on Monday, June 12, 2017.

Ollivier C, Sun H, Amchin W, Beghetti M, Berger RMF, Breitenstein S, Garnett C, Gullberg N, Hassel P, Ivy D, Kawut SM, Klein A, Lesage C, Migdal M, Nije B, Odermarsky M, Strait J, de Graeff PA, Stockbridge N.

J Am Heart Assoc. 2019 May 21;8(10):e011306. doi: 10.1161/JAHA.118.011306. No abstract available.


Meaningful and feasible composite clinical worsening definitions in paediatric pulmonary arterial hypertension: An analysis of the TOPP registry.

Beghetti M, Brand M, Berger RMF, Humpl T, Wheeler JG, Ivy DD, Bonnet D; TOPP investigators.

Int J Cardiol. 2019 Aug 15;289:110-115. doi: 10.1016/j.ijcard.2019.04.062. Epub 2019 Apr 25.


Multicenter Preclinical Validation of BET Inhibition for the Treatment of Pulmonary Arterial Hypertension.

Van der Feen DE, Kurakula K, Tremblay E, Boucherat O, Bossers GP, Szulcek R, Bourgeois A, Lampron MC, Habbout K, Martineau S, Paulin R, Kulikowski E, Jahagirdar R, Schalij I, Bogaard HJ, Bartelds B, Provencher S, Berger RMF, Bonnet S, Goumans MJ.

Am J Respir Crit Care Med. 2019 May 1. doi: 10.1164/rccm.201812-2275OC. [Epub ahead of print]


Right ventricular pressure overload alters cardiac lipid composition.

Koop AMC, Hagdorn QAJ, Bossers GPL, van Leusden T, Gerding A, van Weeghel M, Vaz FM, Koonen DPY, Silljé HHW, Berger RMF, Bartelds B.

Int J Cardiol. 2019 Jul 15;287:96-105. doi: 10.1016/j.ijcard.2019.04.004. Epub 2019 Apr 8.


A novel method optimizing the normalization of cardiac parameters in small animal models: the importance of dimensional indexing.

Hagdorn QAJ, Bossers GPL, Koop AC, Piek A, Eijgenraam TR, van der Feen DE, Silljé HHW, de Boer RA, Berger RMF.

Am J Physiol Heart Circ Physiol. 2019 Jun 1;316(6):H1552-H1557. doi: 10.1152/ajpheart.00182.2019. Epub 2019 Apr 12.


The Natural and Unnatural History of Congenital Aortic Arch Abnormalities Evaluated in an Adult Survival Cohort.

Lodeweges JE, Dikkers FG, Mulder BJM, Roos-Hesselink JW, Vliegen HW, van Dijk APJ, Sieswerda GT, Konings TC, Berger RMF, Slebos DJ, Ebels T, van Melle JP.

Can J Cardiol. 2019 Apr;35(4):438-445. doi: 10.1016/j.cjca.2018.12.004. Epub 2018 Dec 7.


Response: Still puzzling about a clear definition of pulmonary arterial hypertension in newborns.

Rosenzweig EB, Abman SH, Berger RMF.

Eur Respir J. 2019 Mar 28;53(3). pii: 1900135. doi: 10.1183/13993003.00135-2019. Print 2019 Mar. No abstract available.


Converging Paths of Pulmonary Arterial Hypertension and Cellular Senescence.

van der Feen DE, Berger RMF, Bartelds B.

Am J Respir Cell Mol Biol. 2019 Jul;61(1):11-20. doi: 10.1165/rcmb.2018-0329TR.


The Janus-faced Fontan circulation: unravelling its elusive pathophysiology.

Ridderbos FS, Hoendermis ES, Berger RMF, van Melle JP.

Eur J Heart Fail. 2019 Jun;21(6):810-812. doi: 10.1002/ejhf.1415. Epub 2019 Feb 7. No abstract available.


Early Diagnosis in Pulmonary Arterial Hypertension: The Search for the Holy Grail.

Berger RMF, Beghetti M.

Am J Respir Crit Care Med. 2019 Jun 1;199(11):1306-1307. doi: 10.1164/rccm.201812-2314ED. No abstract available.


Necrotizing Enterocolitis Associated with Congenital Heart Disease: a Different Entity?

Bubberman JM, van Zoonen A, Bruggink JLM, van der Heide M, Berger RMF, Bos AF, Kooi EMW, Hulscher JBF.

J Pediatr Surg. 2018 Dec 13. pii: S0022-3468(18)30788-7. doi: 10.1016/j.jpedsurg.2018.11.012. [Epub ahead of print]


Evaluation of Macitentan in Patients With Eisenmenger Syndrome.

Gatzoulis MA, Landzberg M, Beghetti M, Berger RM, Efficace M, Gesang S, He J, Papadakis K, Pulido T, Galiè N; MAESTRO Study Investigators.

Circulation. 2019 Jan 2;139(1):51-63. doi: 10.1161/CIRCULATIONAHA.118.033575.


Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management.

Rosenzweig EB, Abman SH, Adatia I, Beghetti M, Bonnet D, Haworth S, Ivy DD, Berger RMF.

Eur Respir J. 2019 Jan 24;53(1). pii: 1801916. doi: 10.1183/13993003.01916-2018. Print 2019 Jan.


Assessment of reversibility in pulmonary arterial hypertension and congenital heart disease.

van der Feen DE, Bartelds B, de Boer RA, Berger RMF.

Heart. 2018 Nov 22. pii: heartjnl-2018-314025. doi: 10.1136/heartjnl-2018-314025. [Epub ahead of print] Review.


Pulmonary vasodilator therapy as treatment for patients with a Fontan circulation: the Emperor's new clothes?

Ridderbos FS, Hagdorn QAJ, Berger RMF.

Pulm Circ. 2018 Oct-Dec;8(4):2045894018811148. doi: 10.1177/2045894018811148. Epub 2018 Oct 19. No abstract available.


Risk of cardiac tachyarrhythmia in patients with repaired tetralogy of Fallot: a multicenter cardiac MRI based study.

Beurskens NEG, Hagdorn QAJ, Gorter TM, Berger RMF, Vermeulen KM, van Melle JP, Ebels TE, Lui GK, Ceresnak SR, Chan FP, Willems TP.

Int J Cardiovasc Imaging. 2019 Jan;35(1):143-151. doi: 10.1007/s10554-018-1435-9. Epub 2018 Aug 9.


Quality of life and sexual well-being in patients with a Fontan circulation: An explorative pilot study with a mixed method design.

Wolff D, van de Wiel HBM, de Muinck Keizer ME, van Melle JP, Pieper PG, Berger RMF, Ebels T, Weijmar Schultz WCM.

Congenit Heart Dis. 2018 Mar;13(2):319-326. doi: 10.1111/chd.12576. Epub 2018 Mar 12.


Identification of gaps in the current knowledge on pulmonary hypertension in extremely preterm infants: A systematic review and meta-analysis.

Arjaans S, Zwart EAH, Ploegstra MJ, Bos AF, Kooi EMW, Hillege HL, Berger RMF.

Paediatr Perinat Epidemiol. 2018 May;32(3):258-267. doi: 10.1111/ppe.12444. Epub 2018 Jan 17. Review.


Amplitude-integrated electroencephalography during the first 72 h after birth in neonates diagnosed prenatally with congenital heart disease.

Mebius MJ, Oostdijk NJE, Kuik SJ, Bos AF, Berger RMF, Bilardo CM, Kooi EMW, Ter Horst HJ.

Pediatr Res. 2018 Apr;83(4):798-803. doi: 10.1038/pr.2017.311. Epub 2018 Jan 10.


Volume load-induced right ventricular dysfunction in animal models: insights in a translational gap in congenital heart disease.

Bossers GPL, Hagdorn QAJ, Ploegstra MJ, Borgdorff MAJ, Silljé HHW, Berger RMF, Bartelds B.

Eur J Heart Fail. 2018 Apr;20(4):808-812. doi: 10.1002/ejhf.931. Epub 2017 Sep 18. Review. No abstract available.


Cerebral and Renal Oxygen Saturation Are Not Compromised in the Presence of Retrograde Blood Flow in either the Ascending or Descending Aorta in Term or Near-Term Infants with Left-Sided Obstructive Lesions.

van der Laan ME, Mebius MJ, Roofthooft MTR, Bos AF, Berger RMF, Kooi EMW.

Neonatology. 2017;112(3):217-224. doi: 10.1159/000473870. Epub 2017 Jul 14.


Prenatal exposure to serotonin reuptake inhibitors and congenital heart anomalies: an exploratory pharmacogenetics study.

Daud ANA, Bergman JEH, Kerstjens-Frederikse WS, van der Vlies P, Hak E, Berger RMF, Groen H, Wilffert B.

Pharmacogenomics. 2017 Jul;18(10):987-1001. doi: 10.2217/pgs-2017-0036. Epub 2017 Jun 22.


Fontan Circulation over Time.

Wolff D, van Melle JP, Bartelds B, Ridderbos FS, Eshuis G, van Stratum EBHJ, Recinos SJ, Willemse BWM, Hillege H, Willems TP, Ebels T, Berger RMF.

Am J Cardiol. 2017 Aug 1;120(3):461-466. doi: 10.1016/j.amjcard.2017.05.005. Epub 2017 May 11.


Pediatric pulmonary arterial hypertension: on the eve of growing up.

Douwes JM, Berger RMF.

Curr Opin Pulm Med. 2017 Sep;23(5):398-403. doi: 10.1097/MCP.0000000000000406. Review.


Diagnostic value of Doppler echocardiography for identifying hemodynamic significant pulmonary valve regurgitation in tetralogy of Fallot: comparison with cardiac MRI.

Beurskens NEG, Gorter TM, Pieper PG, Hoendermis ES, Bartelds B, Ebels T, Berger RMF, Willems TP, van Melle JP.

Int J Cardiovasc Imaging. 2017 Nov;33(11):1723-1730. doi: 10.1007/s10554-017-1165-4. Epub 2017 May 31.


Right ventricular-vascular coupling in heart failure with preserved ejection fraction and pre- vs. post-capillary pulmonary hypertension.

Gorter TM, van Veldhuisen DJ, Voors AA, Hummel YM, Lam CSP, Berger RMF, van Melle JP, Hoendermis ES.

Eur Heart J Cardiovasc Imaging. 2018 Apr 1;19(4):425-432. doi: 10.1093/ehjci/jex133.


Pulmonary arterial hypertension in congenital heart disease: translational opportunities to study the reversibility of pulmonary vascular disease.

van der Feen DE, Bartelds B, de Boer RA, Berger RMF.

Eur Heart J. 2017 Jul 7;38(26):2034-2041. doi: 10.1093/eurheartj/ehx034. Review.


Right ventricular recovery after bilateral lung transplantation for pulmonary arterial hypertension†.

Gorter TM, Verschuuren EAM, van Veldhuisen DJ, Hoendermis ES, Erasmus ME, Bogaard HJ, Vonk Noordegraaf A, Berger RMF, van Melle JP, Willems TP.

Interact Cardiovasc Thorac Surg. 2017 Jun 1;24(6):890-897. doi: 10.1093/icvts/ivx025.


Pulmonary arterial stiffness indices assessed by intravascular ultrasound in children with early pulmonary vascular disease: prediction of advanced disease and mortality during 20-year follow-up.

Ploegstra MJ, Brokelman JGM, Roos-Hesselink JW, Douwes JM, van Osch-Gevers LM, Hoendermis ES, van den Bosch AE, Witsenburg M, Bartelds B, Hillege HL, Berger RMF.

Eur Heart J Cardiovasc Imaging. 2018 Feb 1;19(2):216-224. doi: 10.1093/ehjci/jex015.


Shunt Surgery, Right Heart Catheterization, and Vascular Morphometry in a Rat Model for Flow-induced Pulmonary Arterial Hypertension.

van der Feen DE, Weij M, Smit-van Oosten A, Jorna LM, Hagdorn QA, Bartelds B, Berger RM.

J Vis Exp. 2017 Feb 11;(120). doi: 10.3791/55065.


Diminished liver microperfusion in Fontan patients: A biexponential DWI study.

Dijkstra H, Wolff D, van Melle JP, Bartelds B, Willems TP, Oudkerk M, Hillege H, van den Berg AP, Ebels T, Berger RM, Sijens PE.

PLoS One. 2017 Mar 3;12(3):e0173149. doi: 10.1371/journal.pone.0173149. eCollection 2017.


A bosentan pharmacokinetic study to investigate dosing regimens in paediatric patients with pulmonary arterial hypertension: FUTURE-3.

Berger RMF, Gehin M, Beghetti M, Ivy D, Kusic-Pajic A, Cornelisse P, Grill S, Bonnet D; FUTURE-3 investigators.

Br J Clin Pharmacol. 2017 Aug;83(8):1734-1744. doi: 10.1111/bcp.13267. Epub 2017 Mar 23.


Physical Activity in Pediatric Pulmonary Arterial Hypertension Measured by Accelerometry. A Candidate Clinical Endpoint.

Zijlstra WMH, Ploegstra MJ, Vissia-Kazemier T, Roofthooft MTR, Sarvaas GDM, Bartelds B, Rackowitz A, van den Heuvel F, Hillege HL, Plasqui G, Berger RMF.

Am J Respir Crit Care Med. 2017 Jul 15;196(2):220-227. doi: 10.1164/rccm.201608-1576OC.


Serially Measured Uric Acid Levels Predict Disease Severity and Outcome in Pediatric Pulmonary Arterial Hypertension.

Leberkühne LJ, Ploegstra MJ, Douwes JM, Bartelds B, Roofthooft MT, Hillege HL, Berger RM.

Am J Respir Crit Care Med. 2017 Feb 1;195(3):401-404. doi: 10.1164/rccm.201606-1152LE. No abstract available.


Pulmonary arterial hypertension in children after neonatal arterial switch operation.

Zijlstra WM, Elmasry O, Pepplinkhuizen S, Ivy DD, Bonnet D, Luijendijk P, Lévy M, Gavilan JL, Torrent-Vernetta A, Mendoza A, Del Cerro MJ, Moledina S, Berger RM.

Heart. 2017 Aug;103(16):1244-1249. doi: 10.1136/heartjnl-2016-310624. Epub 2017 Jan 23.


Maternal-fetal cholesterol transport in the second half of mouse pregnancy does not involve LDL receptor-related protein 2.

Zwier MV, Baardman ME, van Dijk TH, Jurdzinski A, Wisse LJ, Bloks VW, Berger RMF, DeRuiter MC, Groen AK, Plösch T.

Acta Physiol (Oxf). 2017 Aug;220(4):471-485. doi: 10.1111/apha.12845. Epub 2017 Feb 22.


Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry.

Humpl T, Berger RMF, Austin ED, Fasnacht Boillat MS, Bonnet D, Ivy DD, Zuk M, Beghetti M, Schulze-Neick I.

Cardiol Young. 2017 Aug;27(6):1123-1132. doi: 10.1017/S1047951116002493. Epub 2016 Dec 20.


Effects of sildenafil on cardiac structure and function, cardiopulmonary exercise testing and health-related quality of life measures in heart failure patients with preserved ejection fraction and pulmonary hypertension.

Liu LC, Hummel YM, van der Meer P, Berger RM, Damman K, van Veldhuisen DJ, Voors AA, Hoendermis ES.

Eur J Heart Fail. 2017 Jan;19(1):116-125. doi: 10.1002/ejhf.662. Epub 2016 Nov 21.


Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease.

Zijlstra WM, Douwes JM, Ploegstra MJ, Krishnan U, Roofthooft MT, Hillege HL, Ivy DD, Rosenzweig EB, Berger RM.

Pulm Circ. 2016 Sep;6(3):302-12. doi: 10.1086/687764.


KinCor, a national registry for paediatric patients with congenital and other types of heart disease in the Netherlands: aims, design and interim results.

Silva LM, Kuipers IM, van den Heuvel F, Mendes R, Berger RM, van Beynum IM, Rozendaal L, Rammeloo LA, van Iperen GG, Schokking M, Frerich S, Blom NA, Breur JM, Helbing WA.

Neth Heart J. 2016 Nov;24(11):628-639.


Corrigendum to "FUTURE-2: Results from an open-label, long-term safety and tolerability extension study using the pediatric FormUlation of bosenTan in pUlmonary arterial hypeRtEnsion" [Int. J. Cardiol. 202 (2016) 52-58].

Berger RMF, Haworth SG, Bonnet D, Dulac Y, Fraisse A, Galiè N, Ivy DD, Jaïs X, Miera O, Rosenzweig EB, Efficace M, Kusic-Pajic A, Beghetti M.

Int J Cardiol. 2016 Nov 15;223:1072-1073. doi: 10.1016/j.ijcard.2016.08.333. Epub 2016 Aug 24. No abstract available.


NT-proBNP and exercise capacity in adult patients with congenital heart disease and a prosthetic valve: a multicentre PROSTAVA study.

Schoonbeek RC, Pieper PG, van Slooten YJ, Freling HG, Sieswerda GT, van Dijk AP, Jongbloed MR, Post MC, Bouma BJ, Berger RM, Ebels T, van Melle JP.

Neth Heart J. 2016 Nov;24(11):653-665.


Multisite Tissue Oxygenation Monitoring Indicates Organ-Specific Flow Distribution and Oxygen Delivery Related to Low Cardiac Output in Preterm Infants With Clinical Sepsis.

van der Laan ME, Roofthooft MT, Fries MW, Schat TE, Bos AF, Berger RM, Kooi EM.

Pediatr Crit Care Med. 2016 Aug;17(8):764-71. doi: 10.1097/PCC.0000000000000833.


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