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The pulmonary vasculature: Achilles heel of the Fontan circulation.

Ridderbos FS, Ebels T, Berger RMF.

Heart. 2019 Dec;105(24):1922-1923. doi: 10.1136/heartjnl-2019-316136. Epub 2019 Nov 11. No abstract available.


Metabolic Remodeling in the Pressure-Loaded Right Ventricle: Shifts in Glucose and Fatty Acid Metabolism-A Systematic Review and Meta-Analysis.

Koop AC, Bossers GPL, Ploegstra MJ, Hagdorn QAJ, Berger RMF, Silljé HHW, Bartelds B.

J Am Heart Assoc. 2019 Nov 5;8(21):e012086. doi: 10.1161/JAHA.119.012086. Epub 2019 Oct 28.


Reply to: More Insights into the Association Between RVX-208 and Pulmonary Arterial Hypertension.

Provencher S, Boucherat O, Berger RMF, Goumans MJ, Bonnet S; all authors of “Multicenter Preclinical Validation of BET Inhibition for the Treatment of Pulmonary Arterial Hypertension.”.

Am J Respir Crit Care Med. 2019 Sep 27. doi: 10.1164/rccm.201909-1800LE. [Epub ahead of print] No abstract available.


Recommendations from the Association for European Paediatric and Congenital Cardiology for training in pulmonary hypertension.

Sallmon H, Moledina S, Albert DC, Beghetti M, Berger RMF, Bonnet D, Bukova M, Koestenberger M, Meinel K, Reinhardt Z, Tulloh RMR, de Wolf D, Hansmann G.

Cardiol Young. 2019 Nov;29(11):1323-1327. doi: 10.1017/S104795111900235X. Epub 2019 Sep 26.


Failing Homeostasis of Quadriceps Muscle Energy- and pH Balance During Bicycling in a Young Patient With a Fontan Circulation.

Haarman MG, Vos JDL, Berger RMF, Willems TP, Jeneson JAL.

Front Cardiovasc Med. 2019 Aug 21;6:121. doi: 10.3389/fcvm.2019.00121. eCollection 2019.


Pulmonary artery size is associated with functional clinical status in the Fontan circulation.

Ridderbos FS, Bonenkamp BE, Meyer SL, Eshuis G, Ebels T, van Melle JP, Willems TP, Berger RMF.

Heart. 2019 Sep 6. pii: heartjnl-2019-314972. doi: 10.1136/heartjnl-2019-314972. [Epub ahead of print]


The ever-expanding phenotypical spectrum of human TBX4 mutations: from toe to lung.

Haarman MG, Kerstjens-Frederikse WS, Berger RMF.

Eur Respir J. 2019 Aug 22;54(2). pii: 1901504. doi: 10.1183/13993003.01504-2019. Print 2019 Aug. No abstract available.


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 Nov 1;200(9):1190-1191. doi: 10.1164/rccm.201905-1078LE. 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 Nov 15;295:1-6. doi: 10.1016/j.ijcard.2019.07.097. Epub 2019 Aug 1.


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 TR, Hillege HL, Berger RMF.

Am J Respir Crit Care Med. 2019 Nov 15;200(10):1312-1315. doi: 10.1164/rccm.201902-0266LE. 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 Oct 15;293:211-217. doi: 10.1016/j.ijcard.2019.05.021. Epub 2019 May 10.


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 GPL, 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 Oct 1;200(7):910-920. doi: 10.1164/rccm.201812-2275OC.


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. 2019 Sep;54(9):1755-1760. doi: 10.1016/j.jpedsurg.2018.11.012. Epub 2018 Dec 13.


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. 2019 Feb;105(4):276-282. doi: 10.1136/heartjnl-2018-314025. Epub 2018 Nov 22. 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.


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