Imperial College London

DrPiersDaubeney

Faculty of MedicineNational Heart & Lung Institute

Professor of Practice (Paediatric Cardiology)
 
 
 
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Contact

 

+44 (0)20 7351 8430p.daubeney

 
 
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Location

 

Royal BromptonRoyal Brompton Campus

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Summary

 

Publications

Publication Type
Year
to

157 results found

Hatipoglu S, Gatehouse P, Krupickova S, Banya W, Daubeney P, Almogheer B, Izgi C, Weale P, Hayes C, Firmin D, Pennell DJet al., 2021, Reliability of pediatric ventricular function analysis by short-axis "single-cycle-stack-advance" single-shot compressed-sensing cines in minimal breath-hold time, EUROPEAN RADIOLOGY, ISSN: 0938-7994

Journal article

Norrish G, Rance T, Montanes E, Field E, Brown E, Bhole V, Stuart G, Uzun O, McLeod KA, Ilina M, Adwani S, Daubeney P, Delle Donne G, Linter K, Jones CB, Bharucha T, Cervi E, Kaski JPet al., 2021, Friedreich's ataxia-associated childhood hypertrophic cardiomyopathy: a national cohort study, ARCHIVES OF DISEASE IN CHILDHOOD, ISSN: 0003-9888

Journal article

Puricelli F, Gati S, Banya W, Daubeney PEF, Pennell DJ, Voges I, Krupickova Set al., 2021, Normal values of MAPSE and TAPSE in the paediatric population established by cardiovascular magnetic resonance, INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING, ISSN: 1569-5794

Journal article

Norrish G, Kolt G, Cervi E, Field E, Dady K, Ziolkowska L, Olivotto I, Favilli S, Passantino S, Limongelli G, Caiazza M, Rubino M, Baban A, Drago F, Mcleod K, Ilina M, McGowan R, Stuart G, Bhole V, Uzun O, Wong A, Lazarou L, Brown E, Daubeney PEF, Lota A, Delle Donne G, Linter K, Mathur S, Bharucha T, Adwani S, Searle J, Popoiu A, Jones CB, Reinhardt Z, Kaski JPet al., 2021, Clinical presentation and long-term outcomes of infantile hypertrophic cardiomyopathy: a European multicentre study, ESC HEART FAILURE, ISSN: 2055-5822

Journal article

Audain E, Wilsdon A, Breckpot J, Izarzugaza JMG, Fitzgerald TW, Kahlert A-K, Sifrim A, Wünnemann F, Perez-Riverol Y, Abdul-Khaliq H, Bak M, Bassett AS, Benson DW, Berger F, Daehnert I, Devriendt K, Dittrich S, Daubeney PE, Garg V, Hackmann K, Hoff K, Hofmann P, Dombrowsky G, Pickardt T, Bauer U, Keavney BD, Klaassen S, Kramer H-H, Marshall CR, Milewicz DM, Lemaire S, Coselli JS, Mitchell ME, Tomita-Mitchell A, Prakash SK, Stamm K, Stewart AFR, Silversides CK, Siebert R, Stiller B, Rosenfeld JA, Vater I, Postma AV, Caliebe A, Brook JD, Andelfinger G, Hurles ME, Thienpont B, Larsen LA, Hitz M-Pet al., 2021, Correction: Integrative analysis of genomic variants reveals new associations of candidate haploinsufficient genes with congenital heart disease., PLoS Genet, Vol: 17

[This corrects the article DOI: 10.1371/journal.pgen.1009679.].

Journal article

Krupickova S, Hatipoglu S, DiSalvo G, Voges I, Redfearn D, Foldvari S, Eichhorn C, Chivers S, Puricelli F, Delle-Donne G, Barth C, Pennell DJ, Prasad SK, Daubeney PEFet al., 2021, Left ventricular noncompaction in pediatric population: could cardiovascular magnetic resonance derived fractal analysis aid diagnosis?, JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE, Vol: 23, ISSN: 1097-6647

Journal article

Audain E, Wilsdon A, Breckpot J, Izarzugaza JMG, Fitzgerald TW, Kahlert A-K, Sifrim A, Wunnemann F, Perez-Riverol Y, Abdul-Khaliq H, Bak M, Bassett AS, Benson WD, Berger F, Daehnert I, Devriendt K, Dittrich S, Daubeney PEF, Garg V, Hackmann K, Hoff K, Hofmann P, Dombrowsky G, Pickardt T, Bauer U, Keavney BD, Klaassen S, Kramer H-H, Marshall CR, Milewicz DM, Lemaire S, Coselli JS, Mitchell ME, Tomita-Mitchell A, Prakash SK, Stamm K, Stewart AFR, Silversides CK, Siebert R, Stiller B, Rosenfeld JA, Vater I, Postma AV, Caliebe A, Brook JD, Andelfinger G, Hurles ME, Thienpont B, Larsen LA, Hitz M-Pet al., 2021, Integrative analysis of genomic variants reveals new associations of candidate haploinsufficient genes with congenital heart disease, PLOS GENETICS, Vol: 17, ISSN: 1553-7404

Journal article

Topriceanu C-C, Norrish G, Qu C, Fiend E, Walsh H, Ziolkowska L, Olivotto I, Passantino S, Favilli S, Anastasakis A, Vlagkouli V, Weintraub R, King I, Biagini E, Ragni L, Prendiville T, Duignan S, McLeod K, Ilina M, Fernandez A, Bokenkamp R, Baban A, Drago D, Kubus P, Daubeney P, Chivers S, Sarquella-Brugada G, Cesar S, Marrone C, Medrano C, Reyes AG-R, Uzun O, Ipina FG, Garcia FJC, Gimeno JR, Barriales-Villa R, Rueda F, Adwani S, Searle J, Bharucha T, Siles A, Usano A, Rasmussen TB, Jones C, Kubo T, Mogensen J, Reinhardt Z, Cervi E, Elliott P, Omar R, Kaski Jet al., 2021, THE ROLE OF THE ELECTROCARDIOGRAPHIC PHENOTYPE IN RISK STRATIFICATION FOR SUDDEN CARDIAC DEATH IN CHILDHOOD HYPERTROPHIC CARDIOMYOPATHY, Publisher: BMJ PUBLISHING GROUP, Pages: A1-A1, ISSN: 1355-6037

Conference paper

Norrish G, Topriceanu C, Qu C, Field E, Walsh H, Ziółkowska L, Olivotto I, Passantino S, Favilli S, Anastasakis A, Vlagkouli V, Weintraub R, King I, Biagini E, Ragni L, Prendiville T, Duignan S, McLeod K, Ilina M, Fernández A, Bökenkamp R, Baban A, Drago F, Kubuš P, Daubeney PEF, Chivers S, Sarquella-Brugada G, Cesar S, Marrone C, Medrano C, Alvarez Garcia-Roves R, Uzun O, Gran F, Castro FJ, Gimeno JR, Barriales-Villa R, Rueda F, Adwani S, Searle J, Bharucha T, Siles A, Usano A, Rasmussen TB, Jones CB, Kubo T, Mogensen J, Reinhardt Z, Cervi E, Elliott PM, Omar RZ, Kaski JPet al., 2021, The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy., Eur J Prev Cardiol

AIMS: The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. METHODS AND RESULTS: Data from 356 childhood HCM patients with a mean age of 10.1 years (±4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0-7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93-2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of >5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484-0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7. CONCLUSION: In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited.

Journal article

Norrish G, Chubb H, Field E, McLeod K, Ilina M, Spentzou G, Till J, Daubeney PEF, Stuart AG, Matthews J, Hares D, Brown E, Linter K, Bhole V, Pillai K, Bowes M, Jones CB, Uzun O, Wong A, Yue A, Sadagopan S, Bharucha T, Yap N, Rosenthal E, Mathur S, Adwani S, Reinhardt Z, Mangat J, Kaski JPet al., 2021, Clinical outcomes and programming strategies of implantable cardioverter-defibrillator devices in paediatric hypertrophic cardiomyopathy: a UK National Cohort Study, EUROPACE, Vol: 23, Pages: 400-408, ISSN: 1099-5129

Journal article

Krupickova S, Risch J, Gati S, Caliebe A, Sarikouch S, Beerbaum P, Puricelli F, Daubeney PEF, Barth C, Wage R, Boroni Grazioli S, Uebing A, Pennell DJ, Voges Iet al., 2021, Cardiovascular magnetic resonance normal values in children for biventricular wall thickness and mass, JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE, Vol: 23, ISSN: 1097-6647

Journal article

Crossland DS, Ferguson R, Magee A, Jenkins P, Bulock FA, Parry A, Babu-Narayan S, Carroll A, Daubeney PEF, Simpson Jet al., 2021, Consultant staffing in UK congenital cardiac services: a 10-year survey of leavers and joiners, OPEN HEART, Vol: 8, ISSN: 2053-3624

Journal article

Cleary A, Chivers S, Daubeney PE, Simpson JMet al., 2021, Impact of COVID-19 on patients with congenital heart disease, CARDIOLOGY IN THE YOUNG, Vol: 31, Pages: 163-165, ISSN: 1047-9511

Journal article

Sabatino J, Piccinelli E, Borrelli N, Paredes J, Josen M, Avesani M, Sirico D, Fraisse A, Indolfi C, Daubeney P, Di Salvo Get al., 2020, Left ventricular twist predicts prognosis in children with non-compaction cardiomyopathy, 81st National Congress of the Italian-Society-of-Cardiology (SIC), Publisher: OXFORD UNIV PRESS, Pages: N42-N42, ISSN: 1520-765X

Conference paper

Michielon G, DiSalvo G, Fraisse A, Carvalho JS, Krupickova S, Slavik Z, Bartsota M, Daubeney P, Bautista C, Desai A, Burmester M, Macrae Det al., 2020, In-hospital interstage improves interstage survival after the Norwood stage 1 operation, EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY, Vol: 57, Pages: 1113-1121, ISSN: 1010-7940

Journal article

Orban T, Orban NT, Jalahej H, Daubeney PEFet al., 2020, A Novel Quantitative Approach to Staging and Assessing Recovery from Type 1 Diabetes Mellitus: The Type 1 Diabetes Mellitus Metabolic Recovery Index, INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES, Vol: 21

Journal article

Lota AS, Halliday B, Tayal U, Salmi S, Shakur R, Hammersley D, Jones R, Daubeney P, Ware JS, Cleland JG, Cook SA, Pennell DJ, Prasad SKet al., 2019, Epidemiological Trends and Outcomes of Acute Myocarditis in the National Health Service of England, Scientific Sessions of the American-Heart-Association, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322

Conference paper

Chivers SC, Pavy C, Vaja R, Quarto C, Ghez O, Daubeney PEFet al., 2019, The Ozaki procedure with cardioCel patch for children and young adults with aortic valve disease: preliminary experience - a word of caution, World Journal for Pediatric and Congenital Heart Surgery, Vol: 10, Pages: 724-730, ISSN: 2150-1351

Background:The Ozaki procedure is a surgical technique for patients with significant aortic stenosis or regurgitation or both where valve repair cannot be performed. Individual cusps are cut from glutaraldehyde-treated autologous pericardium or bovine pericardium and implanted into the aortic valve position. Encouraging results have been reported within the adult population. There are limited published data on success of this procedure in younger patients.Methods:We present a series of five children and young adults who underwent the Ozaki procedure with neoaortic valve cusps made from CardioCel, a decellularized bovine pericardial patch treated with a monomeric glutaraldehyde.Results:There were no complications in the initial postoperative period and short inpatient stay. At a mean follow-up of 29.6 months (range: 22-36 months), 4 patients had no evidence of stenosis and 3 patients had trivial or no regurgitation from the neoaortic valve. Overall, two patients had complications related to the valve and underwent reintervention during the follow-up period with a Ross procedure. One of these patients who was not taking long-term anticoagulation experienced a transient ischemic attack.Conclusions:Our experience demonstrates that the Ozaki procedure with CardioCel in pediatric and young adult patients should be approached with caution. Further research with larger groups of pediatric patients, comparison of different graft materials, and longer follow-up is required to ascertain long-term success in children.

Journal article

Norrish G, Ding T, Field E, McLeod K, Ilina M, Stuart G, Bhole V, Uzun O, Brown E, Daubeney PEF, Lota A, Linter K, Mathur S, Bharucha T, Kok KL, Adwani S, Jones CB, Reinhardt Z, Omar RZ, Kaski JPet al., 2019, A validation study of the European Society of Cardiology guidelines for risk stratification of sudden cardiac death in childhood hypertrophic cardiomyopathy, EUROPACE, Vol: 21, Pages: 1559-1565, ISSN: 1099-5129

Journal article

Norrish G, Ding T, Field E, Ziolkowska L, Olivotto I, Limongelli G, Anastasakis A, Weintraub R, Biagini E, Ragni L, Prendiville T, Duignan S, McLeod K, Ilina M, Fernandez A, Boekenkamp R, Baban A, Kubus P, Daubeney PEF, Sarquella-Brugada G, Cesar S, Marrone C, Bhole V, Medrano C, Uzun O, Brown E, Gran F, Castro FJ, Stuart G, Vignati G, Barriales-Villa R, Guereta LG, Adwani S, Linter K, Bharucha T, Garcia-Pavia P, Rasmussen TB, Calcagnino MM, Jones CB, De Wilde H, Toru-Kubo J, Felice T, Mogensen J, Mathur S, Reinhardt Z, O'Mahony C, Elliott PM, Omar RZ, Kaski JPet al., 2019, Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids), JAMA CARDIOLOGY, Vol: 4, Pages: 918-927, ISSN: 2380-6583

Journal article

Sabatino J, Di Salvo G, Prota C, Bucciarelli V, Josen M, Paredes J, Borrelli N, Sirico D, Prasad S, Indolfi C, Fraisse A, Daubeney PEFet al., 2019, Left Atrial Strain to Identify Diastolic Dysfunction in Children with Cardiomyopathies, JOURNAL OF CLINICAL MEDICINE, Vol: 8

Journal article

Van Bergen NJ, Guo Y, Rankin J, Paczia N, Becker-Kettern J, Kremer LS, Pyle A, Conrotte J, Ellaway CJ, Procopis P, Prelog K, Homfray T, Baptista J, Baple E, Wakeling M, Massey S, Kay DP, Shukla A, Girisha KM, Lewis LES, Santra SD, Power R, Daubeney P, Montoya J, Ruiz-Pesini E, Kovacs-Nagy R, Pritsch M, Ahting U, Thorburn DR, Prokisch H, Taylor R, Christodoulou J, Linster C, Ellard S, Hakonarson Het al., 2019, NAXDmutations cause a novel neurodegenerative disorder exacerbated by febrile illnesses, 51st Conference of the European-Society-of-Human-Genetics (ESHG) in conjunction with the European Meeting on Psychosocial Aspects of Genetics (EMPAG), Publisher: NATURE PUBLISHING GROUP, Pages: 751-752, ISSN: 1018-4813

Conference paper

Eichhorn C, Voges I, Daubeney PEF, 2019, Out-of-hospital cardiac arrest and survival in a patient with Noonan syndrome and multiple lentigines: a case report, Journal of Medical Case Reports, Vol: 13, ISSN: 1752-1947

BACKGROUND: A 9-year-old Arabic boy attending middle school presented with an out-of-hospital cardiac arrest due to ventricular fibrillation recorded by Holter electrocardiographic monitoring. He had a background history of Noonan syndrome with multiple lentigines (also known as LEOPARD syndrome), a rare condition of autosomal dominant inheritance with approximately 200 cases reported worldwide. CASE PRESENTATION: Apart from characteristic features, the boy was known to have asymmetric septal hypertrophy with a maximum wall thickness of 24 mm measured by cardiovascular magnetic resonance imaging. A day prior to the event, he attended cardiology follow-up at our institution, and Holter monitoring was commenced. Following cardiopulmonary resuscitation by bystanders and paramedics, he reverted back into sinus rhythm after a total downtime of 24 min. He was initially treated in the intensive care unit and underwent implantable cardioverter defibrillator implantation. He has made a full recovery and remains at the top of his class. CONCLUSION: This case demonstrates that sudden cardiac arrest in patients with secondary forms of hypertrophic cardiomyopathy is not necessarily protected by apparently favorable phenotypes and that events may be preceded by non-sustained ventricular tachycardia observed by Holter monitoring. Implantable cardioverter defibrillator implantation plays a critical role in both primary and secondary prevention in patients at high risk of out-of-hospital cardiac arrest.

Journal article

Krupickova S, Hatipoglu S, Di Salvo G, Voges I, Foldvari S, Eichhorn CH, Chivers S, Prasad SK, Daubeney PEFet al., 2019, Quantification of left ventricular trabeculations using fractal analysis in children, Publisher: OXFORD UNIV PRESS, Pages: 139-139, ISSN: 2047-2404

Conference paper

Fidalgo Garcia A, Ahmed R, Nyktari E, Daubeney P, Voges Iet al., 2019, Complicated coarctation repair: The importance of three-dimensional cross-sectional imaging in late postoperative assessment, ANNALS OF PEDIATRIC CARDIOLOGY, Vol: 12, Pages: 178-181, ISSN: 0974-2069

Journal article

Sabatino J, Di Salvo G, Krupickova S, Fraisse A, Prota C, Bucciarelli V, Josen M, Paredes J, Sirico D, Voges I, Indolfi C, Prasad S, Daubeney Pet al., 2019, Left Ventricular Twist Mechanics to Identify Left Ventricular Noncompaction in Childhood, CIRCULATION-CARDIOVASCULAR IMAGING, Vol: 12, ISSN: 1941-9651

Journal article

Sri A, Daubeney P, Prasad S, Baksi J, Kinali M, Voges Iet al., 2019, A case series on cardiac and skeletal involvement in two families with PRKAG2 mutations, Case Reports in Pediatrics, Vol: 2019, ISSN: 2090-6803

Background. PRKAG2 is a rare autosomal dominant syndrome that mainly presents with hypertrophic cardiomyopathy, ventricular preexcitation, and conduction abnormalities. This case report demonstrates that the PRKAG2 mutation presents with various phenotypes already in pediatric patients. Case Summary. We describe the clinical and investigative findings in two families with a PRKAG2 mutation from the different variants in the gene on chromosome 7q36.1, emphasising that the variability of phenotypes and that presentation in childhood is common. Furthermore, we highlight that skeletal myopathy and hypertrophic cardiomyopathy are significant debilitating characteristics of the PRKAG2 mutation. Conclusion. In our report of adult and pediatric patients, early presentation in childhood with hypertrophic cardiomyopathy and skeletal muscle involvement was common, demonstrating the challenges of the clinical management of PRKAG2 mutations.

Journal article

Norrish G, Field E, Mcleod K, Ilina M, Stuart G, Bhole V, Uzun O, Brown E, Daubeney PEF, Lota A, Linter K, Mathur S, Bharucha T, Kok KL, Adwani S, Jones CB, Reinhardt Z, Kaski JPet al., 2019, Clinical presentation and survival of childhood hypertrophic cardiomyopathy: a retrospective study in United Kingdom, European Heart Journal, Vol: 40, Pages: 986-993, ISSN: 1522-9645

Aims: Understanding the spectrum of disease, symptom burden and natural history are essential for the management of children with hypertrophic cardiomyopathy (HCM). The effect of changing screening practices over time has not previously been studied. This study describes the clinical characteristics and outcomes of childhood HCM over four decades in a well-characterized United Kingdom cohort. Methods and results: Six hundred and eighty-seven patients with HCM presented at a median age of 5.2 years (range 0-16). Aetiology was: non-syndromic (n = 433, 63%), RASopathy (n = 126, 18.3%), Friedreich's ataxia (n = 59, 8.6%) or inborn errors of metabolism (IEM) (n = 64, 9%). In infants (n = 159, 23%) underlying aetiology was more commonly a RASopathy (42% vs. 11.2%, P < 0.0001) or IEM (18.9% vs. 6.4% P < 0.0001). In those with familial disease, median age of presentation was higher (11 years vs. 6 years, P < 0.0001), 141 (58%) presented <12 years. Freedom from death or transplantation was 90.6% (87.9-92.7%) at 5 years (1.5 per 100 patient years) with no era effect. Mortality was most frequently sudden cardiac death (SCD) (n = 20, 2.9%). Children diagnosed during infancy or with an IEM had a worse prognosis (5-year survival 80.5% or 66.4%). Arrhythmic events occurred at a rate of 1.2 per 100 patient years and were more likely in non-syndromic patients (n = 51, 88%). Conclusion: This national study describes a heterogeneous disease whose outcomes depend on the age of presentation and aetiology. Overall mortality and SCD rates have not changed over time, but they remain higher than in adults with HCM, with events occurring in syndromic and non-syndromic patients.

Journal article

Van Bergen NJ, Guo Y, Rankin J, Paczia N, Becker-Kettern J, Kremer LS, Pyle A, Conrotte J-F, Ellaway C, Procopis P, Prelog K, Homfray T, Baptista J, Baple E, Wakeling M, Massey S, Kay DP, Shukla A, Girisha KM, Lewis LES, Santra S, Power R, Daubeney P, Montoya J, Ruiz-Pesini E, Kovacs-Nagy R, Pritsch M, Ahting U, Thorburn DR, Prokisch H, Taylor RW, Christodoulou J, Linster CL, Ellard S, Hakonarson Het al., 2019, NAD(P)HX dehydratase (NAXD) deficiency: a novel neurodegenerative disorder exacerbated by febrile illnesses, BRAIN, Vol: 142, Pages: 50-58, ISSN: 0006-8950

Journal article

Sabatino J, Daubeney P, Fraisse A, Josen M, Paredes J, Uy MP, Prota C, Bucciarelli V, Sirico D, Di Salvo Get al., 2018, Left Atrial Strain Classification of Diastolic Dysfunction in Pediatric Cardiomyopathies, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322

Conference paper

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