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2. Sudden cardiac death in childhood RASopathy-associated hypertrophic cardiomyopathy: Validation of the HCM risk-kids model and predictors of events

Author

Boleti, Olga D., Roussos, Sotirios, Norrish, Gabrielle, Field, Ella, Oates, Stephanie, Tollit, Jennifer, Nepali, Gauri, Bhole, Vinay, Uzun, Orhan, Daubeney, Piers E.F., Stuart, Graham A., Fernandes, Precylia, McLeod, Karen, Ilina, Maria, Liaqath, Muhammad Najih Ali, Bharucha, Tara, Delle Donne, Grazia, Brown, Elspeth, Linter, Katie, Khodaghalian, Bernadette, Jones, Caroline, Searle, Jonathan, Mathur, Sujeev, Boyd, Nicola, Reindhardt, Zdenka, Duignan, Sophie, Prendiville, Terence, Adwani, Satish, Zenker, Martin, Wolf, Cordula Maria, and Kaski, Juan Pablo

Published
2023
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3. Management of aortic disease in children with FBN1-related Marfan syndrome: A joint statement from the paediatric subgroup of the European Reference Network of Vascular Diseases (VASCERN, Heritable Thoracic Aortic Disease working group) and the Association for European Paediatric and Congenital Cardiology (AEPC)

Author

Muiño-Mosquera, Laura, Cervi, Elena, Groote, Katya De, Dewals, Wendy, Fejzic, Zina, Kazamia, Kalliopi, Mathur, Sujeev, Milleron, Olivier, Mir, Thomas S, Nielsen, Dorte G, Odermarsky, Michal, Sabate-Rotes, Anna, van der Hulst, Annelies, Valenzuela, Irene, and Jondeau, Guillaume

Subjects
ASCENDING aorta dissection, MARFAN syndrome, PEDIATRIC cardiology, SPORTS participation, THERAPEUTICS
Abstract

Marfan syndrome (MFS) is a hereditary connective tissue disorder with an estimated prevalence of 1:5000–1:10 000 individuals. It is a pleiotropic disease characterized by specific ocular, cardiovascular, and skeletal features. The most common cardiovascular complication is aortic root dilatation which untreated can lead to life-threatening aortic root dissection, mainly occurring in adult patients. Prompt diagnosis, appropriate follow-up, and timely treatment can prevent aortic events. Currently there are no specific recommendations for treatment of children with MFS, and management is greatly based on adult guidelines. Furthermore, due to the scarcity of studies including children, there is a lack of uniform treatment across different centres. This consensus document aims at bridging these gaps of knowledge. This work is a joint collaboration between the paediatric subgroup of the European Network of Vascular Diseases (VASCERN, Heritable Thoracic Aortic Disease Working Group) and the Association for European Paediatric and Congenital Cardiology (AEPC). A group of experts from 12 different centres and 8 different countries participated in this effort. This document reviews four main subjects, namely, (i) imaging of the aorta at diagnosis and follow-up, (ii) recommendations on medical treatment, (iii) recommendations on surgical treatment, and (iv) recommendations on sport participation. [ABSTRACT FROM AUTHOR]

Published
2024
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4. Irbesartan in Marfan syndrome (AIMS): a double-blind, placebo-controlled randomised trial

Author

Dean, John, Was, Bartosz, Gow, Heather, Murray, Jane, D'Allessandro, Mariella, Christie, Michael, Cooper, Patricia, Booth, Philip, Burns, Sharon, Paterson, Yvonne, Chikermane, Ashish, Assing, Anthony, Cotter, Catherine, Atkins, Gillian, Williamson, Helen, Barclay, Justin, Jennison, Alan, Henderson, Alex, McSkeane, Anna, Fairlamb, Helen, Kelly, Julie, Kelsall, Nicola, Prentice, Scott, O'Sullivan, John, Head-Baister, Alison, Phillipson, Angela, Johnson, Anna, Crossland, D, Oliver, Jack, Davison, Jade, Wake, Jill, Quinn, Louise, Foreman, Maureen, Wealleans, Vera, Walker, Niki, Duncan, Alexis, Tibbs, Evelyn, Kelly, Ruth, Khambadkone, Sachin, Zotti, Bridget, Brady, Cassie, Cervi, Elena, Field, Ella, Szepezvary, Eszter, Mantey, Florence, Riley, Gillian, Titmus, Heather, Bo, Ilaria, Kaski, Juan Pablo, Green, Loren, Jones, Nigel, Banks, Rebecca, Kiesewetter, Christopher, Mathur, Sujeev, Frigiola, Alessandra, Savis, Alex, Belfield, Holly, Guzman, Josephine, Harris, Julia, Wilson, Karen, Peacock, Kelly, Gibson, Kirsty, Wellman, Paul, Simpson, John, Kabir, Saleha, Mushemi, Sitali, Stewart, Michael, Atkinson, Bev, Richardson, Cath, Leng, Elaine, Brennan, Paul, Nixon, Annabel, Spencer, Collette, Oliver, James, Forster, Jan, Turner, Louise, Bainbridge, Samantha, Choy, Anna Maria, Dawson, Adelle, Kiddie, Gwen, Kerr, Heather, Mordi, Ify, Duff, Jackie, Dunlop, Jacqueline, Berg, Jonathan, Armory, Pauline, Freeman, Leisa, Anwar, Amir, Graham, Charles, London, Clare, Healey, Gail, Gallagher, Ian, Ilsley, Mary, Ahmed, Rizwan, Wood, Sheila, Wheeldon, Nigel, Mason, Cecilia, Nassim, Farook, Middle, Janet, Adams, Justin, Angelini, Karen, Housley, Kay, Ryalls, Kim, Agyemang, Michael, Walker, Rachel, Batigan, Robina, Bennett, Tina, Clift, Paul, Alvior, Amor Mia, Nilsson, Annette, Green, Carole, Crook, Charlotte, Palmer, Connie Becani, Dwenger, Elizabeth, Doherty, Phillipa, Igbokwe, Rebecca, Sharif, Saba, MacDonald, Sonia, West, Cathy, Kirby, Kevin, Naqvi, Nitha, Welch, Sophie, Warsama, Suad, Li, Wei, Farzad, Zohreh, Smith, Ben, Murday, Victoria, Murtagh, Eamonn, Adams, Emma, Armour, Lesley, Lilley, Stuart, Pandya, Bejal, Richards, Amy, Andiapen, Mervyn, Macrae, Rebecca, Tome, Maite, Hutchinson, Carmel, Angulo, Kameka, Kauppayamootoo, Rooba, Gati, Sabiha, Cruddas, Elizabeth, Newman, William G, Breen, Catherine, Kumar, Dhavendra, Wilson, Dirk G, Farrugia, Adele, Fraser, Alan, Sumers, Jayne, Powell, Jessie, Edwards, Julie, Hale, Terese, Boult, Zoe, Carroll, Aisling, Veldtman, Gruschen, Ho, Andrew, Black, David, Fletcher, Lisa, Mapstone, Sue, Bharucha, Tara, Marsh, Gary, Jones, Joanne, Sheehan, Karen, Selway, Kathleen, Stevenson, Kirsty, Nelson, Martin, Fairweather, Rebecca, Curtis, Stephanie, Simpson, Sue, Denvir, Martin, White, Audrey, Steven, Jill, Munro, Joanna, Lam, Wayne, Toff, William, Petrou, Mario, Silcocks, Paul, MacAllister, Raymond, Mullen, Michael, Jin, Xu Yu, Child, Anne, Stuart, A Graham, Dodd, Matthew, Aragon-Martin, José Antonio, Gaze, David, Kiotsekoglou, Anatoli, Yuan, Li, Hu, Jiangting, Foley, Claire, Van Dyck, Laura, Knight, Rosemary, Clayton, Tim, Swan, Lorna, Thomson, John D R, Erdem, Guliz, Crossman, David, and Flather, Marcus

Published
2019
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5. Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy

Author

Norrish, Gabrielle, Ding, Tao, Field, Ella, Cervi, Elena, Ziółkowska, Lidia, Olivotto, Iacopo, Khraiche, Diala, Limongelli, Giuseppe, Anastasakis, Aris, Weintraub, Robert, Biagini, Elena, Ragni, Luca, Prendiville, Terrence, Duignan, Sophie, McLeod, Karen, Ilina, Maria, Fernández, Adrián, Marrone, Chiara, Bökenkamp, Regina, Baban, Anwar, Kubus, Peter, Daubeney, Piers E.F., Sarquella-Brugada, Georgia, Cesar, Sergi, Klaassen, Sabine, Ojala, Tiina H., Bhole, Vinay, Medrano, Constancio, Uzun, Orhan, Brown, Elspeth, Gran, Ferran, Sinagra, Gianfranco, Castro, Francisco J., Stuart, Graham, Vignati, Gabriele, Yamazawa, Hirokuni, Barriales-Villa, Roberto, Garcia-Guereta, Luis, Adwani, Satish, Linter, Katie, Bharucha, Tara, Garcia-Pavia, Pablo, Siles, Ana, Rasmussen, Torsten B., Calcagnino, Margherita, Jones, Caroline B., De Wilde, Hans, Kubo, Toru, Felice, Tiziana, Popoiu, Anca, Mogensen, Jens, Mathur, Sujeev, Centeno, Fernando, Reinhardt, Zdenka, Schouvey, Sylvie, O’Mahony, Costas, Omar, Rumana Z., Elliott, Perry M., and Kaski, Juan Pablo

Published
2022
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6. Three-dimensional visualisation of the fetal heart using prenatal MRI with motion-corrected slice-volume registration: a prospective, single-centre cohort study

Author

Lloyd, David F A, Pushparajah, Kuberan, Simpson, John M, van Amerom, Joshua F P, van Poppel, Milou P M, Schulz, Alexander, Kainz, Bernard, Deprez, Maria, Lohezic, Maelene, Allsop, Joanna, Mathur, Sujeev, Bellsham-Revell, Hannah, Vigneswaran, Trisha, Charakida, Marietta, Miller, Owen, Zidere, Vita, Sharland, Gurleen, Rutherford, Mary, Hajnal, Joseph V, and Razavi, Reza

Published
2019
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7. Natural history and outcomes in paediatric RASopathy‐associated hypertrophic cardiomyopathy

Author

Boleti, Olga, primary, Norrish, Gabrielle, additional, Field, Ella, additional, Dady, Kathleen, additional, Summers, Kim, additional, Nepali, Gauri, additional, Bhole, Vinay, additional, Uzun, Orhan, additional, Wong, Amos, additional, Daubeney, Piers E. F., additional, Stuart, Graham, additional, Fernandes, Precylia, additional, McLeod, Karen, additional, Ilina, Maria, additional, Ali, Muhammad Najih Liaqath, additional, Bharucha, Tara, additional, Donne, Grazia Delle, additional, Brown, Elspeth, additional, Linter, Katie, additional, Jones, Caroline B., additional, Searle, Jonathan, additional, Regan, William, additional, Mathur, Sujeev, additional, Boyd, Nicola, additional, Reinhardt, Zdenka, additional, Duignan, Sophie, additional, Prendiville, Terence, additional, Adwani, Satish, additional, and Kaski, Juan Pablo, additional

Published
2024
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19. Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy

Author

Norrish, Gabrielle, primary, Cleary, Aoife, additional, Field, Ella, additional, Cervi, Elena, additional, Boleti, Olga, additional, Ziółkowska, Lidia, additional, Olivotto, Iacopo, additional, Khraiche, Diala, additional, Limongelli, Giuseppe, additional, Anastasakis, Aris, additional, Weintraub, Robert, additional, Biagini, Elena, additional, Ragni, Luca, additional, Prendiville, Terence, additional, Duignan, Sophie, additional, McLeod, Karen, additional, Ilina, Maria, additional, Fernandez, Adrian, additional, Marrone, Chiara, additional, Bökenkamp, Regina, additional, Baban, Anwar, additional, Kubus, Peter, additional, Daubeney, Piers E.F., additional, Sarquella-Brugada, Georgia, additional, Cesar, Sergi, additional, Klaassen, Sabine, additional, Ojala, Tiina H., additional, Bhole, Vinay, additional, Medrano, Constancio, additional, Uzun, Orhan, additional, Brown, Elspeth, additional, Gran, Ferran, additional, Sinagra, Gianfranco, additional, Castro, Francisco J., additional, Stuart, Graham, additional, Yamazawa, Hirokuni, additional, Barriales-Villa, Roberto, additional, Garcia-Guereta, Luis, additional, Adwani, Satish, additional, Linter, Katie, additional, Bharucha, Tara, additional, Gonzales-Lopez, Esther, additional, Siles, Ana, additional, Rasmussen, Torsten B., additional, Calcagnino, Margherita, additional, Jones, Caroline B., additional, De Wilde, Hans, additional, Kubo, Toru, additional, Felice, Tiziana, additional, Popoiu, Anca, additional, Mogensen, Jens, additional, Mathur, Sujeev, additional, Centeno, Fernando, additional, Reinhardt, Zdenka, additional, Schouvey, Sylvie, additional, Elliott, Perry M., additional, and Kaski, Juan Pablo, additional

Published
2022
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20. EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Cardiovascular MRI and CT in congenital heart disease

Author

Pushparajah, Kuberan, Duong, Phuoc, Mathur, Sujeev, and Babu-Narayan, Sonya V

Subjects
genetic structures, paediatric cardiology, cardiac magnetic resonance imaging, cardiac CT, Review, CMR, congenital heart disease
Abstract

Cardiac MRI and CT are increasingly used in the diagnosis and management of patients with congenital heart disease as an imaging adjunct to echocardiography. The benefits and limitations of both modalities are highlighted, with a focus on the anatomical, functional and haemodynamic information that can be gained from the different modalities. Deciding on the imaging modality of choice must also take into account patient factors such as age, compliance, the type of congenital heart disease, and previous procedures. Future developments in CT and MRI are also discussed.

Published
2019

23. Cardiac Function After Cardiotoxic Treatments for Childhood Cancer : Left Ventricular Longitudinal Strain in Screening

Author

Niemelä, Jussi, Ylänen, Kaisa, Suominen, Anu, Pushparajah, Kuberan, Mathur, Sujeev, Sarkola, Taisto, Jahnukainen, Kirsi, Eerola, Anneli, Poutanen, Tuija, Vettenranta, Kim, Ojala, Tiina, Tampere University, Department of Paediatrics, Clinical Medicine, HUS Children and Adolescents, Children's Hospital, Clinicum, and Lastentautien yksikkö

Subjects
CARDIOVASCULAR TOXICITY, cardiotoxicity, ADULT SURVIVORS, Cardiovascular Medicine, 3121 Internal medicine, SYSTOLIC FUNCTION, EUROPEAN-ASSOCIATION, WRITING GROUP, speckle tracking, 3123 Gynaecology and paediatrics, 3121 General medicine, internal medicine and other clinical medicine, YOUNG-ADULTS, cardiovascular risk (CV risk), RISK-FACTORS, HEART-FAILURE, childhood cancer, ECHOCARDIOGRAPHY, AMERICAN-SOCIETY, Original Research, longitudinal strain
Abstract

Background: The majority of childhood cancer survivors (CCSs) have been exposed to cardiotoxic treatments and often present with modifiable cardiovascular risk factors. Our aim was to evaluate the value of left ventricular (LV) longitudinal strain for increasing the sensitivity of cardiac dysfunction detection among CCSs. Methods: We combined two national cohorts: neuroblastoma and other childhood cancer survivors treated with anthracyclines. The final data consisted of 90 long-term CCSs exposed to anthracyclines and/or high-dose chemotherapy with autologous stem cell rescue and followed up for > 5 years and their controls (n = 86). LV longitudinal strain was assessed with speckle tracking (Qlab) and LV ejection fraction (EF) by three-dimensional echocardiography (3DE). Results: Of the CCSs, 11% (10/90) had abnormal LV longitudinal strain (i.e., < -17.5%); of those, 70% (7/10) had normal 3DE LV EF. Multivariable linear model analysis demonstrated that follow-up time (p = 0.027), sex (p = 0.020), and BMI (p = 0.002) were significantly associated with LV longitudinal strain. Conversely, cardiac risk group, hypertension, age, cumulative anthracycline dose or exposure to chest radiation were not. Conclusion: LV longitudinal strain is a more sensitive method than LV EF for the detection of cardiac dysfunction among CCSs. Therefore, LV longitudinal strain should be added to the screening panel, especially for those with modifiable cardiovascular risk factors.

Published
2021

24. Clinical presentation and long‐term outcomes of infantile hypertrophic cardiomyopathy: a European multicentre study

Author

Norrish, Gabrielle, primary, Kolt, Gali, additional, Cervi, Elena, additional, Field, Ella, additional, Dady, Kathleen, additional, Ziółkowska, Lidia, additional, Olivotto, Iacopo, additional, Favilli, Silvia, additional, Passantino, Silvia, additional, Limongelli, Giuseppe, additional, Caiazza, Martina, additional, Rubino, Marta, additional, Baban, Anwar, additional, Drago, Fabrizio, additional, Mcleod, Karen, additional, Ilina, Maria, additional, McGowan, Ruth, additional, Stuart, Graham, additional, Bhole, Vinay, additional, Uzun, Orhan, additional, Wong, Amos, additional, Lazarou, Laz, additional, Brown, Elspeth, additional, Daubeney, Piers E.F., additional, Lota, Amrit, additional, Delle Donne, Grazia, additional, Linter, Katie, additional, Mathur, Sujeev, additional, Bharucha, Tara, additional, Adwani, Satish, additional, Searle, Jon, additional, Popoiu, Anca, additional, Jones, Caroline B., additional, Reinhardt, Zdenka, additional, and Kaski, Juan Pablo, additional

Published
2021
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25. Dilated ascending aorta in the fetus

Author

Dumitrascu‐Biris, Ioana, primary, Zidere, Vita, additional, Vigneswaran, Trisha, additional, Charakida, Marietta, additional, Mathur, Sujeev, additional, Kametas, Nick, additional, and Simpson, John, additional

Published
2021
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27. Clinical outcomes and programming strategies of implantable cardioverter-defibrillator devices in paediatric hypertrophic cardiomyopathy: a UK National Cohort Study

Author

Norrish, Gabrielle, primary, Chubb, Henry, additional, Field, Ella, additional, McLeod, Karen, additional, Ilina, Maria, additional, Spentzou, Georgia, additional, Till, Jan, additional, Daubeney, Piers E F, additional, Stuart, Alan Graham, additional, Matthews, Jane, additional, Hares, Dominic, additional, Brown, Elspeth, additional, Linter, Katie, additional, Bhole, Vinay, additional, Pillai, Krishnakumar, additional, Bowes, Michael, additional, Jones, Caroline B, additional, Uzun, Orhan, additional, Wong, Amos, additional, Yue, Arthur, additional, Sadagopan, Shankar, additional, Bharucha, Tara, additional, Yap, Norah, additional, Rosenthal, Eric, additional, Mathur, Sujeev, additional, Adwani, Satish, additional, Reinhardt, Zdenka, additional, Mangat, Jasveer, additional, and Kaski, Juan Pablo, additional

Published
2020
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28. Multimodality cardiac evaluation in children and young adults with multisystem inflammation associated with COVID-19

Author

Theocharis, Paraskevi, primary, Wong, James, additional, Pushparajah, Kuberan, additional, Mathur, Sujeev K, additional, Simpson, John M, additional, Pascall, Emma, additional, Cleary, Aoife, additional, Stewart, Kirsty, additional, Adhvaryu, Kaitav, additional, Savis, Alex, additional, Kabir, Saleha R, additional, Uy, Mirasol Pernia, additional, Heard, Hannah, additional, Peacock, Kelly, additional, and Miller, Owen, additional

Published
2020
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29. Clinical presentation and survival of childhood hypertrophic cardiomyopathy: a retrospective study in United Kingdom

Author

Norrish, Gabrielle, Field, Ella, Mcleod, Karen, Ilina, Maria, Stuart, Graham, Bhole, Vinay, Uzun, Orhan, Brown, Elspeth, Daubeney, Piers E F, Lota, Amrit, Linter, Katie, Mathur, Sujeev, Bharucha, Tara, Kok, Khoon Li, Adwani, Satish, Jones, Caroline B, Reinhardt, Zdenka, and Kaski, Juan Pablo

Subjects
Male, Myocardial Disease, Cardiac & Cardiovascular Systems, Adolescent, Survival, Developmental Disabilities, CHILDREN, AMERICAN-COLLEGE, DIAGNOSIS, Global Burden of Disease, RISK STRATIFICATION, Clinical Research, PEDIATRIC CARDIOMYOPATHY, Humans, EPIDEMIOLOGY, Aetiology, Child, 1102 Cardiorespiratory Medicine and Haematology, Retrospective Studies, CARDIOLOGY, Science & Technology, Infant, Newborn, Infant, Cardiomyopathy, Hypertrophic, United Kingdom, EUROPEAN-SOCIETY, Hypertrophic cardiomyopathy, Editor's Choice, Death, Sudden, Cardiac, Cardiovascular System & Hematology, Friedreich Ataxia, Child, Preschool, Cardiovascular System & Cardiology, Female, Life Sciences & Biomedicine, SUDDEN CARDIAC DEATH, Metabolism, Inborn Errors, TASK-FORCE
Abstract

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

Published
2018

30. Irbesartan in Marfan syndrome (AIMS): a double-blind, placebo-controlled randomised trial

Author

Mullen, Michael, primary, Jin, Xu Yu, additional, Child, Anne, additional, Stuart, A Graham, additional, Dodd, Matthew, additional, Aragon-Martin, José Antonio, additional, Gaze, David, additional, Kiotsekoglou, Anatoli, additional, Yuan, Li, additional, Hu, Jiangting, additional, Foley, Claire, additional, Van Dyck, Laura, additional, Knight, Rosemary, additional, Clayton, Tim, additional, Swan, Lorna, additional, Thomson, John D R, additional, Erdem, Guliz, additional, Crossman, David, additional, Flather, Marcus, additional, Dean, John, additional, Was, Bartosz, additional, Gow, Heather, additional, Murray, Jane, additional, D'Allessandro, Mariella, additional, Christie, Michael, additional, Cooper, Patricia, additional, Booth, Philip, additional, Burns, Sharon, additional, Paterson, Yvonne, additional, Chikermane, Ashish, additional, Assing, Anthony, additional, Cotter, Catherine, additional, Atkins, Gillian, additional, Williamson, Helen, additional, Barclay, Justin, additional, Jennison, Alan, additional, Henderson, Alex, additional, McSkeane, Anna, additional, Fairlamb, Helen, additional, Kelly, Julie, additional, Kelsall, Nicola, additional, Prentice, Scott, additional, O'Sullivan, John, additional, Head-Baister, Alison, additional, Phillipson, Angela, additional, Johnson, Anna, additional, Crossland, D, additional, Oliver, Jack, additional, Davison, Jade, additional, Wake, Jill, additional, Quinn, Louise, additional, Foreman, Maureen, additional, Wealleans, Vera, additional, Walker, Niki, additional, Duncan, Alexis, additional, Tibbs, Evelyn, additional, Kelly, Ruth, additional, Khambadkone, Sachin, additional, Zotti, Bridget, additional, Brady, Cassie, additional, Cervi, Elena, additional, Field, Ella, additional, Szepezvary, Eszter, additional, Mantey, Florence, additional, Riley, Gillian, additional, Titmus, Heather, additional, Bo, Ilaria, additional, Kaski, Juan Pablo, additional, Green, Loren, additional, Jones, Nigel, additional, Banks, Rebecca, additional, Kiesewetter, Christopher, additional, Mathur, Sujeev, additional, Frigiola, Alessandra, additional, Savis, Alex, additional, Belfield, Holly, additional, Guzman, Josephine, additional, Harris, Julia, additional, Wilson, Karen, additional, Peacock, Kelly, additional, Gibson, Kirsty, additional, Wellman, Paul, additional, Simpson, John, additional, Kabir, Saleha, additional, Mushemi, Sitali, additional, Stewart, Michael, additional, Atkinson, Bev, additional, Richardson, Cath, additional, Leng, Elaine, additional, Brennan, Paul, additional, Nixon, Annabel, additional, Spencer, Collette, additional, Oliver, James, additional, Forster, Jan, additional, Turner, Louise, additional, Bainbridge, Samantha, additional, Choy, Anna Maria, additional, Dawson, Adelle, additional, Kiddie, Gwen, additional, Kerr, Heather, additional, Mordi, Ify, additional, Duff, Jackie, additional, Dunlop, Jacqueline, additional, Berg, Jonathan, additional, Armory, Pauline, additional, Freeman, Leisa, additional, Anwar, Amir, additional, Graham, Charles, additional, London, Clare, additional, Healey, Gail, additional, Gallagher, Ian, additional, Ilsley, Mary, additional, Ahmed, Rizwan, additional, Wood, Sheila, additional, Wheeldon, Nigel, additional, Mason, Cecilia, additional, Nassim, Farook, additional, Middle, Janet, additional, Adams, Justin, additional, Angelini, Karen, additional, Housley, Kay, additional, Ryalls, Kim, additional, Agyemang, Michael, additional, Walker, Rachel, additional, Batigan, Robina, additional, Bennett, Tina, additional, Clift, Paul, additional, Alvior, Amor Mia, additional, Nilsson, Annette, additional, Green, Carole, additional, Crook, Charlotte, additional, Palmer, Connie Becani, additional, Dwenger, Elizabeth, additional, Doherty, Phillipa, additional, Igbokwe, Rebecca, additional, Sharif, Saba, additional, MacDonald, Sonia, additional, West, Cathy, additional, Kirby, Kevin, additional, Naqvi, Nitha, additional, Welch, Sophie, additional, Warsama, Suad, additional, Li, Wei, additional, Farzad, Zohreh, additional, Smith, Ben, additional, Murday, Victoria, additional, Murtagh, Eamonn, additional, Adams, Emma, additional, Armour, Lesley, additional, Lilley, Stuart, additional, Pandya, Bejal, additional, Richards, Amy, additional, Andiapen, Mervyn, additional, Macrae, Rebecca, additional, Tome, Maite, additional, Hutchinson, Carmel, additional, Angulo, Kameka, additional, Kauppayamootoo, Rooba, additional, Gati, Sabiha, additional, Cruddas, Elizabeth, additional, Newman, William G, additional, Breen, Catherine, additional, Kumar, Dhavendra, additional, Wilson, Dirk G, additional, Farrugia, Adele, additional, Fraser, Alan, additional, Sumers, Jayne, additional, Powell, Jessie, additional, Edwards, Julie, additional, Hale, Terese, additional, Boult, Zoe, additional, Carroll, Aisling, additional, Veldtman, Gruschen, additional, Ho, Andrew, additional, Black, David, additional, Fletcher, Lisa, additional, Mapstone, Sue, additional, Bharucha, Tara, additional, Marsh, Gary, additional, Jones, Joanne, additional, Sheehan, Karen, additional, Selway, Kathleen, additional, Stevenson, Kirsty, additional, Nelson, Martin, additional, Fairweather, Rebecca, additional, Curtis, Stephanie, additional, Simpson, Sue, additional, Denvir, Martin, additional, White, Audrey, additional, Steven, Jill, additional, Munro, Joanna, additional, Lam, Wayne, additional, Toff, William, additional, Petrou, Mario, additional, Silcocks, Paul, additional, and MacAllister, Raymond, additional

Published
2019
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31. Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids)

Author

Norrish, Gabrielle, primary, Ding, Tao, additional, Field, Ella, additional, Ziólkowska, Lidia, additional, Olivotto, Iacopo, additional, Limongelli, Giuseppe, additional, Anastasakis, Aristides, additional, Weintraub, Robert, additional, Biagini, Elena, additional, Ragni, Luca, additional, Prendiville, Terence, additional, Duignan, Sophie, additional, McLeod, Karen, additional, Ilina, Maria, additional, Fernández, Adrián, additional, Bökenkamp, Regina, additional, Baban, Anwar, additional, Kubuš, Peter, additional, Daubeney, Piers E. F., additional, Sarquella-Brugada, Georgia, additional, Cesar, Sergi, additional, Marrone, Chiara, additional, Bhole, Vinay, additional, Medrano, Constancio, additional, Uzun, Orhan, additional, Brown, Elspeth, additional, Gran, Ferran, additional, Castro, Francisco J., additional, Stuart, Graham, additional, Vignati, Gabriele, additional, Barriales-Villa, Roberto, additional, Guereta, Luis G., additional, Adwani, Satish, additional, Linter, Katie, additional, Bharucha, Tara, additional, Garcia-Pavia, Pablo, additional, Rasmussen, Torsten B., additional, Calcagnino, Margherita M., additional, Jones, Caroline B., additional, De Wilde, Hans, additional, Toru-Kubo, J., additional, Felice, Tiziana, additional, Mogensen, Jens, additional, Mathur, Sujeev, additional, Reinhardt, Zdenka, additional, O’Mahony, Constantinos, additional, Elliott, Perry M., additional, Omar, Rumana Z., additional, and Kaski, Juan P., additional

Published
2019
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32. A validation study of the European Society of Cardiology guidelines for risk stratification of sudden cardiac death in childhood hypertrophic cardiomyopathy

Author

Norrish, Gabrielle, primary, Ding, Tao, additional, Field, Ella, additional, McLeod, Karen, additional, Ilina, Maria, additional, Stuart, Graham, additional, Bhole, Vinay, additional, Uzun, Orhan, additional, Brown, Elspeth, additional, Daubeney, Piers E F, additional, Lota, Amrit, additional, Linter, Katie, additional, Mathur, Sujeev, additional, Bharucha, Tara, additional, Kok, Khoon Li, additional, Adwani, Satish, additional, Jones, Caroline B, additional, Reinhardt, Zdenka, additional, Omar, Rumana Z, additional, and Kaski, Juan Pablo, additional

Published
2019
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33. Multimodality cardiac evaluation in children and young adults with multisystem inflammation associated with COVID-19.

Author

Theocharis, Paraskevi, Wong, James, Pushparajah, Kuberan, Mathur, Sujeev K, Simpson, John M, Pascall, Emma, Cleary, Aoife, Stewart, Kirsty, Adhvaryu, Kaitav, Savis, Alex, Kabir, Saleha R, Uy, Mirasol Pernia, Heard, Hannah, Peacock, Kelly, and Miller, Owen

Subjects
MYOCARDIAL infarction risk factors, HEART disease diagnosis, HEART diseases, VENTRICULAR ejection fraction, COMPUTED tomography, INFLAMMATION, COVID-19 pandemic, COVID-19, ECHOCARDIOGRAPHY, ADOLESCENCE, CHILDREN
Abstract

Aims Following the peak of the UK COVID-19 epidemic, a new multisystem inflammatory condition with significant cardiovascular effects emerged in young people. We utilized multimodality imaging to provide a detailed sequential description of the cardiac involvement. Methods and Results Twenty consecutive patients (mean age 10.6 ± 3.8 years) presenting to our institution underwent serial echocardiographic evaluation on admission (median day 5 of illness), the day coinciding with worst cardiac function (median day 7), and the day of discharge (median day 15). We performed cardiac computed tomography (CT) to assess coronary anatomy (median day 15) and cardiac magnetic resonance imaging (CMR) to assess dysfunction (median day 20). On admission, almost all patients displayed abnormal strain and tissue Doppler indices. Three-dimensional (3D) echocardiographic ejection fraction (EF) was <55% in half of the patients. Valvular regurgitation (75%) and small pericardial effusions (10%) were detected. Serial echocardiography demonstrated that the mean 3D EF deteriorated (54.7 ± 8.3% vs. 46.4 ± 8.6%, P = 0.017) before improving at discharge (P = 0.008). Left main coronary artery (LMCA) dimensions were significantly larger at discharge than at admission (Z score –0.11 ± 0.87 vs. 0.78 ± 1.23, P = 0.007). CT showed uniform coronary artery dilatation commonly affecting the LMCA (9/12). CMR detected abnormal strain in all patients with global dysfunction (EF <55%) in 35%, myocardial oedema in 50%, and subendocardial infarct in 5% (1/20) patients. Conclusions Pancarditis with cardiac dysfunction is common and associated with myocardial oedema. Patients require close monitoring due to coronary artery dilatation and the risk of thrombotic myocardial infarction. [ABSTRACT FROM AUTHOR]

Published
2021
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34. Visualization of coronary arteries in paediatric patients using whole-heart coronary magnetic resonance angiography: comparison of image-navigation and the standard approach for respiratory motion compensation

Author

Instituto de Biomedicina de Sevilla (IBIS), Velasco Forte, Mari Nieves, Valverde, Israel, Prabhu, Nanda, Correia, Teresa, Narayan, Srinivas Ananth, Bell, Aaron, Mathur, Sujeev, Razavi, Reza, Hussain, Tarique, Pushparajah, Kuberan, Henningsson, Markus, Instituto de Biomedicina de Sevilla (IBIS), Velasco Forte, Mari Nieves, Valverde, Israel, Prabhu, Nanda, Correia, Teresa, Narayan, Srinivas Ananth, Bell, Aaron, Mathur, Sujeev, Razavi, Reza, Hussain, Tarique, Pushparajah, Kuberan, and Henningsson, Markus

Abstract

Aims: To investigate the use of respiratory motion compensation using image-based navigation (iNAV) with constant respiratory efficiency using single end-expiratory thresholding (CRUISE) for coronary magnetic resonance angiography (CMRA), and compare it to the conventional diaphragmatic navigator (dNAV) in paediatric patients with congenital or suspected heart disease. Methods: iNAV allowed direct tracking of the respiratory heart motion and was generated using balanced steady state free precession startup echoes. Respiratory gating was achieved using CRUISE with a fixed 50% efficiency. Whole-heart CMRA was acquired with 1.3mm isotropic resolution. For comparison, CMRA with identical imaging parameters were acquired using dNAV. Scan time, visualization of coronary artery origins and mid-course, imaging quality and sharpness was compared between the two sequences. Results: Forty patients (13 females; median weight: 44 kg; median age: 12.6, range: 3 months–17 years) were enrolled. 25 scans were performed in awake patients. A contrast agent was used in 22 patients. The scan time was significantly reduced using iNAV for awake patients (iNAV 7:48 ± 1:26 vs dNAV 9:48 ± 3:11, P = 0.01) but not for patients under general anaesthesia (iNAV = 6:55 ± 1:50 versus dNAV = 6:32 ± 2:16; P = 0.32). In 98% of the cases, iNAV image quality had an equal or higher score than dNAV. The visual score analysis showed a clear difference, favouring iNAV (P = 0.002). The right coronary artery and the left anterior descending vessel sharpness was significantly improved (iNAV: 56.8% ± 10.1% vs dNAV: 53.7% ± 9.9%, P < 0.002 and iNAV: 55.8% ± 8.6% vs dNAV: 53% ± 9.2%, P = 0.001, respectively). Conclusion: iNAV allows for a higher success-rate and clearer depiction of the mid-course of coronary arteries in paediatric patients. Its acquisition time is shorter in awake patients and image quality score is equal or superior to the conventional method in most cases.

Published
2019

35. Visualization of coronary arteries in paediatric patients using whole-heart coronary magnetic resonance angiography: comparison of image-navigation and the standard approach for respiratory motion compensation

Author

Forte, Mari Nieves Velasco, Valverde, Israel, Prabhu, Nanda, Correia, Teresa, Narayan, Srinivas Ananth, Bell, Aaron, Mathur, Sujeev, Razavi, Reza, Hussain, Tarique, Pushparajah, Kuberan, Henningsson, Markus, Forte, Mari Nieves Velasco, Valverde, Israel, Prabhu, Nanda, Correia, Teresa, Narayan, Srinivas Ananth, Bell, Aaron, Mathur, Sujeev, Razavi, Reza, Hussain, Tarique, Pushparajah, Kuberan, and Henningsson, Markus

Abstract

AimsTo investigate the use of respiratory motion compensation using image-based navigation (iNAV) with constant respiratory efficiency using single end-expiratory thresholding (CRUISE) for coronary magnetic resonance angiography (CMRA), and compare it to the conventional diaphragmatic navigator (dNAV) in paediatric patients with congenital or suspected heart disease.MethodsiNAV allowed direct tracking of the respiratory heart motion and was generated using balanced steady state free precession startup echoes. Respiratory gating was achieved using CRUISE with a fixed 50% efficiency. Whole-heart CMRA was acquired with 1.3mm isotropic resolution. For comparison, CMRA with identical imaging parameters were acquired using dNAV. Scan time, visualization of coronary artery origins and mid-course, imaging quality and sharpness was compared between the two sequences.ResultsForty patients (13 females; median weight: 44kg; median age: 12.6, range: 3months-17years) were enrolled. 25 scans were performed in awake patients. A contrast agent was used in 22 patients. The scan time was significantly reduced using iNAV for awake patients (iNAV 7:481:26 vs dNAV 9:48 +/- 3:11, P=0.01) but not for patients under general anaesthesia (iNAV=6:55 +/- 1:50 versus dNAV=6:32 +/- 2:16; P=0.32). In 98% of the cases, iNAV image quality had an equal or higher score than dNAV. The visual score analysis showed a clear difference, favouring iNAV (P=0.002). The right coronary artery and the left anterior descending vessel sharpness was significantly improved (iNAV: 56.8%+/- 10.1% vs dNAV: 53.7%+/- 9.9%, Pamp;lt;0.002 and iNAV: 55.8%+/- 8.6% vs dNAV: 53%+/- 9.2%, P=0.001, respectively).Conclusion p id=Par4 iNAV allows for a higher success-rate and clearer depiction of the mid-course of coronary arteries in paediatric patients. Its acquisition time is shorter in awake patients and image quality score is equal or superior to the conventional method in most cases., Funding Agencies|National Institute for Health Research (NIHR) Cardiovascular Healthcare Technology Cooperative (HTC); Biomedical Research Centre (BRC); Kings College London; Wellcome/EPSRC Centre for Medical Engineering at Kings College London [WT 088641/Z/09/Z]; BHF Centre of Excellence (British Heart Foundation) [RE/08/03]

Published
2019
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36. Clinical presentation and survival of childhood hypertrophic cardiomyopathy: a retrospective study in United Kingdom

Author

Norrish, Gabrielle, primary, Field, Ella, additional, Mcleod, Karen, additional, Ilina, Maria, additional, Stuart, Graham, additional, Bhole, Vinay, additional, Uzun, Orhan, additional, Brown, Elspeth, additional, Daubeney, Piers E F, additional, Lota, Amrit, additional, Linter, Katie, additional, Mathur, Sujeev, additional, Bharucha, Tara, additional, Kok, Khoon Li, additional, Adwani, Satish, additional, Jones, Caroline B, additional, Reinhardt, Zdenka, additional, and Kaski, Juan Pablo, additional

Published
2018
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37. Improved coronary magnetic resonance angiography using gadobenate dimeglumine in pediatric congenital heart disease

Author

Silva Vieira, Miguel, primary, Henningsson, Markus, additional, Dedieu, Nathalie, additional, Vassiliou, Vassilios S., additional, Bell, Aaron, additional, Mathur, Sujeev, additional, Pushparajah, Kuberan, additional, Figueroa, Carlos Alberto, additional, Hussain, Tarique, additional, Botnar, René, additional, and Greil, Gerald F., additional

Published
2018
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38. Axillary Versus Infraclavicular Placement for Endocardial Heart Rhythm Devices in Patients With Pediatric and Congenital Heart Disease

Author

Benjamin H. Hughes, Michael Schaffer, David J. Bradley, Abdul Duke, Christopher M. Rausch, Ian H. Law, Kathryn K. Collins, Mathur Sujeev, and Martin Runciman

Subjects
Heart Defects, Congenital, Male, medicine.medical_specialty, Time Factors, Adolescent, Heart disease, Population, Electric Countershock, Prosthesis Implantation, Young Adult, Heart Rate, Heart rate, Humans, Medicine, education, Retrospective Studies, education.field_of_study, business.industry, Retrospective cohort study, medicine.disease, Clavicle, Defibrillators, Implantable, Surgery, Cardiac surgery, Axilla, Treatment Outcome, medicine.anatomical_structure, Female, Implant, Cardiology and Cardiovascular Medicine, business, Follow-Up Studies
Abstract

Our objective was to evaluate the implant and mid-term outcomes of transvenous pacemaker or internal cardioverter-defibrillator placement by alternative axillary approaches compared to the infraclavicular approach in a pediatric and congenital heart disease population. We conducted a retrospective review of all patients with new endocardial heart rhythm devices placed at 4 pediatric arrhythmia centers. A total of 317 patients were included, 63 had undergone a 2-incision axillary approach, 51 a retropectoral axillary approach, and 203 an infraclavicular approach. Congenital heart disease was present in 62% of the patients. The patients with the 2-incision axillary approach were younger and smaller. The patients with the retropectoral axillary approach were less likely to have undergone previous cardiac surgery and were more likely to have had an internal cardioverter-defibrillator placed. The duration of follow-up was 2.4 ± 1.9 years for the 2-incision axillary, 2.6 ± 2.6 years for retropectoral axillary, and 3.5 ± 1.4 years for the infraclavicular technique (p = 0.01). No differences were seen in implant characteristics, lead longevity, implant complications, lead fractures or dislodgements, inappropriate internal cardioverter-defibrillator discharges, or device infections among the 3 groups. In conclusion, our data support that the outcomes of axillary approaches are comparable to the infraclavicular approach for endocardial heart rhythm device placement and that axillary approaches should be considered a viable option in patients with pediatric and congenital heart disease.

Published
2010
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43. Clinical presentation and survival of childhood hypertrophic cardiomyopathy: a retrospective study in United Kingdom.

Author

Norrish, Gabrielle, Field, Ella, Mcleod, Karen, Ilina, Maria, Stuart, Graham, Bhole, Vinay, Uzun, Orhan, Brown, Elspeth, Daubeney, Piers E F, Lota, Amrit, Linter, Katie, Mathur, Sujeev, Bharucha, Tara, Kok, Khoon Li, Adwani, Satish, Jones, Caroline B, Reinhardt, Zdenka, and Kaski, Juan Pablo

Abstract

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. View large Download slide View large Download slide [ABSTRACT FROM AUTHOR]

Published
2019
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