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5. Arrhythmias May Hide a Genetic Cardiomyopathy in Left Ventricular Hypertrabeculation in Children: A Single-Center Experience.

Author

Battipaglia, Irma, Cantarutti, Nicoletta, Cicenia, Marianna, Adorisio, Rachele, Battista, Virginia, Baban, Anwar, Silvetti, Massimo Stefano, and Drago, Fabrizio

Subjects
ARRHYTHMIA diagnosis, RESEARCH funding, FISHER exact test, CARDIAC hypertrophy, RETROSPECTIVE studies, DESCRIPTIVE statistics, CHI-squared test, SUPRAVENTRICULAR tachycardia, VENTRICULAR fibrillation, ARRHYTHMIA, LEFT ventricular hypertrophy, VENTRICULAR tachycardia, ELECTROCARDIOGRAPHY, MEDICAL records, ACQUISITION of data, VENTRICULAR arrhythmia, IMPLANTABLE cardioverter-defibrillators, DATA analysis software, HEART block, BIOMARKERS, PATIENT aftercare, PHENOTYPES, DISEASE complications, CHILDREN
Abstract

Background. Left ventricular hypertrabeculation (LVHT) is a myocardial disorder with different clinical manifestations, from total absence of symptoms to heart failure, arrhythmias, sudden cardiac death (SCD), and thromboembolic events. It is challenging to distinguish between the benign and pathological forms of LVHT. The aim of this study was to describe the arrhythmic manifestations of LVHT in a large group of pediatric patients and to correlate them with genetic results or other clinical markers. Methods. We retrospectively enrolled 140 pediatric patients with diagnosis of LVHT followed at our Institution from 2013 to 2023. Data regarding family history, instrumental exams, cardiac magnetic resonance, genetic testing and outcomes were collected. Most of them had isolated LVHT (80.7%); in other patients, mixed phenotypes (hypertrophic or dilated cardiomyopathy or congenital heart disease) were present. Results. Arrhythmias were found in 33 children (23.6%): 13 (9.3%) supraventricular tachyarrhythmias; 14 (10%) ventricular arrhythmias (five frequent PVCs (premature ventricular contractions), eight patients with ventricular tachycardia (VT), one ventricular fibrillation (VF)); two (1.4%) sinus node disfunctions; two (1.4%) complete atrio-ventricular blocks (AVB), three (2.1%) paroxysmal complete AVB, one (0.7%) severe I degree AVB. Three patients received an ICD (implantable cardioverter defibrillator). Comparison between LVHT patients with (33 pts) and without (107 pts) arrhythmias as regards genetic testing showed a statistical significance for the presence of class 4 or 5 genetic variants and arrhythmic manifestation (p = 0.037). Conclusions. In our pediatric cohort with LVHT, good outcomes were observed, but arrhythmias were not so rare (23.6%); no SCD occurred. [ABSTRACT FROM AUTHOR]

Published
2024
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7. Long-Term Incidence of Arrhythmias in Extracardiac Conduit Fontan and Comparison Between Systemic Left and Right Ventricle

Author

Di Mambro, Corrado, primary, Yammine, Marie Laure, additional, Tamborrino, Pietro Paolo, additional, Giordano, Ugo, additional, Righi, Daniela, additional, Unolt, Marta, additional, Cantarutti, Nicoletta, additional, Maiolo, Stella, additional, Albanese, Sonia, additional, Carotti, Adriano, additional, Amodeo, Antonio, additional, Galletti, Lorenzo, additional, and Drago, Fabrizio, additional

Published
2024
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10. Long-term incidence of arrhythmias in extracardiac conduit Fontan and comparison between systemic left and right ventricle.

Author

Mambro, Corrado Di, Yammine, Marie Laure, Tamborrino, Pietro Paolo, Giordano, Ugo, Righi, Daniela, Unolt, Marta, Cantarutti, Nicoletta, Maiolo, Stella, Albanese, Sonia, Carotti, Adriano, Amodeo, Antonio, Galletti, Lorenzo, and Drago, Fabrizio

Abstract

Aims The extracardiac conduit-Fontan (ECC) has become the preferred technique for univentricular heart palliation, but there are currently no data on the incidence of long-term arrhythmias. This study investigated the incidence of arrhythmias and relation to single ventricle morphology in the long-term follow-up (FU) in ECC. Methods and results All patients with ECC performed in our Centre between 1987 and 2017 were included (minimum FU 5 years). Of 353 consecutive patients, 303 [57.8% males, aging 8–50 (median 20) years at last FU] were considered and divided into two groups depending on left (194 in Group 1) or right (109 in Group 2) ventricular morphology. Eighty-five (28%) experienced ≥1 arrhythmic complications, with early and late arrhythmias in 17 (5.6%) and 73 (24.1%) patients, respectively. Notably, late bradyarrhythmias occurred after 6 years in 21 (11%) patients in Group 1, and in 15 (13.8%) in Group 2 [ P = 0.48]. Late tachyarrhythmias occurred in 55 (18.2%) patients after 12 years: 33 (17%) in Group 1 and 22 (20.2%) patients in Group 2 [ P   = 0.5]. Ventricular tachycardias (VT) were documented after 12.5 years in 14 (7.2%) patients of Group 1 and 15 (13.8%) of Group 2 [ P = 0.06] with a higher incidence in Group 2 during the FU [ P = 0.005]. Conclusion Extracardiac conduit is related to a significant arrhythmic risk in the long-term FU, higher than previously reported. Bradyarrhythmias occur earlier but are less frequent than tachyarrhythmias. Interestingly, patients with systemic right ventricle have a significantly higher incidence of VT, especially in a very long FU. [ABSTRACT FROM AUTHOR]

Published
2024
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12. ICD outcome in pediatric arrhythmogenic cardiomyopathy

Author

Cicenia, Marianna, primary, Silvetti, Massimo Stefano, additional, Cantarutti, Nicoletta, additional, Battipaglia, Irma, additional, Adorisio, Rachele, additional, Saputo, Fabio Anselmo, additional, Tamburri, Ilaria, additional, Campisi, Marta, additional, Baban, Anwar, additional, and Drago, Fabrizio, additional

Published
2023
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13. Relapsing Myocarditis following Initial Recovery of post COVID-19 Vaccination in two Adolescent Males – Case reports

Author

Amodio, Donato, primary, Manno, Emma, additional, Cotugno, Nicola, additional, Santilli, Veronica, additional, Franceschini, Alessio, additional, Alfonso Perrone, Marco, additional, Chinali, Marcello, additional, Drago, Fabrizio, additional, Cantarutti, Nicoletta, additional, Curione, Davide, additional, Engler, Renata, additional, Secinaro, Aurelio, additional, and Palma, Paolo, additional

Published
2023
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14. Cardiopulmonary Exercise Testing in Children and Young Adolescents after a Multisystem Inflammatory Syndrome: Physical Deconditioning or Residual Pathology?

Author

Gentili, Federica, primary, Calcagni, Giulio, additional, Cantarutti, Nicoletta, additional, Manno, Emma Concetta, additional, Cafiero, Giulia, additional, Tranchita, Eliana, additional, Salvati, Annamaria, additional, Palma, Paolo, additional, Giordano, Ugo, additional, Drago, Fabrizio, additional, and Turchetta, Attilio, additional

Published
2023
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15. Heart rate reduction as a marker to optimize carvedilol treatment and enhance myocardial recovery in pediatric dilated cardiomyopathy

Author

Adorisio, Rachele, primary, Pontrelli, Giuseppe, additional, Cantarutti, Nicoletta, additional, Bellettini, Elisa, additional, Caiazza, Martina, additional, Mencarelli, Erica, additional, Limongelli, Giuseppe, additional, Poli, Daniela, additional, Drago, Fabrizio, additional, Kirk, Richard, additional, and Amodeo, Antonio, additional

Published
2022
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16. Long-Term Cardiovascular Outcome in Children with MIS-C Linked to SARS-CoV-2 Infection—An Italian Multicenter Experience

Author

Cantarutti, Nicoletta, primary, Battista, Virginia, additional, Stagnaro, Nicola, additional, Labate, Marianna Eleonora, additional, Cicenia, Marianna, additional, Campisi, Marta, additional, Vitali, Valerio, additional, Secinaro, Aurelio, additional, Campana, Andrea, additional, Trocchio, Gianluca, additional, and Drago, Fabrizio, additional

Published
2022
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17. Late outcome of extracardiac Fontan patients: 32 years of follow-up

Author

Giannico, Salvatore, primary, Trezzi, Matteo, additional, Cantarutti, Nicoletta, additional, Cafiero, Giulia, additional, Ravà, Lucilla, additional, Adorisio, Rachele, additional, Brancaccio, Gianluca, additional, Albanese, Sonia, additional, Drago, Fabrizio, additional, Carotti, Adriano, additional, Amodeo, Antonio, additional, and Galletti, Lorenzo, additional

Published
2022
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19. ICD Outcome in Pediatric Cardiomyopathies

Author

Silvetti, Massimo Stefano, primary, Tamburri, Ilaria, additional, Campisi, Marta, additional, Saputo, Fabio Anselmo, additional, Cazzoli, Ilaria, additional, Cantarutti, Nicoletta, additional, Cicenia, Marianna, additional, Adorisio, Rachele, additional, Baban, Anwar, additional, Ravà, Lucilla, additional, and Drago, Fabrizio, additional

Published
2022
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20. 245 Arrhythmogenic cardiomyopathy in children according to ‘padua criteria’: single paediatric centre experience

Author

Cicenia, Marianna, primary, Cantarutti, Nicoletta, additional, Adorisio, Rachele, additional, Silvetti, Massimo Stefano, additional, Secinaro, Aurelio, additional, Ciancarella, Paolo, additional, Di Mambro, Corrado, additional, Magliozzi, Monia, additional, Novelli, Antonio, additional, Amodeo, Antonio, additional, Baban, Anwar, additional, and Drago, Fabrizio, additional

Published
2021
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26. Clinical Spectrum Of CACNA1C Variants, Revisited

Author

Baban, Anwar, primary, Corica, Bernadette, additional, Gnazzo, Maria, additional, Calì, Federica, additional, Cantarutti, Nicoletta, additional, Righi, Daniela, additional, Mambro, Corrado Di, additional, Silvetti, Massimo Stefano, additional, Dallapiccola, Bruno, additional, Novelli, Antonio, additional, Amin, Ahmad S., additional, and Drago, Fabrizio, additional

Published
2020
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27. Differences in morbidity and mortality in Down syndrome are related to the type of congenital heart defect

Author

Baban, Anwar, primary, Olivini, Nicole, additional, Cantarutti, Nicoletta, additional, Calì, Federica, additional, Vitello, Carmen, additional, Valentini, Diletta, additional, Adorisio, Rachele, additional, Calcagni, Giulio, additional, Alesi, Viola, additional, Di Mambro, Corrado, additional, Villani, Alberto, additional, Dallapiccola, Bruno, additional, Digilio, Maria Cristina, additional, Marino, Bruno, additional, Carotti, Adriano, additional, and Drago, Fabrizio, additional

Published
2020
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30. Persistent myocardial atrophy despite LV reverse remodeling in Duchenne cardiomyopathy treated by LVAD.

Author

Cantarutti, Nicoletta, Adorisio, Rachele, Baban, Anwar, Di Molfetta, Arianna, Amodeo, Antonio, and Drago, Fabrizio

Subjects
*ATROPHY, *CARDIOMYOPATHIES, *CAUSES of death
Abstract

DCM is the leading cause of death in Duchenne patients. LVADs are considered as therapeutic options as DT in advanced HF. The aim of our study was to evaluate LV remodeling of Duchenne after LVADs and chronic therapy. Demographic and echocardiographic data of 8 Duchenne patients implanted with LVADs were reviewed and analyzed. All measures were collected before LVAD implantation, after 1 month and 1 year. All patients were affected by end‐stage DCM, and mean age at implantation was 16.9 ± 2.9 years. Patients were treated with maximal medical therapy. One‐year post‐implantation HR decreased from a mean of 110 ± 19 bpm to 82 ± 2 bpm (P =.002), and a significant decrease in LV volumes and diameters LVEDD P =.03, LVESD P =.02, EDV P =.01, and ESV P =.02) was noticed together with a significant increase in EF (P =.0036). However, RWT did not change over time, showing an eccentric remodeling pattern pre‐ and post‐LVADs. Our data showed that cardiac atrophy is persistent in Duchenne cardiomyopathy despite the improvement of LV function secondary to a significant ventricular unloading due to LVADs coupled with chronic therapy. [ABSTRACT FROM AUTHOR]

Published
2021
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31. Human B‐cell memory is shaped by age‐ and tissue‐specific T‐independent and GC‐dependent events

Author

Aranburu, Alaitz, primary, Piano Mortari, Eva, additional, Baban, Anwar, additional, Giorda, Ezio, additional, Cascioli, Simona, additional, Marcellini, Valentina, additional, Scarsella, Marco, additional, Ceccarelli, Sara, additional, Corbelli, Sandro, additional, Cantarutti, Nicoletta, additional, De Vito, Rita, additional, Inserra, Alessandro, additional, Nicolosi, Luciana, additional, Lanfranchi, Arnalda, additional, Porta, Fulvio, additional, Cancrini, Caterina, additional, Finocchi, Andrea, additional, and Carsetti, Rita, additional

Published
2016
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33. Human B-cell memory is shaped by age- and tissue-specific T-independent and GC-dependent events.

Author

Aranburu, Alaitz, Piano Mortari, Eva, Baban, Anwar, Giorda, Ezio, Cascioli, Simona, Marcellini, Valentina, Scarsella, Marco, Ceccarelli, Sara, Corbelli, Sandro, Cantarutti, Nicoletta, Vito, Rita, Inserra, Alessandro, Nicolosi, Luciana, Lanfranchi, Arnalda, Porta, Fulvio, Cancrini, Caterina, Finocchi, Andrea, and Carsetti, Rita

Abstract

Switched and IgM memory B cells execute different and noninterchangeable functions. We studied memory B cells in children of different ages, in peripheral blood and spleen and compared them with those of children born asplenic or unable to build germinal centers. We show that, whereas switched memory B cells are mostly generated in the germinal centers at all ages, IgM memory B cells can be distinct in three types with different developmental history. Innate IgM memory B cells, the largest pool in infants, are generated in the spleen by a germinal center-independent mechanism. With age, if the spleen is present and germinal centers are functional, innate IgM memory B cells are remodelled and accumulate somatic mutations. The third type of IgM memory B cell is a by-product of the germinal center reaction. Our data suggest that the B-cell memory developmental program is implemented during the first 5-6 years of life. [ABSTRACT FROM AUTHOR]

Published
2017
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37. Immunodeficiency in Vici syndrome: A heterogeneous phenotype

Author

Finocchi, Andrea, primary, Angelino, Giulia, additional, Cantarutti, Nicoletta, additional, Corbari, Maurizio, additional, Bevivino, Elsa, additional, Cascioli, Simona, additional, Randisi, Francesco, additional, Bertini, Enrico, additional, and Dionisi‐Vici, Carlo, additional

Published
2011
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38. Successful Treatment With Percutaneous Transhepatic Alcoholization of a Liver Abscess in a Child With Chronic Granulomatous Disease

Author

Angelino, Giulia, primary, Natali, Gian Luigi, additional, Falappa, Piergiorgio, additional, Folgori, Laura, additional, Moretti, Roberto, additional, Cantarutti, Nicoletta, additional, Di Matteo, Gigliola, additional, Chiriaco, Maria, additional, Rossi, Paolo, additional, Roos, Dirk, additional, Aiuti, Alessandro, additional, and Finocchi, Andrea, additional

Published
2011
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39. Fulminant Fusobacterium necrophorumMeningitis in an Immunocompetent Adolescent

Author

Angelino, Giulia, Cantarutti, Nicoletta, Chiurchiù, Sara, Amodio, Donato, De Luca, Maia, Lancella, Laura, Coltella, Luana, Russo, Cristina, and Finocchi, Andrea

Abstract

Fusobacterium necrophorumis an anaerobic, gram-negative highly virulent bacillus, isolated from the oropharingeal cavity, the gastrointestinal tract, and the female genital tract. It is responsible of several clinical syndromes, mainly in children or adolescents, ranging from localized abscess, usually in the upper respiratory sites, to severe septicemic diseases, including meningitis.

Published
2012
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41. Cardiomyopathy in Duchenne Muscular Distrophy : Clinical Insights and Therapeutic Implications

Author

Adorisio, Rachele, Mencarelli, Erica, Cantarutti, Nicoletta, and Grandinetti, Maria

Subjects
Medical
Abstract

Duchenne muscular dystrophy cardiomyopathy (DMD-DCM) is characterized by progressive ventricular dilation and dysfunction that can begin at any age and worsens over time. Thanks to the lengthening of life expectancy due to better management of respiratory involvement, end-stage heart failure (HF) is becoming the main cause of death for DMD patients. Therefore, from the time of DMD diagnosis, every effort should be focused to early detect the onset and the worsening of the DMD-DCM, with the aim of starting and modulating the therapy to slow the progression of cardiac dysfunction. In cardiac evaluation, biomarkers, electrocardiograms, and echocardiograms must be considered, but cardiac magnetic resonance (CMR) is now acquiring a leading role due to its sensitivity in the earlier identification of cardiac involvement. The management of DMD-DCM at end stage is a difficult challenge that requires a multidisciplinary team composed of clinical cardiologists, electrophysiologists, cardiac surgeons, neuromuscular specialists, and psychologists. Because of the lack of specific drugs for DMD, we will review the actual cardiovascular armamentarium including drugs used for HF.

Published
2018

42. Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: A systematic review and meta-analysis

Author

Juan Pablo Kaski, Giuseppe Limongelli, Perry M. Elliott, Deborah Ridout, Nicoletta Cantarutti, Gabrielle Norrish, Eleni Pissaridou, Norrish, Gabrielle, Cantarutti, Nicoletta, Pissaridou, Eleni, Ridout, Deborah A, Limongelli, Giuseppe, Elliott, Perry M, and Kaski, Juan Pablo

Subjects
medicine.medical_specialty, Epidemiology, Population, Ventricular outflow tract obstruction, sudden death, 030204 cardiovascular system & hematology, Left ventricular hypertrophy, Ventricular tachycardia, Global Health, Sudden death, Risk Assessment, Sudden cardiac death, 03 medical and health sciences, 0302 clinical medicine, Risk Factors, Internal medicine, Medicine, Humans, 030212 general & internal medicine, Risk factor, education, Child, childhood, education.field_of_study, business.industry, Hypertrophic cardiomyopathy, Cardiomyopathy, Hypertrophic, medicine.disease, Defibrillators, Implantable, Survival Rate, Death, Sudden, Cardiac, risk factor, Cardiology, medicine.symptom, Cardiology and Cardiovascular Medicine, business
Abstract

Aims To perform a systematic literature review and meta-analysis of clinical risk factors for sudden cardiac death (SCD) in childhood hypertrophic cardiomyopathy. Methods Medline and PubMed databases were searched for original articles published in English from 1963 through to December 2015 that included patients under 18 years of age with a primary or secondary end-point of either SCD or SCD-equivalent events (aborted cardiac arrest or appropriate implantable cardioverter-defibrillator discharge) or cardiovascular death (CVD). Results Twenty-five studies (3394 patients) met the inclusion criteria. We identified four conventional major risk factors that were evaluated in at least four studies and that we found to be statistically associated with an increased risk of death in at least two studies: previous adverse cardiac event (pooled hazard ratio [HR] 5.4, 95% confidence interval [CI] 3.67-7.95, p

Published
2017

43. Serratia marcescens Osteomyelitis in a Newborn with Chronic Granulomatous

Author

Irene Salfa, Paolo Palma, Giulia Angelino, Nicoletta Cantarutti, Caterina Cancrini, Valentina Capo, Alessandro Aiuti, Gigliola Di Matteo, Andrea Finocchi, Giada Farinelli, Salfa, Irene, Cantarutti, Nicoletta, Angelino, Giulia, Di Matteo, Gigliola, Capo, Valentina, Farinelli, Giada, Cancrini, Caterina, Aiuti, Alessandro, Palma, Paolo, and Finocchi, Andrea

Subjects
Microbiology (medical), Settore MED/38 - Pediatria Generale e Specialistica, Pathology, medicine.medical_specialty, Serratia infection, biology, business.industry, Osteomyelitis, medicine.disease, biology.organism_classification, Infant newborn, Infectious Diseases, Chronic granulomatous disease, Granulomatous disease, Pediatrics, Perinatology and Child Health, Serratia marcescens, medicine, business
Published
2013

44. The role of Italian pre-participation screening in early detection of cardiomyopathies: what is the meaning of T wave inversion in young athletes?

Author

Tranchita E, Cafiero G, Giordano U, Gentili F, Turchetta A, Cantarutti N, Cicenia M, Battipaglia I, Di Mambro C, Baban A, Secinaro A, and Drago F

Abstract

Introduction: The presence of T wave inversion on screening electrocardiogram may represent an early sign of cardiomyopathies in athletes. This finding even in very young athletes can generate some suspicion and may determine a contraindication to practice competitive sport. The aim of this study is to evaluate the prevalence of T wave inversion in a population of young competitive athletes and determine whether they can be associated with the occurrence of cardiomyopathies in the absence of other pathological features., Methods: A prospective cross-sectional study was carried out and 581 subjects were screened for competitive sport eligibility. Based on inclusion/exclusion criteria, 53 athletes showed T wave inversion and they were selected to undergo further investigations., Results: In 32,1% of cases, we have identified the cause of T wave inversions and we suspended them from competition. In particular, in 15% of athletes who showed T wave inversions, we found cardiomyopathies., Discussion: Prevalence of T wave inversion in this population of athletes was 9,1%. At the end of second and third-level evaluations, eight athletes with T wave inversion showed an early form of cardiomyopathy and were suspended from competitive sport. Most of them showed T wave inversion in infero-lateral leads on electrocardiogram., Conclusion: The probability that competitive athletes have a concealed cardiomyopathy is low, but not negligible. Pre-participation screening for competitive sport activity represents an excellent opportunity to early identify cardiomyopathies and other pathologies that increase the risk of sudden death in apparently healthy young athletes.

Published
2024
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