Advances in Pediatric Heart Failure: Congenital Heart Disease and Cardiomyopathies

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".

Deadline for manuscript submissions: closed (31 January 2024) | Viewed by 2396

Special Issue Editors


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Guest Editor
Department of Pediatric Cardiology, Meyer Hospital, 50139 Florence, Italy
Interests: congenital heart disease (CHD); cardiomyopathies; adult CHD
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Guest Editor
Department of Experimental and Clinical Medicine, University of Florence, 50121 Florence, Italy
Interests: clinical and molecular basis of cardiomyopathies; metabolic pathways involved in cardiomyopathies; innovation in pharmacological treatment and clinical trials in patients with inherited heart diseases
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Guest Editor
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital and University College London Institute of Cardiovascular Science, London WC1E 6DD, UK
Interests: inherited cardiac conditions; cardiomyopathy; cardiovascular genetics; sudden cardiac death

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Guest Editor
Meyer Children’s Hospital, Florence, Italy
Interests: paediatric diseases

Special Issue Information

Dear Colleagues,

In recent decades, we have witnessed extraordinary progress both in the field of congenital heart diseases and of cardiomyopathies. These advancements are largely driven by early cardiac surgery and interventional procedures, currently performed in very young patients, as well as tremendous improvement in diagnostic tools, from advanced imaging to genetics. Nevertheless, heart failure in children and adolescents remains largely uncharted territory in cardiovascular medicine. Research initiatives in pediatric cohorts are limited, and management is often based on ‘adaptation’ of adult guidelines. An age-specific and evidence-based approach is sorely needed. A more comprehensive approach to the problems will require multidisciplinary, concerted efforts, uniting the two most prominent areas: congenital heart disease and genetic cardiomyopathies.

The international symposium, “Advances in Pediatric Heart Failure: Congenital Disease and Cardiomyopathies”, is scheduled to take place in Florence in April 2023. It is supported by an unrestricted grant by Fondazione Menarini (as was the case for the previous edition in 2018) and aims to discuss the state-of-the-art management in children and adolescents with heart failure, bringing together an international faculty of experts in both fields.

The issue will collect a selection of reviews from the participants, combining these two different but complementary perspectives, as anticipated by the title. We hope that the synergy of such rich and diverse areas of expertise will help advance the field and improve the outlook of our youngest patients.

Dr. Silvia Favilli
Prof. Dr. Iacopo Olivotto
Dr. Juan Pablo Kaski
Dr. Alberto Zanobini
Guest Editors

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Published Papers (2 papers)

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Research

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10 pages, 472 KiB  
Article
Prognostic Value of Tpeak–Tend Interval in Early Diagnosis of Duchenne Muscular Dystrophy Cardiomyopathy
by Serra Baskan, Pelin Karaca Ozer, Huseyin Orta, Doruk Ozbingol, Mustafa Lutfi Yavuz, Elif Ayduk Govdeli, Kemal Nisli and Kazim Oztarhan
Diagnostics 2023, 13(14), 2381; https://doi.org/10.3390/diagnostics13142381 - 15 Jul 2023
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Abstract
The most common cause of death in patients with Duchenne muscular dystrophy (DMD) is cardiomyopathy. Our aim was to investigate the relationship between the Tpeak–Tend (Tp-e) interval and the premature ventricular contraction (PVC) burden and therefore early arrhythmic risk and cardiac involvement in [...] Read more.
The most common cause of death in patients with Duchenne muscular dystrophy (DMD) is cardiomyopathy. Our aim was to investigate the relationship between the Tpeak–Tend (Tp-e) interval and the premature ventricular contraction (PVC) burden and therefore early arrhythmic risk and cardiac involvement in DMD patients. Twenty-five patients with DMD followed by pediatric cardiology were included in the study. Those with a frequency of <1% PVC in the 24 h Holter were assigned to Group 1 (n = 15), and those with >1% were assigned to Group 2 (n = 10). Comparisons were made with healthy controls (n = 27). Left ventricular ejection fraction (LVEF) was lowest in Group 2 and highest in the control group (p < 0.001). LV end-diastolic diameter was greater in Group 2 than in Group 1 and the control group (p = 0.005). Pro-BNP and troponin levels were higher in Group 1 and Group 2 than in the control group (p = 0.001 and p < 0.001, respectively). Tp-e interval was longer in Group 2 compared to Group 1 and the control group (p < 0.001). The LVEF (OR 0.879, 95% CI 0.812–0.953; p = 0.002) and Tp-e interval (OR 1.181, 95% CI 1.047–1.332; p = 0.007) were independent predictors of PVC/24 h frequency of >1%. A Tp-e interval > 71.65 ms predicts PVC > 1%, with a sensitivity of 80% and a specificity of 90% (AUC = 0.842, 95% CI (0.663–1.000), p = 0.001). Determination of Tp-e prolongation from ECG data may help in the determination of cardiac involvement and early diagnosis of arrhythmic risk in DMD. Full article
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16 pages, 1424 KiB  
Review
Left Ventricular Non-Compaction in Children: Aetiology and Diagnostic Criteria
by Emanuele Monda, Gianantonio De Michele, Gaetano Diana, Federica Verrillo, Marta Rubino, Annapaola Cirillo, Adelaide Fusco, Federica Amodio, Martina Caiazza, Francesca Dongiglio, Giuseppe Palmiero, Pietro Buono, Maria Giovanna Russo and Giuseppe Limongelli
Diagnostics 2024, 14(1), 115; https://doi.org/10.3390/diagnostics14010115 - 4 Jan 2024
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Abstract
Left ventricular non-compaction (LVNC) is a heterogeneous myocardial disorder characterized by prominent trabeculae protruding into the left ventricular lumen and deep intertrabecular recesses. LVNC can manifest in isolation or alongside other heart muscle diseases. Its occurrence among children is rising due to advancements [...] Read more.
Left ventricular non-compaction (LVNC) is a heterogeneous myocardial disorder characterized by prominent trabeculae protruding into the left ventricular lumen and deep intertrabecular recesses. LVNC can manifest in isolation or alongside other heart muscle diseases. Its occurrence among children is rising due to advancements in imaging techniques. The origins of LVNC are diverse, involving both genetic and acquired forms. The clinical manifestation varies greatly, with some cases presenting no symptoms, while others typically manifesting with heart failure, systemic embolism, and arrhythmias. Diagnosis mainly relies on assessing heart structure using imaging tools like echocardiography and cardiac magnetic resonance. However, the absence of a universally agreed-upon standard and limitations in diagnostic criteria have led to ongoing debates in the scientific community regarding the most reliable methods. Further research is crucial to enhance the diagnosis of LVNC, particularly in early life stages. Full article
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