Diagnosis and Management of Rare Cardiomyopathies in Adult and Paediatric Patients. A Position Paper of the Italian Society of Cardiology (SIC) and Italian Society of Paediatric Cardiology (SICP)

Limongelli, Giuseppe; Adorisio, Rachele; Baggio, Chiara; Bauce, Barbara; Biagini, Elena; Castelletti, S; Favilli, Silvia; Imazio, Massimo; Lioncino, Michele; Merlo, Marco; Monda, Emanuele; Olivotto, Iacopo; Parisi, Vanda; Pelliccia, Francesco; Basso, Cristina; Sinagra, Gianfranco; Indolfi, Ciro; Autore, Camillo

doi:10.1016/j.ijcard.2022.03.050

Cited by 47 publications

(56 citation statements)

References 171 publications

(211 reference statements)

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“…Furthermore, genetic testing may play a role in the development of viral and/or autoimmune myocarditis and a potential progression to DCM [ 4 ]. Recently, a current position paper [ 44 ] underlined the necessity of a more tailored investigation and management in patients with specific cardiomyopathies, strengthening the role of additional genetic testing, which might have an impact on the patient’s prognosis. Moreover, genetic testing should be considered in all familial forms of myocarditis, in familial cardiomyopathy or when signs of arrhythmogenic cardiomyopathy are present in imaging or electrophysiological tests [ 45 ].…”

Section: Discussionmentioning

confidence: 99%

Using Multiparametric Cardiac Magnetic Resonance to Phenotype and Differentiate Biopsy-Proven Chronic from Healed Myocarditis and Dilated Cardiomyopathy

Krumm

Brendel

Klingel

et al. 2022

JCM

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(1) Objectives: To discriminate biopsy-proven myocarditis (chronic vs. healed myocarditis) and to differentiate from dilated cardiomyopathy (DCM) using cardiac magnetic resonance (CMR). (2) Methods: A total of 259 consecutive patients (age 51 ± 15 years; 28% female) who underwent both endomyocardial biopsy (EMB) and CMR in the years 2008–2021 were evaluated. According to right-ventricular EMB results, patients were divided into either chronic (n = 130, 50%) or healed lymphocytic myocarditis (n = 60, 23%) or DCM (n = 69, 27%). The CMR protocol included functional, strain, and late gadolinium enhancement (LGE) imaging, T2w imaging, and T2 mapping. (3) Results: Left-ventricular ejection fraction (LV-EF) was higher, and the indexed end-diastolic volume (EDV) was lower in myocarditis patients (chronic: 42%, median 96 mL/m²; healed: 49%, 86 mL/m²) compared to the DCM patients (31%, 120 mL/m²), p < 0.0001. Strain analysis demonstrated lower contractility in DCM patients vs. myocarditis patients, p < 0.0001. Myocarditis patients demonstrated a higher LGE prevalence (68% chronic; 59% healed) than the DCM patients (45%), p = 0.01. Chronic myocarditis patients showed a higher myocardial edema prevalence and ratio (59%, median 1.3) than healed myocarditis (23%, 1.3) and DCM patients (13%, 1.0), p < 0.0001. T2 mapping revealed elevated values more frequently in chronic (90%) than in healed (21%) myocarditis and DCM (23%), p < 0.0001. T2 mapping yielded an AUC of 0.89 (sensitivity 90%, specificity 76%) in the discrimination of chronic from healed myocarditis and an AUC of 0.92 (sensitivity 86%, specificity 91%) in the discrimination of chronic myocarditis from DCM, both p < 0.0001. (4) Conclusions: Multiparametric CMR imaging, including functional parameters, LGE and T2 mapping, may allow differentiation of chronic from healed myocarditis and DCM and therefore help to optimize patient management in this clinical setting.

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Section: Discussionmentioning

confidence: 99%

Using Multiparametric Cardiac Magnetic Resonance to Phenotype and Differentiate Biopsy-Proven Chronic from Healed Myocarditis and Dilated Cardiomyopathy

Krumm

Brendel

Klingel

et al. 2022

JCM

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“…Cardiomyopathies (CMP) are a group of myocardial disorders that structurally and functionally affect the heart muscle in the absence of a disease that could better explain the extent of myocardial damage in the affected heart [ 40 ]. This definition has undergone significant changes from the time it was introduced in 1957 as we became more knowledgeable about the etiology and pathophysiology of this disease [ 41 ].…”

Section: Molecular Biology Of Cardiovascular Diseasesmentioning

confidence: 99%

Deciphering the Basis of Molecular Biology of Selected Cardiovascular Diseases: A View on Network Medicine

Alhajri¹,

Rustom²,

Adegbile³

et al. 2022

IJMS

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Cardiovascular diseases are the leading cause of death across the world. For decades, researchers have been studying the causes of cardiovascular disease, yet many of them remain undiscovered or poorly understood. Network medicine is a recently expanding, integrative field that attempts to elucidate this issue by conceiving of disease as the result of disruptive links between multiple interconnected biological components. Still in its nascent stages, this revolutionary application of network science facilitated a number of important discoveries in complex disease mechanisms. As methodologies become more advanced, network medicine harbors the potential to expound on the molecular and genetic complexities of disease to differentiate how these intricacies govern disease manifestations, prognosis, and therapy. This is of paramount importance for confronting the incredible challenges of current and future cardiovascular disease research. In this review, we summarize the principal molecular and genetic mechanisms of common cardiac pathophysiologies as well as discuss the existing knowledge on therapeutic strategies to prevent, halt, or reverse these pathologies.

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“…As miocardiopatias (MCPs) são doenças miocárdicas nas quais o músculo cardíaco é estrutural e funcionalmente anormal na ausência de doença arterial coronariana, hipertensão, doença valvar e cardiopatia congênita o suficiente para causar a anomalia miocárdica observada, como definem a Sociedade Europeia de Cardiologia e a World Heart Federation (WHF) (LI-MONGELLI, 2022). Pensadas por muito tempo como doenças raras, hoje está claro que a maioria das MCPs podem ser facilmente observadas na prática clínica (LIMONGELLI, 2022).…”

Section: Definição E Classificaçãounclassified

“…Isso pode sugerir um avanço rápido da doença em pacientes jovens, com maior agressividade da doença em comparação com os adultos, em termos de gravidade da insuficiência cardíaca e necessidade de transplante cardíaco ou de suporte circulatório mecânico (LIMONGELLI, 2022). Uma possível explicação é a presença de mutações genéticas particularmente agressivas que levam a uma doença de início precoce, mais arrítmica e rapidamente progressiva (LIMONGELLI, 2022). Uma história familiar de MCD em pacientes pediátricos, por exemplo, foi relatada como sendo duas vezes mais comum que entre os adultos e uma história familiar positiva para MCD resultou em um preditor de eventos arrítmicos (LIMONGELLI, 2022).…”

Section: Miocardiopatia Dilatadaunclassified

“…Uma história familiar de MCD em pacientes pediátricos, por exemplo, foi relatada como sendo duas vezes mais comum que entre os adultos e uma história familiar positiva para MCD resultou em um preditor de eventos arrítmicos (LIMONGELLI, 2022). Assim, é importante assegurar uma abordagem individualizada e holística a estes doentes, com um acompanhamento próximo e um tratamento mais agressivo (LIMONGELLI, 2022).…”

Section: Miocardiopatia Dilatadaunclassified

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Miocardiopatias

LOPES¹,

VAZ²,

BRAZ³

et al. 2022

Livro: Fundamentos E Práticas Pediátricas E Neonatais - Edição IV

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Diagnosis and Management of Rare Cardiomyopathies in Adult and Paediatric Patients. A Position Paper of the Italian Society of Cardiology (SIC) and Italian Society of Paediatric Cardiology (SICP)

Cited by 47 publications

References 171 publications

Using Multiparametric Cardiac Magnetic Resonance to Phenotype and Differentiate Biopsy-Proven Chronic from Healed Myocarditis and Dilated Cardiomyopathy

Using Multiparametric Cardiac Magnetic Resonance to Phenotype and Differentiate Biopsy-Proven Chronic from Healed Myocarditis and Dilated Cardiomyopathy

Deciphering the Basis of Molecular Biology of Selected Cardiovascular Diseases: A View on Network Medicine

Miocardiopatias

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