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European Journal of Pediatrics Aug 2019Dilated cardiomyopathy is the most common form of childhood cardiomyopathy and is known to result in significant morbidity and mortality. This study aims to review the...
Dilated cardiomyopathy is the most common form of childhood cardiomyopathy and is known to result in significant morbidity and mortality. This study aims to review the aetiology and associated outcomes of DCM. The median age at diagnosis was 6 months (0-42 months); n = 23 (43.3%) were idiopathic; n = 11 (20.9%) secondary to a viral infection; n = 12 (22.6%) genetic disorders and n = 7 (13.2%) as a result of vitamin D deficiency. There was a significant correlation between aetiology and mortality, r = 0.85, with a lower survival rate in idiopathic and genetic cohorts. Males were significantly less likely to survive to 1 year of age, p = 0.035. The age at diagnosis did not alter survival to 1 year and the predicted survival beyond 1 year was 84.3% (95% CI, 71.3 to 94.5%). Severely impaired left ventricular fractional shortening at presentation (< 15%) was an independent predictor of death, p = 0.002, (95% CI, 11.2 to 14.2%).Conclusion: Paediatric DCM is a heterogeneous disease resulting in significant morbidity. The aetiology alters the age of presentation. Identification of a specific cause is a useful for risk stratification and prognostication. The first year after diagnosis is a critical time period reflected by the significant morbidity and mortality. What is Known: •Paediatric dilated cardiomyopathy (DCM) is the commonest of the childhood cardiomyopathies, with significant associated morbidity and mortality. •DCM is most commonly idiopathic. What is New: •Identifying the aetiology of DCM in the paediatric population aids risk stratification and prognostication. •The first year after diagnosis of DCM is associated with significant mortality.
Topics: Cardiomyopathy, Dilated; Child, Preschool; Databases, Factual; Female; Humans; Infant; Infant, Newborn; Male; Prognosis; Retrospective Studies; United Kingdom
PubMed: 31187263
DOI: 10.1007/s00431-019-03404-w -
JACC. Cardiovascular Imaging Jul 2021
Topics: Cardiomyopathies; Cardiomyopathy, Dilated; Fibrosis; Humans; Predictive Value of Tests
PubMed: 34023259
DOI: 10.1016/j.jcmg.2021.03.015 -
ESC Heart Failure Feb 2020The aim of this study is to reveal the causal relationship between dilated cardiomyopathy and aldosteronoma. A 44-year-old male dilated cardiomyopathy patient with...
The aim of this study is to reveal the causal relationship between dilated cardiomyopathy and aldosteronoma. A 44-year-old male dilated cardiomyopathy patient with aldosteronoma, who demonstrated a worse cardiac function after 1 year therapy with optimized dosage of sacubirtil/valsartan, furosemide, metoprolol, and spironolactone. The patient shows a promising prognosis after aldosteronoma removal procedure. Aldosteronoma may cause dilated cardiomyopathy. We assume that the optimal treatment for aldosteronoma-induced dilated cardiomyopathy is surgical removal combined with drugs.
Topics: Adrenal Gland Neoplasms; Adult; Biopsy; Cardiomyopathy, Dilated; Diagnosis, Differential; Echocardiography; Humans; Hyperparathyroidism; Male; Prognosis; Tomography, X-Ray Computed; Ventricular Function, Left
PubMed: 31856419
DOI: 10.1002/ehf2.12579 -
Circulation Dec 2016
Review
Topics: American Heart Association; Cardiology; Cardiomyopathy, Dilated; Consensus; Evidence-Based Medicine; Humans; Prognosis; Risk Factors; Time Factors; United States
PubMed: 27832612
DOI: 10.1161/CIR.0000000000000455 -
Clinical Screening for Dilated Cardiomyopathy in At-Risk First-Degree Relatives: Who, When, and How?Journal of the American College of... May 2023
Topics: Humans; Cardiomyopathy, Dilated
PubMed: 37225359
DOI: 10.1016/j.jacc.2023.04.001 -
JAAPA : Official Journal of the... May 2011
Topics: Cardiomyopathy, Dilated; Diagnosis, Differential; Genetic Predisposition to Disease; Genetic Testing; Genomics; Humans; Prevalence; Risk
PubMed: 21574539
DOI: 10.1097/01720610-201105000-00017 -
Cardiology Journal 2014Dilated cardiomyopathy (DCM) is one of the leading causes of heart failure and heart transplant. Mutations in 60 genes have been associated with DCM. Approximately 6% of... (Review)
Review
Dilated cardiomyopathy (DCM) is one of the leading causes of heart failure and heart transplant. Mutations in 60 genes have been associated with DCM. Approximately 6% of all DCM cases are caused by mutations in the lamin A/C gene (LMNA). LMNA codes for type-V intermediate filaments that support the structure of the nuclear membrane and are involved in chromatin structure and gene expression. Most LMNA mutations result in striated muscle diseases while the rest affects the adipose tissue, peripheral nervous system, multiple tissues or lead to progeroid syndromes/overlapping syndromes. Patients with LMNA mutations exhibit a variety of cellular and physiological phenotypes. This paper explores the current phenotypes observed in LMNA-caused DCM, the results and implications of the cellular and animal models of DCM and the prevailing theories on the pathogenesis of laminopathies.
Topics: Animals; Cardiomyopathy, Dilated; Genetic Predisposition to Disease; Humans; Lamin Type A; Mutation; Phenotype; Prognosis
PubMed: 24846508
DOI: 10.5603/CJ.a2014.0037 -
JACC. Cardiovascular Imaging Feb 2020
Topics: Cardiomyopathy, Dilated; Genetic Testing; Humans; Pedigree
PubMed: 31864986
DOI: 10.1016/j.jcmg.2019.09.004 -
Current Problems in Cardiology Oct 1987This monograph begins and ends with a statement of uncertainty regarding many aspects of dilated cardiomyopathy. Natural history studies identify patients with widely... (Review)
Review
This monograph begins and ends with a statement of uncertainty regarding many aspects of dilated cardiomyopathy. Natural history studies identify patients with widely differing outcomes. A host of prognostic factors have emerged, yet it would appear that the major determinants of survival are as yet unrecognized. The diagnosis remains primarily one of exclusion, and management is largely nonspecific and supportive. The frequency of sudden cardiac death is well documented, but the ability to accurately identify patients at risk and the efficacy of antiarrhythmic therapy is unestablished. The emerging success of cardiac transplantation is a source of encouragement. The causes of dilated cardiomyopathy remain a source of intense investigation. Accumulating evidence (much of it circumstantial) does, however, implicate a viral etiology and perhaps altered function of the immunoregulatory system. However, the disparity between the severity of functional disturbance with the relative lack of histologic markers of cellular necrosis implies a disturbance at a cellular level. The etiology or etiologies remain elusive. Future investigation directed at fundamental aspects of cardiac cellular biology may provide the answers.
Topics: Adolescent; Adult; Aged; Cardiomyopathy, Dilated; Female; Humans; Male; Middle Aged; Myocarditis
PubMed: 3322687
DOI: 10.1016/0146-2806(87)90002-8 -
Nursing Standard (Royal College of...This article provides an overview of alcoholic dilated cardiomyopathy. It aims to increase awareness of the condition among nurses, and help in early diagnosis and... (Review)
Review
This article provides an overview of alcoholic dilated cardiomyopathy. It aims to increase awareness of the condition among nurses, and help in early diagnosis and appropriate treatment referrals. The key message is that all patients with alcoholic dilated cardiomyopathy should be advised and assisted to stop drinking alcohol.
Topics: Alcoholism; Cardiomyopathy, Dilated; Humans; Prognosis
PubMed: 18578120
DOI: No ID Found