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Journal of the American Heart... Sep 2019Background Limited data are available on mid-range ejection fraction (mrEF) patients with dilated cardiomyopathy. We sought to define the characteristics, evolution, and...
Background Limited data are available on mid-range ejection fraction (mrEF) patients with dilated cardiomyopathy. We sought to define the characteristics, evolution, and long-term prognosis of dilated cardiomyopathy patients with mrEF at diagnosis. Methods and Results We analyzed all dilated cardiomyopathy patients consecutively evaluated in the Trieste Heart Muscle Disease Registry from 1988 to 2013. mrEF and reduced ejection fraction (rEF) were defined as baseline left ventricular (LV) ejection fraction values between 40% and 49% and <40%, respectively. All-cause mortality or heart transplantation, sudden cardiac death, or major ventricular arrhythmias were considered as outcome measures. Worsening LV ejection fraction (reduction to <40%) during follow-up was also considered to identify possible predictors of adverse remodeling. Among 812 enrolled patients, 175 (22%) presented with mrEF at presentation. At baseline, as compared with the rEF group, mrEF patients had lower rates of moderate-severe mitral regurgitation and restrictive LV filling pattern. During a median follow-up period of 120 (60-204) months, the mrEF group presented a lower rate of death/heart transplantation (9% versus 36%, P<0.001) and sudden cardiac death or major ventricular arrhythmias (4.5% versus 15%, P<0.001) than rEF patients. Moreover, 29 out of 175 mrEF patients (17%) evolved to rEF. Restrictive LV filling pattern emerged as the strongest predictor of rEF development following multivariable analysis. Conclusions mrEF identified a consistent subgroup of dilated cardiomyopathy patients diagnosed in an earlier stage with subsequent apparent better long-term evolution. However, 17% of these patients evolved into rEF despite the use of medical therapy. A baseline restrictive LV filling pattern was independently associated with subsequent evolution to rEF.
Topics: Adult; Arrhythmias, Cardiac; Cardiomyopathy, Dilated; Cause of Death; Death, Sudden, Cardiac; Disease Progression; Early Diagnosis; Echocardiography, Doppler; Female; Heart Transplantation; Humans; Incidence; Italy; Male; Middle Aged; Prognosis; Registries; Retrospective Studies; Risk Assessment; Risk Factors; Stroke Volume; Time Factors; Ventricular Function, Left
PubMed: 31431100
DOI: 10.1161/JAHA.118.010705 -
ESC Heart Failure Feb 2024Cardiomyopathies (CMPs) are a heterogeneous group of diseases that are defined by structural and functional abnormalities of the cardiac muscle. Dilated cardiomyopathy... (Observational Study)
Observational Study
AIMS
Cardiomyopathies (CMPs) are a heterogeneous group of diseases that are defined by structural and functional abnormalities of the cardiac muscle. Dilated cardiomyopathy (DCM), the most common CMP, is defined by left ventricular dilation and impaired contractility and represents a common cause of heart failure. Different phenotypes result from various underlying genetic and acquired causes with variable effects on disease development and progression, prognosis, and response to medical treatment. Current treatment algorithms do not consider these different aetiologies, due to lack of insights into treatable drivers of cardiac failure in patients with DCM. Our study aims to precisely phenotype and genotype the various subtypes of DCM and hereby lay the foundation for individualized therapy.
METHODS AND RESULTS
The Geno- And Phenotyping of PrImary Cardiomyopathy (GrAPHIC) is a currently ongoing prospective observational monocentric cohort study that recruits patients with DCM after exclusion of other causes such as coronary artery disease, valvular dysfunction, myocarditis, exposure to toxins, and peripartum CMP. Patients are enrolled at our heart failure outpatient clinic or during hospitalization at the University Hospital Hamburg. Clinical parameters, multimodal imaging and functional assessment, cardiac biopsies, and blood samples are obtained to enable an integrated genomic, functional, and biomarker analysis.
CONCLUSIONS
The GrAPHIC will contribute to a better understanding of the heterogeneous nature of primary CMPs focusing on DCM and provide improved prognostic approaches and more individualized therapies.
Topics: Humans; Cardiomyopathy, Dilated; Cohort Studies; Cardiomyopathies; Heart Failure; Genotype
PubMed: 37964758
DOI: 10.1002/ehf2.14544 -
International Journal of Molecular... May 2021Non-ischemic dilated cardiomyopathy encompasses a wide spectrum of myocardial disorders, characterized by left ventricular dilatation with systolic impairment and... (Review)
Review
Non-ischemic dilated cardiomyopathy encompasses a wide spectrum of myocardial disorders, characterized by left ventricular dilatation with systolic impairment and increased risk of sudden cardiac death. In spite of all the therapeutic progress that has been made in recent years, dilated cardiomyopathy continues to be an important cause of cardiac transplant, being associated with an enormous cost burden for health care systems worldwide. Predicting the prognosis of patients with dilated cardiomyopathy is essential to individualize treatment. Late gadolinium enhancement-cardiac magnetic resonance imaging, microvolt T-wave alternans, and genetic testing have emerged as powerful tools in predicting sudden cardiac death occurrence and maximizing patient's selection. Despite all these new diagnostic modalities, additional tests to complement or replace current tools are required for better risk stratification. Therefore, biomarkers are an easy and important tool that can help to detect patients at risk of adverse cardiovascular events. Additionally, identifying potential biomarkers involved in dilated cardiomyopathy can provide us important information regarding the diagnostic, prognostic, risk stratification, and response to treatment for these patients. Many potential biomarkers have been studied in patients with dilated cardiomyopathy, but only a few have been adopted in current practice. Therefore, the aim of our review is to provide the clinicians with an update on the well-known and novel biomarkers that can be useful for risk stratification of patients with non-ischemic dilated cardiomyopathy.
Topics: Biomarkers; Cardiomyopathy, Dilated; Contrast Media; Gadolinium; Humans; Magnetic Resonance Imaging; Risk Assessment
PubMed: 34073616
DOI: 10.3390/ijms22115688 -
Journal of the American College of... Apr 2005Idiopathic dilated cardiomyopathy (IDC) is characterized by left ventricular dilatation and systolic dysfunction after known causes have been excluded. Idiopathic... (Review)
Review
Idiopathic dilated cardiomyopathy (IDC) is characterized by left ventricular dilatation and systolic dysfunction after known causes have been excluded. Idiopathic dilated cardiomyopathy occurring in families, or familial dilated cardiomyopathy (FDC), may occur in 20% to 50% of IDC cases. Sixteen genes have been shown to cause autosomal dominant FDC, but collectively may account for only a fraction of genetic causation; it is anticipated that additional genes causative of FDC will be discovered. Familial dilated cardiomyopathy demonstrates incomplete penetrance, variable expression, and significant locus and allelic heterogeneity, making clinical and genetic diagnosis complex. Echocardiographic and electrocardiographic screening of first-degree relatives of individuals with IDC and FDC is indicated, as detection and treatment are possible before the onset of advanced symptomatic disease. Genetic counseling for IDC and FDC is also indicated to assist with family evaluations for genetic disease and with the uncertainty and anxiety surrounding the significance of clinical and genetic evaluation. Genetic testing is not yet commonly available, but its emergence will provide new opportunities for presymptomatic diagnosis.
Topics: Cardiomyopathy, Dilated; Echocardiography; Electrocardiography; Family; Genetic Counseling; Genetic Testing; Humans
PubMed: 15808750
DOI: 10.1016/j.jacc.2004.11.066 -
Clinical Cardiology Dec 1998Prior to 1972, the increased cardiovascular morbidity and mortality that diabetics endure had been attributed to vascular disease. In 1972, Rubler et al. proposed the... (Review)
Review
Prior to 1972, the increased cardiovascular morbidity and mortality that diabetics endure had been attributed to vascular disease. In 1972, Rubler et al. proposed the existence of a diabetic cardiomyopathy based on their expereince with four adult diabetic patients who suffered from congestive heart failure (CHF) in the absence of discernable coronary artery disease, valvular or congenital heart disease, hypertension, or alcoholism. Alternative explanations for CHF, such as anemia and vascular and renal disease in these four patients, gave rise to criticisms, but a wave of subsequent studies in the 1970s and 1980s provided credence to this new disease entity. This review of the studies done since 1972 appears to support the concept of a diabetic cardiomyopathy independent of atherosclerotic cardiovascular disease. The exact mechanism is still questionable, and several mechanisms have been proposed including small and microvascular disease, autonomic dysfunction, metabolic derangements, and interstitial fibrosis. However, the weight of evidence leans toward the development of fibrosis, possibly caused by the accumulation of a peroxidase acid schiff (PAS)-positive glycoprotein, leading to myocardial hypertrophy and diastolic dysfunction.
Topics: Cardiomyopathy, Dilated; Diabetes Complications; Humans
PubMed: 9853179
DOI: 10.1002/clc.4960211205 -
JACC. Cardiovascular Imaging Feb 2020
Topics: Cardiomyopathy, Dilated; Genetic Testing; Humans; Pedigree
PubMed: 31864986
DOI: 10.1016/j.jcmg.2019.09.004 -
Revista Chilena de Pediatria Dec 2020Pediatric cardiomyopathies are infrequent diseases of the cardiac muscle, with an annual inciden ce of 1.1 to 1.2 per 100,000 children. Dilated cardiomyopathy (DCM) is...
Pediatric cardiomyopathies are infrequent diseases of the cardiac muscle, with an annual inciden ce of 1.1 to 1.2 per 100,000 children. Dilated cardiomyopathy (DCM) is the predominant form, characterized by ventricular dilatation and systolic dysfunction. Etiologies are multiple, with at least 50%-70% of cases being idiopathic. When assessing a child with DCM, secondary potentially reversible causes must be ruled out. The main diagnostic tool is the echocardiogram which allows the identification of cardiac phenotype, to establish the degree of functional compromise, and res ponse to medical therapy. Prognosis is limited but more favorable in infants younger than 1 year at the onset, post myocarditis, or with a lesser degree of ventricular dysfunction. At least 20% of patients may recover ventricular function in the first 2 years after the onset and 40%-50% may die or need heart transplant in the first 5 years. Medical therapy is mainly based on adult experience with limited scientific evidence in children. Heart transplant is the therapy of choice in patients with end-stage disease, with excellent short- and medium-term survival. A significant proportion of patients may require stabilization on the waiting list, including the use of mechanical circulatory support as a bridge to transplantation. The purpose of this revision is to update the available infor mation on etiology, physiopathological mechanisms, prognostic factors, and management of DCM in children.
Topics: Age Factors; Cardiomyopathy, Dilated; Child; Child, Preschool; Echocardiography; Heart Transplantation; Humans; Infant; Prognosis; Waiting Lists
PubMed: 33861821
DOI: 10.32641/rchped.vi91i6.2851 -
BMJ (Clinical Research Ed.) Apr 1990
Review
Topics: Adult; Aged; Cardiomyopathy, Dilated; Humans
PubMed: 2186831
DOI: 10.1136/bmj.300.6729.890 -
BMJ Case Reports Sep 2012Tachycardia-induced cardiomyopathy (TIC) is an important cause of heart failure as it is potentially reversible after ventricular rate control. A 66-year-old...
Tachycardia-induced cardiomyopathy (TIC) is an important cause of heart failure as it is potentially reversible after ventricular rate control. A 66-year-old hypertensive woman presented with a 15-day history of tachycardia, dyspnoea and oedema. ECG revealed atrial fibrillation with ventricular frequency of 130 beats per minute (bpm). Echocardiogram showed dilated left ventricle (LV) with 0.39 ejection fraction. Angiography revealed non-obstructed coronary arteries. Heart rate and cardiac failure were controlled with amiodarone, digoxine, captopril, metoprolol and furosemide. During follow-up, despite drug dose optimisation, the patient kept complaining of tachycardia and dyspnoea with a ventricular rate between 108 and 120 bpm. Medical staff suspected she was not taking her medicines properly. Two months later, the patient was asymptomatic and had converted to sinus rhythm (heart rate of 76 bpm). Echocardiogram showed normal LV size and function. Patient's diagnosis was TIC. Although rare, TIC should be considered in all cases of systolic dysfunction associated with tachyarrhythmia.
Topics: Aged; Cardiomyopathy, Dilated; Diagnosis, Differential; Dyspnea; Electrocardiography; Female; Heart; Humans; Tachycardia
PubMed: 23001096
DOI: 10.1136/bcr-2012-006587 -
International Journal of Molecular... Feb 2022Left ventricular assist device (LVAD) use in patients with dilated cardiomyopathy (DCM) can lead to a differential response in the LV and right ventricle (RV), and RV...
Left ventricular assist device (LVAD) use in patients with dilated cardiomyopathy (DCM) can lead to a differential response in the LV and right ventricle (RV), and RV failure remains the most common complication post-LVAD insertion. We assessed transcriptomic signatures in end-stage DCM, and evaluated changes in gene expression (mRNA) and regulation (microRNA/miRNA) following LVAD. LV and RV free-wall tissues were collected from end-stage DCM hearts with ( = 8) and without LVAD ( = 8). Non-failing control tissues were collected from donated hearts ( = 6). Gene expression (for mRNAs/miRNAs) was determined using microarrays. Our results demonstrate that immune response, oxygen homeostasis, and cellular physiological processes were the most enriched pathways among differentially expressed genes in both ventricles of end-stage DCM hearts. LV genes involved in circadian rhythm, muscle contraction, cellular hypertrophy, and extracellular matrix (ECM) remodelling were differentially expressed. In the RV, genes related to the apelin signalling pathway were affected. Following LVAD use, immune response genes improved in both ventricles; oxygen homeostasis and ECM remodelling genes improved in the LV and, four miRNAs normalized. We conclude that LVAD reduced the expression and induced additional transcriptomic changes of various mRNAs and miRNAs as an integral component of the reverse ventricular remodelling in a chamber-specific manner.
Topics: Adult; Cardiomyopathy, Dilated; Female; Heart Ventricles; Heart-Assist Devices; Humans; Male; Middle Aged; Transcriptome
PubMed: 35216165
DOI: 10.3390/ijms23042050