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2.
Comput Math Methods Med ; 2021: 4186648, 2021.
Article En | MEDLINE | ID: mdl-34795790

Dilated cardiomyopathy (DCM) is a cardiomyopathy with left ventricle or double ventricle enlargement and systolic dysfunction. It is an important cause of sudden cardiac death and heart failure and is the leading indication for cardiac transplantation. Major heart diseases like heart muscle damage and valvular problems are diagnosed using cardiac MRI. However, it takes time for cardiologists to measure DCM-related parameters to decide whether patients have this disease. We have presented a method for automatic ventricular segmentation, parameter extraction, and diagnosing DCM. In this paper, left ventricle and right ventricle are segmented by parasternal short-axis cardiac MR image sequence; then, related parameters are extracted in the end-diastole and end-systole of the heart. Machine learning classifiers use extracted parameters as input to predict normal people and patients with DCM, among which Random forest classifier gives the highest accuracy. The results show that the proposed system can be effectively utilized to detect and diagnose DCM automatically. The experimental results suggest the capabilities and advantages of the proposed method to diagnose DCM. A small amount of sample input can generate results comparable to more complex methods.


Algorithms , Cardiomyopathy, Dilated/diagnostic imaging , Cardiomyopathy, Dilated/diagnosis , Heart Ventricles/diagnostic imaging , Cardiomyopathy, Dilated/classification , Case-Control Studies , Computational Biology , Diagnosis, Computer-Assisted , Functional Neuroimaging/statistics & numerical data , Humans , Machine Learning , Magnetic Resonance Imaging/statistics & numerical data , Magnetic Resonance Imaging, Cine/statistics & numerical data
3.
Rev. bras. ciênc. vet ; 26(2): 28-33, abr./jun. 2019. tab
Article Pt | LILACS, VETINDEX | ID: biblio-1491637

A presente revisão tem por objetivo aprimorar o conhecimento sobre Dilated Cardiomyopathy (DCM) em cães, visando à compreensão dos aspectos clínicos, diagnóstico e tratamento.  A DCM é caracterizada por dilatação ventricular, disfunção sistólica e arritmias que podem culminar em insuficiência cardíaca e morte. É a segunda cardiopatia mais frequente em cães, acometendo principalmente animais de grande porte e machos. A etiologia é idiopática, mas alguns genes associados à doença já foram identificados. A manifestação clínica é dividida basicamente em estágios oculto e sintomático. O estágio oculto é caracterizado pela presença de alterações elétricas e/ou morfológicas e ausência de sinais clínicos. Os cães podem apresentar o estágio oculto longo até o desenvolvimento de insuficiência cardíaca de forma aguda ou morte súbita. O estágio sintomático é definido pela presença de insuficiência cardíaca esquerda ou biventricular. O diagnóstico somente é confirmado por meio de ecocardiograma e/ou Holter.  Estes exames são considerados padrão-ouro, uma vez que apresentam alta sensibilidade na identificação precoce da doença. Cães de raças predispostas devem ser monitorados anualmente a partir dos três anos de idade. O tratamento tem o intuito de minimizar os efeitos da insuficiência cardíaca, sendo instituído de acordo com a fase em que o animal se encontra. O prognóstico após início dos


The aim of the present review is to improve the knowledge about Cardiomyopathy dilata (CMD) in dogs, in order to understanding clinical aspects, diagnosis and treatment. CMD is characterized by ventricular dilation, systolic dysfunction, and arrhythmias that may culminate in heart failure and death. It is the second most common heart disease in dogs, affecting mainly large animals and males. The etiology is idiopathic, but some genes associated with the disease have already been identified. The clinical manifestation is basically divided into occult and symptomatic stages. The occult stage is characterized by the presence of electrical and/or morphological changes and absence of clinical signs. Dogs may present the long occult stage to the development of acute heart failure or sudden death. The symptomatic stage is defined by the presence of left or biventricular heart failure. The diagnosis is only confirmed by echocardiography and/or Holter. These exams are considered gold standard, since they present high sensitivity in the early identification of the disease. Dogs of predisposed breeds should be monitored annually from the age of three. The treatment is intended to minimize the effects of heart failure, and is instituted according to the stage in which the animal is. The prognosis after onset of clinical signs is worse. Some factors may influence survival in a positive or negative way. Periodic examinations are great importance to obtain early diagnosis and interpose in order to delay the progression of the disease.


Animals , Dogs , Cardiomyopathy, Dilated/classification , Cardiomyopathy, Dilated/diagnosis , Cardiomyopathy, Dilated/veterinary , Dogs/abnormalities
4.
Cell Physiol Biochem ; 52(3): 435-438, 2019.
Article En | MEDLINE | ID: mdl-30873819

BACKGROUND/AIMS: Tachycardiomyopathy (TCM) is a largely reversible form of non-ischemic heart failure. The underlying mechanism are, however, still today poorly understood. Recent data indicate distinct changes in mitochondrial distribution in these patients, compared to other non-ischemic cardiomyopathies.This study investigated underlying mechanisms in mitochondrial dynamics in endomyocardial biopsy samples (EMB) from patients with TCM and compared them to patients with dilated cardiomyopathy (DCM), which show similar clinical features. METHODS: Focused mRNA analyses were performed on routinely obtained paraffinfixed EMB specimen from patients fulfilling TCM diagnosis criteria, as well as patients with DCM to elucidate regulatory changes in mitochondrial fusion, fission and mitophagy. RESULTS: In patients with TCM we were able to identify mRNA of Mitofusin 1 and 2, two effector proteins regulating mitochondrial fusion, to be strongly upregulated compared to patients with DCM. Conclusively, we did not find differences in the mRNA expression of mitochondrial fission regulators including DRP1, Fis1, MFF, MiD49, and MiD51. Furthermore, we did not find significant changes in PINK1 expression, an important mediator for mitochondrial autophagy. CONCLUSION: The mRNA upregulation of Mitofusin 1 and 2 provides first insight into the complex changes of mitochondrial dynamics in cardiomyocytes of patients with reversible heart failure due to TCM.


Cardiomyopathy, Dilated/genetics , GTP Phosphohydrolases/genetics , Mitochondria/genetics , Mitochondrial Dynamics/genetics , Mitochondrial Membrane Transport Proteins/genetics , Mitochondrial Proteins/genetics , RNA, Messenger/genetics , Biopsy , Cardiomyopathy, Dilated/classification , Cardiomyopathy, Dilated/diagnosis , Cardiomyopathy, Dilated/physiopathology , Dynamins , GTP Phosphohydrolases/metabolism , Gene Expression Regulation , Heart Rate/physiology , Humans , Membrane Proteins/genetics , Membrane Proteins/metabolism , Microtubule-Associated Proteins/genetics , Microtubule-Associated Proteins/metabolism , Mitochondria/metabolism , Mitochondria/pathology , Mitochondrial Membrane Transport Proteins/metabolism , Mitochondrial Proteins/metabolism , Mitophagy/genetics , Myocytes, Cardiac/metabolism , Myocytes, Cardiac/pathology , Peptide Elongation Factors/genetics , Peptide Elongation Factors/metabolism , Protein Kinases/genetics , Protein Kinases/metabolism , RNA, Messenger/metabolism
6.
Int Heart J ; 59(5): 959-967, 2018 Sep 26.
Article En | MEDLINE | ID: mdl-30101845

The degree or nature of functional mitral regurgitation (MR) is not necessarily correlated with the size or function of the left ventricle (LV). We hypothesized that the anatomical structure of the mitral valve (MV) complex might play a role in functional MR in ischemic or nonischemic dilated cardiomyopathy (DCM).The structure of the LV and MV complex in DCM patients (n = 29) was assessed using electrocardiogram-gated 320-slice computed tomography and was compared with that in healthy patients (n = 12). Twenty-five DCM patients with mild or low MR (DCM-lowMR) had markedly greater length, diameter, and sphericity index of the LV and a larger tenting area than the controls. The distance between the papillary muscle (PM) tip and the mitral annular plane was not different between DCM-lowMR and normal hearts despite the greater LV length observed in DCM-lowMR. Furthermore, DCM-lowMR had markedly longer chordae tendineae (DCM-lowMR: 24 [20-26] mm; controls: 14 [13-16] mm; P < 0.01) and larger anterior leaflets (DCM-lowMR: 30 [27-31] mm; controls: 22 [20-24] mm; P < 0.01), thus suggesting the adaptive remodeling of the MV complex. Four DCM patients with moderate-severe MR had unbalanced remodeling, such as excessive LV dilatation, short anterior mitral leaflets, and short chordae tendineae.The development of functional MR might be associated with the remodeling of LV and MV components, such as the PMs, chordae tendineae, or anterior MV leaflets. Detailed anatomical assessments of the LV and MV complex would contribute to the adequate staging of ischemic or nonischemic DCM.


Atrial Remodeling/physiology , Cardiomyopathy, Dilated/diagnostic imaging , Chordae Tendineae/diagnostic imaging , Heart Ventricles/diagnostic imaging , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve/diagnostic imaging , Aged , Cardiomyopathy, Dilated/classification , Cardiomyopathy, Dilated/pathology , Cardiomyopathy, Dilated/physiopathology , Chordae Tendineae/anatomy & histology , Chordae Tendineae/physiopathology , Echocardiography/methods , Electrocardiography/methods , Female , Heart Ventricles/anatomy & histology , Heart Ventricles/physiopathology , Humans , Male , Middle Aged , Mitral Valve/anatomy & histology , Mitral Valve/physiopathology , Mitral Valve Insufficiency/physiopathology , Papillary Muscles/anatomy & histology , Papillary Muscles/diagnostic imaging , Papillary Muscles/physiopathology , Retrospective Studies , Tomography, X-Ray Computed
7.
Eur Heart J ; 38(13): 942-954, 2017 04 01.
Article En | MEDLINE | ID: mdl-27147609

The terms used to describe different states of myocardial health and disease are poorly defined. Imprecision and inconsistency in nomenclature can lead to difficulty in interpreting and applying trial outcomes to clinical practice. In particular, the terms 'viable' and 'hibernating' are commonly applied interchangeably and incorrectly to myocardium that exhibits chronic contractile dysfunction in patients with ischaemic heart disease. The range of inherent differences amongst imaging modalities used to define myocardial health and disease add further challenges to consistent definitions. The results of several large trials have led to renewed discussion about the classification of dysfunctional myocardial segments. This article aims to describe the diverse myocardial pathologies that may affect the myocardium in ischaemic heart disease and cardiomyopathy, and how they may be assessed with non-invasive imaging techniques in order to provide a taxonomy of myocardial dysfunction.


Cardiomyopathy, Dilated/pathology , Cardiomyopathy, Hypertrophic/pathology , Myocardial Ischemia/pathology , Acute Disease , Cardiac Imaging Techniques/methods , Cardiomyopathy, Dilated/classification , Cardiomyopathy, Dilated/metabolism , Cardiomyopathy, Hypertrophic/classification , Cardiomyopathy, Hypertrophic/metabolism , Chronic Disease , Heart/physiology , Heart Failure, Diastolic/classification , Heart Failure, Diastolic/metabolism , Heart Failure, Diastolic/pathology , Humans , Myocardial Infarction/classification , Myocardial Infarction/metabolism , Myocardial Infarction/pathology , Myocardial Ischemia/classification , Myocardial Ischemia/metabolism , Myocardial Stunning/classification , Myocardial Stunning/metabolism , Myocardial Stunning/pathology , Myocardium/pathology , Myocytes, Cardiac/metabolism , Myocytes, Cardiac/physiology , Terminology as Topic
8.
JACC Cardiovasc Imaging ; 9(7): 858-872, 2016 07.
Article En | MEDLINE | ID: mdl-27388665

The almost 50-year odyssey of cardiac imaging in hypertrophic cardiomyopathy (HCM), revisited and described here, has been remarkable, particularly when viewed in the timeline of advances that occurred during a single generation of investigators. At each step along the way, from M-mode to 2-dimensional echocardiography to Doppler imaging, and finally over the last 10 years with the emergence of high-resolution tomographic cardiac magnetic resonance (CMR), evolution of the images generated by each new technology constituted a paradigm change over what was previously available. Together, these advances have transformed the noninvasive diagnosis and management of HCM in a number of important clinical respects. These changes include a more complete definition of the phenotype, resulting in more reliable clinical identification of patients and family members, defining mechanisms (and magnitude) of left ventricular outflow obstruction, and novel myocardial tissue characterization (including in vivo detection of fibrosis/scarring); notably, these advances afford more precise recognition of at-risk patients who are potential candidates for life-saving primary prevention defibrillator therapy. This evolution in imaging as applied to HCM has indelibly changed cardiovascular practice for this morphologically and clinically complex genetic disease.


Cardiomyopathy, Dilated/diagnostic imaging , Cardiomyopathy, Hypertrophic/diagnostic imaging , Echocardiography, Doppler , Echocardiography, Three-Dimensional , Echocardiography, Transesophageal , Magnetic Resonance Imaging, Cine , Cardiomyopathy, Dilated/classification , Cardiomyopathy, Dilated/history , Cardiomyopathy, Dilated/therapy , Cardiomyopathy, Hypertrophic/classification , Cardiomyopathy, Hypertrophic/history , Cardiomyopathy, Hypertrophic/therapy , Echocardiography, Doppler/history , Echocardiography, Three-Dimensional/history , Echocardiography, Transesophageal/history , History, 20th Century , History, 21st Century , Humans , Magnetic Resonance Imaging, Cine/history , Phenotype , Predictive Value of Tests , Prognosis
9.
Arq. bras. cardiol ; 106(6): 502-509, tab, graf
Article En | LILACS | ID: lil-787319

Abstract Background: Idiopathic dilated cardiomyopathy (IDCM), most common cardiac cause of pediatric deaths, mortality descriptor: a low left ventricular ejection fraction (LVEF) and low functional capacity (FC). FC is never self reported by children. Objective: The aims of this study were (i) To evaluate whether functional classifications according to the children, parents and medical staff were associated. (iv) To evaluate whether there was correlation between VO2 max and Weber's classification. Method: Prepubertal children with IDCM and HF (by previous IDCM and preserved LVEF) were selected, evaluated and compared. All children were assessed by testing, CPET and functional class classification. Results: Chi-square test showed association between a CFm and CFp (1, n = 31) = 20.6; p = 0.002. There was no significant association between CFp and CFc (1, n = 31) = 6.7; p = 0.4. CFm and CFc were not associated as well (1, n = 31) = 1.7; p = 0.8. Weber's classification was associated to CFm (1, n = 19) = 11.8; p = 0.003, to CFp (1, n = 19) = 20.4; p = 0.0001and CFc (1, n = 19) = 6.4; p = 0.04). Conclusion: Drawing were helpful for children's self NYHA classification, which were associated to Weber's stratification.


Resumo Fundamento: A cardiomiopatia dilatada idiopática (CMDid) possui poucos preditores de mortalidade descritos: a baixa fração de ejeção de ventrículo esquerdo (FEVE) e a baixa capacidade funcional, sendo esta subjetiva. Objetivo: Os objetivos desse estudo foram (i) Avaliar se as classes funcionais propostas pela NYHA, modificada para crianças, estiveram associadas entre a percepção médica (CFm), dos pais ou representantes (CFp) e das próprias crianças avaliadas (CFc). (ii) Avaliar se houve correlação entre VO2 max e a classificação proposta por Weber. Método: Crianças com CMDid e com IC por CMDid prévia com FEVE preservada, na fase pré-puberdade foram selecionadas submetidas a avaliações de ergoespirometria e classificação da classe funcional. As crianças utilizaram uma representação gráfica para se intitular quanto à classe funcional. Resultado: O teste Chi-quadrado mostrou que houve associação ente a CFm e CFp (1, n = 31) = 20,6; p = 0,002. Não houve associação significativa entre CFp e CFc (1, n = 31) = 6,7; p = 0,4. As CF segundo médico e CFc não foram, tampouco, associadas (1, n = 31) = 1,7; p = 0,8. A classificação de Weber foi significativamente associada às três classes funcionais (classificação de Weber e CFm (1, n = 19) = 11,8; p = 0,003; classificação de Weber e CFp (1, n = 19) = 20,4; p = 0,0001; classificação de Weber e CFc (1, n = 19) = 6.4; p = 0.04.). Conclusão: A representação gráfica serviu para que as crianças pudessem se classificar segundo a NYHA, que se demonstrou associada com a estratificação de Weber.


Humans , Male , Female , Child, Preschool , Child , Oxygen Consumption/physiology , Stroke Volume/physiology , Cardiomyopathy, Dilated/physiopathology , Ventricular Function, Left/physiology , Cardiomyopathy, Dilated/classification , Random Allocation , Pilot Projects , Maximal Voluntary Ventilation/physiology , Cross-Sectional Studies , Prospective Studies , Exercise Test
10.
Arq Bras Cardiol ; 106(6): 502-9, 2016 Jun.
Article En, Pt | MEDLINE | ID: mdl-27168472

BACKGROUND: Idiopathic dilated cardiomyopathy (IDCM), most common cardiac cause of pediatric deaths, mortality descriptor: a low left ventricular ejection fraction (LVEF) and low functional capacity (FC). FC is never self reported by children. OBJECTIVE: The aims of this study were (i) To evaluate whether functional classifications according to the children, parents and medical staff were associated. (iv) To evaluate whether there was correlation between VO2 max and Weber's classification. METHOD: Prepubertal children with IDCM and HF (by previous IDCM and preserved LVEF) were selected, evaluated and compared. All children were assessed by testing, CPET and functional class classification. RESULTS: Chi-square test showed association between a CFm and CFp (1, n = 31) = 20.6; p = 0.002. There was no significant association between CFp and CFc (1, n = 31) = 6.7; p = 0.4. CFm and CFc were not associated as well (1, n = 31) = 1.7; p = 0.8. Weber's classification was associated to CFm (1, n = 19) = 11.8; p = 0.003, to CFp (1, n = 19) = 20.4; p = 0.0001and CFc (1, n = 19) = 6.4; p = 0.04). CONCLUSION: Drawing were helpful for children's self NYHA classification, which were associated to Weber's stratification.


Cardiomyopathy, Dilated/physiopathology , Oxygen Consumption/physiology , Stroke Volume/physiology , Ventricular Function, Left/physiology , Cardiomyopathy, Dilated/classification , Child , Child, Preschool , Cross-Sectional Studies , Exercise Test , Female , Humans , Male , Maximal Voluntary Ventilation/physiology , Physical Exertion/physiology , Pilot Projects , Prospective Studies , Random Allocation
11.
J Am Coll Cardiol ; 66(12): 1313-23, 2015 Sep 22.
Article En | MEDLINE | ID: mdl-26383716

BACKGROUND: The multifactorial pathogenesis leading to dilated cardiomyopathy (DCM) makes stratification difficult. The recent MOGE(S) (morphofunctional, organ involvement, genetic or familial, etiology, stage) classification addresses this issue. OBJECTIVES: The purpose of this study was to investigate the applicability and prognostic relevance of the MOGE(S) classification in patients with DCM. METHODS: This study used patients from the Maastricht Cardiomyopathy Registry in the Netherlands and excluded patients with ischemic, valvular, hypertensive, and congenital heart disease. All other patients underwent a complete diagnostic work-up, including genetic evaluation and endomyocardial biopsy. RESULTS: A total of 213 consecutive patients with DCM were included: organ involvement was demonstrated in 35 (16%) and genetic or familial DCM in 70 (33%) patients, including 16 (8%) patients with a pathogenic mutation. At least 1 cause was found in 155 (73%) patients, of whom 48 (23%) had more than 1 possible cause. Left ventricular reverse remodeling was more common in patients with nongenetic or nonfamilial DCM than in patients with genetic or familial DCM (40% vs. 25%; p = 0.04). After a median follow-up of 47 months, organ involvement and higher New York Heart Association functional class were associated with adverse outcome (p < 0.001 and p = 0.02, respectively). Genetic or familial DCM per se was of no prognostic significance, but when it was accompanied by additional etiologic-environmental factors such as significant viral load, immune-mediated factors, rhythm disturbances, or toxic triggers, a worse outcome was revealed (p = 0.03). A higher presence of MOGE(S) attributes (≥2 vs. ≤1 attributes) showed an adverse outcome (p = 0.007). CONCLUSIONS: The MOGE(S) classification in DCM is applicable, and each attribute or the gene-environment interaction is associated with outcome. Importantly, the presence of multiple attributes was a strong predictor of adverse outcome. Finally, adaptation of the MOGE(S) involving multiple possible etiologies is recommended.


Cardiomyopathy, Dilated/classification , Cardiomyopathy, Dilated/etiology , Gene-Environment Interaction , Adult , Cardiomyopathy, Dilated/diagnosis , Female , Humans , Male , Middle Aged , Prognosis , Severity of Illness Index
13.
Cardiovasc Pathol ; 24(6): 351-8, 2015.
Article En | MEDLINE | ID: mdl-26382083

BACKGROUND: The appropriate condition of the coronary microcirculation is essential for proper cardiac muscle activity. The understanding of the pathological microcirculation changes in different stages of idiopathic dilated cardiomyopathy (IDCM) could provide a reliable background for proper therapeutic decisions and prognosis. METHODS AND RESULTS: The study population consisted of 116 patients (86.2% males, mean age 50.4±13.2 years) with IDCM and heart failure. In samples from left ventricular endomyocardial biopsy, the coronary microcirculation was evaluated by staining with hematoxylin and eosin, Masson's trichrome, and anti-CD34 antibody. The microvessel density (MVD) was calculated. Also, the electron microscopic evaluation of the extracellular matrix capillaries was performed. Samples were assigned to one of four types according to the microcirculation condition: 1, normal microvessels (MVs) (18 patients); 2, mostly normal, some MVs with slightly decreased lumen diameter and thickened wall, absent/mild intravascular fibrosis, and MVD decrease (37 patients); 3, MVs with moderately decreased lumen diameter and thickened wall, moderate intravascular fibrosis, and MVD decrease (45 patients); and 4, MVs with significantly decreased lumen diameter and thickened wall, significant intravascular fibrosis, and MVD decrease (16 patients). Taking all types of the proposed classification into consideration, in type 4, clinical (incidence of New York Heart Association 3 and 4, dyspnea on exertion, pulmonary congestion) and echocardiographic (left atrial and right ventricular diameter, left ventricular mass and ejection fraction, tricuspid annular plane systolic excursion, early diastolic mitral annular velocity measured at the interventricular-septal annulus [E'med], ratio of early diastolic mitral inflow velocity to E'med) parameters were worst. Only atrial fibrillation, diabetes, tricuspid annular plane systolic excursion, and the type of the microcirculation significantly correlated with the incidence of cardiovascular hospitalizations in the linear regression models. CONCLUSION: The condition of the coronary microcirculation corresponds with the heart failure progression in patients with IDCM.


Cardiomyopathy, Dilated/complications , Coronary Vessels/pathology , Heart Failure/etiology , Microvessels/pathology , Terminology as Topic , Adult , Aged , Biopsy , Cardiomyopathy, Dilated/classification , Cardiomyopathy, Dilated/pathology , Cardiomyopathy, Dilated/physiopathology , Cardiomyopathy, Dilated/therapy , Chi-Square Distribution , Coronary Circulation , Coronary Vessels/physiopathology , Coronary Vessels/ultrastructure , Disease Progression , Female , Fibrosis , Heart Failure/classification , Heart Failure/pathology , Heart Failure/physiopathology , Heart Failure/therapy , Hospitalization , Humans , Linear Models , Male , Microcirculation , Microscopy, Electron , Microvessels/physiopathology , Microvessels/ultrastructure , Middle Aged , Multivariate Analysis , Predictive Value of Tests , Proportional Hazards Models , Prospective Studies , Risk Factors , Stroke Volume , Ventricular Function, Left
14.
Lancet ; 386(9995): 813-25, 2015 Aug 22.
Article En | MEDLINE | ID: mdl-25865865

Left ventricular non-compaction, the most recently classified form of cardiomyopathy, is characterised by abnormal trabeculations in the left ventricle, most frequently at the apex. It can be associated with left ventricular dilation or hypertrophy, systolic or diastolic dysfunction, or both, or various forms of congenital heart disease. Affected individuals are at risk of left or right ventricular failure, or both. Heart failure symptoms can be induced by exercise or be persistent at rest, but many patients are asymptomatic. Patients on chronic treatment for compensated heart failure sometimes present acutely with decompensated heart failure. Other life-threatening risks of left ventricular non-compaction are ventricular arrhythmias or complete atrioventricular block, presenting clinically as syncope, and sudden death. Genetic inheritance arises in at least 30-50% of patients, and several genes that cause left ventricular non-compaction have been identified. These genes seem generally to encode sarcomeric (contractile apparatus) or cytoskeletal proteins, although, in the case of left ventricular non-compaction with congenital heart disease, disturbance of the NOTCH signalling pathway seems part of a final common pathway for this form of the disease. Disrupted mitochondrial function and metabolic abnormalities have a causal role too. Treatments focus on improvement of cardiac efficiency and reduction of mechanical stress in patients with systolic dysfunction. Further, treatment of arrhythmia and implantation of an automatic implantable cardioverter-defibrillator for prevention of sudden death are mainstays of therapy when deemed necessary and appropriate. Patients with left ventricular non-compaction and congenital heart disease often need surgical or catheter-based interventions. Despite progress in diagnosis and treatment in the past 10 years, understanding of the disorder and outcomes need to be improved.


Isolated Noncompaction of the Ventricular Myocardium/diagnosis , Animals , Anticoagulants/therapeutic use , Arrhythmias, Cardiac/etiology , Cardiomyopathy, Dilated/classification , Cardiomyopathy, Dilated/diagnosis , Cardiomyopathy, Dilated/therapy , Defibrillators, Implantable , Disease Models, Animal , Echocardiography , Electrocardiography , Female , Humans , Isolated Noncompaction of the Ventricular Myocardium/classification , Isolated Noncompaction of the Ventricular Myocardium/therapy , Magnetic Resonance Angiography , Male , Mice , Mutation/genetics , Signal Transduction/genetics , Treatment Outcome
15.
Rev. Méd. Clín. Condes ; 26(2): 210-216, mar. 2015.
Article Es | LILACS | ID: biblio-1128819

En la última década se ha producido un incremento sustancial en el conocimiento de las bases genéticas de las cardiomiopatías, en consideración a lo cual las Sociedades Americanas y Europea de Cardiología, han propuesto una nueva clasificación con el fin de incluir tales aspectos, Aunque se carece de datos precisos, aproximadamente el 30% de los pacientes con miocardiopatías referidos para pruebas genéticas presentan una variante genética patogénica. En la herencia de la miocardiopatía dilatada familiar, la modalidad predominante es la autosómica dominante, siendo mucho menos frecuentes las ligadas al cromosoma X, la herencia autosómica recesiva y la mitocondrial. A su vez, pruebas provenientes de ensayos clínicos y experimentales indican que la infección, la inflamación y el sistema inmunológico están de alguna manera interrelacionados en los mecanismos patogénicos implicados en la miocardiopatía dilatada, No hay duda que en un futuro próximo las mejoras en la secuenciación de genes y el mayor conocimiento de la patogenia influirán decididamente en el diagnóstico, evaluación y tratamiento de esta entidad.


In the last time, a substantial increase in the knowledge of the genetic basis of cardiomyopathy has occurred. Therefore in the last decade the American Heart Association, the American College of Cardiology and the European Society of Cardiology have proposed a new revision of the classification of cardiomyopathies in order to include the genetic basis on the etiology, Although precise data are lacking, approximately 30% of patients currently referred for clinical genetic testing will be found to have a pathogenic genetic variant. The predominant mode of inheritance for familial dilated cardiomyopathy is autosomal dominant, with X linked autosomal recessive and mitochondrial inheritance being less frequent. Evidence from experimental and clinical trials indicates that infection, inflammation and the immune system are in some way interrelated on the pathogenic mechanisms involved in the dilated cardiomyopathy, There is no doubt that in the future, improvements in sequencing genes and insight into pathogenesis will influence the diagnosis, evaluation and management of familial dilated cardiomyopathy.


Humans , Cardiomyopathy, Dilated/genetics , Cardiomyopathy, Dilated/immunology , Cardiomyopathy, Dilated/microbiology , Cardiomyopathy, Dilated/classification , Cardiomyopathy, Dilated/parasitology , Inflammation
19.
Arq. bras. cardiol ; 101(5): 457-465, nov. 2013. ilus, tab
Article Pt | LILACS | ID: lil-696886

FUNDAMENTO: A insuficiência mitral (IM) é frequente nos pacientes com cardiomiopatia dilatada. Não se sabe se os critérios para classificação da IM são adequados para pacientes com cardiomiopatia dilatada OBJETIVO: Avaliar a concordância entre os quatro métodos ecocardiográficos mais utilizados para classificação da IM. MÉTODOS: Noventa pacientes com cardiomiopatia dilatada foram incluídos. A IM foi classificada por quatro métodos ecocardiográficos: área do jato regurgitante (AJ), vena contracta (VC), área do orifício regurgitante (AOR) e volume regurgitante (VR). A IM foi classificada em leve, moderada ou importante segundo os critérios da American Society of Echocardiography e também foi dividida em tercis conforme os valores absolutos. O teste de Kappa foi utilizado para avaliar a concordância entre os métodos. O coeficiente de Pearson foi utilizado para avaliar a correlação entre os valores absolutos por cada método. RESULTADOS: A classificação da IM, de acordo com cada método, foi a seguinte: AJ: 26 leve, 44 moderada, 20 importante; VC: 12 leve, 72 moderada, 6 importante; AOR: 70 leve, 15 moderada, 5 importante; VR: 70 leve, 16 moderada, 4 importante. A concordância entre os métodos foi ruim (kappa = 0,11; p < 0,001), porém foi observada uma forte correlação entre os valores absolutos de cada método (0,70 a 0,95; p < 0,01). A concordância foi melhor com a divisão dos valores em tercis (kappa = 0,44; p < 0,01). CONCLUSÃO: Os critérios para classificação da IM não são adequados para os pacientes com cardiomiopatia dilatada. É necessário estabelecer novos valores de corte para classificar a IM nestes pacientes.


BACKGROUND: Mitral regurgitation (MR) is common in patients with dilated cardiomyopathy (DCM). It is unknown whether the criteria for MR classification are inadequate for patients with DCM. OBJECTIVE: We aimed to evaluate the agreement among the four most common echocardiographic methods for MR classification. METHODS: Ninety patients with DCM were included. Functional MR was classified using four echocardiographic methods: color flow jet area (JA), vena contracta (VC), effective regurgitant orifice area (ERO) and regurgitant volume (RV). MR was classified as mild, moderate or important according to the American Society of Echocardiography criteria and by dividing the values into terciles. The Kappa test was used to evaluate whether the methods agreed, and the Pearson correlation coefficient was used to evaluate the correlation between the absolute values of each method. RESULTS: MR classification according to each method was as follows: JA: 26 mild, 44 moderate, 20 important; VC: 12 mild, 72 moderate, 6 important; ERO: 70 mild, 15 moderate, 5 important; RV: 70 mild, 16 moderate, 4 important. The agreement was poor among methods (kappa=0.11; p<0.001). It was observed a strong correlation between the absolute values of each method, ranging from 0.70 to 0.95 (p<0.01) and the agreement was higher when values were divided into terciles (kappa = 0.44; p < 0.01) CONCLUSION: The use of conventional echocardiographic criteria for MR classification seems inadequate in patients with DCM. It is necessary to establish new cutoff values for MR classification in these patients.


Adult , Female , Humans , Male , Middle Aged , Cardiomyopathy, Dilated , Mitral Valve Insufficiency , Cardiomyopathy, Dilated/classification , Echocardiography/methods , Echocardiography/standards , Mitral Valve Insufficiency/classification
20.
Arq Bras Cardiol ; 101(5): 457-65, 2013 Nov.
Article En, Pt | MEDLINE | ID: mdl-24100692

BACKGROUND: Mitral regurgitation (MR) is common in patients with dilated cardiomyopathy (DCM). It is unknown whether the criteria for MR classification are inadequate for patients with DCM. OBJECTIVE: We aimed to evaluate the agreement among the four most common echocardiographic methods for MR classification. METHODS: Ninety patients with DCM were included. Functional MR was classified using four echocardiographic methods: color flow jet area (JA), vena contracta (VC), effective regurgitant orifice area (ERO) and regurgitant volume (RV). MR was classified as mild, moderate or important according to the American Society of Echocardiography criteria and by dividing the values into terciles. The Kappa test was used to evaluate whether the methods agreed, and the Pearson correlation coefficient was used to evaluate the correlation between the absolute values of each method. RESULTS: MR classification according to each method was as follows: JA: 26 mild, 44 moderate, 20 important; VC: 12 mild, 72 moderate, 6 important; ERO: 70 mild, 15 moderate, 5 important; RV: 70 mild, 16 moderate, 4 important. The agreement was poor among methods (kappa=0.11; p<0.001). It was observed a strong correlation between the absolute values of each method, ranging from 0.70 to 0.95 (p<0.01) and the agreement was higher when values were divided into terciles (kappa = 0.44; p < 0.01) CONCLUSION: The use of conventional echocardiographic criteria for MR classification seems inadequate in patients with DCM. It is necessary to establish new cutoff values for MR classification in these patients.


Cardiomyopathy, Dilated/diagnostic imaging , Mitral Valve Insufficiency/diagnostic imaging , Adult , Cardiomyopathy, Dilated/classification , Echocardiography/methods , Echocardiography/standards , Female , Humans , Male , Middle Aged , Mitral Valve Insufficiency/classification
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