Cardiomyopathy: a necessary revision of the WHO classification

Boffa, Giovanni; Thiene, Gaetano; Nava, Andrea; Volta, Sergio Dalla

doi:10.1016/0167-5273(91)90117-8

Cited by 40 publications

(13 citation statements)

References 27 publications

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“…Some investigators consider fatty and fibrofatty patterns to be consecutive stages of disease, mediated by myocarditis, in which the fatty form is an early feature and fibrofatty repair results from myocarditis-induced injury. 21,22 Consistent with this hypothesis, RV inflammatory infiltrates were pronounced in boxer dogs with the fibrofatty form, whereas areas of myocarditis were small and uncommon when associated with purely fatty replacement. It is also noteworthy that in the feline model of ARVC, RV myocarditis was also a characteristic feature in the fibrofatty pattern.…”

Section: Discussionmentioning

confidence: 73%

Arrhythmogenic Right Ventricular Cardiomyopathy Causing Sudden Cardiac Death in Boxer Dogs

et al. 2004

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Background-Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary familial heart muscle disease associated with substantial cardiovascular morbidity and risk of sudden death. Efforts to discern relevant pathophysiological mechanisms have been impaired by lack of a suitable animal model. Methods and Results-ARVC was diagnosed in 23 boxer dogs (12 male; 9.1Ϯ2.3 years old). Clinical events alone or in combination included sudden death (nϭ9; 39%), ventricular arrhythmias of suspected right ventricular (RV) origin (nϭ19; 83%), syncope (nϭ12, 52%), and heart failure (nϭ3; 13%). Right ventricular enlargement or aneurysms occurred in 10 (43%). Striking histopathological abnormalities were present in each boxer dog but not in controls, including severe RV myocyte loss with replacement by fatty (nϭ15, 65%) or fibrofatty (nϭ8, 35%) tissue. Focal fibrofatty lesions were also present in both atria (nϭ8) and the left ventricle (LV) (nϭ11). Fatty replacement occupied substantially greater RV wall area in ARVC dogs than controls (40.4Ϯ18.8% versus 13.8Ϯ3.4%, respectively) (PϽ0.001); residual myocardium was correspondingly reduced (56.6Ϯ19.2% versus 84.8Ϯ3.8% in controls) (PϽ0.001). MRI demonstrated bright anterolateral and/or infundibular RV myocardial signals, confirmed as fat by histopathology. Myocarditis appeared in the RV (nϭ14, 61%) and LV (nϭ16, 70%) and in each dog with sudden death, but not in controls. Familial transmission was evident in 10 of the 23. Key Words: models, animal Ⅲ cardiomyopathy Ⅲ pathology Ⅲ death, sudden A rrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary familial cardiomyopathy associated with substantial cardiovascular morbidity and sudden death in the young. 1-3 ARVC is transmitted as an autosomal dominant trait, and 2 mutations have been identified at the cardiac ryanodine receptor 2 gene (ARVD2) and the desmoplakin gene (ARVD8). 4,5 It has been noted for many years that the boxer canine breed is predisposed to ventricular arrhythmias and sudden death, 6,7 but the underlying disease responsible for these clinical features has been incompletely defined. In light of advances in genomic mapping of the domestic dog, 8 a spontaneous canine model of ARVC and sudden death would provide a unique opportunity to study this disease genome and contribute valuable insights into its pathogenesis. Therefore, the purpose of the present study was to define the clinical and pathological features of a naturally occurring myocardial disease in boxer dogs and assess its suitability as an animal model of ARVC. Conclusions-We Methods Selection of Animal SubjectsAs part of an ongoing study to evaluate the heritability of ventricular arrhythmias in boxer dogs, 239 such animals, including 6 large families, were prospectively recruited for Holter ECG between 1997 and 1999 at the Ohio State University College of Veterinary Medicine. Of those with substantial ectopy or syncope that died or were euthanized, 23 boxer dogs (12 male, 11 female) had autopsy examination and constitute the study group....

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Section: Discussionmentioning

confidence: 73%

Arrhythmogenic Right Ventricular Cardiomyopathy Causing Sudden Cardiac Death in Boxer Dogs

et al. 2004

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“…The criteria used for patient listing for HTx or high-urgency HTx are of vital importance, thus the need for more reliable predictors of the time course of heart failure (HF) has become increasingly important. Although many studies analyzing the long term predictors of HF course have been published (2)(3)(4)(5), the data on short term prognostic markers in patients with DCM, which could be helpful in HTx listing, are scarce.…”

Section: Preamblementioning

confidence: 99%

“…The World Health Organization (WHO) defines DCM as a condition in which the ventricular chambers exhibit increased diastolic and systolic volume and a low (<40%) ejection fraction (1,2). The prevalence of DCM in the adult population in Western countries is 1-1,5% and as already mentioned it is considered as the most common cause of chronic HF syndrome.…”

Section: Definition and Epidemiologymentioning

confidence: 99%

Prediction of Short-Term Outcomes in Patients With Idiopathic Dilated Cardiomyopathy Referred for Transplantation Using Standard Echocardiography and Strain Imaging

Jasaitytė

Dandel

Lehmkuhl

et al. 2009

Transplantation Proceedings

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“…The hallmark of differential diagnosis of pediatric cardiomyopathies is the assessment of myocardial abnormalities in systolic function (contractile ability), diastolic function (relaxation and compliance), and myocardial growth (hypertrophy and atrophy) [43][44][45][46][47]. Still, diagnostic evaluation of pediatric cardiomyopathies remains an important clinical challenge because of a large number of idiopathic genetic etiologies, a broad range of clinical manifestations and large numbers of specialized clinical and biochemical tests complicating confirmatory diagnosis [27].…”

Section: Differential Diagnosismentioning

confidence: 99%

Pediatric cardiomyopathies: A review of literature on clinical status and meta-analysis of diagnosis and clinical management methods

Albakri¹

2018

Pediatr Dimensions

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Cardiomyopathies define a heterogeneous group of heart muscle disorders. They are an important cause of heart failure and a leading indication for heart transplantation in pediatric patients. Research on cardiomyopathy has however focused on the adult populations with fewer dedicated clinical trials investigating pediatric cardiomyopathy. Diagnosis and clinical management of pediatric cardiomyopathy are also challenging because of the lack of specific methods for diagnosis and treatment. Assessment of ventricular structure using absolute values in adults are not useful in children because of their constantly changing body surface area. Therapies developed for adults applied to pediatric patients also have had varied clinical outcomes. This paper therefore reviews existing research evidence on pediatric cardiomyopathy, with the objective of broadening its clinical understanding and management.

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Cardiomyopathy: a necessary revision of the WHO classification

Cited by 40 publications

References 27 publications

Arrhythmogenic Right Ventricular Cardiomyopathy Causing Sudden Cardiac Death in Boxer Dogs

Arrhythmogenic Right Ventricular Cardiomyopathy Causing Sudden Cardiac Death in Boxer Dogs

Prediction of Short-Term Outcomes in Patients With Idiopathic Dilated Cardiomyopathy Referred for Transplantation Using Standard Echocardiography and Strain Imaging

Pediatric cardiomyopathies: A review of literature on clinical status and meta-analysis of diagnosis and clinical management methods

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