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PubMed1985

Myocarditis and cardiomyopathy in the dog and cat.

Myocarditis and cardiomyopathy were diagnosed in 36 dogs from 11 litters, and myoendocarditis and restrictive cardiomyopathy were diagnosed in 51 cats. Most of the dogs and cats died unexpectedly. Spontaneous parvoviral infection in the dogs caused acute (myocytolysis with presence of intranuclear inclusion bodies in the myocytes), subacute (inflammatory reaction and myocytolysis), and chronic (fibrosis and myocytolysis) myocarditis, which led to extensive myocardial fibrosis and abnormality of the myocytes, similar to dilated cardiomyopathy in man. Spontaneous acute, subacute, and chronic myoendocarditis in the cats led to granulation, extensive fibrosis, and necrosis of the myoendocardium, i.e., like restrictive cardiomyopathy which occurs in man without eosinophilia. Thus, the dog and cat are important animal models of primary myocardial disease.

Acute Disease↗

PubMedDate not supplied

Transient right sided hypertrophic cardiomyopathy in an infant born to a diabetic mother.

Hypertrophic cardiomyopathy (HCM) is a rare primary myocardial disease, characterized by hypertrophy of the left and/or right ventricle. Infants of diabetic mothers (IDM) are at risk for development of HCM, respiratory distress and persistent pulmonary hypertension. A case of severe right sided HCM in an infant born to a diabetic mother is presented. The patient's findings were complementary to the previous observations reporting HCM in IDM. The presence of disproportionate septal hypertrophy in the echocardiography of an infant born to a diabetic mother is highly suggestive of HCM in IDM. In our opinion, further cardiac evaluation is not indicated unless other cardiac abnormalities are suspected.

Cardiomyopathy, Hypertrophic↗

PubMedDate not supplied

Systolic time intervals in diabetes mellitus.

Systolic time intervals (STI) were evaluated in 45 diabetics without any cardiac signs and symptoms in order to study the left ventricular function. The STI parameters revealed an impairment of left ventricular function, thus implying preclinical myocardial involvement. This left ventricular dysfunction was more marked in patients in poor metabolic control and in cases with evidence of microangiopathy in kidney and retina, thus favoring the view that the primary myocardial disease is the result of metabolic and vascular components. Significant improvement in STI parameters was observed when good metabolic control was achieved, thus indicating that strict metabolic control is indeed worthwhile.

Age Factors↗

PubMedDate not supplied

Recent advances in the treatment of congestive heart failure.

Treatment of heart failure should include correction of the underlying cause. These causes include large left to right shunts, obstructive lesions, arrhythmias, primary myocardial disease etc. The main pharmacological therapy includes inotropic agents, vasodilators and diuretics. Inotropic agents increase myocardial contractility and include digoxin, intravenous dopamine, dobutamine and isoproterenol. Vasodilators improve cardiac pump performance by decreasing the vascular resistance and/or increasing the venous capacitance. Commonly used vasodilator agents include angiotensin converting enzyme inhibitors (captopril, enalapril etc.), hydralazine, prazosin hydrochloride etc. Diuretics inhibit salt and water reabsorption promoting their excretion. Furosemide, thiazide diuretics, aldactone, are commonly used diuretics. Electrolyte and acid-base imbalance can occur on chronic diuretic therapy. Cardiac transplantation is considered for patients where all medical management has failed.

Cardiovascular Agents↗

PubMed2005

[Familial dilated cardiomyopathy].

Dilated cardiomyopathy (DCM) is the most frequent form of primary myocardial diseases and the third most common cause of heart failure. Clinically, DCM is characterized by a progressive course of ventricular dilatation and systolic dysfunction. The life expectancy is limited and varies according to the underlying etiology with a median survival time of about 5 years after diagnosis. Myocarditis, immunologic abnormalities, toxic myocardial damage, and genetic factors are all assumed to be causes. Familial occurrence of DCM, mostly as an autosomal dominant trait, is more common than generally believed and is responsible for 20-30% of all cases of DCM. Candidate gene screening and linkage analyses in large families were successful in identifying 24 disease genes. There is a wide variability in the onset, course and severity of the disease even within the same family. In addition, genotype-phenotype correlations included only small numbers of affected. This implies that in most cases no conclusion can be drawn from the clinical manifestation of DCM to the responsible disease gene. Mutations in the beta-myosin heavy chain and in cardiac troponin T are common causes of pure familial DCM. DCM associated with conduction disease is mainly due to mutations in lamin A/C and X-linked DCM is often caused by mutations in dystrophin. All other disease genes are rare causes of familial DCM. Genetic screening in all known disease genes is not possible, but more efficient screening methods are awaited in the near future. Until then, clinical examination of family members and, in case of familial DCM, genetic counseling are recommended in the work-up of patients with idiopathic DCM.

Cardiomyopathy, Dilated↗

PubMed2006

The diabetic myocardium.

Heart failure and diabetes mellitus are frequently associated, with diabetes potentiating the development of heart failure after other myocardial insults. This review documents the evidence in support of a specific primary myocardial disease in diabetes. The strongest clinical evidence relates to the detection of otherwise unexplained diastolic dysfunction in apparently healthy diabetic subjects, but recent studies with sensitive echocardiographic markers have shown systolic disturbances as well. The mechanism of this myocardial disease is multifactorial, with contributions from metabolic effects on the myocyte, structural changes in the myocardium and interstitium, autonomic neuropathy, and perhaps coronary vascular disease. The common pathway appears to be related to glycemic control and new evidence suggests better metabolic control to be beneficial, as well as angiotensin-converting enzyme inhibition and cross-link breakers.

Diabetes Mellitus↗

PubMed2002

Hypertrophic cardiomyopathy: who plays and who sits.

Hypertrophic cardiomyopathy (HCM) is a genetically determined, primary myocardial disease associated with an increased risk for sudden cardiac death during physical exertion. In the United States, HCM is the most frequent cause of exertion-related sudden cardiac death (SCD). Current recommendations provided by the 26th Bethesda Conference entitled Recommendations for Determining Eligibility for Competition in Athletes with Cardiovascular Abnormalities restrict participation for patients with HCM to sports requiring low levels of dynamic and isometric exertion. Such recommendations are prudent given the association of the disease with disastrous cardiovascular consequences. Nevertheless, because the prognosis of HCM and its risk of sudden death is typified by great variability among patients, these recommendations may be overly restrictive for many patients with this disease, and it is possible that a subset of low-risk patients can be identified who may continue to engage in more vigorous exercise activities. This article presents our current understanding and approach to evaluating and advising athletes with HCM.

Cardiomyopathy, Hypertrophic↗

PubMed1983

Relationship of angiographic and echographic dimensions in chronic left ventricular dilatation.

Recently discrepancies have been reported between echocardiographic (Echo) % delta D and angiocardiographic (Angio) ejection fraction (EF), particularly in valvular regurgitation. One hundred and twelve patients with varying degrees of LV dilatation were studied in the right anterior oblique position with M-mode Echo. None had localized contraction abnormalities. There were 20 normals, 33 with primary myocardial disease, and 59 with mitral and/or aortic regurgitation. Echo end-diastolic diameter (EDD) and end-systolic diameter (ESD) were consistently smaller than Angio calculated EDD and ESD, and the difference was magnified at larger EDDs (p less than 0.01). The result is a relatively poor correlation of Echo % delta D and Angio EF (r = 0.69), compared to r = 0.98 for Angio % delta D and EF, both from Angio visualization. However, if valvular disease patients are excluded, the correlation improves to 0.82. The mechanisms for these disparities include increased sphericity as the ventricle dilates, and in the case of valvular disease where the EF is better for any degree of dilatation, the echo measurement errors for EDD and ESD are different.

Adolescent↗

PubMed1980

Value and limitations of two dimensional echocardiography in assessment of cardiomyopathy.

Primary myocardial diseases have traditionally been classified into congestive, hypertrophic and restrictive varieties. M mode echocardiography has proved extremely valuable in distinguishing congestive cardiomyopathy with major ventricular dilation and poor contractile performance from hypertrophic cardiomyopathy with predominant left ventricular hypertrophy (septum greater than posterior wall) and normal pump performance, and restrictive disorders with symmetric ventricular wall thickening and normal or diminished contractile function. The contributions of two dimensional echocardiography to the evaluation of patients with congestive or restrictive cardiomyopathy have been limited to certain specific situations, such as the recognition of left ventricular mural thrombi. Twenty-five patients with hypertrophic cardiomyopathy were studied to assess two dimensional echocardiography in the evaluation of patients with this disorder. Two dimensional echocardiography indicated that hypertrophy of the interventricular septum is not uniform from apex to base in all patients but may be greatest in the apical, mid or basal third. In addition, the anterior free wall of the left ventricle was involved in the hypertrophic process in approximately 50 percent of patients. Two dimensional echocardiography documented that the location of systolic anterior motion of the mitral valve in patients with hypertrophic cardiomyopathy is most often at the junction of the mitral valve leaflets and chordae tendineae, although the chordae themselves and even th papillary muscles may be involved in this movement. The heterogeneity of these characteristics may enable patients with hypertrophic cardiomyopathy to be classified into subsets of patients in whom meaningful therapeutic and prognostic implications may be derived. Finally, data derived from two dimensional echocardiography have shown that, although the left ventricle in hypertrophic cardiomyopathy conforms in some degree to the configuration of a catenoid, this geometric conformation is unlikely to account for the genesis of this order.

Cardiomegaly↗

PubMedDate not supplied

The use of echocardiography for quantitative evaluation of left ventricular function.

Echocardiography is a valuable technique for the diagnosis and serial follow-up of patients with impaired cardiac function. It is subject to certain limitations due to the assumptions inherent in deriving ventricular volume from a one-dimensional measurement and must be interpreted with caution in cases of suspected regional abnormalities of contraction. Given these caveats, echocardiography is valuable in the quantitative assessment of cardiac size and the level of compensation in patients with primary myocardial disease, valvular heart disease, and left ventricular hypertrophy. It can detect abnormal contraction in some patients with ischemic heart disease and provides an accurate method to serially follow changes produced as a result of drug or surgical therapy. Finally, two-dimensional techniques promise to provide a new perspective on the evaluation of patients with regional wall motion abnormalities.

Cardiac Volume↗

PubMed1993

Infantile histiocytoid cardiomyopathy--myocardial or conduction system hamartoma: what is the cell type involved?

Primary myocardial diseases in the pediatric age group encompass a variety of metabolic, infectious, congenital, and acquired disorders, one of which is "histiocytoid cardiomyopathy." We describe clinical and pathologic features in two infants. Autopsy findings in the first case were consistent with sudden cardiac death. The second infant has survived for 2 years on antiarrhythmic therapy with amiodarone. Microscopically, cells with vacuolated to granular cytoplasm were grouped in fascicles, imparting a pseudonodular appearance, but following a distribution reminiscent of conduction fibers. Ultrastructurally, the cells lack a T-tubule system, possess scattered lipid droplets and desmosomes rather than side-to-side junctions, and contain leptomeric fibrils that predominantly marginate to the cell periphery without sarcomeres. Immunostaining of paraffin-embedded tissue reveals perimembranous immunoreactivity for muscle-specific actin, but not for the histiocytic markers CD68 (KP1) and lysozyme. Immunohistochemistry may be a practical alternative when tissue is not saved for electron microscopy. The clinical and pathologic features of this disorder in light of the current literature suggest that it may be hamartoma, possibly of conduction system origin.

Actins↗

PubMed2004

Unique features of early diastolic mitral annulus velocity in constrictive pericarditis.

BACKGROUND: In most primary myocardial diseases, early diastolic mitral annulus velocity (E') decreases with disease progression. To our knowledge, constrictive pericarditis (CP) is the only condition without this phenomenon. OBJECTIVE: This study was performed to evaluate the diagnostic and pathophysiologic role of mitral annulus velocity in patients with CP. METHODS: In all, 17 patients with CP (9 men; mean age 46.5 +/- 14.3 years), 8 patients with cardiac tamponade (Tamp) (2 men; mean age 44.5 +/- 15.0 years), and age- and sex-matched control subjects for CP and Tamp were recruited for the study. Early mitral inflow velocity and E' were obtained while simultaneously recording respiration. In 8 patients with CP and in all patients with Tamp, these measurements were repeated after the relief of constrictive physiology or after pericardiocentesis. RESULTS: In patients with CP, E' was significantly higher than it was for control subjects (12.9 +/- 3.0 cm/s vs 9.8 +/- 2.4 cm/s, P <.01). An E' of 2 cm/s higher than the predicted normal E' could differentiate patients with CP from control subjects with a sensitivity of 76% and specificity of 82%. In 12 of 17 patients (71%), inspiratory E' was higher than expiratory E'-the opposite of mitral inflow variation. In 8 patients, E' decreased significantly after the relief of constrictive physiology (13.8 +/- 2.5 cm/s vs 9.3 +/- 3.1 cm/s, P <.05). In contrast, E' in the Tamp group was significantly lower than in the control group (6.8 +/- 1.6 cm/s vs 10.2 +/- 2.5 cm/s, P <.01), did not show significant respiratory variation, and increased significantly after pericardiocentesis (6.8 +/- 1.6 cm/s vs 9.5 +/- 3.0 cm/s, P <.05). CONCLUSION: E' is exaggerated in CP, which is helpful for diagnosis. The opposite phenomenon was noted in Tamp, a dissimilarity that might contribute to different hemodynamics.

Adult↗

PubMed2004

Cardiomyopathies: is it time for a molecular classification?

Primary myocardial diseases have always attracted the interest of the scientific community because of their obscure aetiopathogenesis. For years there was a confusion and controversy over their definition and classification. The 1995 WHO classification led to major advancements such as the introduction of a unified terminology, the official recognition of novel entities (arrhythmogenic right ventricular and idiopathic restrictive cardiomyopathies) and the definitive clarification that inflammatory heart disease has to be regarded as a cardiomyopathy. However, according to the new definition of cardiomyopathies as diseases of the myocardium associated with cardiac dysfunction, they should include not only forms with depressed contractility and impaired diastolic function, but also conduction and rhythm disturbances and enhanced arrhythmogenicity. Moreover, the recent development of molecular genetics, with the discovery of a genetic background in several forms previously defined of unknown origin, raises the need of a debate on a possible classification based on genomics.

Cardiomyopathies↗

PubMed2006

Molecular genetics of sudden cardiac death in small animals - a review.

Sudden cardiac death in small animals is uncommon but often occurs due to cardiac conduction defects or myocardial diseases. Primary cardiac conduction defects are mainly caused by mutations in genes involved in impulse conduction processes (e.g., gap-junction genes and transcription factors) or repolarisation processes (e.g., ion-channel genes), whereas primary cardiomyopathies are mainly caused by defective force generation or force transmission due to gene mutations in either sarcomeric or cytoskeleton proteins. Although over 50 genes have been identified in humans directly or indirectly related to sudden cardiac death, no genetic aetiologies have been identified in small animals. Sudden cardiac deaths have been also reported in German Shepherds and Boxers. A better understanding of molecular genetic aetiologies for sudden cardiac death will be required for future study toward unveiling aetiology in sudden cardiac death in small animals.

Animals↗

PubMed1987

Noninvasive assessment of left ventricular diastolic function by pulsed Doppler echocardiography in patients with hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy is a primary myocardial disease in which symptoms may frequently result from impaired left ventricular relaxation, filling and compliance. In the present investigation, Doppler echocardiography was utilized to measure transmitral flow velocity and thereby assess left ventricular diastolic performance noninvasively in a group of 111 patients representative of the broad clinical spectrum of hypertrophic cardiomyopathy. In patients with hypertrophic cardiomyopathy, all Doppler indexes of diastolic relaxation and filling differed significantly (p less than 0.001) from those obtained in 86 control subjects without heart disease, namely, prolongation of isovolumic relaxation (94 +/- 24 versus 78 +/- 12 ms) and of the early diastolic peak of flow velocity (244 +/- 55 versus 220 +/- 28 ms), as well as slower deceleration (3.4 +/- 1.4 versus 4.9 +/- 1.3 m/s2) and reduced maximal flow velocity in early diastole (0.5 +/- 0.2 versus 0.6 +/- 0.1 m/s). As an apparent compensation for impaired relaxation and early diastolic filling, the atrial contribution to left ventricular filling was increased, as shown by increased late diastolic flow velocity (0.4 +/- 0.3 versus 0.3 +/- 0.1 m/s) and reduced ratio of maximal flow velocity in early diastole to that in late diastole (1.4 +/- 0.8 versus 2.1 +/- 0.9). The vast majority of patients with hypertrophic cardiomyopathy (91 [82%] of 111) showed evidence of impaired left ventricular diastolic performance, as assessed from the Doppler waveform. Abnormal Doppler diastolic indexes were identified with similar frequency in patients with (78%) or without (83%) left ventricular outflow obstruction, as well as in patients with (84%) or without (80%) cardiac symptoms. However, patients with nonobstructive hypertrophic cardiomyopathy showed more severe alterations in the Doppler indexes of diastolic function than did patients with obstruction. Thus, abnormal diastolic performance as assessed by Doppler echocardiography was apparent in the vast majority of the study patients with hypertrophic cardiomyopathy, independent of the presence or absence of cardiac symptoms or a subaortic pressure gradient. The high frequency with which diastolic abnormalities are identified in asymptomatic patients with hypertrophic cardiomyopathy suggests that impaired diastolic performance may be present at a time in the natural history of the disease when functional limitation is not yet evident.

Adolescent↗

PubMed1999

Upregulated expression of cardiac endothelin-1 participates in myocardial cell growth in Bio14.6 Syrian cardiomyopathic hamsters.

OBJECTIVES: The purpose of this study is to investigate the role of endogenous endothelin-1 (ET-1) in myocardial growth in Bio 14.6 Syrian cardiomyopathic hamsters (Bio). BACKGROUND: While ET-1, as a growth-promoting peptide, has been implicated in the development of secondary cardiac hypertrophy, the role of endogenous ET-1 in cardiac growth in primary myocardial disease is unknown. METHODS: We measured left ventricular ET-1 levels by a specific sandwich enzyme-linked immunosorbent assay. Furthermore, we examined the chronic effect of T0201, an ET type A receptor-specific antagonist. RESULTS: The ET-1 levels in the left ventricles were 1.8-fold higher (p < 0.0005) at 20 weeks and 6.4-fold higher (p < 0.0001) at 35 weeks in the Bio compared to age-matched control F1B hamsters (F1B). The Bio ET-1 levels in the lungs exhibited an only 1.3-fold elevation at 35 weeks. Immunohistochemistry demonstrated the localization of ET-1 mainly in the cardiac myocytes. The treatment with T0201 significantly reduced the heart weight/body weight ratio in the Bio, but did not affect the heart weight/body weight ratio in the F1B. Histologically, T0201 reduced the myocyte diameter of Bio to a level similar with that of F1B. However, T0201 did not affect the extent of fibrosis in Bio or F1B. CONCLUSIONS: The ET-1 level in the heart of cardiomyopathic hamsters increases in stage-dependent and organ-specific manners. Though myocyte degeneration and subsequent replacement fibrosis do not require an ET-1 pathway, the accelerated synthesis of ET-1 in the heart may contribute to the pathological growth of remaining myocytes in this animal model.

Animals↗

PubMed1998

Predictors of myocardial dysfunction in human immunodeficiency virus-infected patients.

BACKGROUND: Some of the most frequent manifestations of heart involvement in human immunodeficiency virus (HIV) infection include right and left ventricular dysfunction. The pathogenesis remains obscure. METHODS AND RESULTS: This prospective clinical and echocardiographic study involved 181 patients at all stages of HIV infection. We tested a set of clinical variables using a backward logistic regression model to assess their ability to independently predict the presence of ventricular dysfunction. The presence of pulmonary infections (all etiologies mixed) was the only variable independently associated with isolated right ventricular dysfunction (odds ratio = 4.08; P = .02). Signs suggestive of pulmonary arterial hypertension were present in 71% of the patients with right ventricular dilation. History of previous opportunistic infections (all etiologies mixed) (odds ratio = 10.9; P = .0026) and time since the diagnosis of acquired immunodeficiency syndrome more than 12 months (odds ratio = 6.6; P = .03) were the only two independent predictors of left ventricular dysfunction. CONCLUSIONS: Isolated right ventricular dysfunction may be secondary to pulmonary hypertension caused by repetitive pulmonary infections and not to primary myocardial disease. The aggressive treatment of opportunistic infections may become an important element of heart failure prophylaxis in HIV infection because they may be associated with left ventricular dysfunction.

Acquired Immunodeficiency Syndrome↗

PubMed2004

Transcription factor AP-2alpha triggers apoptosis in cardiac myocytes.

Idiopathic-dilated cardiomyopathy (IDC) is a common primary myocardial disease of unknown etiology associated with apoptosis, cardiac dilatation, progressive heart failure and increased mortality. An elevation of the transcription factor activator protein 2alpha (AP-2alpha) is involved in vertebrate embryonic development and oncogenesis. Here, we show that AP-2alpha protein is expressed in the human heart and increased in human failing myocardium with IDC. Adenovirus-mediated overexpression of human AP-2alpha triggered apoptosis and increased mRNA levels of Bcl-2 family members Bax and Bcl-x in rat cardiomyocytes. Immunohistological analysis of human myocardium revealed an increased percentage of AP-2alpha-positive nuclei in IDC and, interestingly, a colocalization of AP-2alpha-positive but not -negative cells with a caspase-cleaved fragment of poly(ADP-ribose)polymerase. We suggest AP-2alpha as a novel cardiac regulator implicated in the activation of apoptosis in IDC.

Adenoviridae↗