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PubMed1987

Cardiomyopathies in the elderly.

Cardiomyopathies in the elderly have certain characteristic features. The dilated form appears to be less common than in younger patients. Hypertrophic cardiomyopathy is more often associated with severe and concentric hypertrophy. The prognosis of hypertrophic cardiomyopathy seems to be better in the elderly, because they appear to have a lower incidence of sudden death. Restrictive cardiomyopathies are not common in the elderly, and senile amyloid heart disease rarely, if ever, results in congestive heart failure. A syndrome of clinical heart failure with reduced diastolic compliance and preserved systolic function is more common in elderly patients.

Aged↗
PubMed1998

[Unclassified cardiomyopathies: subspecies and their transformation].

Some cases with hypertrophic cardiomyopathy (HCM) progress to dilated cardiomyopathy (DCM), therefore, we hypothesized that a transforming-type phase between HCM and DCM could exist. This study was made based on a retrospective analysis of 471 of 1,388 patients with cardiomyopathy who underwent diagnostic myocardial biopsy in several hospitals between 1977 to 1995, and who were not diagnosed with restrictive cardiomyopathy, arrhythmogenic right ventricular dysplasia, specific heart muscle diseases, or electrical disturbance type of heart muscle diseases. Based on echocardiographic measurements, the 471 patients were classified into eight subgroups according to the presence or absence of three parameters. A: left ventricular hypertrophy (septal or posterior wall thickness > 12 mm), B: left ventricular dilation (left ventricular end-diastolic dimension > 55 mm), C: systolic dysfunction (left ventricular ejection fraction < 50%), is signified by plus or minus ([ABC]). HCM, DCM and normal heart are defined as [+(-)-], [-(+2)] and [-(-)-], respectively. Unclassified cardiomyopathy (UCM) was indicated as cardiomyopathy not diagnosed as HCM or DCM. Therefore, unclassified cardiomyopathies are signified as either [+2-], [+(-)+], [+3], [-(+)-] or [-(-)+]. Patients in each subgroup of UCM were followed up for 6.4 +/- 6.0 years and their clinical courses compared with the histological findings. Of the 471 patients, 111 (24%) were classified as UCM, 240 as HCM, and 120 as DCM. Severe myocardial disarray was noted more frequently in UCM [17 of 111 cases (15%)] than in DCM [7 of 120 cases (6%)] (p < 0.05), and not significantly higher than in HCM [34 of 240 cases (14%)]. Patients with UCM whose conditions deteriorated had positive pathological findings (15 of 26 cases) more often than those without deterioration (8 of 29 cases; p < 0.05). UCM could be a transforming type of cardiomyopathy for some patients with HCM who progress to DCM. In addition, there may be a positive correlation between the histopathologic findings and the clinical course.

Cardiomyopathies↗
PubMed1979

OBLITERATIVE RESTRICTIVE ENDOMYOCARDIAL FIBROSIS: A SURGICAL APPROACH.

Restrictive cardiomyopathies have been variously classified and interpreted. Although their etiologic and anatomic features may vary, their common denominator is indicated by restrictive hemodynamic behavior of either the left or right ventricle or both. This report describes a case in which such restriction was caused by obliterative endomyocardial fibrosis of unknown etiology. Because of significant symptomatology, including congestive failure and syncope, the patient underwent surgical excision of the endomyocardial overgrowth.

Journal Article↗
PubMed1983

Primary cardiac amyloidosis: an angiocardiographic clue to early diagnosis.

Restrictive cardiomyopathy produced by primary cardiac amyloidosis may have a clinical syndrome identical to noncalcific constrictive pericarditis. We report two patients with amyloidosis and restrictive physiology who had enlarged papillary muscles shown on left ventricular angiocardiogram. Rapid volume expansion with normal saline failed to produce ventricular filling pressure equilibration as in occult constrictive pericarditis. The absence of diastolic pressure equilibration and enlarged papillary muscles may permit early diagnosis of cardiac amyloidosis.

Amyloidosis↗
PubMed2005

Beta-blockade intolerance in anthracycline-induced cardiomyopathy.

Beta-blockade efficiency and safety in anthracycline induced cardiomyopathy (AIC) are poorly documented. Cardiac Heart Failure (CHF) due to an AIC has haemodynamic and histologic particularities: only mild ventricular dilatation, restriction pattern and myocardial and endocardial fibrous thickening. Therefore, beta blockade therapy initiation may cause heart failure decompensation by absence of the usual left ventricular adaptation (improvement of left ventricular compliance allowing maintenance of stroke volume). We describe an AIC patient in whom a first beta-blockade initial administration caused a global cardiac failure; after stabilisation, one month later, a second attempt caused a new cardiac failure. We raise the question of beta-blockade safety in restrictive cardiomyopathies.

Adrenergic beta-Antagonists↗
PubMed1991

Cardiomyopathies in children with neuromuscular disorders.

In this paper eight patients with myogenic or neurogenic muscle disorders are presented, in whom cardiomiopathy was also found. Six patients developed a dilated cardiomiopathy associated with neurogenic atrophies or progressive muscular dystrophy. In patients with Mb. Friedreich and HSNM type II together with the total dilatation of the septum hypertrophy was observed and in patients with spinal muscular amyotrophy of scapuloperoneal type atriomyopathy dominated. In two patients with mitochondrial disorders a hypertrophic cardiomyopathy was found. One of them had mitochondrial encephalomyoneuropathy and the other mitochondrial encephalopathy with myoclonic epilepsy. In none of them a restrictive cardiomyopathy was found. From the presentation could be concluded that in neurogenic muscle diseases and progressive muscular dystrophy respectively most frequently dilated cardiomyopathies have been developed. Hypertrophic cardiomyopathies are usually found in children with mitochondrial disorders.

Adolescent↗
PubMed1982

Real time two dimensional echocardiography in constrictive pericarditis.

Real time two dimensional echocardiography was performed in five patients with constrictive pericarditis. The echocardiogram showed several typical features: (1) The heart was normal in size or decreased; the ventricles were small and the atria enlarged. (2) The pericardium was seen as a single or double dense rigid shell surrounding both ventricles and the apex; its immobility was striking. (3) The inferior vena cava and hepatic veins were greatly dilated in keeping with the severe clinical venous congestion. (4) Both interventricular and interatrial septa bulged into the left side of the heart on inspiration. (5) The atrioventricular valves were hypermobile. (6) A prominent ventricular diastolic filling half was seen. The two dimensional echocardiogram excluded other causes of severe cardiac failure such as congestive cardiomyopathy and valvular heart disease, while the dense immobile pericardial echo or echoes and the "normal" texture of the myocardial echoes made the diagnosis of restrictive cardiomyopathy unlikely.

Adolescent↗
PubMed2006

Use of denaturing high-performance liquid chromatography to detect mutations in pediatric cardiomyopathies.

This chapter describes the use of denaturing high-performance liquid chromatography as a high-throughput method to detect genetic mutations in pediatric cardiomyopathies. An overview of the classification, incidence, and etiologies of the major cardiomyopathies is provided, with emphasis on the special circumstances of the pediatric patient. During the past 15 yr, the genetic bases of inherited dilated, hypertrophic, and restrictive cardiomyopathy have been elucidated. As the list of known and candidate cardiomyopathy genes continues to grow and our ability to screen for genetic mutations improves, the cause of cardiomyopathy will be identified in a larger percentage of cases. This outcome is highly relevant to children with cardiomyopathy as well as those at risk for developing the disease because of their family history.

Cardiomyopathies↗
PubMed2002

A case of hypereosinophilic syndrome presenting mid-ventricular obstruction.

A 59-year-old man with hypereosinophilic syndrome (HES) who had been maintained with low-dose prednisolone for 5 years developed the characteristic features of hypertrophic cardiomyopathy. Left ventricular endomyocardial biopsy revealed no eosinophilic infiltration but extensive myocardial fibrosis. Cardiac involvement in HES presents as endocardial fibrosis, resulting in a clinical presentation of restrictive cardiomyopathy. HES heart disease can also present dilated cardiomyopathy, but myocardial hypertrophy has only rarely been noted in conjunction with HES. This report concerns a patient with HES who had clinical and hemodynamic evidence of asymmetric septal hypertrophy with mid-ventricular obstruction.

Cardiomyopathies↗
PubMed2001

[Diagnosis and treatment of childhood cardiomyopathies].

Cardiomyopathies have either a primary (without associated anomalies) or a secondary origin. They are classified in three groups according to their anatomy and function: hypertrophic, dilated or restrictive. We review here the relevant diagnostic points of each type as well as their treatment. Restrictive cardiomyopathies, arrhythmogenic right ventricle, non compaction and Uhl's anomaly will not be dealt with in detail as they are very seldom in children.

Cardiac Surgical Procedures↗
PubMedDate not supplied

A case of massive dystrophic cardiac calcinosis with increased bone resorption markers: a novel pathophysiologic link?

We report a 72-year-old man who presented to our emergency room with congestive heart failure that was resistant to initial medical therapy. He had suffered from anterior myocardial infarction 20 years prior. Echocardiography and computed tomography revealed marked cardiac calcification including myocardium, chordal structures, mitral annulus, and aortic valve. Neither chronic renal insufficiency nor hypercalcemia were present in our patient. Bone resorption markers were increased and bone mineral density was consistent with severe osteoporosis. We suggested a novel mechanism, that increased bone resorption may lead to accumulation of calcium into avascular tissues in the heart (ie, chordal structures, mitral annulus, aortic valve, and fibronecrotic myocardium) especially in the setting of high left ventricular end-diastolic pressure. Dystrophic cardiac calcinosis, an age-related cardiomyopathy, is associated with elevated bone resorption markers and it may cause alterations in cellular calcium hemostasis with initiation of deleterious events leading to aggravate dilated and restrictive cardiomyopathy and may result in intractable congestive heart failure. The implication of this case report needs to be reemphasized.

Aged↗
PubMed2005

[Cardiomyopathies due to defective energy metabolism: morphological and functional features].

Cardiomyopathies are defined as diseases of the myocardium associated with cardiac dysfunction and are classified by morphological characteristics as hypertrophic (HCM), dilated (DCM) arrhithmogenic right ventricular (ARVC) and restrictive cardiomyopathy. These were once considered as specific diagnoses but there is now considerable evidence that many different gene mutations can cause these pathologies. In recent years, big emphasis has been given to the possibility that deregulation of cardiac metabolism may play a role in the mechanisms that lead to cardiac maladaptive remodelling. Cardiac energy metabolism is tightly controlled in mammalian organisms during development and in response to diverse dietary, physiologic, and pathologic conditions. The cardiac phenotype of many genetic diseases caused by mutations in proteins involved in mitochondrial energy production and/or homeostasis, underscores the importance of energetic pathway on cardiac function. For example, inborn errors in nuclear-encoded mitochondrial fatty acid oxidation (FAO) pathway enzymes and defects in fatty acid uptake are an important cause of childhood HCM and sudden death. Abnormalities in mitochondrial respiratory chain function, particularly those caused by mitochondrial DNA (mtDNA) mutations, are responsible for a heterogeneous group of clinical disorders, including isolated HCM. Mitochondrial cardiomyopathies (MCM) are characterized by an adverse clinical course with biventricular dilation and failure, even at a young age. Mutations in genes encoding the gamma2 subunit of AMP-activated protein kinase (PRKAG2), alpha-galactosidase A (GLA) and lysosome-associated membrane proteine-2 (LAMP2) can cause profound myocardial hypertrophy in association with electrophysiological defects. Unlike HCM due to sarcomere gene mutations, which is characterized by myofiber disarray and fibrosis, large cytosolic vacuoles characterize cardiomyopathy due to defect in energy metabolism. Ultrastructural analysis revealed massive mitochondrial proliferation in MCM and glycogen in complexes with protein and/or lipids in cardiomyopathy due to PRKAG2, GLA and LAMP2 mutations.

Cardiomyopathies↗
PubMed2001

[Doppler myocardial velocity gradient as a new diagnostic index for the assessment of myocardial disease].

Currently, echocardiography is the technique of choice in the noninvasive assessment of left ventricular (LV) function. Unlike the assessment of LV systolic function, its role is limited in the quantification of LV diastolic function where only global LV filling can be assessed. Tissue Doppler echocardiography (TDE) is a recently introduced imaging technique which allows for direct assessment of LV myocardial contraction and relaxation. Therefore, parameters derived from TDE showed provide a more accurate assessment of LV systolic and diastolic function. Doppler myocardial velocity gradient (MVG) is a new parameter derived from TDE, which enables the measurement of the spatial distribution of transmyocardial velocities over the cardiac cycle. The most studied application of this technique relates to the earlier and more sensitive diagnosis of different forms of cardiomyopathy. Based on the Doppler MVG measurement, patients with ischaemic cardiomyopathy can be differentiated from those with idiopathic dilated cardiomyopathy. In both diseases MVG is reduced in systole, but as opposed to dilated cardiomyopathy, MVG in patients with ischaemic cardiomyopathy is also markedly reduced in early diastole. MVG derived from TDE also provides new diagnostic insights into genetically determined heart muscle diseases. It has been shown that an early diastolic MVG < or = 7 s-1 is characteristic for patients with hypertrophic cardiomyopathy and differentiates this group of patients from 'athletes' heart, independently of the degree of LV hypertrophy. A recent study has also shown that in Friedreich ataxia related cardiomyopathy there is a close relation between the degree of genotype abnormalities and the degree of reduction in the MVG. Also, the assessment of Doppler MVG enables the differentiation of restrictive cardiomyopathy from constrictive pericarditis. Based on available literature it appears that TDE derived MVG enhances the information available from conventional echocardiography and helps to establish an earlier diagnosis in patients with primary and secondary myocardial diseases.

Cardiomyopathies↗
PubMed2003

[Usefulness of magnetic resonance imaging in the assessment of endomyocardial disease].

Endomyocardial disease is a restrictive cardiomyopathy that includes Löffler endocarditis, which is characterized by hypereosinophilia, and endomyocardial fibrosis, which is not. Echocardiography enables cardiac function and anatomy to be assessed and the differential diagnosis of other causes of restrictive disease, but magnetic resonance imaging provides information about the tissue itself. Furthermore, paramagnetic contrast agents are useful in detecting myocardial abnormalities. We report three cases of endomyocardial disease and the typical findings of magnetic resonance imaging.

Adolescent↗
PubMedDate not supplied

[Churg-Strauss syndrome in a 15-year old boy].

A 15 year-old boy was admitted in our hospital with the clinical signs of cardiopulmonary deterioration. His history showed bronchial asthma since 3 years. Furthermore he revealed signs of polyneuropathy, exanthema and a transitory pulmonary infiltration. Elevation of eosinophilic granulocytes and IgE confirmed the diagnosis of a Churg-Strauss syndrome. A rapid improvement of the symptoms was achieved by high-dose steroids and symptomatic therapy. The impaired systolic and diastolic myocardial function improved, but a cardiomyopathy with a shortening fraction below 25% persisted. The Churg-Strauss syndrome is a rare disease in childhood and is classified as a systemic granulomatosis of the vessels. The marked perimyocarditis with consecutive restrictive cardiomyopathy is a rare manifestation in childhood and adolescence. The prognosis of this disease is mainly influenced by the cardiac involvement.

Adolescent↗
PubMed1976

[Alcoholic Cardiomyopathy (author's transl)].

Alcoholic cardiomyopathy is a consequence of toxic effects of ethyl alcohol. Acute effects must be distinguished from chronic effects over many years. Chronic abuse of alcohol of 1.5-2 g ethyl alcohol per kg body weight (i.e. about 100-150 g/70 kg) per day for years can cause congestive cardiomyopathy in predisposed persons, usually between 30 and 50 years of age. The diagnosis is associated with some criteria for exclusion, i.e. coronary heart disease, hypertension, valvular heart disease, in addition all obstructive and restrictive cardiomyopathy must be excluded. On the other hand, a specific constellation of findings can be considered characteristic of alcoholic cardiomyopathy, namely the coincidence of a radiologically established cardiomegaly in the form of a congestive cardiomyopathy with a raised serum concentration of immunoglobulin A and a negative myocardial immunofluorescence test. Therapeutically, in addition to the classical principles of the treatment of heart failure, absolute abstention from alcohol and physical stress seemed to be effective.

Acute Disease↗
PubMed1975

Cardiac amyloidosis. Diagnosis by transvenous endomyocardial biopsy.

Endomyocardial tissue, obtained from two patients presenting with restrictive cardiomyopathies, demonstrated amyloid infiltration. The percutaneous transvenous cardiac biopsy technic, using a modified Konno-Sakakibara cardiac bioptome, was safe and quick. Physical examination and catheterization data may not provide a definite differential diagnosis between restrictive and constrictive myocardial disease. Confirmation by biopsy of the cardiac amyloidosis assisted in providing optimum diagnostic and therapeutic care for these patients.

Aged↗
PubMed1990

Diabetic cardiomyopathy.

The purpose of this article was to review the clinical and experimental features of diabetic cardiomyopathy, with particular relevance to the Black population. One hundred thirty-seven studies were identified, of which 57 were selected as references for this article. Diabetes is associated with the development of cardiomyopathy, independent of coronary atherosclerosis. Pathological studies show myocardial hypertrophy and fibrosis; microvascular pathology is also present, but all of these pathological findings have an uncertain relationship to myocardial failure. Hemodynamic findings of both congestive and restrictive cardiomyopathy have been described. Noninvasive studies revealed abnormal systolic and diastolic function in many diabetic subjects, particularly in the presence of diabetic complications and/or hypertension. Experimental studies have focused on the mildly diabetic dog and the severely diabetic rat. One year of diabetes in dogs resulted in decreased left ventricular compliance and increased interstitial connective tissue. Studies in the diabetic rat showed a marked slowing of contraction and relaxation. Chronic insulin therapy reversed the changes in the rat model. Combining hypertension with diabetes in the rat resulted in increased myocardial and coronary microvascular pathology and greater changes in isolated muscle function, electrophysiology, and contractile protein biochemistry. Many hypertensive diabetic rats died spontaneously, showing signs of congestive heart failure. Diabetic cardiomyopathy is a significant cause of heart failure in diabetic subjects and occurs more frequently in those with microvascular complications and/or hypertension. Clinical studies are needed to clarify the natural history of this disorder, focusing on the benefits of tight control of hyperglycemia and treatment of associated hypertension. Experimental studies will clarify the pathophysiology and contribute to improved therapy. The high prevalence of diabetes and hypertension in Blacks makes these considerations especially relevant to this population.

Animals↗