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PubMed1990

An autopsy case of cardiomyopathy with restrictive physiology in a child.

An autopsy case of cardiomyopathy with restrictive physiology associated with subendocardial myocardial necrosis and fibrosis in a seven-year-old child is described. Cardiac catheterization showed high left ventricular end-diastolic pressure with a dip-and-plateau pattern. Macroscopically, marked dilatation with fibroelastosis in both atria and mild dilatation in both ventricles were observed. Histologic examination revealed extensive subendocardial necrosis with marked disorganization of myocardial cells and moderate arteriolosclerosis. We speculate that these extensive myocardial lesions, which contributed to the restrictive hemodynamic changes, could have been induced by anoxia or a Ca2+ transport abnormality of unknown cause.

Cardiomyopathy, Restrictive↗

PubMed1987

Cardiac and skeletal muscle abnormalities in cardiomyopathy: comparison of patients with ventricular tachycardia or congestive heart failure.

Results of cardiac muscle and skeletal muscle biopsies were compared in 22 patients with cardiomyopathy; 11 patients presented with symptoms secondary to ventricular tachycardia (Group 1) and 11 had symptoms of severe congestive heart failure (Group 2). No patient had structural or ischemic cardiac disease. In Group 1 patients, hemodynamic abnormalities were subtle, but invasive study demonstrated dilated cardiomyopathy in two patients and restrictive cardiomyopathy in nine. In Group 2, eight patients had dilated cardiomyopathy and three had restrictive cardiomyopathy. Cardiac biopsy results were abnormal in all 22 patients and the abnormalities were similar for the two groups. Cardiac histologic study revealed a spectrum of abnormalities including fibrosis, dilated sarcoplasmic reticulum, increased numbers of intercalated discs and mitochondrial abnormalities. Histologic abnormalities of skeletal muscle were similar in each group, consisting of endomysial fibrosis and increased lipid deposits. Slightly more than half of the Group 1 and Group 2 patients also had a low concentration of skeletal muscle long chain acylcarnitine. These data demonstrate that abnormalities of both cardiac and skeletal muscle are common in patients with cardiomyopathy; abnormalities are similar whether initial symptoms are due to ventricular tachycardia or congestive heart failure. It is suggested that these patients with cardiomyopathy may have a generalized myopathy.

Adolescent↗

PubMed2002

Epidemiology of cardiomyopathies in children and adolescents: a retrospective study over the last 10 years.

We conducted a retrospective study at the Department of Paediatric Cardiology of the University Hospital Centre Rebro, Zagreb, over the period from 1988 to 1998, so as to assess the epidemiology of childhood cardiomyopathies. The patients were categorized according to the guidelines of the Task Force on Cardiomyopathies of the World Health Organization and the International Society and Federation of Cardiology. We identified 121 infants, children and adolescents as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10,000 patients examined in our outpatient clinics for paediatric cardiology. Of the patients, 50 were female (41.3%) and 71 were male (58.7%). The cardiomyopathy was of the dilated variant in 52 patients (42.9%), with 43 patients (35.5%) having hypertrophic cardiomyopathy, and 6 patients (4.8%) identified with restrictive cardiomyopathy. We encountered no patients with arrhythmogenic right ventricular cardiomyopathy. In nine patients (7.4%), it proved impossible to classify the cardiomyopathy. We placed 11 patients (9.0%) in the group of specific cardiomyopathies. Most of those with dilated cardiomyopathy had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4-2.47). There were no statistically significant differences in the incidences of dilated as compared to hypertrophic cardiomyopathy (Z 0.923, p = 0.1779), but we encountered a significantly lower occurrence of restrictive cardiomyopathy (Z 6.044, p < 0.001). Of those with hypertrophic cardiomyopathy, 15 patients (34.8%) had the asymmetric variant, while 28 patients (65.2%) exhibited the concentric form. During the period of follow-up, 10 patients died, 4 with dilated cardiomyopathy, 4 with hypertrophic cardiomyopathy, 1 with restrictive cardiomyopathy, and 1 with a specific cardiomyopathy. We encountered 12 (9.9%) patients who, besides cardiomyopathies, also suffered from neuromuscular disorders. Most of these had dilated cardiomyopathy. Mitochondrial disorders, in contrast, were more frequently found in patients with hypertrophic cardiomyopathy.

Adolescent↗

PubMed1991

Cardiomyopathy: a necessary revision of the WHO classification.

The classification of myocardial disease proposed by the WHO/ISFC task force in 1980 distinguishes specific heart muscle diseases from myocardial diseases of unknown origin, termed cardiomyopathies, and differentiated into the dilated, hypertrophic and restrictive forms. This last group includes endomyocardiofibrosis and fibroblastic parietal endocarditis. In more recent years, two new forms of heart muscle disease have been recognized: so-called "primary" restrictive cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy. Primary restrictive cardiomyopathy is characterized anatomically by normally sized, non-hypertrophic ventricles with dilated atria, and functionally by impaired diastolic compliance due to myocardial stiffness. The clinical picture is that of chronic congestive heart failure; histology shows interstitial fibrosis and myocardial disarray, but not hypereosinophilia. In arrhythmogenic right ventricular cardiomyopathy, the myocardium of the right ventricular free wall is substituted by fibrous and/or adipose tissue, which results in regional dynamic alterations and ominous ventricular arrhythmias. The left ventricle is usually spared. Both forms should be classified as heart muscle diseases of unknown origin, and kept clearly distinct from the other cardiomyopathies listed in the WHO classification.

Arrhythmias, Cardiac↗

PubMed1983

Cardiomyopathy: how far have we come in 25 years, how far yet to go?

Twenty-five years ago clinical investigators began to appreciate that cardiomyopathy is an important and reasonably common form of heart disease. Since then, several functional classifications have been proposed, the specific myocardial diseases have been classified and chronic ischemic ventricular failure has been described. The boundary separating myocarditis from dilated cardiomyopathy remains hazy and, despite intensive research, the causes of dilated cardiomyopathy remain obscure. In particular, we still do not understand the role that may be played by viral infection and alcohol. Myocardial biopsy has proved useful in patients with specific myocardial disorders, heart transplant recipients and patients receiving Adriamycin, but is disappointing in patients with dilated cardiomyopathy. It has become increasingly evident that exercise capacity does not correlate with ventricular function, being highly dependent on peripheral factors. Measurements of oxygen consumption during exercise promise to be useful in assessing treatment of dilated cardiomyopathy. True restrictive cardiomyopathy is uncommon, and the term should be reserved for cardiomyopathies that meet strict criteria. A restrictive component to filling is common to many cardiac disorders, including some cases of cardiac amyloidosis. The concept of hypertrophic cardiomyopathy has evolved rapidly over the past 25 years, and continues to evolve. The importance of arrhythmia as a cause of sudden death is becoming increasingly clear. The place of calcium channel blocking agents in the treatment of hypertrophic cardiomyopathy will probably emerge soon. Amiodarone is finding an increasing role in the treatment of dilated and hypertrophic cardiomyopathy. Surgical treatment is still required for some patients despite unanswered questions on how it works.

Arrhythmias, Cardiac↗

PubMed1988

[Diastolic opening of the pulmonary valve in a case of cardiomyopathy with restrictive dysfunction. Case report and review of the literature].

In a case of cardiomyopathy with restrictive ventricular dysfunction a complete diastolic opening of the pulmonary valve immediately following the atrial contraction could be visualized by echocardiography and at the same time a forward blood flow into the pulmonary artery could be shown by conventional and color Doppler. The velocity of this flow (0.37 m/s) was as high as the maximal velocity of the systolic outflow. As an expression of the unpaired compliance of the right ventricle and a compensatory hyperkinetic right atrium the heart catheterization revealed a high a-wave of 18 mm Hg, which was transmitted into the right ventricle and the pulmonary artery. This produced a short diastolic pressure drop between the right ventricle and pulmonary artery causing an opening of the pulmonary valve which could be shown by echocardiography. In connection with similar cases in the literature the pathophysiology of this phenomenon is discussed.

Adult↗

PubMed1982

[The electrocardiogram in myocardial diseases].

From 1970 to 1980 75 patients with dilatative, 51 patients with hypertrophic, and 8 patients with restrictive cardiomyopathy (endomyocardial fibrosis) have been investigated. The routine electrocardiogram showed premature ventricular beats in patients with dilatative (53%) and hypertrophic (59%) cardiomyopathy. Atrial fibrillation was observed in 16% of the patients with dilatative and 37% of the patients with restrictive cardiomyopathy. Left axis deviation was seen in dilatative (45%) cardiomyopathy and right axis deviation in restrictive (37%) cardiomyopathy. A left bundle branch block was seen in 33% of the patients with dilatative cardiomyopathy and left ventricular hypertrophy in 55% of the patients with dilatative and 67% of the patients with hypertrophic cardiomyopathy. Left and right atrial hypertrophy was observed in patients with restrictive cardiomyopathy (50%) and left atrial hypertrophy in patients with dilatative cardiomyopathy (40%). It is concluded that the electrocardiogram in patients with dilatative cardiomyopathy is characterized by premature ventricular beats, left ventricular and left atrial hypertrophy and a left bundle branch block. Hypertrophic cardiomyopathy is characterized by left ventricular hypertrophy with negative T-waves and premature ventricular beats, and restrictive cardiomyopathy by atrial fibrillation, right axis deviation and left and right atrial hypertrophy.

Adult↗

PubMed1985

Surgical treatment of constrictive pericarditis: analysis of outcome and diagnostic error.

The records of 81 patients with a diagnosis of constrictive pericarditis who underwent surgical treatment were examined to assess the effectiveness of diagnosis and therapy. A false-positive diagnosis occurred in 10 patients (12%); seven had restrictive cardiomyopathy. Of 51 variables examined, only a low right ventricular end-diastolic pressure (RVEDP) significantly and independently predicted diagnostic error. Seventy-one patients with constrictive pericarditis underwent pericardiectomy. Mean follow-up was 4.7 years (maximum 12), and only two patients were untraceable. The study population was notable: 42 patients had visceral as well as parietal pericardial involvement; 32 had idiopathic disease and 25 had pericarditis related to radiation therapy. Results were favorable in 83% of the population. There were seven in-hospital deaths (10%). Actuarial survival estimates were 74% and 64% at 5 and 10 years, respectively. Compared with a normal population, the survival rate of patients with postradiation constrictive pericarditis was significantly inferior, whereas the survival rate of the remaining patients was not significantly different. Patients with constrictive pericarditis and restrictive cardiomyopathy did no better than those with restrictive cardiomyopathy alone. Additionally, patients in NYHA functional class IV had a significantly worse prognosis. Multivariate analysis of 38 preoperative variables identified high RVEDP as a significant independent predictor of in-hospital death, and renal dysfunction and diuretic use were significant independent predictors of overall poor outcome. Differentiation between the diagnosis of constrictive pericarditis and restrictive cardiomyopathy remains a problem. Radiation therapy, pericarditis with restrictive cardiomyopathy, high RVEDP, NYHA class IV status, renal dysfunction, and diuretic use adversely influenced outcome in patients undergoing pericardiectomy.

Actuarial Analysis↗

PubMed1994

Left atrial dilatation in constrictive pericarditis: a pre and post-operative echocardiographic study.

Thirty-three surgically proven cases of constrictive pericarditis were studied pre-operatively by echocardiography for left atrial dilatation and 18 of them underwent post-operative study at a mean follow-up period of 229 +/- 105 days. The degree of left atrial dilatation in these patients was compared with patients of restrictive cardiomyopathy (n = 8) and sex matched controls of similar age (n = 33). Significant left atrial dilatation was present in patients of constrictive pericarditis compared to controls and it was of a similar or greater degree compared to patients of restrictive cardiomyopathy (the left atrium to aorta ratio was 1.7 +/- 0.31, 1.53 +/- 0.18 and 1.07 +/- 0.1, in constrictive pericarditis, restrictive cardiomyopathy and controls, respectively, P = NS for constrictive pericarditis vs. restrictive cardiomyopathy and P < 0.001 for constrictive pericarditis vs. controls). There was no correlation of degree of left atrial dilatation with clinical and hemodynamic variables. Postoperatively, there was regression of left atrial size in patients with normal hemodynamics (n = 12, pre- vs. post-operative left atrium to aorta ratio 1.65 +/- 0.23 vs. 1.32 +/- 0.14, P < or = 0.001) and persisting or increasing left atrial dilatation in patients with persisting hemodynamic abnormality (n = 6, left atrium to aorta ratio 1.66 +/- 0.23 vs. 1.82 +/- 0.15, P = NS). We conclude that significant left atrial dilatation is a consistent echocardiographic feature of constrictive pericarditis. Hemodynamic normalization following successful pericardiectomy is associated with regression of atrial size.

Adolescent↗

PubMed2006

Amyloidosis and the heart: a comprehensive review.

Infiltration of the heart from insoluble protein deposits in amyloidosis often results in restrictive cardiomyopathy that manifests late in its course with heart failure and conduction abnormalities. While the rare primary amyloidosis-related heart disease has been well characterized, senile amyloidosis occurring in the seventh decade of life most frequently affects the heart. Early diagnosis of cardiac amyloidosis may improve outcomes but requires heightened suspicion and a systematic clinical approach to evaluation. Demonstration of tissue infiltration of biopsy specimens using special stains, followed by immunohistochemical studies and genetic testing, is essential in defining the specific protein involved. The therapeutic strategy depends on the characterization of the type of amyloid protein and extent of disease and may include chemotherapy, stem cell transplantation, and liver transplantation. Heart transplantation is controversial and is generally performed only at isolated centers.

Amyloidosis↗

PubMedDate not supplied

Isolated noncompaction of the ventricular myocardium.

Isolated noncompaction of the ventricular myocardium is a recently described anomaly. We report the first case of this anomaly presenting as a restrictive cardiomyopathy, and the first association of this entity with endocardial fibrosis.

Cardiomyopathy, Restrictive↗

PubMed2005

The enlarging spectrum of desminopathies: new morphological findings, eastward geographic spread, novel exon 3 desmin mutation.

A 52-year-old man, who had developed distal muscle weakness in legs and arms, was found to have distal muscle atrophy as well as cardiac arrhythmia. His 10-year younger brother developed restrictive cardiomyopathy at the age of 20 years, which required cardiac transplantation at the age of 41 years. Skeletal muscle biopsy specimens of the older brother revealed granulofilamentous material and plaques containing numerous proteins, foremost desmin, as did cardiac biopsy tissue. The explanted heart of the younger brother showed similar protein-rich plaques and granulofilamentous material within cardiac myocytes. A novel heterozygous Glu245Asp (E245D) missense mutation in exon 3 of the desmin gene (DES) at 2q35 was found in the older brother. While clinical data and muscle biopsy pathology of the older brother conform to the nosological spectrum of desminopathies, the early-onset cardiomyopathy, a similar cardiac pathology as in skeletal muscle tissues and a novel missense mutation in the DES gene, enlarge the nosological spectrum of desminopathies.

Adult↗

PubMed1985

Mechanisms in cardiomyopathies.

The three main clinical types of cardiomyopathy are: hypertrophic cardiomyopathy; dilated cardiomyopathy; and restrictive cardiomyopathy. In each case the basic cellular mechanisms still remain to be defined.

Adrenergic beta-Antagonists↗

PubMed1985

Unsuspected amyloidosis and cardiac arrest following mitral valve replacement.

We performed an autopsy on a 58-year-old female with previously unsuspected cardiac amyloidosis. One day prior to expiration the patient underwent a mitral valve replacement with a Bjork-Shiley prosthesis for mitral valve regurgitation. The valvular defect along with the massive myocardial amyloidosis which created a restrictive cardiomyopathy, were the etiology of the patient's poor cardiac output and subsequent demise.

Amyloidosis↗

PubMed2006

A point mutation (R192H) in the C-terminus of human cardiac troponin I causes diastolic dysfunction in transgenic mice.

Cardiac troponin I (cTnI) mutations have been linked to the development of restrictive cardiomyopathy (RCM) in human patients. We modeled one mutation in human cTnI C-terminus, arginine192-->histidine (R192H) by cardiac specific expression of the mutated protein (cTnI(193His) in mouse sequence) in transgenic mice. Heart tissue sections revealed neither significant hypertrophy nor ventricular dilation in cTnI(193His) mice. The main functional alteration detected in cTnI(193His) mice by ultrasound cardiac imaging examinations was impaired cardiac relaxation manifested by a decreased left ventricular end diastolic dimension (LVEDD) and an increased end diastolic dimension in both atria. The cardiac ejection fraction (EF) was not significant changed in 6- to 8-week-old cTnI(193His) mice, however, the EF was significantly decreased in cTnI(193His) mice at age of 11 months. These data indicate that individual genetic conditions and environmental factors participate together in the development of the cTnI mutation based-cardiac muscle disorders. This mouse model provides us with a tool to further investigate the pathophysiology and the development of RCM.

Animals↗

PubMed2006

[Original case report of amyloidosis with pleural involvement: the role of echocardiography in the diagnosis].

INTRODUCTION: Amyloidosis is often difficult to diagnose and cardiac involvement worsens the prognosis. CLINICAL CASE: We report the case of a 72-year old man consulting for cardiac failure with pleural effusion. A restrictive cardiomyopathy was discovered by echocardiography, and amyloidosis was then suspected. First histological localization was pleural. Cardiac involvement was confirmed. The diagnosis was supported by digestive and cutaneous localizations. It was an AL amyloidosis. Treatment with melphalan and dexamethasone allowed stabilization during more than six months. DISCUSSION: This is an original case report, because of the first clinical signs (cardiac failure), the histological proof (pleural histology). Echocardiography is particularly helpful in internal medicine.

Aged↗

PubMed1999

Constrictive pericarditis: clinical and pathophysiologic characteristics.

Constrictive pericarditis is an uncommon disorder with various causes. Although most often idiopathic, it may also occur after cardiovascular surgery, radiation therapy, and tuberculosis, especially in developing countries. The encasement of the heart by a rigid, nonpliable pericardium results in characteristic pathophysiologic effects, including impaired diastolic filling of the ventricles, exaggerated ventricular interdependence, and dissociation of intracardiac and intrathoracic pressures during respiration. Constrictive pericarditis typically presents with chronic insidious signs and symptoms of predominantly systemic venous congestion. Notoriously difficult to diagnose and distinguish from restrictive cardiomyopathy (RCM), the use of cardiac catheterization, echocardiography (transthoracic and transesophageal), central venous (hepatic and pulmonary) and transvalvular Doppler measurements, and magnetic resonance imaging should secure the diagnosis in most cases, eliminating the need for diagnostic thoracotomy. Although medical treatment may temporarily alleviate symptoms of heart failure, patients do poorly without pericardiectomy.

Animals↗

PubMed1998

The role of cytoskeletal proteins in cardiomyopathies.

Cardiomyopathies are serious heart muscle disorders in children and adults, which result in morbidity and premature death. These disorders include hypertrophic cardiomyopathy, dilated cardiomyopathy and restrictive cardiomyopathy. Recently, mutations in seven genes, all encoding sarcomeric proteins, have been identified as causes of familial hypertrophic cardiomyopathy. The genes include those encoding the beta-myosin heavy chain, alpha-tropomyosin, cardiac troponin T, myosin binding protein-C, myosin essential light chain, myosin regulatory light chain, and troponin I. Advances in the understanding of dilated cardiomyopathy have been made recently as well and it appears as if cytoskeletal proteins play a central role. Dystrophin has been identified as the gene responsible for X-linked dilated cardiomyopathy and this protein, which is also responsible for Duchenne and Becker muscular dystrophy, plays an important role in myocyte and cardiomyocyte function. Mutations in other cytoskeletal proteins such as metavinculin, alpha-dystroglycan, alpha- and gamma-sarcoglycan, and muscle LIM protein have also been found to result in dilated cardiomyopathy, suggesting that cytoskeletal proteins play a central role in cardiac function.

Cardiomyopathies↗