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PubMed1980

Cardiomyopathies characterized by evidence of resistance to left ventricular inflow.

Restrictive cardiomyopathy characterized by evidence of left ventricular inflow obstruction was studied in four cases--one child and three adults. In three of the cases, including the child, there was pathologic evidence of hypertrophic cardiomyopathy with asymmetric septal hypertrophy. In the fourth case, no cardiac disease was identified as the anatomic basis for the inflow restriction. This latter case broadens the concept of restrictive cardiomyopathies.

Adult↗
PubMed1992

HLA gene analysis in a Japanese family with hypertrophic cardiomyopathy by restriction fragment length polymorphism.

A large Japanese family with hypertrophic cardiomyopathy (HCM) was examined (n = 61). Ten of 14 affected family members who showed HCM on the basis of electrocardiography and two-dimensional echocardiography were subjected to human leukocyte antigen (HL-A)-A,B,C typing by the lymphocyte cytotoxicity test and D, DR typing by restriction fragment length polymorphism (RFLP). As control, the HLA genotype of 14 non-affected family members was analyzed. HLA typing of the affected subjects with HCM revealed a very close association (67%) of HLA-DR (4w8) among A,B,C,D and DR. However, the LOD scores of HLA-DR4 and -DR w8 were 0.693 (theta = 0.35) and 0.642 (theta = 0.30) respectively. These data suggest that HLA-loci, analyzed on the basis of RFLP, may not be related with familial HCM with or without obstruction.

Cardiomyopathy, Hypertrophic↗
PubMed2000

[Echocardiography of idiopathic cardiomyopathies].

Echocardiography is useful to demonstrate cardiac abnormalities seen in idiopathic cardiomyopathies. Dilated cardiomyopathy is characterized by dilated cardiac chambers and diffusely hypokinetic wall motion. Hypertrophic cardiomyopathy is characterized by left ventricular hypertrophy with no known cause. Restrictive cardiomyopathy has diastolic failure with preserved systolic function. Echocardiography demonstrates the normal sized ventricles and the dilated atria. Recent advances in echocardiography include second harmonic imaging and tissue Doppler echocardiography. Second harmonic imaging can produce good quality images with high S/N ratio and is useful to delineate the endocardial border clearly and to show smoke-like echo in cardiac chambers. Tissue Doppler echocardiography can measure wall motion velocity. One of the potential application of this method would be to differentiate constrictive pericarditis from restrictive cardiomyopathy.

Cardiomyopathy, Dilated↗
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↗
PubMed2001

Cardiac amyloidosis associated with the transthyretin Ile122 mutation in a Caucasian family.

The Ile122 transthyretin variant associated with restrictive cardiomyopathy has been described in African-Americans and estimated to be present in approximately 4% of the Black population. We report the first American-Caucasian family with cardiomyopathy due to the TTR Ile122 mutation. The high prevalence of this mutation in the Black population and the discovery that it may cause disease in other ethnic populations highlights the importance of considering this autosomal dominant systemic amyloidosis in all individuals with restrictive cardiomyopathy. Inadequate diagnosis combined with inappropriate treatment may have a significant impact on morbidity and mortality.

Aged↗
PubMed2000

[Contribution of echocardiography to the diagnosis of patients with chronic heart failure].

The echocardiographic examination is generally performed in patients with heart failure and it often gives a significant contribution to the differential diagnosis. Firstly, the evaluation of left ventricular pump function by measuring the ejection fraction (EF) can distinguish patients with heart failure into two different groups, with depressed or preserved EF. The most frequent causes of heart failure and depressed EF are coronary artery disease, idiopathic dilated cardiomyopathy (DCM) and hypertensive heart disease. Although the echocardiographic features of coronary artery disease versus idiopathic DCM may be similar, the demonstration of inducible ischemia at dobutamine echocardiographic test suggests the presence of significant coronary artery disease and may be useful in the selection of cases for coronary arteriography. The association of left ventricular hypertrophy, hypokinesis and, sometimes, significant dilation is compatible with hypertensive heart disease or end-stage hypertrophic cardiomyopathy. No useful echocardiographic findings can identify the patients with genetic DCM or affected by myocarditis from other cases with idiopathic DCM. Some advanced cases of right ventricular dysplasia/cardiomyopathy may show a biventricular involvement and mimic DCM; these patients are usually characterized at echo by predominant right ventricular dilation and multiple a-dyskinetic bulges in the absence of pulmonary hypertension. Very difficult to manage are the patients with significant left ventricular dysfunction and severe valvular heart disease (such as aortic stenosis or mitral regurgitation). According to the literature, the left ventricular systolic function is relatively preserved (EF > 40%) in 30-40% of patients with heart failure. In these cases a diastolic dysfunction may be hypothesized. Echo-Doppler evaluation can be helpful in the recognition of signs of increased left ventricular stiffness ("restrictive filling pattern") and of increased filling pressures. In the differential diagnosis one must first consider the most frequent heart disorders that may present with this clinical syndrome, coronary artery disease and hypertensive heart disease. Furthermore, other less common diseases characterized by heart failure due to predominant diastolic dysfunction are the following: hypertrophic and restrictive cardiomyopathies, infiltrative heart diseases, such as amyloidosis, and constrictive pericarditis. Restrictive cardiomyopathy is characterized by heart failure and preserved left ventricular EF in the absence of significant ventricular dilation and hypertrophy; typical, although not pathognomonic, echocardiographic features are atrial enlargement ad restrictive filling pattern. In distinguishing constrictive pericarditis from restrictive cardiomyopathy useful Doppler signs are the wide respiratory variability in flow velocities at mitral and tricuspid levels, due to increased ventricular interdependence caused by the presence of an abnormally rigid pericardium.

Cardiomegaly↗
PubMed2006

Different types of cardiomyopathy associated with isolated ventricular noncompaction.

Although mainly described in the context of dilated and hypokinetic left ventricles, it is unclear whether isolated ventricular noncompaction (IVNC) is a distinct cardiomyopathy, a subtype of dilated cardiomyopathy, or a morphogenetic disorder. To investigate the spectrum of cardiomyopathies associated with IVNC, children and adults with stringent echocardiographic diagnoses of IVNC were reviewed. Seventy-three patients (12 children aged <15 years) seen since 1994 satisfied stringent echocardiographic criteria for IVNC. Sixty-five patients (89%; 11 children) had dilated cardiomyopathy, 2 adults had clear-cut hypertrophic cardiomyopathy, 1 adult had restrictive cardiomyopathy (to the investigators' knowledge, the first reported example of this particular association), and 5 patients (1 child) had normal left ventricular morphology and function. In conclusion, knowledge that IVNC can co-exist with restrictive and hypertrophic cardiomyopathy (in addition to the dilated form) supports the concept that IVNC is a morphologic trait rather than a distinct cardiomyopathy. This knowledge should be taken into account during echocardiographic examination and encourage the use of contrast echocardiography (and magnetic resonance) and could also orient molecular biology studies.

Adolescent↗
PubMed1999

Constrictive pericarditis.

The diagnosis of constrictive pericarditis remains a challenge because its physical findings and hemodynamics mimic restrictive cardiomyopathy. Various diagnostic advances over the years enable us to differentiate between these two conditions. This review begins with a case report of constrictive pericarditis, followed by a brief history and discussions of etiologies. Clinical features, radiologic, electrocardiographic, angiographic findings, and hemodynamics of constrictive pericarditis are reviewed. The echocardiographic findings are detailed and the recent advances in Doppler flow velocity patterns of pulmonary, mitral, tricuspid valves and hepatic veins are reported. Nuclear ventriculograms depict rapid ventricular filling in constrictive pericarditis and differentiate it from restrictive cardiomyopathy. Endomyocardial biopsy helps further in recognizing the various types of restrictive cardiomyopathies. Computed tomography and magnetic resonance imaging delineate abnormal pericardial thickness in constrictive pericarditis. Association of characteristic hemodynamic changes and abnormal pericardial thickness > 3 mm usually confirms the diagnosis of constrictive pericarditis. Effusive and occult varieties of constrictive pericarditis are briefly described. This review concludes with emphasizing the importance of pericardial resection.

Adult↗
PubMed2003

[Unusual evolution of myopericarditis: a case report].

Presenting features of constrictive pericarditis, a rare evolution of acute pericardial inflammation, may resemble those of restrictive cardiomyopathy or liver diseases. We describe the case of a 32-year-old man, with a history of myocardial and pericardial inflammation disease, who showed recurrent symptoms and signs of heart failure. For this reason, he underwent complete diagnostic evaluation, including computed tomography, echocardiography with tissue Doppler imaging, and endomyocardial biopsy. Constrictive pericarditis could be correctly diagnosed and the patient underwent pericardiectomy with complete resolution of heart failure. The present case report provides evidence that tissue Doppler echocardiography yields diagnostic information helpful to differentiate between constrictive pericarditis and restrictive cardiomyopathy. Moreover, non-invasive imaging is of value to choose the optimal therapeutic strategy in constrictive cardiomyopathy.

Adult↗
PubMed2006

Cardiac diseases as a risk factor for stroke in Saudi children.

OBJECTIVE: To ascertain the role of cardiac diseases as a risk factor for stroke in a cohort of Saudi children who were evaluated in a retrospective and prospective study. METHODS: Children with cardiac diseases were identified from within a cohort of 104 Saudi children who presented with stroke. They were seen as inpatients in the Pediatric Wards or evaluated at the Outpatient Clinics of the Division of Pediatric Neurology (DPN), and the Division of Pediatric Cardiology at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia during the periods July 1992 to February 2001 (retrospective study) and February 2001 to March 2003 (prospective study). A comprehensive form for clinical, neuroimaging, neurophysiological and laboratory data retrieval was designed and completed for each patient. Cardiac evaluation included 12-lead ECG and serial echocardiograms. Cardiac catheterization and 24-hour ambulatory ECG (Holter) were conducted on clinical discretion. RESULTS: Cardiac diseases were the underlying risk factor for stroke in 6 (5.8%) of the 104 children (aged one month to 12 years). The patients (4 males and 2 females) were evaluated at the DPN at a mean age of 5.3 years (range = 1-8 years; median 6.5 years). Onset of stroke was at a mean age of 34 months (range = 4 months-8 years; median = 30 months). Five patients had stroke in association with congenital heart disease (CHD), whereas the sixth had restrictive cardiomyopathy. The identified CHD consisted of membranous ventricular septal defect in a 5-year-old boy who had moyamoya syndrome and sickle cell beta(0)-thalassemia, asymptomatic patent ductus arteriosus (PDA) in a 17-month-old girl, atrioventricular canal defect and PDA in an 8-year-old boy who also had Down syndrome, partial anomalous pulmonary venous drainage in a one-year-old boy, and Tetralogy of Fallot in an 8-year-old boy. The latter patient developed hemiparesis secondary to a septic embolus, which evolved into brain abscess involving the right fronto-parietal region. This was successfully managed surgically. The sixth patient was an 8 1/2-year-old girl who had hemiparesis and complex partial seizure in association with restrictive cardiomyopathy. Serial echocardiograms depicted resolution of the cardiac abnormalities within 5 years and subsequent normal findings. CONCLUSIONS: Cardiac diseases, as a group, constitute a significant risk factor for stroke in Saudi children. Early diagnosis of these diseases is important to prevent further recurrences of stroke, and because some of them are potentially curable.

Cardiomyopathy, Restrictive↗
PubMedDate not supplied

Septal bounce, vena cava plethora, and pericardial adhesion: informative two-dimensional echocardiographic signs in the diagnosis of pericardial constriction.

To assess the diagnostic value of three different two-dimensional echocardiographic signs of pericardial constriction (early diastolic septal bounce, plethora of the inferior vena cava with blunted respiratory response, and pericardial adhesion), two independent observers retrospectively evaluated echocardiograms in 100 patients, 39 of whom had pericardial constriction, 15 had hemodynamically insignificant pericardial thickening, 16 had restrictive cardiomyopathy, and 30 had normal hearts. Causes of pericardial disease included cardiac surgery, malignancy, and uremia. Sensitivity and specificity of the three signs for constriction were 62% and 93% for septal bounce, 79% and 80% for vena cava plethora, and 79% and 90% for pericardial adhesion, respectively. The presence of either vena cava plethora or pericardial adhesion increased sensitivity, whereas the presence of both plethora and adhesion increased specificity. Between the two readers, septal bounce was the most consistent and pericardial adhesion the least consistent sign. False positive results included right ventricular pacing or left bundle branch block (septal bounce), postpericardiotomy (pericardial adhesion), and right heart failure (vena cava plethora). False negative results were often caused by technical problems with imaging. We conclude that these three two-dimensional echocardiographic signs are useful in differentiating pericardial constriction from hemodynamically insignificant pericardial thickening or restrictive cardiomyopathy.

Aged↗
PubMed2007

[Patterns of delayed-enhancement in MRI of ischemic and non-ischemic cardiomyopathies].

Contrast-enhanced MRI using the delayed-enhancement technique (DE-MRI) is widely applied in the clinical work-up of myocardial diseases. Myocardial diseases of varying etiology result in myocardial changes, such as necrosis, fibrosis, edema and metabolite deposition, which can be visualized by DE-MRI. Acute and chronic ischemic diseases based on a coronary artery disease as well as non-ischemic cardiomyopathies display DE. Cardiomyopathies often show a characteristic enhancement pattern. While ischemic lesions are localized in the subendocardium, non-ischemic cardiomyopathies often display an intramyocardial or subepicardial pattern. The typical pattern for dilated cardiomyopathies is band-like and intramyocardial with septal involvement. Arrhythmogenic right-ventricular dysplasias/cardiomyopathies are frequently associated with right-ventricular DE. In the case of amyloid cardiomyopathies which are often restrictive cardiomyopathies, subendocardial and circular DE is typically observed. Hypertrophic cardiomyopathies display patchy intramyocardial DE usually in the anteroseptal region. Acute myocarditis is typically accompanied by intramyocardial or subepicardial DE affecting the lateral wall. In the case of chronic myocarditis, intramyocardial or subepicardial DE is observed most frequently. Cardiac sarcoidosis typically entails patchy subepicardial DE with right- and left-ventricular involvement. Since there is an overlap between the enhancement patterns of cardiomyopathies, the diagnostic accuracy of DE-MRI is limited and the diagnosis must be based on additional clinical and MRI findings. The amount of DE often corresponds with cardiac functional parameters as well as with the frequency of cardiac events so that DE-MRI may be useful for risk stratification. Furthermore, DE-MRI can be helpful in the planning and evaluation of myocardial biopsies and electrophysiological examinations.

Acute Disease↗
PubMed2005

Marginal dietary copper restriction induces cardiomyopathy in rats.

Prior studies have provided evidence of marginal dietary copper restriction in humans. The present study was undertaken to examine in a rat model the effect of a long-term marginal dietary Cu deficiency on the heart. Male adult Sprague-Dawley rats were fed AIN-76 diet containing 6.0 (control), 3.0, or 1.5 mg Cu/kg starting at 11 wk of age. Groups of rats were killed at 6, 9, 12, 15, or 18 mo after initiation of feeding, and the same experiment was repeated once. The only systemic change induced by marginal dietary Cu restriction (P < 0.05) was depression of organ Cu concentrations in rats fed 1.5 mg Cu/kg diet. Cardiac pathological manifestations in rats fed lower Cu diets were evidenced by histopathological, ultrastructural, and functional alterations. Myocyte hypertrophy and excessive collagen deposition in the heart occurred in rats fed 1.5 mg Cu/kg diet. Ultrastructural changes, including increased number and volume of mitochondria along with disruption of cristae structure, diastolic and systolic dysfunction, and electrocardiograph alterations, occurred in rats fed 1.5 or 3.0 mg Cu/kg diet. These results demonstrate that, in the absence of most indications of systemic Cu deficiency, heart morphology and function are sensitive to marginal Cu deficiency.

Animals↗
PubMed1998

A clinical, echocardiographic and genetic characterization of a Danish kindred with familial amyloid transthyretin methionine 111 linked cardiomyopathy.

AIMS: To identify carriers and non-carriers of the mutant transthyretin methionine 111 linked familial amyloid disease, to detect early signs of the restrictive cardiomyopathy and other clinical manifestations characteristic of this inheritable disease. METHODS AND RESULTS: Out of 125 living family members 99 were available for clinical, echocardiographic and genetic examination. Twenty-five family members were heterozygous carriers of the mutant transthyretin methionine 111 genotype, while 74 were non-carriers. Among the 25 carriers, none had overt clinical signs of heart disease. Eight carriers, all above the age of 35, showed echocardiographic abnormalities suggestive of developing or manifest restrictive cardiomyopathy. Three had biopsy-verified transthyretin-related amyloid cardiomyopathy. None of the 15 carriers in the younger age group exhibited aberrant echocardiographic patterns. Nine carriers had carpal tunnel syndrome as opposed to none of the non-carriers. CONCLUSION: For early detection of familial amyloid cardiomyopathy, echocardiography is the investigation of choice. The first sign is diastolic dysfunction detected as an abnormal relaxation pattern. The appearance of echocardiographic aberrations solely in the older age group suggests that the cardiomyopathy is a late onset disease. Carpal tunnel syndrome appears to be the earliest presenting clinical symptom. A curative treatment seems to be an early liver transplantation.

Adolescent↗
PubMed2000

[Idiopathic cardiomyopathy: pathophysiology and hemodynamics].

Idiopathic cardiomyopathy is classified into three categories, namely, hypertrophic, dilated and restrictive cardiomyopathy. Pathophysiology and hemodynamics are quite different between the three types of cardiomyopathies. Hypertrophic cardiomyopathy is hemodynamically characterized by outflow obstruction and diastolic dysfunction. In dilated cardiomyopathy, progressive dilatation and systolic dysfunction of the left ventricle are predominant. This left ventricular remodeling is strongly associated with neurohormonal activation occurred in this disease. In restrictive cardiomyopathy, left ventricular distensibility is markedly impaired and differential diagnosis from constrictive pericarditis is often difficult.

Cardiomyopathies↗
PubMed1984

Possibilities of two-dimensional echocardiography in cardiomyopathies.

Findings in 20 patients with congestive, in 14 with hypertrophic and in one with restrictive cardiomyopathy are described. In congestive cardiomyopathy, there predominated left ventricular dilatation and diffuse hypokinesis with decreased ejection fraction. The main criteria are reported for differentiating it from advanced diffuse ischaemic heart disease. More detailed characterization is presented of the type of asymmetric septal hypertrophy and of the systolic anterior motion of the mitral apparatus and the possible causes of this finding are discussed. In a patient with restrictive cardiomyopathy there predominated a symmetric thickening of the left ventricle, with diffusely decreased kinetics and increased density of all structures.

Adolescent↗
PubMed1989

Noninvasive assessment of cardiomyopathy in normotensive diabetic patients between 20 and 50 years old.

PURPOSE: To further the understanding of diabetic heart disease, we tested the hypothesis that an asymptomatic group of normotensive diabetic patients between 20 and 50 years old had a restrictive cardiomyopathy independent of clinically significant coronary artery disease. PATIENTS AND METHODS: Quantitative two-dimensional echocardiography and stress myocardial perfusion scintigraphy were performed to detect and characterize the cardiac abnormalities in this study group comprising 88 patients with rigorously classified diabetes and 65 volunteer control subjects. RESULTS: Diabetic patients were shown to have a mildly reduced left ventricular end-diastolic volume index: 50.1 +/- 8.2 and 52.1 +/- 14.7 mL/m2 for patients with type I and type II diabetes, respectively, versus 58.9 +/- 11.7 mL/m2 for control subjects. The left ventricular diastolic filling was also impaired in diabetic patients as reflected by a lower atrial emptying index: 0.73 +/- 0.24 and 0.76 +/- 0.3 for type I and type II diabetics, respectively, compared with 1.14 +/- 0.24 for control subjects. Exercise tolerance was normal in subjects with type I diabetes and slightly reduced in subjects with type II diabetes. Only one patient developed regional ischemia on thallium exercise testing. CONCLUSION: Using a comprehensive, noninvasive approach, we have shown that asymptomatic normotensive patients with type I or type II diabetes who were between 20 and 50 years old had a restrictive cardiomyopathy characterized by mildly reduced left ventricular end-diastolic volume and altered left ventricular compliance independent of critical coronary artery disease.

Adult↗
PubMed1989

[Cardiomyopathy in Italy today: extent of the problem].

Since the number of patients suffering from cardiomyopathy in Italy is unknown at present, a multicentre survey was carried out in 15 University and Hospital cardiological centres. Each centre was asked to report the number of cases of dilated, hypertrophic or restrictive cardiomyopathy observed between 1970 and 1987. Information was also sought regarding the residence of the patient at the time of diagnosis. Diagnostic criteria for each type of cardiomyopathy, based on those published in the current literature, were sent to the participating centres. For the entire period, a total of 2,229 patients with dilated, 913 with hypertrophic, and 64 with restrictive cardiomyopathy were reported. The number of cases observed between 1980-87 was about four times greater than that observed between 1970-79 for dilated cardiomyopathy and three times greater for hypertrophic cardiomyopathy. In the period 1970-79, a minimal annual incidence of dilated cardiomyopathies was calculated. In the centres this ranged from 0.005 to 1.8 per 100,000 inhabitants, although no systematic evaluation was made. On the contrary, minimal prevalence for hypertrophic cardiomyopathy ranged from 0.4 to 5.6 between 1970-79. These rates increased to 0.09-3.4 for dilated cardiomyopathy and to 1.0-23.0 for hypertrophic cardiomyopathy in the period 1980-87. On the basis of this survey the number of patients with a diagnosis of dilated and hypertrophic cardiomyopathy seems to be on the increase in our country. This increase may be due to the diagnostic facilities available, such as echocardiography, and possibly to a better knowledge and a greater interest in cardiomyopathies.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiomyopathy, Dilated↗