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Results for “Cardiomyopathy, Restrictive”

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At least 667 records · Page 37Linked to original sources
PubMed2002

[Isolated non-compaction of the ventricular myocardium].

Isolated non-compaction of the ventricular myocardium is a rare disorder of endomyocardial morphogenesis characterized by numerous, excessively prominent ventricular trabeculations, probably due to arrest of normal endomyocardial embryogenesis. We report two cases of isolated non-compaction of the ventricular myocardium.The first patient had a brother who died in the first year of life after heart transplantation due to refractory heart failure caused by restrictive biventricular cardiomyopathy associated with a right ventricular diverticulum. This could have been a case of isolated non-compaction of the ventricular myocardium that was not diagnosed. The patient, who is 8 years old, is asymptomatic and echocardiogram (ECG) showed multiple prominent ventricular trabeculations in the apical portion of the left ventricle, slightly depressed systolic and diastolic function and restrictive ventricular filling pattern.The second patient is a 7-year-old boy who presents recurrent syncopes. ECG showed multiple prominent ventricular trabeculations, restrictive ventricular filling pattern and normal systolic function. The Holter ECG recording showed multiple premature ventricular complexes and nonsustained ventricular tachycardia. An implantable subcutaneous Holter recorder was inserted, which was associated with an episode of aborted sudden death and polymorphic ventricular tachycardia followed by asystole. The patient was then treated with an implantable defibrillator.

Defibrillators, Implantable↗
PubMed1998

[Restrictive myocardiopathy and pulmonary Raynaud's syndrome].

Idiopathic restrictive myocardiopathy is an uncommon condition. The differential diagnosis with constrictive pericarditis may require endomyocardial biopsy or surgical exploration. The prognosis of idiopathic restrictive myocardiopathy reported in the literature is to the order of several years. In this case the association with pulmonary Raynaud syndrome appears to be fortuitous and has not been previously reported.

Cardiomyopathy, Restrictive↗
PubMed1999

[Case of cardiac amyloidosis associated with IgG-K multiple myeloma in the framework of restrictive myocardiopathy].

Primary amyloidosis, due to amassing of fragments of light chains of IgG, often causes cardiac involvement. We describe a 65-year-old woman with multiple myeloma efficaciously treated with chemotherapy. Amyloidosis had been supported by myelic biopsy. The patient came to our observation because of right heart failure, hypotension and syncope: she was treated with a dopamine i.v. and was in cachectic status. She had a moderate pericardial effusion. ECG showed reduction of QRS amplitude, I degree atrioventricular block, posterior fascicular and right bundle branch block. Right cardiac catheterization showed a restrictive situation. After 1 week exitus occurred by asystole. In this case, there were other involvements by amyloidosis, besides the cardia one: that of autonomic nervous system and, probably, surrenal.

Aged↗
PubMed2006

Alpha-lipoic acid prevents lipotoxic cardiomyopathy in acyl CoA-synthase transgenic mice.

Alpha-lipoic acid (alpha-LA) mimics the hypothalamic actions of leptin on food intake, energy expenditure, and activation of AMP-activated protein kinase (AMPK). To determine if, like leptin, alpha-LA protects against cardiac lipotoxicity, alpha-LA was fed to transgenic mice with cardiomyocyte-specific overexpression of the acyl CoA synthase (ACS) gene. Untreated ACS-transgenic mice died prematurely with increased triacylglycerol content and dilated cardiomyopathy, impaired systolic function and myofiber disorganization, apoptosis, and interstitial fibrosis on microscopy. In alpha-LA-treated ACS-transgenic mice heart size, echocardiogram and TG content were normal. Plasma TG fell 50%, hepatic-activated phospho-AMPK rose 6-fold, sterol regulatory element-binding protein-1c declined 50%, and peroxisome proliferator-activated receptor-gamma cofactor-1alpha mRNA rose 4-fold. Since food restriction did not prevent lipotoxicity, we conclude that alpha-LA treatment, like hyperleptinemia, protects the heart of ACS-transgenic mice from lipotoxicity.

Animals↗
PubMed2004

Cardiac sarcoidosis: two cases with autopsy findings.

Cardiac sarcoidosis is a disease of young adults. In most cases it presents with sudden death, arrhythmias, conduction disorders, heart failure or cardiomyopathy. The authors describe two cases of myocardial involvement by sarcoidosis that lead to death of the patients. Case one was a 26-year-old Indian man who was previously well and presented with sudden death. Autopsy showed nodules of sarcoid granuloma involving the heart, lungs and lymph nodes. Case two was a 47-year-old Indian lady who complained of reduced effort tolerance. Echocardiography showed that she had restrictive hypertrophic cardiomyopathy with heart failure. Seven months after initial presentation, she developed worsening of heart failure and died. Autopsy revealed involvement of the heart, lungs and liver by sarcoidosis.

Adult↗
PubMedDate not supplied

Regional diastolic function in hypertrophic cardiomyopathy: a tissue Doppler echocardiographic study.

BACKGROUND: Tissue Doppler echocardiography (TDE) is a recent ultrasonic technique. One of its potential clinical uses is the assessment of regional diastolic function. OBJECTIVES: To compare left ventricular global diastolic function with interventricular septum regional diastolic function in patients with hypertrophic cardiomyopathy with different levels of global diastolic dysfunction. METHODOLOGY: 10 normal subjects (group 1), eight hypertrophic cardiomyopathy patients with abnormal relaxation (group 2) and 12 patients with hypertrophic cardiomyopathy with restrictive pattern (group 3) on the mitral inflow profile (pulsed wave Doppler) were studied. Each patient performed an M-mode color; TDE of the basal septum in the four chamber view. Velocities during the rapid filling phase (RF), and during atrial contraction (AC) were measured and RF/AC ratio calculated in each patient. RESULTS: While in normal subjects (group 1) the RF/AC ratio was always higher than 1, in patients with hypertrophic cardiomyopathy patients with different levels of global diastolic dysfunction (groups 2 and 3), the RF/AC ratio was always lower than 1 (velocities during atrial contraction always higher than during rapid filling), despite the level of global diastolic dysfunction. CONCLUSIONS: A ratio between myocardial velocities during the rapid filling phase and during atrial contraction lower than one (RF/AC < 1) is a tissue Doppler echocardiography index of regional diastolic dysfunction, independently of the severity of global diastolic dysfunction.

Adult↗
PubMedDate not supplied

Doppler transmitral flow pattern is an independent prognostic factor in acute myocardial infarction.

We analyzed the transmitral flow pattern on admission in 95 patients with acute myocardial infarction. Nineteen patients had a restrictive pattern, defined as a peak early to peak atrial filling wave ratio greater than 1.6, plus an early wave deceleration time shorter than 180 ms. Restrictive pattern, compared with nonrestrictive one, was associated with the development of heart failure (42 versus 11%, p = 0.001) and greater in-hospital mortality (32 versus 7%, p = 0.002). After multivariate analysis, it remained significantly predictive of death (p = 0.028). We conclude that a restrictive pattern of left ventricular filling in patients with acute myocardial infarction is an independent predictor of early outcome.

Adult↗
PubMed1993

[Prognosis and treatment of chronic cor pulmonale].

We have report one hundred cases of chronic pulmonary heart observed between 1982 and 1991. Mean age was 63 years, 61% were men. COPD was the most frequent etiology of CP (84%) before restrictive (14%) and thromboembolic diseases (2%). Diuretics were used in all patients, nifedipine in 32% and digitalis in 16%. Eleven patients died during their first admission, and six died later. Only 31 patients are still regularly.

Adult↗
PubMed2005

Laminopathies: multisystem dystrophy syndromes.

Laminopathies are a heterogeneous group of genetic disorders due to abnormalities in type A lamins and can manifest varied clinical features affecting many organs including the skeletal and cardiac muscle, adipose tissue, nervous system, cutaneous tissue, and bone. Mutations in the gene encoding lamins A and C (LMNA) cause primary laminopathies, including various types of lipodystrophies, muscular dystrophies and progeroid syndromes, mandibuloacral dysplasia, dilated cardiomyopathies, and restrictive dermopathy. The secondary laminopathies are due to mutations in ZMPSTE24 gene which encodes for a zinc metalloproteinase involved in processing of prelamin A into mature lamin A and cause mandibuloacral dysplasia and restrictive dermopathy. Skin fibroblast cells from many patients with laminopathies show a range of abnormal nuclear morphology including bleb formation, honeycombing, and presence of multi-lobulated nuclei. The mechanisms by which mutations in LMNA gene cause multisystem dystrophy are an active area of current investigation. Further studies are needed to understand the underlying mechanisms of marked pleiotropy in laminopathies.

Cardiomyopathy, Dilated↗
PubMed2005

Amyloid heart disease. New frontiers and insights in pathophysiology, diagnosis, and management.

Amyloidosis comprises a unique group of diseases that share in common the extracellular deposition of insoluble fibrillar proteins in organs and tissues. Cardiovascular amyloidosis can be primary, a part of systemic amyloidosis, or a result of chronic systemic diseases elsewhere in the body. The most common presentations are congestive heart failure-mainly a restrictive infiltrative pattern--and conduction system disturbances. Recent developments in imaging techniques and extracardiac tissue sampling have minimized the need for invasive endomyocardial biopsy for amyloidosis. Despite advances in treatment, the prognosis for patients with amyloidosis is still poor and depends on the underlying disease type. Herein, we present new insights and recent advances in cardiovascular amyloidosis.

Amyloid↗
PubMed1994

Abnormal subendocardial function in restrictive left ventricular disease.

OBJECTIVE: To study possible disturbances in left ventricular long axis function in patients with a restrictive filling pattern. DESIGN: Prospective examination of the left ventricular transverse and longitudinal axes, transmitral flow, and the apexcardiogram. SETTING: A tertiary referral centre for cardiac diseases. SUBJECTS: 21 normal subjects, age (SD) 51(11); 30 patients of similar age with a restrictive left ventricular filling pattern, defined as short early diastolic deceleration time less than the lower 95% confidence limit of the normal value (120 ms). 20 patients had a normal and 10 had an increased left ventricular end diastolic cavity size. RESULTS: Mitral Doppler echocardiography: E wave velocity was high only in patients with a normal cavity size. A wave velocity was greatly reduced in the two groups (P < 0.001) so that the E/A ratio was abnormally high. The relative A wave amplitude on the apexcardiogram was greatly increased in the two groups: 46(15)% (mean (SD)) and 54(4)% v 15(5)%. Minor axis: Fractional shortening was reduced from 30(10)% to 17(7)% in patients with normal cavity size and to 13(4.2)% in those with a dilated cavity (P < 0.001), as was the posterior wall thickening fraction from 100(30)% to 42(20)% and 50(25)% respectively (P < 0.001). Total systolic epicardial motion was normal and isovolumic relaxation time was short in the two groups. Long axis: Left ventricular abnormalities included reduced total amplitude of motion and its component during atrial systole (P < 0.001 for the two groups at both sites). Peak long axis shortening and lengthening were decreased at both left ventricular sites (P < 0.001). The time intervals from q wave of the electrocardiogram and A2 (aortic valve closure) to the onset of shortening and lengthening respectively were increased (both P < 0.001). Right ventricular long axis function was similarly affected but to a lesser extent. CONCLUSION: Left ventricular long axis function is consistently abnormal in patients with restrictive disease whether or not cavity size is increased. Not only are the extent and peak velocity of shortening reduced, but during diastole the peak early diastolic lengthening rate and amplitude during atrial systole are impaired. Early diastolic long axis motion is asynchronous with respect to transmitral flow and left ventricular minor axis. These effects will impair the overall left ventricular systolic and diastolic function independently of any decrease in passive cavity compliance. Unlike fibrosis, these long axis abnormalities are potentially amenable to treatment.

Cardiomyopathy, Restrictive↗
PubMed2002

Restrictive left ventricular filling pattern with preserved systolic function assessed by Doppler echocardiography: clinical, echocardiographic and prognostic implications.

From an echocardiographic laboratory database, echocardiographic characteristics of clinical and cardiovascular mortality were compared in patients with restrictive pattern of left ventricular filling (RPLVF) and left ventricular ejection fraction (LVEF) >50% (n = 36), with patients with RPLVF and LVEF <50% (n = 120) and echocardiographic controls (n = 160). Preserved LVEF was found in 21% of the patients with RPLVF. Mortality in patients with RPLVF was worst compared with echocardiographic controls regardless of their underlying LVEF after 2.7 +/- 1 years of follow-up. Patients with RPLVF had distinctive predictors of cardiovascular death, with clinical and echocardiographic characteristics according to their LVEF.

Adult↗
PubMed2004

[Idiopathic hypereosinophilia with cardiac involvement].

HISTORY AND CLINICAL FINDINGS: A 34-year-old previously healthy woman was admitted to another hospital because of abdominal pain, cough and dyspnea. Peripheral eosinophilia was present. Two months later she was admitted to the cardiology department with signs of mitral regurgitation. Dyspnea, fatigue, skin rashes with pruritus and a systolic murmur were noted. INVESTIGATIONS: Laboratory tests showed 11.4/nl leukocytes (normal range 4.8-10.8/nl) with an eosinophilia of 19% (normal range < 4%) corresponding to 2.2/nl. Cardiac magnetic resonance imaging revealed endomyocardial fibrosis involving the posterior mitral leaflet with resulting valvular regurgitation. Doppler ultrasound showed restrictive heart failure. DIAGNOSIS, THERAPY AND FURTHER COURSE: The diagnosis of idiopathic hypereosinophilia most likely as part of hypereosinophilic syndrome with cardiac involvement was made. The patient was treated with digitalis, diuretics and peptidyl dipeptidase (PDP) inhibitor. The treatment with glucocorticoids and cytotoxic agent to achieve a reduction of eosinophil count was ended by the patient a few weeks later. CONCLUSION: The hypereosinophilic syndrome with endomyocardial fibrosis is rare, and its prognosis is grave. The pathophysiological mechanisms are not entirely clear, nearly 70 years after Löffler first described fibrous endocarditis with eosinophilia. Patients receive symptomatic medical therapies. Additional surgical treatment has been reported,. Antihypereosinophilic therapy is used to control the disease.

Adult↗
PubMed2004

Hyperleptinemia prevents lipotoxic cardiomyopathy in acyl CoA synthase transgenic mice.

The physiologic function of the progressive hyperleptinemia of diet-induced obesity is unknown. However, that lipotoxicity in nonadipose tissues of congenitally unleptinized obese rodents is far greater than in hyperleptinemic diet-induced obesity rodents has suggested an antilipotoxic role. To test this hypothesis, mice with severe lipotoxic cardiomyopathy, induced transgenically by cardiomyocyte-specific overexpression of the acyl CoA synthase (ACS) gene, were made hyperleptinemic by treatment with recombinant adenovirus containing the leptin cDNA. Normoleptinemic control ACS-transgenic mice developed severe dilated cardiomyopathy with thickened left ventricular walls and profound impairment of systolic function on echocardiogram; histologically, there was severe myofiber disorganization and interstitial fibrosis, with intracytoplasmic lipid vacuoles identifiable by electron microscope. By contrast, the hearts of hyperleptinemic ACS-transgenic mice appeared normal, with normal echocardiograms and cardiac triglyceride (TG) contents. Their lower myocardial TG content was ascribed primarily to profound lowering of plasma TG and free fatty acids; free fatty acids were 17% of normal at 8 weeks. Additionally, enhanced myocardial AMP-activated protein kinase phosphorylation may have increased fatty acid oxidation, thereby contributing to the lowering of lipid stores. We conclude that obesity-level hyperleptinemia protects the heart from lipotoxicity.

Animals↗
PubMed2004

Inherited cardiomyopathies as a troponin disease.

Troponin, one of the sarcomeric proteins, plays a central role in the Ca(2+) regulation of contraction in vertebrate skeletal and cardiac muscles. It consists of three subunits with distinct structure and function, troponin T, troponin I, and troponin C, and their accurate and complex intermolecular interaction in response to the rapid rise and fall of Ca(2+) in cardiomyocytes plays a key role in maintaining the normal cardiac pump function. More than 200 mutations in the cardiac sarcomeric proteins, including myosin heavy and light chains, actin, troponin, tropomyosin, myosin-binding protein-C, and titin/connectin, have been found to cause various types of cardiomyopathy in human since 1990, and more than 60 mutations in human cardiac troponin subunits have been identified in dilated, hypertrophic, and restrictive forms of cardiomyopathy. In this review, we have focused on the mutations in the genes for human cardiac troponin subunits and discussed their functional consequences that might be involved in the primary mechanisms for the pathogenesis of these different types of cardiomyopathy.

Cardiomyopathy, Dilated↗
PubMedDate not supplied

Occult restrictive hemodynamics after pediatric heart transplantation.

BACKGROUND: Although resting hemodynamics after pediatric heart transplantation are generally within normal limits, we hypothesized that occult restrictive hemodynamics suggesting diastolic dysfunction may be unmasked by acute volume loading (fluid challenge) during cardiac catheterization. We wished to determine the incidence of diastolic dysfunction and to assess whether it progressed over time. METHODS: From 1988 through 1993, a total of 100 fluid challenges were performed at the time of surveillance endomyocardial biopsy in 31 survivors of orthotopic heart transplantation. Cyclosporine-based immunosuppression was used in 16 patients, and FK506 was used in 15 patients. Right heart hemodynamics and cardiac output (thermodilution) were obtained at baseline and after a fluid challenge with 10 ml/kg of normal saline solution. The data were analyzed to determine whether type of immunosuppression or time elapsed since transplantation predicted the response to fluid challenge. RESULTS: Baseline hemodynamics were normal; however, a marked increase in atrial filling pressures occurred after fluid challenge (p < 0.001). Findings were similar in cyclosporine- and FK506-treated patients. Hemodynamic response to fluid challenge was not related to duration of time since transplantation, including studies on patients surviving more than 4 years. CONCLUSIONS: Diastolic dysfunction after heart transplantation is common; however, the abnormalities do not progress in severity, suggesting stable long-term graft function.

Adolescent↗