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Results for “Cardiomyopathy, Restrictive”

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PubMed1984

Idiopathic restrictive cardiomyopathy.

This report details the clinical, hemodynamic, and morphologic data from four patients 59 to 77 years old (mean 66) with a primary restrictive cardiomyopathy. All patients had symptoms of congestive heart failure, jugular venous distention, and murmurs of mitral and tricuspid regurgitation. Four patients required pacemakers, three for the brady-tachy syndrome and one for complete heart block. Chest x-ray demonstrated cardiomegaly in all four patients and pulmonary congestion and/or pleural effusions in three. Echocardiographic examination documented left atrial enlargement in all patients, along with normal left ventricular internal dimensions. Global left ventricular systolic function was normal in all, and left and right ventricular filling pressures were elevated and similar in three. A dip and plateau pattern was present in the pressure tracings of two of three patients. Unlike previous reports of restrictive cardiomyopathy, in our four patients there was no specific morphologic cause noted at necropsy. Pathologic evaluation demonstrated biatrial dilatation in all patients, with thrombi present in the atrial appendages in three. Normal ventricular cavity size and mild right ventricular hypertrophy were present in all patients and mild-to-moderate left ventricular hypertrophy was present in two. There were no significant pericardial, endocardial, or valvular abnormalities and no infiltrative myocardial disorders were present. Light and electron microscopic studies demonstrated only interstitial fibrosis of the myocardium. A restrictive hemodynamic profile may be observed in the absence of specific infiltrative disorders and affected patients may exhibit a prolonged clinical course of 4 to 14 years (mean 9). However, in these patients congestive heart failure responded poorly to medical therapy or surgical correction of valvular regurgitation, which is common in this disorder.

Aged↗

PubMed1985

Laser photoablation of pathological endocardium: in vitro findings suggesting a new approach to the surgical treatment of refractory arrhythmias and restrictive cardiomyopathy.

In selected patients, malignant ventricular tachyarrhythmias have been successfully abolished by excision of subendocardial arrhythmogenic foci. Likewise, in certain patients in whom restrictive cardiomyopathy is due to endocardial thickening, endocardial resection has resulted in hemodynamic improvement. The present study was designed to explore the utility, in vitro, of laser photoablation of pathologically thickened endocardium. Endocardial photoablation was easily accomplished regardless of etiological or anatomical variations using either the focused beam of a carbon dioxide laser or argon laser light delivered through a 200-microns optical fiber. Photoablation of areas as large as 3.9 X 1.3 cm was performed within 40 seconds. The extent or depth of endocardial photoablation could be limited to 2 mm2 in area or 1 mm in depth using either form of laser therapy. These in vitro results suggest that either carbon dioxide or argon laser phototherapy can be successfully applied to the surgical treatment of refractory arrhythmias and restrictive cardiomyopathy. Advantages of laser photoablation include speed and precision. Furthermore, laser photoablation obviates the difficulty associated with conventional techniques in establishing tissue planes.

Argon↗

PubMed1998

Echocardiographic Aspects of Restrictive Cardiomyopathy: Their Relationship with Pathophysiology.

BACKGROUND AND METHODS: We studied 17 patients with restrictive cardiomyopathy; eight had biventricular restriction (type A), four had left ventricular restriction (type B), and five had only right ventricular restriction (type C). RESULTS: Type A disease was characterized by pulmonary and systemic venous congestion. The restrictive pattern was found in the inlet of both ventricles. Both atria were enormous, with small or normal-size ventricles. Differential diagnosis included constrictive pericarditis and systolic pump dysfunction. Type B restriction disease was characterized by venous pulmonary congestion, pulmonary hypertension, and important dilatation of the left atrium and right cavities with a small or normal-size left ventricle; the restrictive pattern was found only in the affected left ventricle. CONCLUSIONS: The clinical picture resembles that of rheumatic mitral valve disease with right ventricular failure. Type C disease had restriction only in the inlet of right ventricle, with giant right atrium, systemic venous hypertension with low flow, and normal pressure of pulmonary artery and left heart. Differential diagnosis included Ebstein's anomaly of tricuspid valve. The etiology of type A disease was amyloid, endomyocardial fibrosis of ventricles and idiopathic interstitial fibrosis. Asymmetric types were always caused by Davies' disease.

Journal Article↗

PubMed1993

Restrictive cardiomyopathy caused by chloroquine.

A 59 year old white woman who had been treated with chloroquine phosphate for 25 years presented with signs of congestive heart failure and was diagnosed as having restrictive cardiomyopathy by non-invasive methods. Electron microscopy of a biopsy specimen of skeletal muscle showed lesions compatible with chloroquine myopathy. The patient died five weeks after presentation. Electron microscopy of heart tissue showed similar lesions to those of the skeletal muscle.

Cardiomyopathy, Restrictive↗

PubMed1988

Severe constrictive pericarditis as an unsuspected cause of death in a patient with idiopathic hypereosinophilic syndrome and restrictive cardiomyopathy.

A 43-year-old man with idiopathic hypereosinophilic syndrome survived a relatively long term (6 1/2 years) before he succumbed to intractable heart failure. Six months before death, his chronic heart failure from restrictive cardiomyopathy was well compensated. Autopsy demonstrated severe constrictive pericarditis which was not suspected antemortem. Constrictive pericarditis as a late complication of idiopathic hypereosinophilic syndrome is discussed.

Adult↗

PubMed1991

Constrictive pericarditis versus restrictive cardiomyopathy: a reappraisal and update of diagnostic criteria.

Distinguishing constrictive pericarditis from restrictive cardiomyopathy is a difficult clinical challenge. We review published reports in which hemodynamic criteria were used to differentiate these two diagnoses. There were 82 cases of constriction and 37 cases of restriction. The overall predictive accuracy of the difference between right and left ventricular end-diastolic pressures (RVEDP and LVEDP), RV systolic pressure, and the ratio of RVEDP to RV systolic pressure were 85%, 70%, and 76%, respectively. If all three criteria were concordant, the probability of having correctly classified the patient was greater than 90%. However, one fourth of patients could not be classified by hemodynamic criteria. There are few data to support the use of hemodynamic measurements after exercise or volume infusion to separate these two groups. Numerous recent studies have reported on the ability of left ventriculography, Doppler echocardiography, or radionuclide angiography to distinguish constriction from restriction. Many of the proposed indices appear promising, but these studies suffer from small sample size, potential selection bias, and complexity of the proposed criteria, which have limited their widespread application. New imaging technologies, such as CT scanning or MRI have been applied in a limited number of cases, but appear to be a sensitive means of detecting abnormal pericardium. Endomyocardial biopsy has proven useful in establishing the diagnosis of infiltrative cardiomyopathies, eliminating in those cases the need for surgical intervention. The finding of myocarditis must be considered a nonspecific finding that does not preclude thoracotomy. Since constrictive pericarditis is a surgically curable condition, the distinction between constrictive and restrictive disease is of critical importance. Taking into account the relative contribution of data derived from hemodynamic, imaging,and biopsy studies, we propose an algorithm for the selection of appropriate candidates for pericardial biopsy and stripping.

Algorithms↗

PubMed1997

Multicore myopathy with restrictive cardiomyopathy.

A 10-y-old girl is presented who suffered mild muscular weakness and exercise intolerance from the age of 1 y onwards, with progression appearing from the age of about 8 y. Multicore myopathy and restrictive cardiomyopathy were diagnosed. Literature concerning the coexistence of multicore myopathy and cardiomyopathy is reviewed.

Cardiomyopathy, Restrictive↗

PubMed1996

Idiopathic restrictive cardiomyopathy in childhood. A diastolic disorder characterized by delayed relaxation.

Six children with idiopathic restrictive cardiomyopathy were evaluated. Electrocardiographic evaluation disclosed left atrial dilatation and repolarization abnormalities. Echocardiographic examination showed gross left atrial enlargement (182 +/- 29% of predicted values, P < 0.001) in the presence of normal left ventricular cavity dimensions (99 +/- 6%, P: ns). Left ventricular wall thickness varied from normal to mild concentric hypertrophy (septum: 116 +/- 16%, P < 0.05). Global left ventricular systolic function was normal or slightly subnormal; however, the relaxation was significantly delayed throughout diastole. E/A ratio was 4.1 +/- 1.4 and deceleration time 94 +/- 7 ms. Marked ventricular filling occurred in mid-diastole as could be deduced from a prominent mid-diastolic mitral L wave on the Doppler flow tracing. Early filling contributed 56 +/- 6%, mid-diastolic filling 28 +/- 4% and atrial contraction 16 +/- 3% to total ventricular filling as estimated by determining E-area, L-area and A-area, respectively. The left ventricular pressure curve showed a steady decline during mid-diastolic filling. This implies that the driving force for mid-diastolic filling is not the increased left atrial pressure but suction by the ventricle. The restrictive haemodynamics are therefore not caused by increased intrinsic stiffness of the ventricular wall, but most likely result from serious dysfunction and delay of the active relaxation of the ventricle. Progression of the disease was observed in three out of six patients, resulting in death or extreme low cardiac output. The three other patients remained clinically stable during the follow-up period of 6-10 years.

Adolescent↗

PubMed2006

[Restrictive cardiomyopathy due to myocardial cysticercosis].

There is no description of cysticercosis affecting heart function. In the present report, the authors describe the case of a 46-year-old woman with cardiac cysticercosis and heart failure, presenting with echocardiographic findings suggestive of restrictive cardiomyopathy and myocardial microcalcifications suggestive of cardiac infiltration by the disease.

Cardiomyopathy, Restrictive↗

PubMedDate not supplied

Favorable response to high-dose interferon-alpha in idiopathic hypereosinophilic syndrome with restrictive cardiomyopathy--case report and literature review.

The idiopathic hypereosinophilic syndrome is characterized by a prolonged overproduction of eosinophils of unknown cause in addition to specific organ damage due to eosinophil-derivated protein toxicity. Its prognosis is correlated with heart involvement that results in a restrictive cardiomyopathy. This is frequently biventricular. Isolated left ventricular infiltration has rarely been reported. The authors report a case of idiopathic hypereosinophilic syndrome with cardiac involvement obliterating the left ventricular cavity with a favorable clinical outcome following a combination therapy with interferon-alpha, hydroxyurea and prednisone. Data from other studies dealing with the treatment of idiopathic hypereosinophilic syndrome with interferon-alpha are reviewed and discussed.

Adult↗

PubMed1982

Restrictive cardiomyopathy with pseudotumor formation of the left ventricle.

A 4-year-old girl had the insidious onset of congestive heart failure without apparent cause. Evaluation by echocardiography, thallous chloride TI 201 scintigraphy, and angiography suggested the presence of either a neoplasm or restrictive cardiomyopathy with a localized mass effect on the left ventricle. Pathological specimens obtained by transvascular endomyocardial biopsy and at surgery defined the pathology to be restrictive cardiomyopathy. Analysis of myocardium by electron microscopy demonstrated a previously undescribed abnormality of the contractile elements involving the myofilaments and Z bands, with generalized secondary glycogen deposition.

Cardiomyopathies↗

PubMed1988

Restrictive cardiomyopathy with kappa light chain deposits in myocardium as a complication of multiple myeloma. Histochemical and electron microscopic observations.

kappa Light chain deposits occurring in myocardium as a complication of multiple myeloma were identified ultrastructurally and immunohistochemically in a right ventricular endomyocardial biopsy specimen from a patient who presented with clinical and hemodynamic findings of restrictive cardiomyopathy. These deposits were not evident on routine histopathologic examination; they were Congo red-negative and gave a positive immunoperoxidase reaction for kappa light chains and a negative reaction for lambda chains. They consisted of amorphous, electron-dense granules that formed discontinuous layers adjacent to the plasma membranes of cardiac myocytes, arteriolar endothelial and smooth-muscle cells, and neural elements. These observations underscore the need for critical study of endomyocardial biopsy specimens, using electron microscopy and immunohistochemical reagents, for the precise identification of protein components in tissue deposits in patients suspected of having cardiac amyloidosis or related disorders.

Aged↗

PubMed1997

[Primary amyloidosis AL as a cause of restrictive cardiomyopathy].

In a 71-year-old woman with macroglossia and heart failure, a monoclonal protein (kappa-light chains) was found in the serum. Congestive heart failure was diagnosed on the basis of heart catheterization and echocardiography. The tongue biopsy showed numerous amyloid fibrils, and plasmacytosis was present in the bone marrow. All these findings indicated a restrictive cardiomyopathy as part of a primary systemic amyloidosis.

Aged↗

PubMed1990

Restrictive cardiomyopathy following acute myocarditis--a case report.

A fifty-five-year-old man developed intractable heart failure four weeks after upper respiratory infection. His central venous pressure was as high as 300 mmH2O. Two-dimensional echocardiogram revealed no muscular hypertrophy of the ventricles, no pericardial thickness, and no pericardial effusion. On catheterization, hemodynamic data were compatible with restrictive cardiomyopathy. Transvenous endomyocardial biopsy of the left ventricle was performed. Histologic examination strongly suggested that cardiomyopathy developed after acute myocarditis.

Biopsy↗

PubMed2006

Hydroxychloroquine-induced restrictive cardiomyopathy.

Chloroquine (Hydroxychloroquine)-induced cardiomyopathy is a rare but potentially life-threatening condition. Cessation of the culprit drug, along with aggressive afterload reduction therapy, has been associated with halting of disease progress and even improvement in patients' clinical and histologic status. Echocardiography is a fundamental tool in the identification and assessment of patients with cardiomyopathy, with particular utility in the detailed assessment of biventricular systolic and diastolic function. It also provides an objective and non-invasive means of assessing treatment response. We present a case of a 51-year-old woman with hydroxychloroquine-induced restrictive cardiomyopathy and correlate clinical, echocardiographic and anatomic pathologic findings both at initial presentation and following treatment.

Antirheumatic Agents↗

PubMed2002

[Bilateral phrenic nerve paralysis, dysautonomia and restrictive cardiomyopathy in a case of POEMS syndrome].

We report a case of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes) with unusual clinical features. A 62-year-old woman presented a severe polyneuropathy with dysphonia and vegetative symptoms, including bradycardia and sphincterial disorders. The clinical examination showed facial hyperpigmentation, cachexia, anasarca and splenomegaly. She also presented restrictive cardiomyopathy and endocrine disturbances. Nerve conduction studies revealed a severe demyelinating sensorimotor neuropathy. Cerebrospinal fluid analysis showed an elevated protein level. We detected a biclonal gammapathy (Ig G and Ig A with lambda light chain) and lytic pelvic bone lesions. Later, she developed a severe ventilatory failure due to a bilateral phrenic nerve paralysis leading to a mechanical ventilation. Steroids followed by localized radiotherapy partially improved the respiratory status and stabilized the neuropathy. Phrenic nerve paralysis, restrictive cardiomyopathy, vegetative symptoms and cranial nerve palsy are exceptional in POEMS syndrome. Moreover, this case emphasizes the importance of radiological investigations since the discover of plasmocytoma may improve the prognosis of POEMS syndrome.

Anti-Inflammatory Agents↗

PubMed2004

Troponin I, cardiac diastolic dysfunction and restrictive cardiomyopathy.

Cardiomyopathies are diseases of heart muscle that are associated with cardiac dysfunction. Molecular genetic studies performed to date have demonstrated that the damage or mutations in several sarcomeric contractile protein genes are associated with the development of the diseases. In this review, cardiac troponin I, one of the sarcomeric thin filament protein, will be discussed regarding its role in cardiac function, its deficiency-related diastolic dysfunction, and the mutation of this protein-mediated restrictive cardiomyopathy.

Animals↗

PubMed1995

Idiopathic restrictive cardiomyopathy in childhood: diagnostic features and clinical course.

OBJECTIVE: To describe the clinical course and outcome of children with idiopathic restrictive cardiomyopathy (IRCM) and to present the Doppler echocardiographic features of this disease in childhood. DESIGN: We reviewed the Mayo Clinic patient database for the period from 1975 to 1993 to identify children who underwent assessment for IRCM. MATERIAL AND METHODS: Clinical records and diagnostic studies, including two-dimensional (2-D), M-mode, and Doppler echocardiograms, were reviewed for each patient. Characteristics were analyzed statistically to determine potential predictors of outcome. RESULTS: Eight children (five girls and three boys) were diagnosed with IRCM between 1975 and 1993 at our institution. The median age at diagnosis was 11 years, and the median duration of follow-up was 11.5 years. Of the eight patients, five died (the median time from initial examination to death was 1 year). All five of these patients had clinical and radiographic evidence of pulmonary venous congestion. In all patients, 2-D and M-mode echocardiography revealed atrial enlargement without ventricular dilatation or hypertrophy. The four patients who underwent detailed diastolic Doppler assessment had findings consistent with restrictive filling and increased left ventricular end-diastolic pressure: (1) short mitral deceleration time, (2) increased pulmonary vein atrial reversal velocity and duration, and (3) pulmonary vein atrial reversal duration greater than mitral A-wave duration. CONCLUSION: The prognosis for children with IRCM is poor. In this small group of patients, absence of pulmonary venous congestion most consistently predicted extended survival. A combined 2-D and Doppler echocardiographic examination provides a reliable noninvasive means of assessing the physiologic and morphologic features of IRCM in children.

Adolescent↗