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PubMed1992

Controversial issues in restrictive cardiomyopathy.

The topic of restrictive cardiomyopathy remains controversial for many reasons. The term cardiomyopathy is unfortunately sometimes used interchangeably with diastolic heart failure. Furthermore, diastolic heart failure is sometimes linked with other causes of diastolic dysfunction such as hypertrophic cardiomyopathy and mitral valve disease. Restrictive cardiomyopathy is a clinical entity of primary or secondary myocardial disease presenting a picture that closely simulates that of constrictive pericarditis. In the majority of cases the correct diagnosis can be arrived at following a careful paradigm that begins with history and may end with endomyocardial biopsy. Many of the old teachings about how to distinguish restrictive cardiomyopathy from constrictive pericarditis have not held up with time and clinical experience: in particular equal diastolic pressures on both sides of the heart are compatible with either restrictive cardiomyopathy or constrictive pericarditis.

Biopsy↗

PubMed2001

Clinical and molecular studies of a large family with desmin-associated restrictive cardiomyopathy.

Patients with restrictive cardiomyopathy (RC) have impaired diastolic function, but intact systolic function until later stages of the disease, ultimately leading to heart failure. Primary RC is often sporadic, but also may be inherited in an autosomal dominant fashion, particularly the idiopathic forms. Recently there has been great interest in inherited cardiomyopathy associated with myocyte desmin deposition ('desminopathies'). In some such families, desmin or alpha-B crystallin gene mutation is the underlying cause, and the desmin accumulation affects skeletal muscle as well, usually causing skeletal myopathy. We describe a large family with apparent autosomal dominant inheritance of desmin-associated RC spanning four generations, with the age of onset and severity/rate of progression being highly variable. This family is relatively unique in that there is no symptom-based evidence of skeletal muscle involvement, and the known desminopathy and cardiomyopathy genes/loci have been ruled out. These data support literature suggesting that desmin deposition may be associated with different underlying gene defects, and that a novel desminopathy gene is responsible for the condition in this family.

Adolescent↗

PubMed1996

[Anesthetic management of a patient complicated with restrictive cardiomyopathy for gastrectomy].

Restrictive cardiomyopathy is a rare condition characterized with endomyocardial fibrosis, which interferes diastolic ventricular filling. A 52-year-old man with a 38 year history of dyspnea on effort presented with advanced gastric cancer. Subtotal gastrectomy under general anesthesia was scheduled. Preoperative examination showed biventricular dysfunction, impaired liver function and chronic renal failure. General anesthesia was induced using fentanyl, pancuronium and isoflurane, and maintained with nitrous oxide/oxygen, isoflurane and fentanyl. Extensive invasive monitoring included arterial blood pressure, central venous pressure, pulmonary artery pressure and oxygen saturation of mixed venous blood as indices of left-sided pump function. Dopamine and nitroglycerin infusion was also started after the tracheal intubation. Although a transient improvement of cardiac function was noted after the removal of ascites 5 liter, restricted fluid administration induced desaturation of mixed venous blood and tachycardia. The start of low dose prostaglandin E1 markedly improved cardiac output without hypotension, while an increase of intravenous nitroglycerin did not prove to be beneficial. PGE1 was more selective in decreasing left ventricular afterload, while nitroglycerin produces greater decrease of preload.

Alprostadil↗

PubMed2005

Differentiating constrictive pericarditis from restrictive cardiomyopathy.

Constrictive pericarditis and restrictive cardiomyopathy are 2 forms of diastolic dysfunction with similar presentation but different treatment options. Whereas constrictive pericarditis has the potential of being cured with pericardiectomy, restrictive cardiomyopathy is usually incurable. It is therefore crucial to differentiate between the 2 disorders. In the last few years, new diagnostic techniques have become available to differentiate these causes of diastolic dysfunction from each other. This review provides a complete, in-depth comparison of the 2 disorders with regard to their symptoms and clinical features, etiology, pathophysiology, hemodynamics, echocardiographic presentation, and finally the different available management options.

Cardiomyopathy, Restrictive↗

PubMed2002

Does rapid volume loading during transesophageal echocardiography differentiate constrictive pericarditis from restrictive cardiomyopathy?

BACKGROUND: Respiratory variation of the pulmonary venous (PV) peak flow velocities can be used to distinguish constrictive pericarditis (constriction) from restrictive cardiomyopathy (restriction). Rapid volume expansion has been used successfully to enhance diastolic pressure equalization in occult constriction. The effect of volume on the respiratory variation in constriction has not been studied previously. This study assessed the utility of volume in enhancing the PV respiratory variation of constriction to further separate it from restriction. METHODS: The study population consisted of 15 patients referred to the echocardiography laboratory for further evaluation of clinically suspected diastolic dysfunction. Pulsed-Doppler transesophageal echocardiography (TEE) of the left or right upper pulmonary vein and mitral inflow was performed with respiratory monitoring before and after infusion of 1 liter of normal saline over 5 to 10 minutes. The classification of patients as constriction (n = 8) or restriction (n = 7) was confirmed independently by cardiac catheterization or surgery. Peak velocities of the PV systolic and diastolic waves and the mitral inflow E were measured during inspiration and expiration. A mean of 3-6 respiratory cycles was obtained for each value before and after volume loading. The percent change from expiration to inspiration (%E) was calculated using the formula %E = expiration - inspiration / expiration. RESULTS: At baseline, patients with constrictive pericarditis can be separated reliably from those with restrictive cardiomyopathy based on a higher systolic/diastolic ratio and greater respiratory variation of their PV diastolic flow velocity. There were no complications in any patient due to volume expansion. Although the change from baseline to volume expansion was not statistically significant in either constriction or restriction, the %E of the PV diastolic wave became significantly higher in constriction than in restriction (P < 0.05). CONCLUSIONS: Rapid volume expansion is relatively safe during TEE and can be used for further separation of constrictive pericarditis from restrictive cardiomyopathy by significantly enhancing the respiratory variation of the PV diastolic flow velocity in constrictive pericarditis.

Adult↗

PubMed1991

Acute hemodynamic effects of captopril in children with a congestive or restrictive cardiomyopathy.

The acute hemodynamic effects of captopril were evaluated at cardiac catheterization in 16 children (age, 0.3-18 years) with cardiomyopathy. Twelve children had congestive cardiomyopathy, whereas four had restrictive cardiomyopathy. Hemodynamic measurements were obtained 30 and 60 minutes after the oral administration of captopril (0.5 mg/kg). Blood pressures were measured in the aorta, pulmonary artery, right atrium, and pulmonary capillary wedge position; cardiac outputs were measured by the thermodilution technique. Hemodynamic data could not be obtained after the administration of captopril in one child with congestive cardiomyopathy because of an immediate, severe hypotensive response. In 11 of 12 children with congestive cardiomyopathy, cardiac index increased by 22%, from 2.3 to 2.8 l/min/m2 (p less than 0.05), and stroke volume increased by 22%, from 23 to 28 ml/m2 (p less than 0.05). Systemic vascular resistance decreased from 32 to 21 units.m2 (p less than 0.01), but the mean aortic pressure did not change significantly. In contrast, four children with restrictive cardiomyopathy had no change in cardiac output after captopril, but there was a trend toward significant arterial hypotension (mean aortic pressure decreased from 78 to 59 mm Hg). Thus, captopril acutely reduced systemic vascular resistance and increased both cardiac output and stroke volume in children with congestive cardiomyopathy. In children with restrictive cardiomyopathy, however, captopril did not affect cardiac output, but it did decrease aortic pressure. These data indicate that captopril may benefit children with a congestive cardiomyopathy but that captopril probably should not be used in children with restrictive disease.

Captopril↗

PubMed2005

[Restrictive cardiomyopathy].

The group of restrictive cardiomyopathies (RKMP) includes a number of myocardial and endomyocardial diseases. These entities are characterized by stiffening of both ventricles resulting in a deterioration of ventricular filling and severe diastolic dysfunction. Systolic ventricular function is usually normal or only mildly impaired. Amyloidosis is the most prevalent underlying cause, but also sarcoidosis, hypereosinophilic syndrome with endocardial fibrosis and some inherited metabolic and storage diseases are common. Constrictive pericarditis is the main differential diagnosis, but noninvasive (magnetic resonance imaging, echocardiography, natriuretic peptides) and invasive diagnostic procedures allow separation of these two entities. Diagnosis of the underlying disease is mandatory for therapy of RKMP, as there are specific therapeutic options for symptom relief. Heart transplantation may be an option in end-stage heart failure.

Cardiomyopathy, Restrictive↗

PubMed1993

Multiple myeloma complicated by restrictive cardiomyopathy and cardiac tamponade.

Restrictive cardiomyopathy from amyloid deposition within the myocardium is a well-described complication of multiple myeloma; however, myelomatous involvement of pericardium with subsequent cardiac tamponade has rarely been described. Optimal treatment for malignant involvement of the pericardium by myeloma cells has yet to be established. The following description is of a patient with myocardial and pericardial manifestations of multiple myeloma. Treatment of the malignant pericardial effusion was implemented with intrapericardial administration of bleomycin. This therapy resulted in no recurrence of pericardial effusion at nine days follow-up. Despite the absence of detectable recurrent effusion, the patient died suddenly from causes felt unrelated to pericardial disease.

Aged↗

PubMedDate not supplied

Restrictive cardiomyopathy in dermatomyositis.

The association between dermatomyositis and restrictive cardiomyopathy has not been reported before. We present here the clinical, echocardiographic and muscle biopsy data for a patient with dermatomyositis and restrictive cardiomyopathy. In a 78-year-old male with a history of arterial hypertension, recurrent episodes of atrial fibrillation and syncopes, rupture of an infra-renal aortic aneurysm with complications (recurrent QT-prolongation, lumbo-sacral plexopathy, transient ischaemic attack, peripheral embolism), monoclonal gammopathy, subdural haematoma, focal seizures, megaloblastic anaemia, leucopenia, eosinophilia, elevated muscle enzymes and increasing tiredness, dermatomyositis was diagnosed upon clinical presentation, muscle enzyme and muscle biopsy findings. Cardiological examination revealed atrial fibrillation, left anterior hemiblock and restrictive cardiomyopathy. After the exclusion of various differentials for restrictive cardiomyopathy, a causative relationship between restrictive cardiomyopathy and dermatomyositis was assumed. This case suggests the need for suspecting restrictive cardiomyopathy in patients with dermatomyositis. Patients with dermatomyositis should undergo a comprehensive cardiological investigation as soon as the neurological diagnosis is established.

Aged↗

PubMed1990

An atypical case of cardiomyopathy in a child: hypertrophic or restrictive cardiomyopathy?

A case of an atypical form of cardiomyopathy, in which biopsy showed bizarre myocardial hypertrophy with disorganization, and in which there was no obvious hypertrophy of the interventricular septum and left ventricular free wall, is presented. Ventricular filling was critically impaired, and consequently pulmonary and systemic venous congestion predominated in the clinical presentation which was similar to that of restrictive cardiomyopathy.

Biopsy↗

PubMed1997

Restrictive cardiomyopathies in childhood. Etiologies and natural history.

Restrictive cardiomyopathy is rare in childhood and little is known about the causes and outcome. This lack of information results in extrapolation of adult data to the care and management of children, who might require different treatment from that of adults. This study was undertaken retrospectively to evaluate the causes and natural history of restrictive cardiomyopathy in childhood. Twelve cases of restrictive cardiomyopathy were identified by database review of patient records from 1967 to 1994. The cases were selected on the basis of echocardiographic and cardiac catheterization criteria. Charts were reviewed for the following variables: age, sex, cause, right-and left-sided hemodynamics, pulmonary vascular resistance index, shortening fraction, therapy, and outcome. There were 6 males and 6 females with a mean age of 4.6 years at presentation (median, 3.4 yr; range, 0.9 to 12.3 yr). Etiologies included hypertrophic cardiomyopathy in 3 patients, cardiac hypertrophy with restrictive physiology in 3, idiopathic in 2, familial in 2 (twins), "chronic eosinophilia" in 1, and "post inflammatory" with no definitive causes in 1. At presentation the mean shortening fraction was 33% +/- 2% (mean +/- SEM), average right ventricular pressures were 44/13 +/- 3/1, average left ventricular pressures were 88/25 +/- 4/3, and the mean pulmonary vascular resistance index was 3.4 +/- 1.3 U.m2 (n = 9), but increased to 9.9 +/- 3.1 U.m2 (n = 5, p = 0.04) by 1 to 4 years after diagnosis. Four of the 12 patients had embolic events (1, recurrent pulmonary emboli; 1, saddle femoral embolus; 2, cerebrovascular accidents) and 9 of 12 died within 6.3 years despite medical therapies, which included diuretics, verapamil, propranolol, digoxin, and captopril. In conclusion, restrictive cardiomyopathy in childhood is commonly idiopathic or associated with cardiac hypertrophy, and the prognosis is poor. Embolic events occurred in 33% of our patients, and 9 of 12 patients died within 6.3 years. Within 1 to 4 years of diagnosis, patients may develop a markedly elevated pulmonary vascular resistance index; therefore, transplantation should be considered early.

Cardiac Catheterization↗

PubMed2004

Differentiation of constrictive pericarditis from restrictive cardiomyopathy using mitral annular velocity by tissue Doppler echocardiography.

This study evaluated the diagnostic role of early diastolic mitral annular velocity (E') by tissue Doppler echocardiography for differentiating constrictive pericarditis from restrictive cardiomyopathy (primary restrictive cardiomyopathy and cardiac amyloidosis). The study group consisted of 75 patients (53 men, 22 women; mean age 62 years, range 27 to 87). Of these, 23 patients had surgically confirmed constrictive pericarditis, 38 had biopsy-proved systemic amyloidosis and typical echocardiographic features of cardiac involvement, and 14 had primary restrictive cardiomyopathy. Standard mitral inflow characteristics were measured. Tissue Doppler echocardiography was used to measure E' at the septal annulus. E' was significantly higher in patients with constrictive pericarditis than in those with primary restrictive cardiomyopathy or cardiac amyloidosis (12.3 vs 5.1 cm/second, p <0.001). An E' cut-off value > or =8 cm/second resulted in 95% sensitivity and 96% specificity for the diagnosis of constrictive pericarditis. There was no overlap of E' between patients who had constrictive pericarditis and those who had cardiac amyloidosis. In a subgroup analysis of restrictive cardiomyopathy, E' of patients who had cardiac amyloidosis was significantly lower than that of patients who had primary restrictive cardiomyopathy (4.6 vs 6.3 cm/second, p <0.001). Thus, E' velocity can distinguish between constrictive pericarditis and restrictive cardiomyopathy with a specific cut-off value in patients with clinical and echocardiographic evidence of diastolic heart failure.

Aged↗

PubMed2005

Desmin accumulation restrictive cardiomyopathy and atrioventricular block associated with desmin gene defects.

BACKGROUND: Primary desminopathies are caused by desmin gene [DES (MIM*125660)] mutations. The clinical spectrum includes pure myopathies, cardiomuscular diseases and cardiomyopathies. Patients with restrictive cardiomyopathy (RCM) plus atrioventricular block (AVB) due to DES defects are frequently unrecognized unless desmin accumulation is specifically investigated in endomyocardial biopsy (EMB) by ultrastructural study. AIMS: To describe a cardiological phenotype characterized by RCM plus AVB due to desmin accumulation caused by DES defects. METHODS AND RESULTS: Desmin accumulation was diagnosed by means of ultrastructural and immunocytochemical studies of EMB in four unrelated probands with RCM and AVB. Candidate genes [DES and alphaB-crystallin (CRYAB)] were screened using sequence analysis. Four DES gene mutations were identified: three new (R16C, T453I and a 10 bp deletion at the exon-intron boundary of exon 3 disrupting the donor splice site) and one known (R406W). The disease was autosomal dominant in two families, recessive in one and associated with a de novo mutation in one. The mutations cosegregated with phenotype in all patients. CRYAB gene screening was negative. CONCLUSIONS: A cardiac phenotype characterized by RCM and AVB caused by desmin accumulation is associated with DES mutations. Although the mutations affected different domains, the cardiac phenotype was identical.

Adolescent↗

PubMed1991

Primary restrictive cardiomyopathy: clinical and pathologic characteristics.

Twenty-four patients with restrictive cardiomyopathy were identified at St. Thomas' Hospital during a 17-year period. All had endomyocardial biopsy, but in two patients the biopsy specimens were small and nondiagnostic. Seven patients had amyloidosis and five had other specific heart muscle diseases. The remaining 10 patients with primary restrictive cardiomyopathy had myocyte hypertrophy or interstitial fibrosis, or both. Patients with primary restrictive cardiomyopathy presented earlier but survived longer after presentation than did those with amyloidosis. In each group, survival after cardiac catheterization was related to cardiac index but not to filling pressures. Primary restrictive cardiomyopathy was associated with complete heart block in four patients, two of whom had skeletal myopathy. One had a family history of dominantly inherited skeletal myopathy. Primary restrictive cardiomyopathy was present in a mother and daughter. Two other patients had a family history of heart failure, sudden death or complete heart block, alone or in combination, at a young age. Restrictive hemodynamics and complete heart block were present in patients even in the absence of significant fibrosis. The data suggest that primary restrictive cardiomyopathy may be a distinct myopathy with dominant inheritance and incomplete penetrance that is expressed morphologically as myocyte hypertrophy and interstitial fibrosis. Skeletal myopathy may be associated with the cardiomyopathy.

Adolescent↗

PubMed2003

Constrictive pericarditis and restrictive cardiomyopathy: similarities and differences.

Constrictive pericarditis and restrictive cardiomyopathy, two relatively uncommon clinical conditions, create a diagnostic dilemma primarily because of the many similarities in both their clinical and hemodynamic presentations. However, considerable differences exist in the pathophysiology, management, and prognosis between these two syndromes. Furthermore, the precise diagnosis of constrictive pericarditis and restrictive cardiomyopathy is mandatory, as the former is often curable whereas only palliative treatments are available for the latter. In this brief review, similarities and differences in the various aspects of constrictive pericarditis and restrictive cardiomyopathy will be discussed.

Cardiomyopathy, Restrictive↗

PubMed1987

Restrictive cardiomyopathy in children. Ultrastructural findings.

Restrictive cardiomyopathy is usually related to fibrosis of the endocardium or to an infiltrative disorder. However, in few cases, it can be due to isolated pathology of the myocytes but such alterations are not well characterized. This paper reports the disease in two 7 year old patients. There was severe venous congestion and catheterisation revealed increased end diastolic pressure in the ventricles. Both pericardial and myocardial biopsies were performed, as the clinical and haemodynamic data were indistinguishable from constrictive pericarditis. The structure of the pericardium was normal. The endocardium was not thickened. The interstitium of the myocardial tissue was not increased. Electron microscopic examination revealed intracellular masses of disorganized myofilaments. These large deposits may have produced decrease compliance of the myocytes and of the ventricular walls.

Biopsy↗

PubMed1993

[Idiopathic restrictive cardiomyopathy].

PURPOSE: To characterize the idiopathic restrictive cardiomyopathy (RCM) through clinical manifestations and complementary tests and to analyze the surgical benefit on the correction of tricuspid regurgitation. METHODS: Five patients with RCM idiopathic were retrospectively studied, 4 female and 1 male, with ages ranging from 30 to 59 (mean 40) years. The patients presented heart failure due to restrictive syndrome and were submitted to chest X-ray, EKG, echocardiogram, right ventricular endomyocardial biopsy, hemodynamic study and surgery. RESULTS: X-ray showed heart enlargement in all patients, but only one had pulmonary venous capillary congestion. EKG presented atrial fibrillation, complex QRS with low voltage on frontal axis, abnormal ventricular repolarization, right bundle branch block, inactive area and right ventricular hypertrophy. Complete atrioventricular block was observed in one patient. The echocardiogram study showed biatrial enlargement in all patients, and left ventricular dilatation in one patient with left ventricular dysfunction. Mild tricuspid regurgitation were observed in one patient and moderate in four. Two patients had mild mitral regurgitation. Diastolic dysfunction were observed at Doppler-cardiography in all patients. Right ventricular endomyocardial biopsy showed interstitial fibrosis, myocytes hypertrophy and myofibrils disarray. The hemodynamic study disclosed right ventricular hypertension in all patients. Three patients had hypotension in left ventricle and aorta. The left ventricular end diastolic pressure and pulmonary capillary pressure (pulmonary wedge pressure) were elevated in all patients. Dilated left ventricle with mild hypokinesia and right ventricular inferior hypokinesia and diastolic restriction were observed in one patient. One patient had similar pattern without diastolic restriction. Mild tricuspid regurgitation was observed in one patient, moderate in three and severe in one. Mild mitral regurgitation were observed in two patients. The restrictive pattern (deep and plateau) was present in three patients. Surgery were performed in all patients, and confirmed the mild tricuspid regurgitation in one and the moderate in four. De Vega plastia were performed in four patients. CONCLUSION: Among the complementary methods, echocardiogram and hemodynamic study were those of higher contribution to the diagnosis of idiopathic RCM. The disease has a poor evolution, even with clinical or surgical treatment. Heart transplantation may would be the best procedure on patients with symptomatic idiopathic restrictive cardiomyopathy.

Adult↗

PubMed2002

Criterion for fetal primary spongiform cardiomyopathy: restrictive pathophysiology.

BACKGROUND: Most cardiomyopathies recognizable in utero are the dilated type-with dilated, poorly contractile left ventricle. We propose a diagnostic criterion for the rare spongiform (noncompacted) cardiomyopathy. CASES: Three perinatal cases with echocardiography and autopsy are presented. The apical ventricular myocardium was thickened and markedly trabeculated. The ventricles were not dilated in two, and the atria were enlarged in all. Hydrops and bradycardia were present in all three despite normal or only mildly diminished contractility. Although the cardiomyopathy was familial in two siblings, two of three cases were female, ruling out Barth syndrome (with sex-linked recessive inheritance). Although all three of our cases with hydrops died, rare survivors have been reported in the eighth decade. CONCLUSION: Although spongiform cardiomyopathy may eventually develop into a dilated cardiomyopathy, its early characteristic is relatively diagnostic: a restrictive cardiomyopathy with no enlargement of the ventricles and prominent atria.

Autopsy↗