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PubMedDate not supplied

Clinical potential of ultrasound tissue characterization in cardiomyopathies.

Standard methods of echocardiographic analysis permit accurate categorization of cardiomyopathies into dilated, hypertrophic, and restrictive types. However, ascertainment of the cause of a particular cardiomyopathy (e.g., ischemia versus inflammation as the cause of a dilated myopathy) with ultrasound would be greatly facilitated by analysis of myocardial composition with ultrasound tissue characterization techniques. Qualitative observations have identified unusual echocardiographic image texture in hypertrophic cardiomyopathy and in amyloidosis. Quantitative observations have verified these findings and have identified increased ultrasound backscatter in regions of myocardial fibrosis, calcification, and anthracycline-induced cardiomyopathy. Although several technical problems remain unsolved and further research is needed in the mechanisms of normal and abnormal ultrasound/tissue interactions, tissue characterization with ultrasound has the potential to contribute independent information on myocardial composition in patients with cardiomyopathy.

Animals↗
PubMedDate not supplied

[Specific dilated myocardiopathy. Chronic chagasic cardiopathy at the National Institute of Cardiology Ignacio Chávez].

Cardiomyopathies are a heterogenous group of heart ailments. Some of them are primary myocardial diseases and are classified as dilated, hypertrophic, restrictive and arryhithmogenic. Dilated cardiomyopathies (DCs) are the most common. Sometimes it is possible to identify an etiologic agent, in that case we talk about a specific dilated cardiomyopathy. Here in, we review one of these specific DCs, the so called Chronic Chagasic Cardiopathy (CCC) from the point of view of our personal experience at the Instituto Nacional de Cardiología "Ignacio Chávez". Chagas' disease is present in Mexico, therefore CCC is also present. We estimate that 5,000 people, suffer CCC with severe symptoms. In Mexico, Chagas' disease occurs below the Tropic of Cancer and between 2,000-2,500 m above sea level, in this area there is a real risk for vectorial infection, mainly in rural villages. Clinical diagnosis should be supported by epidemiological and seroepidemiological confirmatory data. There is not appropriate therapy yet for this condition.

Cardiomyopathy, Dilated↗
PubMed2005

Heart failure in beta-thalassemia syndromes: a decade of progress.

The thalassemias are common monogenic disorders of hemoglobin synthesis. beta-thalassemias are the most important among the thalassemia syndromes and have become a worldwide clinical problem due to an increasing immigrant population. In beta-thalassemia major, regular blood transfusions are necessary early in life. Beta-thalassemia intermedia refers to a less severe phenotype, whereas beta-thalassemia/hemoglobin E disease encompasses a broad phenotypic spectrum. Blood transfusions and increased gastrointestinal iron absorption result in iron overload and tissue damage. Among patients with beta-thalassemia major, biventricular, dilated cardiomyopathy remains the leading cause of mortality. In some patients, a restrictive type of left ventricular cardiomyopathy or pulmonary hypertension is noted. The clinical course, although variable and occasionally fulminant, is more benign in recent than in older series. Myocarditis has been described as a cause of left-sided heart failure in younger patients. Pulmonary arterial hypertension is the principal cause of heart failure in beta-thalassemia intermedia. Chelation therapy has improved prognosis in beta-thalassemia major both by reducing the incidence of heart failure and by reversing cardiomyopathy. Estimation of the patient's cardiac risk is mainly based on clinical criteria and serial echocardiography. A new cardiovascular magnetic resonance technique will probably fulfill the need for more precise risk stratification in beta-thalassemia syndromes. By increasing the proportion of patients on optimal chelation, survival in beta-thalassemia major may further improve. Recent advances in gene therapy are expected to result in the long-awaited cure of this disease.

Chelating Agents↗
PubMed1998

Transgenic expression of replication-restricted enteroviral genomes in heart muscle induces defective excitation-contraction coupling and dilated cardiomyopathy.

Numerous studies have implicated Coxsackievirus in acute and chronic heart failure. Although enteroviral nucleic acids have been detected in selected patients with dilated cardiomyopathy, the significance of such persistent nucleic acids is unknown. To investigate the mechanisms by which restricted viral replication with low level expression of Coxsackieviral proteins may be able to induce cardiomyopathy, we generated transgenic mice which express a replication-restricted full-length Coxsackievirus B3 (CVB3) cDNA mutant (CVB3DeltaVP0) in the heart driven by the cardiac myocyte-specific myosin light chain-2v (MLC-2v) promoter. CVB3DeltaVP0 was generated by mutating infectious CVB3 cDNA at the VP4/VP2 autocatalytic cleavage site from Asn-Ser to Lys-Ala. Cardiac-specific expression of this cDNA leads to synthesis of positive- and negative-strand viral RNA in the heart without formation of infectious viral progeny. Histopathologic analysis of transgenic hearts revealed typical morphologic features of myocardial interstitial fibrosis and in some cases degeneration of myocytes, thus resembling dilated cardiomyopathy in humans. There was also an increase in ventricular atrial natriuretic factor mRNA levels, demonstrating activation of the embryonic program of gene expression typical of ventricular hypertrophy and failure. Echocardiographic analysis demonstrated the presence of left ventricular dilation and decreased systolic function in the transgenic mice compared with wild-type littermates, evidenced by increased ventricular end-diastolic and end-systolic dimensions and decreased fractional shortening. Analysis of isolated myocytes from transgenic mice demonstrate that there is defective excitation-contraction coupling and a decrease in the magnitude of isolated cell shortening. These data demonstrate that restricted replication of enteroviral genomes in the heart can induce dilated cardiomyopathy with excitation-contraction coupling abnormalities similar to pressure overload models of dilated cardiomyopathy.

Animals↗
PubMed1996

[Prognostic significance of doppler-derived and left ventricular diastolic filling variables in patients with dilated cardiomyopathy].

The purpose of this echocardiographic study was to evaluate the frequency of restrictive left ventricular filling pattern in dilated cardiomyopathy, as well as its clinical and echocardiographic correlations and prognostic implications. The study included 49 pts (39 males, 10 females) aged 32 to 56 years (mean age: 46 years), with a diagnosis of dilated cardiomyopathy, according to the World Health Organization criteria. Pulsed Doppler echocardiographic analysis was performed at discharge and during a two year follow-up and compared with clinical and 2-dimensional echocardiographic findings. The patients were divided into two groups according to measurement of E decelaration time (DT) of transmitral flow patterns: a restrictive group (Group A-21 pts) with DT < 115 ms. and a non-restrictive group (Group B-28 pts) with DT > 115 ms. Of 49 pts, 15 died during a two-year follow-up, 12 in the restrictive group and only 3 in the non-restrictive group. Eleven of these pts (73%) died of worsening of congestive heart failure and four (27%) died suddenly. The restrictive filling pattern was associated at Doppler study with a higher E wave velocity, lower A wave velocity and higher E/A ratio. Pts in the restrictive group were in a higher New York Heart Association functional class, and had a lower ejection fraction and more severe mitral regurgitation. In addition, they had a significantly larger right ventricle and higher mean pulmonary artery pressure. Mortality rate in the restrictive group was markedly higher than that in the non-restrictive group at 1 year (24% vs. 0%, respectively, p < 0.001) and at 2 years (57% vs. 11%, respectively, p < 0.001). The results from this study indicate that a restrictive left ventricular filling pattern is frequent in dilated cardiomyopathy, and is associated with more severe disease and is the single best clinical predictor for cardiac death in pts with dilated cardiomyopathy.

Adult↗
PubMed2003

The incidence of pediatric cardiomyopathy in two regions of the United States.

BACKGROUND: Population-based data on the incidence of pediatric cardiomyopathy are rare because of the lack of large, prospective studies. METHODS: Since 1996 the Pediatric Cardiomyopathy Registry sponsored by the National Heart, Lung, and Blood Institute has collected data on all children with newly diagnosed cardiomyopathy in New England and the Central Southwest region (Texas, Oklahoma, and Arkansas) of the United States. We report on all children in these regions who received this diagnosis between 1996 and 1999. RESULTS: We identified 467 cases of cardiomyopathy, for an overall annual incidence of 1.13 per 100,000 children (95 percent confidence interval, 1.03 to 1.23). The incidence was significantly higher among infants younger than 1 year old than among children and adolescents who were 1 to 18 years old (8.34 vs. 0.70 per 100,000, P<0.001). The annual incidence of cardiomyopathy was lower among white children (upper-bound estimate, 1.06 cases per 100,000) than among black children (lower-bound estimate, 1.47 per 100,000; P=0.02) and higher among boys than among girls (1.32 vs. 0.92 per 100,000, P<0.001). The incidence also varied significantly by region: 1.44 cases per 100,000 in New England and 0.98 per 100,000 in the Central Southwest region (P<0.001). When categorized according to type, dilated cardiomyopathy made up 51 percent of the cases, hypertrophic cardiomyopathy 42 percent, and restrictive or other types 3 percent; 4 percent were unspecified. There was no significant difference in the incidence rates according to the year. CONCLUSIONS: The estimated incidence of pediatric cardiomyopathy in two large regions of the United States is 1.13 cases per 100,000 children. Most cases are identified at an early age, and the incidence varies according to sex, region, and racial or ethnic origin.

Adolescent↗
PubMed2005

Is it time to include ion channel diseases among cardiomyopathies?

Heart muscle diseases are traditionally classified according to their peculiar pathophysiologic features such as "dilated," "hypertrophic," "restrictive," and "arrhythmogenic right ventricular" cardiomyopathy. The extraordinary advances accomplished in the last two decades in molecular genetics have allowed the identification of the genetic background of most of these conditions. According to the 1995 World Health Organization definition of cardiomyopathies as "diseases of the myocardium associated with cardiac dysfunction," they should include not only forms with hemodynamic dysfunction, but also conduction and rhythm disturbances. Arrhythmias are per se a sign of cardiac dysfunction and may reflect an underlying myocardial electrical disease with or without structural abnormalities as features. Nonstructural arrhythmogenic heart diseases include long and short QT syndromes, Brugada syndrome, Lènegre disease, and catecholaminergic polymorphic ventricular tachycardia. These conditions are defined as "channelopathies" because they are the consequence of cardiac ion channel gene mutations. Long and short QT syndromes are mostly caused by either sodium or potassium ion channel gene mutations; Brugada syndrome and Lènegre disease are both related to a defective sodium channel gene; and polymorphic ventricular tachycardia is the result of an abnormal ryanodine receptor regulating calcium release from the sarcoplasmic reticulum. These nonstructural inherited arrhythmic conditions should be regarded as cardiomyopathies because the myocyte is abnormal, although the heart is apparently intact. It is time for a new classification of cardiomyopathies taking into account the underlying gene mutations and the cellular level of expression of encoded proteins, thus distinguishing cytoskeleton (cytoskeletalopathies), desmosomal (desmosomalopathies), sarcomeric (sarcomyopathies), and ion channel (channelopathies) cardiomyopathies.

Arrhythmias, Cardiac↗
PubMed1995

Recent advances in the etiology, diagnosis, and treatment of myocarditis and cardiomyopathies in children.

The cardiomyopathies are usually classified as dilated, hypertrophic, or restrictive. Although clinical presentations vary among these myopathies, systolic or diastolic dysfunction characterizes the principal pathophysiologic features of each. Advances in genetic linkage analysis and mutation screening have recently allowed investigators to establish a genetic basis for several forms of dilated and hypertrophic cardiomyopathy. Although traditional treatment modalities and newer strategies (such as atrioventricular pacing in patients with dilated cardiomyopathy and pacing or amiodarone therapy for patients with hypertrophic cardiomyopathy) may provide short-term relief, cardiac transplantation may be the only intervention that can alter the natural history and poor prognosis for these potentially lethal conditions. Myocarditis, which usually presents as an acute form of dilated cardiomyopathy, may have a broad spectrum of presentation and is currently treated with nonspecific supportive measures. Although the polymerase chain reaction may provide rapid diagnosis of viral etiologies, newer therapies have not yet been developed. Clinical trials with intravenous immunoglobulin, however, are currently in progress. In patients whose symptoms do not resolve, cardiac transplantation may afford the only potential for long-term survival.

Cardiomyopathies↗
PubMed2006

Inducible cardiac-restricted expression of enteroviral protease 2A is sufficient to induce dilated cardiomyopathy.

BACKGROUND: Enterovirus infection is a cause of cardiomyopathy. We previously demonstrated that enteroviral protease 2A directly cleaves the cytoskeletal protein dystrophin. However, the direct effect of protease 2A in enteroviral cardiomyopathy is less clear because other viral proteins are also expressed with viral infection. METHODS AND RESULTS: A transgenic mouse with inducible cardiac-restricted expression of enteroviral protease 2A was generated. In the transgenic mouse, a tamoxifen-regulated Cre-loxP system, MerCreMer (MCM), was used to induce genetic recombination in cardiac myocytes, which led to protease 2A expression. Protease 2A and MCM double transgenic (2AxMCM) mice were treated with tamoxifen; the controls included 2AxMCM mice treated with diluents for tamoxifen and tamoxifen-treated MCM littermates. Protease 2A activity was significantly induced after tamoxifen in the 2AxMCM mice compared with controls. Echocardiographic analysis demonstrated an increase in left ventricular end-diastolic and end-systolic chamber size, with decreased fractional shortening in tamoxifen-treated 2AxMCM mice. There was an increase in heart weight-to-body weight ratio in 2AxMCM mice treated with tamoxifen. Only a small increase in interstitial fibrosis and inflammation was found in tamoxifen-treated 2AxMCM mice; however, ultrastructural analysis demonstrated myofibrillar collapse with abnormalities of intercalated discs and sarcolemmal membranes. Evans blue dye-positive myocytes with disruption of dystrophin were present in 2AxMCM mice treated with tamoxifen. Disruption of dystrophin was also found in cultured myocytes isolated from 2AxMCM mice with Cre in the nucleus. CONCLUSIONS: Protease 2A has a significant role in enteroviral cardiomyopathy and alone is sufficient to induce dilated cardiomyopathy, which is associated with disruption of the sarcolemmal membrane and cleavage of dystrophin with protease 2A expression.

Animals↗
PubMed1986

Myocardial diseases of animals.

In this review we have attempted a comprehensive compilation of the cardiac morphologic changes that occur in spontaneous and experimental myocardial diseases of animals. Our coverage addresses diseases of mammals and birds and includes these diseases found in both domesticated and wild animals. A similar review of the myocardial diseases in this broad range of animal species has not been attempted previously. We have summarized and illustrated the gross, microscopic, and ultrastructural alterations for these myocardial diseases; and, whenever possible, we have reviewed their biochemical pathogenesis. We have arranged the myocardial diseases for presentation and discussion according to an etiologic classification with seven categories. These include a group of idiopathic or primary cardiomyopathies recognized in man (hypertrophic, dilated, and restrictive types) and a large group of secondary cardiomyopathies with known causes, such as inherited tendency; nutritional deficiency; toxicity; physical injury and shock; endocrine disorders, and myocarditides of viral, bacterial, and protozoal causation. Considerable overlap exists between each of the etiologic groups in the spectrum of pathologic alterations seen in the myocardium. These include various degenerative changes, myocyte necrosis, and inflammatory lesions. However, some diseases show rather characteristic myocardial alterations such as vacuolar degeneration in anthracycline cardiotoxicity, myofibrillar lysis in furazolidone cardiotoxicity, calcification in calcinosis of mice, glycogen accumulation in the glycogenoses, lipofuscinosis in cattle, fatty degeneration in erucic acid cardiotoxicity, myofiber disarray in hypertrophic cardiomyopathy, and lymphocytic inflammation with inclusion bodies in canine parvoviral myocarditis. The myocardial diseases represent the largest group in the spectrum of spontaneous cardiac diseases of animals. Pericardial and endocardial diseases and congential cardiac diseases are seen less frequently; and, in contrast to man, coronary artery disease and myocardial ischemia are rather infrequent in animals. The present review shows clearly that the spectrum of myocardial diseases in animals is enlarging and that many newly recognized diseases are emerging and assuming considerable importance. For example, various heritable cardiomyopathies have recently been described in the KK mouse, cattle, and rats. Increasingly recognized myocardial diseases include cardiomyopathies in cats, dogs, and birds; anthracycline cardiotoxicity; furazolidone cardiotoxicity; ionophore cardiotoxicity; myocardial damage associated with central nervous system injuries; myocardial hypertrophy in

Animals↗
PubMed1995

Anaesthetic induction in a child with end-stage cardiomyopathy.

The purpose of this clinical report is to discuss the anaesthetic management of a patient with end-stage cardiomyopathy. An eight-year-old boy sustained electro-mechanical dissociation following a "standard anaesthetic induction" but was subsequently successfully anaesthetized using a combination of etomidate, midazolam, lidocaine, vecuronium and glycopyrrolate. Anaesthetic techniques and their relationship to the specific category of cardiomyopathy (dilated, hypertrophic or restrictive) are discussed. The potential detrimental effects of anaesthetic sympatholysis, myocardial depression and negative chronotropy on cardiac output are explained.

Anesthesia, Intravenous↗
PubMedDate not supplied

A retrospective epidemiological and etiological study of metabolic disorders in children with cardiomyopathies.

Cardiomyopathies may be idiopathic or secondary to an underlying definable systemic disorder, especially in a young population. A number of inherited metabolic disorders will lead to hypertrophic or dilated cardiomyopathy. Identifying the metabolic etiologies of cardiomyopathy is important for genetic counseling and identification of appropriate therapeutic interventions. Fifty-eight children aged from 1 to 18 years were diagnosed as suffering from cardiomyopathy over six years. Cardiomyopathies (dilated, hypertrophic or restrictive) were confirmed by echocardiography together with extensive clinical and metabolic investigations. The patients could be divided into two groups, etiology known (50.0%) and idiopathic (50.0%). Among the definable underlying diseases, 31.0% were diagnosed as inherited metabolic disorders, including mitochondrial disorders (n=4), primary systemic carnitine deficiency (n=1), Pompe disease (n=3) and mucopolysaccharidosis (MPS) I (n=1). In this study, there was a large component of idiopathic cardiomyopathies (50.0%), and it is likely that a proportion of these "unknowns" have a diagnosable disorder. Therefore, for cardiomyopathies in children, after exclusion of non-genetic causes, genetic studies and metabolic screening, such as tandem mass spectrometry, urine gas chromatography/mass spectrometry, carnitine level and mitochondrial DNA screening, are recommended.

Adolescent↗
PubMed2006

Long-term survival of a patient with isolated noncompaction of the ventricular myocardium.

Isolated noncompaction of the myocardium (INCM) is a rare, congenital, unclassified cardiomyopathy. It is caused by a disorder in endomyocardial morphogenesis in the absence of other structural disease. INCM is characterized by numerous prominent trabeculations and deep intertrabecular recesses in the myocardium. Frequently, INCM is associated with an increased incidence of heart failure, arrhythmias, and cardioembolic events with high morbidity and mortality. We describe a 28-year-old woman experiencing symptoms of heart failure since she was 4 years old. She had been intensively investigated and misdiagnosed as having dilated, restrictive, and apical hypertrophic cardiomyopathies. Cardiac magnetic resonance and echocardiography recently revealed the actual diagnosis of INCM. The patient is alive and well, taking vasodilators and warfarin. Herein, we describe the long-term follow-up of this patient and demonstrate that some patients have a favorable prognosis. In addition, the improvement in noninvasive cardiac imaging has revealed a higher prevalence of INCM, previously undetected.

Anticoagulants↗
PubMed2005

Effect of dobutamine stress on left ventricular filling in ischemic dilated cardiomyopathy: pathophysiology and prognostic implications.

OBJECTIVES: The purpose of this research was to study the effect of dobutamine on left ventricular (LV) filling in ischemic cardiomyopathy (ICM) and to determine whether restrictive filling pattern (RFP) at peak stress has prognostic value. BACKGROUND: The prognostic value of RFP at peak stress in ICM is unknown. METHODS: A total of 69 patients with ICM were studied by Doppler echocardiography at rest and stress; RFP was defined as transmitral E:A ratio > or =1.0, isovolumic relaxation time (IVRT) <80 ms, and E-wave deceleration time (EDT) <120 ms. RESULTS: A total of 42 of 69 had RFP at rest, which reverted to non-RFP at stress in 24 (EA), but persisted in 18 (EE); 27 of 69 had non-RFP at rest and peak stress (AA). In EA, IVRT and EDT lengthened (by 43 ms and 46 ms), and tricuspid regurgitation (TR) decreased (by 26 mm Hg, p < 0.01), suggesting a fall in left atrial (LA) pressure. The stress response in AA was similar to EA. In EE, IVRT and EDT shortened (by 21 ms) and TR increased (by 13 mm Hg, p < 0.01), suggesting a rise in LA pressure. Peak aortic acceleration (LV inotropy) increased by 0.8 g in EA but only by 0.2 g in EE (difference p < 0.001). Median follow-up (interquartile range) was 34 (20 to 57) months. Three-year survival for EE, EA, and AA was 49%, 79%, and 89%, respectively (p < 0.001). Compared with AA, the hazard ratio for EE was 9.5 (p < 0.001) and for EA was 1.9 (p = 0.30). CONCLUSIONS: In ischemic cardiomyopathy, persistence of restrictive filling during stress implies a striking rise in LA pressure, greatly attenuated LV inotropic response, and markedly reduced survival. Stress echocardiography uniquely identifies these high-risk patients.

Aged↗
PubMed1996

[Left ventricular systolic dysfunction in cardiomyopathies].

Of the three physiopathological types of cardiomyopathy, dilated, hypertrophic and restrictive, it is the first which characteristically shows major left ventricular systolic dysfunction. The left ventricular volumes are increased, the ventricle becomes spherical and global ejection fraction decreases with diffuse or segmental wall motion abnormalities. The left ventricular mass is increased in an excentrical fashion with wall thinning. Isovolumic contraction is slower, the ejection time is shorter and, above all, the indices of contractility such as maximal velocity of the contractile elements ... are very abnormal and do not improve after positive inotropic stimulation.

Adrenergic beta-Antagonists↗
PubMed1997

High prevalence of mitochondrial diabetes mellitus in Japanese patients with major risk factors.

To identify diabetes mellitus caused by the mitochondrial gene substitution at genomic nucleotide pair 3243 (M3243A-->G) we selected 87 diabetic patients with high risk factors such as maternal inheritance and hearing loss. Total DNA was extracted from peripheral leukocytes, and mitochondrial DNA fragments containing M3243A-->G were amplified by polymerase chain reaction (PCR). The amplified fragments were digested with a restriction endonuclease Apa1 and analyzed by agarose gel electrophoresis. The incidence of the M3243A-->G mutation was 4.6% (four of 87) in diabetic patients with maternal inheritance and/or hearing loss. In a subgroup with both maternal inheritance and hearing loss, the incidence of the mutation was as high as 21.4% (three of 14). Cardiac disorders were also present in all four diabetic patients with the mutation. This study suggests that maternal inheritance and hearing loss are useful clinical findings to identify diabetic patients with the mutation, and that cardiac involvement is a high risk factor for the M3243A-->G mutation.

Adult↗
PubMed1996

Aspects of cardiomyopathy are exacerbated by elevated dietary fat in copper-restricted rats.

The objective of this study was to determine if a high fat diet having a 2:1 saturated-polyunsaturated fatty acid ratio exacerbates signs of copper deficiency. Male weanling Long-Evans rats were randomly placed into one of the following treatment groups: adequate copper low fat or deficient copper high fat. The levels of fat used were 31 or 12% of daily energy, and copper concentrations were 94.5 micromol/kg and <15.8 micromol/kg in the copper-adequate and copper-deficient diets, respectively. Cardiac hypertrophy as well as lower liver copper levels and superoxide dismutase activity were observed in both groups of copper-deficient rats. Irrespective of copper level, consumption of the high fat diet resulted in the thickening of the interventricular septum and left ventricular free wall. Electrocardiograms revealed that the copper-deficient high fat diet led to a significantly smaller QT interval compared with all other groups. Significantly greater S-wave voltage due to copper deficiency was observed. Significantly lower heart cytochrome c oxidase (CCO) activity was found in the copper-deficient groups with the copper deficient high fat group showing the lowest activity. Western blots of the cardiac non-myofibrillar fraction demonstrated lower amounts of CCO nuclear encoded peptides in the copper-deficient groups, with the least amount seen in the copper-deficient high fat treatment. These data suggest that a high level of dietary fat exacerbates some of the signs of copper deficiency.

Animals↗
PubMed2005

The additive prognostic value of restrictive pattern and dipyridamole-induced contractile reserve in idiopathic dilated cardiomyopathy.

BACKGROUND: Diastolic dysfunction and lack of contractile reserve are unfavorable prognostic predictors in patients with dilated cardiomyopathy (DCM). AIMS: This study aims to assess whether diastolic dysfunction and lack of dipyridamole-induced contractile reserve were additive predictors of poor outcome in patients with DCM. METHODS: A total of 116 patients with DCM and ejection fraction (EF<35%) were studied by dipyridamole echo (0.84 mg/kg over 10 min). At rest, a restrictive filling pattern was defined as: E/A ratio >2 and an E-wave deceleration time of <140 ms on transmitral flow velocity profile. RESULTS: Rest wall motion score index (WMSI) was 2.2+/-0.3 and decreased to 1.9+/-0.41 after dipyridamole (p<0.001). During follow-up (median 26.5 months), 22 cardiac deaths occurred. At multivariate analysis, dipyridamole-induced contractile reserve yielded significant incremental prognostic value (RR=0.275, p<0.006) over NYHA class (RR=1.971, p<0.03), angiotensin-converting enzyme inhibitor therapy (RR=0.173, p<0.001), and left ventricular end-diastolic diameter (RR=1.131, p<0.001). The worst prognostic combination was the presence of restrictive pattern at rest and the absence of contractile reserve (deltaWMSI<0.15). CONCLUSION: In patients with DCM, the ominous combination of restrictive transmitral flow pattern and lack of contractile reserve during dipyridamole stress predicts an unfavourable outcome.

Aged↗