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Diabetes mellitus-related cardiomyopathy (DMCMP) was originally described as a dilated phenotype with eccentric left ventricular (LV) remodelling and systolic LV dysfunction. Recently however, clinical studies on DMCMP mainly describe a restrictive phenotype with concentric LV remodelling and diastolic LV dysfunction. Both phenotypes are not successive stages of DMCMP but evolve independently to respectively heart failure with preserved left ventricular ejection fraction (HFPEF) or reduced left ventricular ejection fraction (HFREF). Phenotype-specific pathophysiological mechanisms were recently proposed for LV remodelling and dysfunction in HFPEF and HFREF consisting of coronary microvascular endothelial dysfunction in HFPEF and cardiomyocyte cell death in HFREF. A similar preferential involvement of endothelial or cardiomyocyte cell compartments explains DMCMP development into distinct restrictive/HFPEF or dilated/HFREF phenotypes. Diabetes mellitus (DM)-related metabolic derangements such as hyperglycaemia, lipotoxicity, and hyperinsulinaemia favour development of DMCMP with restrictive/HFPEF phenotype, which is more prevalent in obese type 2 DM patients. In contrast, autoimmunity predisposes to a dilated/HFREF phenotype, which manifests itself more in autoimmune-prone type 1 DM patients. Finally, coronary microvascular rarefaction and advanced glycation end-products deposition are relevant to both phenotypes. Diagnosis of DMCMP requires impaired glucose metabolism and exclusion of coronary, valvular, hypertensive, or congenital heart disease and of viral, toxic, familial, or infiltrative cardiomyopathy. In addition, diagnosis of DMCMP with restrictive/HFPEF phenotype requires normal systolic LV function and diastolic LV dysfunction, whereas diagnosis of DMCMP with dilated/HFREF phenotype requires systolic LV dysfunction. Treatment of DMCMP with restrictive/HFPEF phenotype is limited to diuretics and lifestyle modification, whereas DMCMP with dilated/HFREF phenotype is treated in accordance to HF guidelines., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy postulated to be a defect in endomyocardial morphogenesis due to the embryonic arrest of the compaction of myocardial fibers. It could be isolated, without other structural heart defects; or associated with congenital heart defects. It is characterized by prominent ventricular myocardial trabeculations and deep intertrabecular recesses. The clinical manifestations, i.e., heart failure, arrhythmias or thromboembolism, overlap with those of other cardiac disorders. It is often misdiagnosed as restrictive or dilated cardiomyopathy. The high mortality and morbidity associated with it and familial occurrence make diagnosis important. Only 3 pediatric cases have been reported from India. We present 2 cases, that of an 11-year-old girl (familial case) with embolism (documented but rare in children) and atrial flutter (not yet reported), with mother having asymptomatic LVNC; and that of a 4-month-old girl. Both presented with heart failure. The 11-year-old child had sudden death, known to occur in LVNC.

Objective: To explore the imaging and related clinical characteristics of magnetic resonance (MR) delayed enhancement in patients with ischemic or nonischemic heart disease., Methods: Thirty-two cases who underwent MR myocardial cine and delayed enhancement imaging from January 2004 to October 2006 were retrospectively analyzed. The cine sequence imaging included the four-chamber view and the left ventricular short axis view. The delayed enhancement imaging was taken 10 minutes after the infusion of gadolinium from the antecubital vein with a segmented inversion-recovery-prepared T1-weighted fast gradient echo sequence. Patients underwent coronary computed tomography angiography (CTA) two weeks before or after the MR imaging examination. Combined with clinical history, the clinical and MR imaging characteristics of the patients who had delayed enhancement were analyzed., Results: MR delayed enhancement could be found in 16 cases. Among them, 12 cases had ischemic heart disease. Their coronary CTA showed one to three vessel diseases. The delayed enhancement was transmural or subendocardium, and the area of delayed enhancement corresponded well with one or more coronary arteries which had severe stenosis or occlusion. Four cases had nonischemic heart diseases. One case was dilated cardiomyopathy, with diffuse small midwall spots in delayed enhancemen and only 30% stenosis of the anterior descending coronary artery in coronary CTA. One case was hypertrophic cardiomyopathy, with delayed enhancement of strip- and patch-shaped at midwall of the hypertrophic myocardium. One case was restrictive cardiomyopathy, and the delayed enhancement was located in the area of subendocardium of both the right and left ventricles. Coronary CTA of these two cases were normal. The other case was a mass of the lateral wall of the left ventricle, and the delayed enhancement with a clumpy shape was located in the lateral wall of the left ventricle., Conclusions: MR myocardial delayed enhancement is not a specific sign of myocardial infarction of ischemic heart disease. Nonischemic heart diseases including all kinds of primary cardiomyopathy and some other diseases affecting myocardium can also cause delayed enhancement, but their characteristics are different. The differentiation of the etiology of the nonischemic heart disease with delayed enhancement relies upon the intimate connection with clinical history and the cine sequence MR images.

Cardiomyopathies include a wide variety of cardiac diseases. The value of MR imaging is not only to provide information about cardiac function, but also to detect the underlying cause of the disease.

Cardiomyopathies are diseases of the myocardium of unknown etiology associated with cardiac dysfunction. On the grounds of their morphology and pathophysiology, primary or idiopathic cardiomyopathies may be classified into a number of disorders; namely, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy, and restrictive cardiomyopathy. The term "secondary cardiomyopathies" is reserved to specific heart muscle diseases clinically very similar to primary cardiomyopathies. Cardiac magnetic resonance imaging has long been used to study cardiac morphology and, more recently, to assess blood flow, perfusion, and contractile function. The emerging role of magnetic resonance imaging for the understanding and treatment of primary cardiomyopathies cannot be underestimated. From a clinical point of view, an examination based on a single, efficient, and noninvasive MR study focusing on the clinically relevant features of cardiomyopathies is an objective and reproducible means for diagnosing and monitoring hypertrophic, arrhythmogenic, dilated, and restrictive cardiomyopathies.

These heart muscle diseases, associated with systolic or diastolic dysfunction, are associated with a high prevalence and morbidity, because of the frequent evolution towards congestive heart failure or sudden death. Dilated cardiomyopathy is the first cause of heart transplantation and Hypertrophic cardiomyopathy is the first cause of sudden death in athletes. Familial and monogenic forms of these diseases are frequent and this should lead to a systematic cardiological familial inquest in first degree relatives of a patient. Although they were previously called as "idiopathic", the pathophysiology of the disease is actually under investigation thanks to molecular genetics. This completely new knowledge should lead to the development of new therapeutic strategies.

Three major types of cardiomyopathies affect adolescents: dilated, hypertrophic, and restrictive. At the present time the etiologies of the majority of these cardiomyopathies in children remain elusive. Treatment of these diseases is generally directed toward improving symptoms, survival, myocardial performance, and hemodynamics. Most treatment strategies are extrapolated from studies in adults with these types of cardiomyopathies, although prospective, randomized therapeutic trials are currently underway in children. All three types of cardiomyopathy can be associated with a wide range of symptoms (from none to severe) and also are associated with sudden death. For those in whom medical management fails, heart transplantation is a therapeutic option with an excellent intermediate-term success rate.

Two new noninvasive imaging modalities, electron beam computed tomography (EBCT) and magnetic resonance imaging(MRI), have emerged in recent years and have provided images of the heart with high temporal and spatial resolution. They provides accurate information of myocardial thickness, ventricular shape and volume, wall motion, and ventricular function in patients with cardiomyopathy. In addition, myocardial damage can be evaluated using contrast media. They can give unique diagnostic information above and beyond that of more traditional noninvasive modalities such as two-dimensional echocardiography, left ventriculography and radionuclide technique because both modalities acquire images in three dimensions. They have a possibility to become an alternative modality of the left ventriculography in the diagnosis of cardiomyopathy.

Dilated cardiomyopathy is characterized by systolic dysfunction and cardiac enlargement of unknown origin. Various Doppler modalities are useful to detect and quantitate atrioventricular regurgitation, which is common and contributes to clinical symptoms. Pulsed Doppler assessment of mitral and tricuspid inflow velocities shows a spectrum of findings indicative of abnormal diastolic function and hemodynamic status. When mitral regurgitation is more than moderate and heart failure is severe, the ratio between early inflow E wave to atrial inflow A wave peak velocities is increased. Mitral deceleration time may be short. When mitral regurgitation is trivial and left atrial pressure is not increased, abnormal relaxation may be detected as a low E:A ratio. Mitral deceleration time and isovolumic relaxation time are prolonged. In restrictive cardiomyopathy, there is an abrupt limitation in early ventricular filling due to abnormal compliance of endocardial or endomyocardial origin. Mitral and tricuspid inflow velocities show normal to increased early peak velocity, rapid deceleration time, low peak atrial velocity, and an increased E:A ratio. Differentiation between restriction and constriction might be possible by the demonstration in pericardial constriction of inspiratory decreases in mitral early inflow peak velocities and in prolongation of isovolumic relaxation time, with reciprocal changes on tricuspid inflow velocity profiles. In constriction, these respiratory variations are caused by the ventricular limitation to accommodate changes in venous return due to the pericardial shell. Doppler abnormalities and two-dimensional echocardiographic assessment of ventricular and atrial size and ejection fraction provide the practicing physician with valuable diagnostic information.

As many as 60 patients with hypereosinophilia of different genesis were examined. 40 of them manifested the clinical (congestive heart failure in 6, tachycardia in 10, cardialgias in 3, murmur in the heart in 13, pericardial murmur in 3) and/or ECG signs (disorders of repolarization in 25, His bundle block in 4, rhythm disorders in 7, pathologic Q waves in 2) of heart injury. In 15 out of the 20 examined, the echocardiography data corresponded to the dilated pattern of heart injury despite the fact that the changes in contractile function of the left ventricle were less appreciable than in 28 patients with dilated cardiomyopathy. In 2 patients who died (one from congestive heart failure and one from anaphylactic shock) the presence of endomyocardial fibrosis with heart dilatation was confirmed on postmortem examination. One female patient with right ventricular failure manifested the characteristic signs of isolated injury to the right ventricle. In 3 patients, the endocardium appeared thickened. None of the cases showed the signs of restrictive heart injury. The data obtained indicate that hypereosinophilia may be one of the etiologic factors of dilated cardiomyopathy.

The study comprised 14 patients with systemic lupus erythematosus (SLE) and 12 patients with diffuse scleroderma. Echocardiography ascertained 5 cases of congestive and 1 case of restrictive cardiomyopathy among the patients with SLE, as well as 6 cases with restrictive cardiomyopathy among those with scleroderma. The authors specify two important echocardiographic variables: the E-septum distance and the septal and wall motion after amyl-nitrite test, which reveal the intrinsic contractile properties of the myocardium, allowing an early diagnosis of myocardial impairment, as well as the selection of patients for an efficient treatment with vasodilator drugs.

Congestive cardiomyopathy was recognized in eight patients with only mildly dilated ventricles (an echocardiographic ventricular diastolic dimension index of less than 10% to 15% above the normal range) but with other features typical of end-stage congestive cardiomyopathy. Such patients with mildly dilated cardiomyopathy (MDCM) represented 5% of heart transplant recipients with cardiomyopathy, who were analyzed by us. The clinical, echocardiographic, and hemodynamic data, as well as the gross and microscopic pathologic findings of the hearts in this group were compared with similar data in five patients with idiopathic restrictive cardiomyopathy (IRCM) and 10 patients with typical dilated cardiomyopathy (DCM). Compared with IRCM and DCM, patients with MDCM had a higher incidence of familial cardiomyopathy (p less than 0.009). The echocardiographic features of patients with MDCM and DCM were virtually identical, showing globular hearts with predominant left ventricular dilation and diffuse poor left ventricular contraction. Patients with IRCM had marked atrial dilation but less abnormality of left ventricular size and contraction parameters. On the basis of echocardiographic data, patients with IRCM and MDCM could be well segregated. Cardiac index and light microscopic examinations were similar in the three groups. However, electron microscopy showed a lack of myofibrillar loss in patients with IRCM, little or no myofibrillar loss in those with MDCM, and almost total myofibrillar loss in those with DCM. Patients with MDCM have a high prevalence of familial cardiomyopathy. Absolute heart size and electron microscopic features of the MDCM group were intermediate between IRCM and DCM, but other clinical, echocardiographic and hemodynamic findings were similar to typical DCM.

Using the records linkage system of the Mayo Clinic and of the Rochester Epidemiology Project, which accesses diagnostic data on the entire population of Olmsted County, Minnesota, we identified 45 new cases of idiopathic dilated cardiomyopathy (DCM) and 19 new cases of hypertrophic cardiomyopathy (HCM) among county residents for the years 1975-1984. Overall age- and sex-adjusted incidence rates were 6.0/100,000 and 2.5/100,000 person-years, respectively. The incidence of DCM doubled from 3.9/100,000 in the first 5 years to 7.9/100,000 person-years in the last 5 years of study. The corresponding change for HCM was from 1.4 to 3.6/100,000 person-years. Age- and sex-adjusted prevalence rates as of January 1, 1985, for DCM and HCM were 36.5/100,000 and 19.7/100,000 population, respectively. The prevalence of DCM in persons less than 55 years old was 17.9/100,000, over a third of whom were New York Heart Association functional Class III or IV at diagnosis. These estimates may be of value in determining the potential use of health care resources, particularly cardiac transplantation.