HOCM (4) Takotsubo cardiomyopathy or Takotsubo syndrome (TTS), also known as stress cardiomyopathy, is a type of non-ischemic cardiomyopathy in which there is a sudden temporary weakening of the muscular portion of the heart. Because radiation is not required and the risk of MRI is low, cardiac MRI is often used to supplement evaluation by echocardiography. Note the transmural infarction of the basal septum (arrow). There is an inverse relationship between the transmural extent of hyperenhancement, and the likelihood of wall motion recovery following revascularization. Eur Radiol 2006; 16:944-951, McCrohon JA, Moon JC, Prasad SK, McKenna WJ, Lorenz CH, Coats AJ, Pennell DJ. Images can be enlarged by clicking on them. Hypertrophic cardiomyopathy (HCM) is characterized by a hypertrophied left ventricle, defined as diastolic wall thickness 15mm or more, without any identifiable cause such as hypertension or valvular disease. In nonischemic heart failure a history of hypertension, diabetes and excessive alcohol intake may be present. Non Ischemic cardiomyopathy is defined as a myocardial disorder in which the heart muscle is structurally and functionally abnormal, in the absence of other causes of heart dysfunction, like coronary artery disease, hypertension, valvular disease and congenital heart disease. AJR Am J Roentgenol 2007; 189:1344-1352, Vanden Driesen RI, Slaughter RE, Strugnell WE. The modified Mayo Clinic criteria for diagnosis of takotsubo cardiomyopathy: On the left an angiogram of a patient with Tako-Tsubo cardiomyopathy. If MRI and other tests are negative, patients return yearly for follow-up MRI. MR imaging of ischemic heart disease. However, more obvious morphologic changes of dilatation and aneurysm formation are much more common in the right heart than the left. More than 800 different mutations have been identified in at least 11 contraction-encoding genes in HCM. On the left a short axis movie in a patient with ARVC. In normal myocardium there will be early wash out of contrast. Cardiovascular magnetic resonance, fibrosis, and prognosis in dilated cardiomyopathy. Dilated cardiomyopathy (DCM) Dilated cardiomyopathy (DCM) is defined as dilation of one or both ventricles. For patients with suspected hypertrophic cardiomyopathy, MRI may detect abnormal wall thickness that is otherwise missed by echocardiography (discussed later). Late enhancement image shows enhancement over the entire subendocardial circumference, variably extending into the neighboring myocardium . Usually there is asymmetric thickening of the wall most prominently involving the ventricular septum without abnormal enlargement of the ventricular cavities. J Am Coll Cardiol 2003; 42:895-901, Kim RJ, Wu E, Rafael A, Chen EL, Parker MA, Simonetti O, Klocke FJ, Bonow RO, Judd RM. MRI can show segmental hypokinesis, dilatation, fatty infiltration in the right ventricular myocardium, small aneurysms and late enhancement of the myocardium [5,27]. The first is noncoronary artery distribution. Using T2*-weighted imaging, the presence of significant amounts of iron in the heart can be determined. If the transmural extent of late enhancement is less than 50% the function is likely to improve after revascularization . On the far left a 3-chamber late enhancement image which nicely demonstrates the enhancement of the hypertrophic basal septum (arrow). However, individual ICD trials … This enhancement is explained by the presence of fibrosis. In injured myocardium the wash out is very slow resulting in delayed enhancement after 10 - 15 minutes compared to the normal myocardium. Both conditions have been identified as having a genetic basis and both are associated with sudden cardiac death. In nonischemic myocardial disease the delayed enhancement usually does not occur in a coronary artery distribution and is often midwall or epicardial rather than subendocardial or transmural. Myocarditis (2) Fat infiltration is seldom the only abnormality seen on MRI in ARVC, it should coincide with right ventricular regional dysfunction . The arrow points to the hypertrophic basal septum. Subgroup analysis in non-CRT patients showed that ICD use reduced sudden cardiac death by 73% (RR, 0.27; 95% CI, 0.15-0.50; p . The myocardium has a very limited response to cellular injury and can be replaced by either fibrosis or fat tissue. The underlying abnormality in HCM is a mutation involving sarcomere contractile proteins. Patients with nonischemic cardiomyopathy may be managed medically, but myocardial transplantation or defibrillator implantation may be the only options in certain severe cardiomyopathies. However, in a small number of people with HCM, the … Because of the adverse risk of the condition, cardiologists may aggressively pursue the diagnosis of ARVD when other tests, including ECG, Holter monitor, and echocardiography, are normal. No reflow zones are identified on late-enhancement images as a dark core surrounded by an enhancing rim. The definitions of DCM and ischemic cardiomyopathy, with the latter defined as a “dilated cardiomyopathy with impaired contractile performance not explained by the extent of the coronary artery disease or ischemic damage,” have been controversial in the past. Role of magnetic resonance imaging in arrhythmogenic right ventricular dysplasia: insights from the North American arrhythmogenic right ventricular dysplasia (ARVD/C) study. Finally, there are other tissue characteristics that can be detected by MRI for nonischemic cardiomyopathy. We will discuss the cardiomyopathies listed in the table on the left. The presence of a 'no reflow' zone is associated with worse functional outcome, larger infarcts and adverse clinical outcome [8,9]. Transmural enhancement of the inferior wall, which can be diagnosed as an old infarction (yellow arrow). MR scans may be overinterpreted since the RV has substantial normal variations including variable trabeculation and small outward bulges near the insertion of the moderator band. Circulation 2006; 113:1807-1816, Elliott P, Andersson B, Arbustini E, et al. Dilated cardiomyopathy (2) In summary, cardiac MRI readily visualizes all portions of the myocardium with high spatial resolution with the use of steadystate free precession cine imaging. When the cause of the myocardial dysfunction is unknown, a major benefit of MRI is to help separate an ischemic (atherosclerotic) cause from a nonischemic cause. If scar is absent, a further explanation for the patient's dysfunction must be sought. If biventricular disease is present, other causes for myocardial dysfunction should be sought. The cine images show: On the left the long axis late enhancement image in the same patient. Am Heart J 2008; 155:147-153, McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist C, Fontaine G, Camerini F. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. On the left an end-diastolic image. If a person is diagnosed to have early stage non-ischemic cardiomyopathy, then his life expectancy is much better because proper treatment begins earlier. Implantable cardioverter-defibrillator (ICD) therapy has been the primary intervention for managing individuals at high risk for SCD. Nonischemic cardiomyopathy is damage to the heart muscle that is not associated with interruptions to the heart's blood supply, as seen in cases of coronary artery disease. J Am Coll Cardiol 2003; 41:1561-1567, Adabag AS, Maron BJ, Appelbaum E, Harrigan CJ, Buros JL, Gibson CM, Lesser JR, Hanna CA, Udelson JE, Manning WJ, Maron MS. The thickened heart muscle can make it harder for the heart to pump blood.Hypertrophic cardiomyopathy often goes undiagnosed because many people with the disease have few, if any, symptoms and can lead normal lives with no significant problems. A multidisciplinary team that is expert in understanding the manifestations of ARVD is helpful to avoid a false-positive MRI diagnosis . Thus, it is difficult to determine the accuracy of MRI for the diagnosis. Just to review that, I think the salient points, younger 60-year-old man, non-ischemic, dilated cardiomyopathy. The main types of this condition include dilated, hypertrophic, and restrictive cardiomyopathy. Other MRI characteristics of importance are the imaging correlates of histologically identified fibrofatty infiltration. et al Radiographics 2006; 26:795-810, White JA, Patel MR. Our current concept of the disease is that early ARVD is manifested by subtle contraction abnormalities that may involve both the left and right ventricles. Br Heart J 1994; 71:215-218, Mahrholdt H, Goedecke C, Wagner A, Meinhardt G, Athanasiadis A, Vogelsberg H, Fritz P, Klingel K, Kandolf R, Sechtem U. Cardiovascular Magnetic Resonance Assessment of Human Myocarditis. Emerging evidence suggests that myocardial scar is often the genesis for cardiac arrhythmia that can be life threatening . Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. Nonischemic cardiomyopathy may be either primary (confined to the heart) or secondary to systemic diseases (Table 1), and many of these conditions recently have been extensive reviewed [2–4]. For patients with a prior myocardial infarction, myocardial scar is readily identified as transmural or subendocardial focal regions of delayed enhancement. 4). Hypertrophic cardiomyopathy (HCM) is a disease in which the heart muscle (myocardium) becomes abnormally thick (hypertrophied). Thus, whereas nonischemic myocardial disease usually can be distinguished from ischemic disease, determining the particular cause of nonischemic cardiomyopathy requires clinical correlation and often myocardial biopsy. MR findings in cardiac amyloidosis. According to the guidelines of ACC/AHA/HRS 2008  there is an indication for an automated implantable cardioverter-defibrillator (AICD) if: On the left the 4-chamber view of a patient with the idiopathic dilated cardiomyopathy. MRI can differentiate between those two diagnoses: On the left the 4-chamber movie in a patient with constrictive CM. 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