1/55. Rapid progression of cardiomyopathy in mitochondrial diabetes.Cardiac involvement and its clinical course in a diabetic patient with a mitochondrial tRNA(Leu)(UUR) mutation at position 3243 is reported in a 54-year-old man with no history of hypertension. At age 46, an electrocardiogram showed just T wave abnormalities. At age 49, it fulfilled SV1 RV5 or 6>35 mm with strain pattern. At age 52, echocardiography revealed definite left ventricular (LV) hypertrophy, and abnormally increased mitochondria were shown in biopsied endomyocardial specimens. He was diagnosed as having developed hypertrophic cardiomyopathy associated with the mutation. However, at age 54, SV1 and RV5,6 voltages were decreased, and echocardiography showed diffuse decreased LV wall motion and LV dilatation. Because he had mitochondrial diabetes, the patient's heart rapidly developed hypertrophic cardiomyopathy, and then it seemed to be changing to a dilated LV with systolic dysfunction. Rapid progression of cardiomyopathy can occur in mitochondrial diabetes.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
2/55. Apical hypokinesis in a patient with hypertrophic cardiomyopathy and myocardial bridging: reversal with beta-blockade--a case report.A 42-year-old man presented with effort angina pectoris of 20 minutes' duration. Hypertrophic obstructive cardiomyopathy, severe myocardial bridging involving the midleft anterior descending coronary artery, and apical hypokinesis were identified. Regional wall motion normalized following the initiation of beta blockade.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
3/55. Fixed left ventricular outflow tract obstruction in presumed hypertrophic obstructive cardiomyopathy: implications for therapy.BACKGROUND: A subset of patients presenting with a presumed diagnosis of hypertrophic obstructive cardiomyopathy (HOCM) have a fixed left ventricular outflow tract (LVOT) obstruction. Recognition of this pathophysiologic abnormality is important in choosing therapy. methods: Of patients referred for treatment of HOCM, 4 had fixed LVOT obstruction. Clinical and echocardiographic data and surgical findings were reviewed. RESULTS: In the 4 patients with clinical features consistent with HOCM or HOCM-like conditions, echocardiography showed fixed LVOT obstruction with an early-peaking LVOT Doppler signal or absence of severe systolic anterior motion of the mitral valve. The causes of fixed obstruction included accessory mitral tissue with associated fibrous ring (1 patient), fixed subaortic tunnel stenosis (2 patients), and a discreet subaortic ridge (1 patient). After surgical relief of the fixed LVOT obstruction, all patients had relief of the ventricular outflow tract gradient. CONCLUSIONS: Not all patients with a presumed diagnosis of HOCM have isolated dynamic LVOT obstruction but may have isolated or additional fixed obstruction. Careful two-dimensional and Doppler echocardiography are needed to identify this subset of patients who are best treated surgically.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
4/55. Dynamic outflow obstruction due to the transient extensive left ventricular wall motion abnormalities caused by acute myocarditis in a patient with hypertrophic cardiomyopathy: reduction in ventricular afterload by disopyramide.A 65-year-old woman was admitted to the coronary care unit because of acute pulmonary edema. Immediate 2-dimensional and Doppler echocardiograms revealed extensive left ventricular wall motion abnormalities and left ventricular hypertrophy with extreme outflow obstruction. Although an ECG showed ST-segment elevation in the anterolateral leads, a coronary arteriogram revealed normal epicardial arteries. heart failure was relieved after diminishing the dynamic outflow obstruction with disopyramide administration. An endomyocardial biopsy from the right ventricle on the 8th hospital day showed borderline myocarditis. Wall motion abnormalities gradually normalized within 2 weeks. It is speculated that her pulmonary edema would not have been relieved so readily without the immediate reduction in ventricular afterload by disopyramide. These clinical changes over time were observed with serial echo-Doppler examinations.- - - - - - - - - - ranking = 6keywords = motion (Clic here for more details about this article) |
5/55. diagnosis of apical hypertrophic cardiomyopathy using magnetic resonance imaging.Apical hypertrophic cardiomyopathy is an uncommon variant of non-obstructive hypertrophic cardiomyopathy with low prevalence outside East asia. A case is reported of a non-Asian (European) 51 year old man with characteristic ECG and morphological changes of apical hypertrophic cardiomyopathy. Although the patient underwent catheterisation three years previously because of suggested coronary ischaemic heart disease, apical hypertrophic cardiomyopathy was not diagnosed. More recently, a regional wall motion abnormality was noticed at the apex on echocardiography. To rule out an ischaemic injury a stress perfusion scintigraphy was performed; no perfusion defect was present but an apical tracer enhancement was noted. Further evaluation by magnetic resonance imaging revealed the pathognomonic "ace of spades" configuration of the left ventricle with systolic obliteration of the apical region, which led to the diagnosis of apical hypertrophic cardiomyopathy.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
6/55. Hypertrophic obstructive cardiomyopathy. Assessment by echocardiographic and Doppler ultrasound techniques.Hypertrophic obstructive cardiomyopathy is a disease of the myocardium that can be assessed echocardiographic and transcutaneous Doppler ultrasound techniques. Four patients are presented with various patterns of the disease, and the frequently familial incidence is illustrated. The importance of ultrasonic evidence for asymmetric septal hypertrophy in all stages is emphasized and evidence of reduced septal contractility demonstrated. Abnormalities of mitral valve motion, slow diastolic closure rate and systolic anterior movement of the anterior leaflet, are shown in the obstructive form of the disease. Also partial mid-systolic aortic valve closure and aortic cusp flutter are shown with outflow obstruction. The outflow tract gradient can be calculated from mitral valve to septum systolic distances. Transcutaneous Doppler ultrasound shows a normal aortic velocity pattern in nonobstructive disease while consistent abnormalities are present with severe resting obstruction. Isoprenaline can be used to alter the normal velocity pattern associated with a minimal resting gradient to an abnormal pattern indicating the development of significant obstruction.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
7/55. Multislice cardiac spiral CT evaluation of atypical hypertrophic cardiomyopathy with a calcified left ventricular thrombus.We report a case of a 43-year-old male patient with an atypical nonobstructive hypertrophic cardiomyopathy and a calcified left ventricular thrombus, and present results of multislice computed tomography (MSCT) using retrospective electrocardiograph gating, which is a new modality in cardiac imaging. Obtaining virtually motion-free images with a temporal resolution of 250 ms in an optimized heart scan MSCT allows functional imaging with evaluation of impaired systolic and diastolic left ventricular wall motion.- - - - - - - - - - ranking = 2keywords = motion (Clic here for more details about this article) |
8/55. Anteroapical stunning and left ventricular outflow tract obstruction.Dynamic left ventricular outflow tract (LVOT) obstruction is typically observed in the setting of hypertrophic cardiomyopathy. It has also been reported with concentric LV hypertrophy, excessive sympathetic stimulation, and acute myocardial infarction. We describe 3 patients with chest discomfort after emotional stress, who had pronounced abnormalities on electrocardiograms, insignificant obstructive coronary disease and hemodynamic instability with LVOT obstruction, and regional wall motion abnormalities. Suppression of contractility with beta-blockers resulted in resolution of the gradient and in clinical improvement. On follow-up, functional recovery was excellent, and ventricular function had normalized. The conditions and mechanisms that may produce this sequence of events are discussed. The most probable scenario is that an acute ischemic insult secondary to vasospasm, LV stunning, and acute geometric remodeling produced a substrate for LVOT obstruction that was exacerbated by basal LV hypercontractility. The importance of this observation is that routine treatment of cardiogenic shock cannot be used and that conservative management results in excellent prognosis.- - - - - - - - - - ranking = 2keywords = motion (Clic here for more details about this article) |
9/55. Quantitation of localized abnormal deformation in asymmetric nonobstructive hypertrophic cardiomyopathy: a velocity, strain rate, and strain Doppler myocardial imaging study.We report a case of a 10-year-old child with nonobstructive hypertrophic cardiomyopathy in whom two-dimensional echocardiography showed asymmetric septal hypertrophy with a localized thickening in the mid-septal segment. Systolic regional longitudinal motion and deformation indices were quantified by the new ultrasound-based parameters velocity, strain rate, and strain. Regional longitudinal myocardial function indices were normal for the basal and apical septal segments. The deformation parameters strain rate and strain (not the regional velocity profile) were abnormal only in the hypertrophied mid-septal segment with myofibril disarray. The concepts and advantages and clinical implications behind this quantitative approach to localizing and quantifying areas of abnormal deformation related to such myocardial disarray in localized hypertrophy are discussed.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
10/55. Perforated aneurysms of left side valves during active infective endocarditis complicating hypertrophic obstructive cardiomyopathy.The most frequent site of vegetative lesion in patients with hypertrophic cardiomyopathy is anterior mitral leaflet, due to chronic endocardial trauma arising from systolic anterior motion. We describe three cases of serious infective endocarditis complicated lesions (vegetation, aneurysm and perforation) on aortic and mitral valves, in patients with obstructive hypertrophic cardiomyopathy. In particular, we observed how severe valvular damage and dysfunction, combined with particular hemodynamic conditions, are followed by adverse clinical outcome. We performed transthoracic echocardiogram and transoesophageal echocardiography studies to define morphologic and hemodynamic features of infection, deciding the proper therapy and we planned the echocardiographic follow-up.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
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