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1/240. Pacemaker therapy in a pediatric patient with hypertrophic obstructive cardiomyopathy and rapid intrinsic atrioventricular conduction.

    A 13-year-old boy with hypertrophic obstructive cardiomyopathy was treated with dual-chamber pacing after severe progression of left ventricular outflow tract obstruction and of clinical symptoms despite drug therapy. Rapid intrinsic atrioventricular conduction was overcome and complete preexcitation of the septum achieved by omitting atrial sensing and programming constant atrial pacing with a short atrioventricular delay of 70 msec. After 8 weeks of therapy, a reduction of the left ventricular outflow tract gradient from 125 to 16 mmHg and remodeling of the left ventricle were demonstrated.
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ranking = 1
keywords = outflow, obstruction
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2/240. Fatal neonatal hypertrophic cardiomyopathy with severe stenosis of the right ventricular outflow tract: echocardiographic diagnosis and potential therapy.

    We discuss the clinical course of aneonate with idiopathic hypertrophic cardiomyopathy who showed rapid progression of stenosis of the right ventricular outflow tract and reduction in size of the right ventricular cavity due to thicken ing of the ventricular septum. Medical treatment proved unsuccessful. We suggest that balloon atrial septostomy combined with construction of a Blalock-Taussig shunt may be effective in view of the hemodynamics, which mimic pulmonary atresia with intact interventricular septum.
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ranking = 2.3601372172682
keywords = outflow
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3/240. Subaortic obstruction after the use of an intracardiac baffle to tunnel the left ventricle to the aorta.

    Postoperative hemodynamic studies in five patients document subaortic obstruction after surgical repair utilizing an intracardiac baffle to establish continuity between the left ventricle and the aorta. Four of the patients had a Rastelli procedure for D-transposition of the great arteries with a ventricular septal defect and pulmonary stenosis; one patient had repair of double outlet right ventricle with a ventricular septal defect and pulmonary stenosis. The left ventricular outflow was shown to be a long narrow tunnel by angiography in four of five patients and by echocardiography in one patient. Resting aortic peak systolic pressure gradient ranged from 10 to 42 mm Hg (mean 24). The obstruction was localized to the proximal end of the left ventricule to aorta tunnel (i.e., at the site of ventricular septal defect) in five patients. One patient with a gradient of 42 mm Hg has angina and decreased exercise tolerance. Subaortic obstruction is a newly described sequelae after the Rastelli procedure for transposition or repair of double outlet right ventricle. The obstruction may be hemodynamically significant and should be searched for at postoperative cardiac catheterization.
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ranking = 0.91958834819548
keywords = outflow, obstruction
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4/240. Persistent ST segment elevation: a new ECG finding in hypertrophic cardiomyopathy.

    Hypertrophic cardiomyopathy is a primary disease of myocardium resulting in myocardial hypertrophy without any inciting pressure or volume overload. The typical triad of symptoms includes exertional angina, syncope, and shortness of breath. Sudden cardiac death, the most dreadful complication of this disorder, can be the first manifestation of the disease and is more common in young patients. Elderly patients, on the other hand, may have a relatively benign course with normal or near-normal life span. The electrocardiogram (ECG) and echocardiography are the two most useful measures to diagnose hypertrophic cardiomyopathy. The electrocardiographic features of hypertrophic cardiomyopathy are numerous, including ST segment elevation that may simulate other ST segment elevation syndromes, including acute myocardial infarction, variant angina pectoria, acute pericarditis, bundle branch blocks, ventricular paced rhythm, dyskinetic ventricular segment, ventricular aneurysm, left ventricular hypertrophy, wolff-parkinson-white syndrome, and early repolarization syndrome. This report describes a case of an asymptomatic patient who presented with ST segment elevation of acute injury type and, therefore, was admitted to rule out silent myocardial infarction. myocardial infarction was ruled out by cardiac enzyme levels, but ST segment elevation remained persistent in all of the subsequent ECGs. echocardiography was performed, which clearly showed hypertrophic cardiomyopathy with left ventricular outflow tract obstruction and a high intracavity pressure gradient. Subsequently, retrieval of old ECGs showed a similar type of ST segment elevation in the patient's previous ECGs.
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ranking = 0.52797255654637
keywords = outflow, obstruction
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5/240. Hypertrophic cardiomyopathy in Friedreich's ataxia.

    The cardiac findings in two sibs with Friedreich's ataxia are described. The clinical signs were suggestive of hypertrophic obstructive cardiomyopathy. During left heart catheterization a systolic pressure gradient across the left ventricular outflow tract could be provoked by an infusion of isoprenaline. Left ventricular angiocardiograms and echocardiograms showed gross thickening of the interventricular septum. In one patient a systolic anterior movement of the anterior leaflet of the mitral valve was seen. The importance of serial echocardiographic examination for patients with Friedreich's ataxia is emphasized.
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ranking = 0.47202744345363
keywords = outflow
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6/240. Pre- and postoperative echocardiographic features of discrete subaortic stenosis.

    In two patients with discrete membranous subaortic stenosis, partial early systolic closure of the aortic valve was noted on the preoperative record. Postoperatively, this abnormality was found to be less pronounced. Narrowing of the left ventricular outflow tract was seen in the preoperative tracing in each patient. Echocardiograms taken after resection of the subaortic membrane showed widening of the left ventricular outflow tract as compared with the preoperative tracing. Thus, echocardiography may be of value in distinguishing between discrete subaortic stenosis and other forms of left ventricular outflow tract obstruction.
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ranking = 1.4720274434536
keywords = outflow, obstruction
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7/240. pericardium-covered stent for septal myocardial ablation in hypertrophic obstructive cardiomyopathy.

    This report describes a patient with severe hypertrophic obstructive cardiomyopathy in new york Heart association functional class III. Complete reduction of left ventricular outflow tract gradient was achieved by the selective occlusion of three target septal arteries with a pericardium-covered stent. The patient's in-hospital course was uneventful and has improved to functional class I.
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ranking = 0.47202744345363
keywords = outflow
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8/240. 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.
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ranking = 3.4475609047418
keywords = outflow, obstruction
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9/240. Non-surgical reduction of septal myocardial mass in a patient with hypertrophic obstructive cardiomyopathy.

    Non-surgical reduction of septal myocardial mass was performed with success in a 48-year-old male with hypertrophic obstructive cardiomyopathy. angioplasty balloon inflated in the first septal branch of the left anterior descending artery dramatically reduced the pressure gradient across the outflow tract of the left ventricle. Following injections of absolute alcohol into the artery with the balloon kept inflated induced a small septal myocardial infarction. By hemodynamic evaluation, the peak pressure gradient of 108 mmHg before the procedure was decreased to 30 mmHg. Clinical improvement in new york Heart association (NYHA) functional class was obtained. At 3-month follow-up, the patient was doing quite well; the gradient was only 10 mmHg at rest and 25 mmHg with valsalva maneuver.
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ranking = 0.47202744345363
keywords = outflow
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10/240. 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.
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ranking = 46.24393048769
keywords = outflow obstruction, outflow, obstruction
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