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1/43. 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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2/43. 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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3/43. Intra-aortic balloon pump associated with dynamic left ventricular outflow tract obstruction after valve replacement for aortic stenosis.

    An unstable patient with critical aortic stenosis had an intra-aortic balloon pump placed preoperatively for hemodynamic support and alleviation of symptoms. After separation from cardiopulmonary bypass following aortic valve replacement, the patient was hypotensive with increased pulmonary artery pressures. Transesophageal echocardiography revealed left ventricular outflow tract obstruction associated with systolic anterior motion of the mitral valve and severe mitral regurgitation. This pathophysiology was present when ventricular systole was preceded by balloon counterpulsation, but was absent during unassisted systole. This case report demonstrates a potentially significant untoward effect of intra-aortic balloon pump augmentation after aortic valve replacement for aortic stenosis. The timely diagnosis of this iatrogenic condition in the operating room permitted the prompt implementation of appropriate management strategies and avoided unnecessary surgical intervention.
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4/43. Dynamic left ventricular outflow tract obstruction in a patient with pheochromocytoma.

    Symmetric left ventricular hypertrophy or asymmetric septal hypertrophy associated with pheochromocytoma simulating hypertrophic obstructive cardiomyopathy have been rarely reported. In this report, we present a case with pheochromocytoma that had dynamic left ventricular outflow tract obstruction without asymmetric septal hypertrophy. A surface echo revealed resolution of the systolic anterior motion of the mitral valve and all Doppler evidence of left ventricular outflow tract obstruction following removal of the tumor. Dynamic left ventricular outflow tract obstruction seen in this patient was probably due to excessive secretion of cathecolamines by the tumor.
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5/43. Transformation of mitral valve prolapse to dynamic left ventricular outflow tract obstruction and back again in a patient with acute transient myocardial depression.

    We describe an unusual case of transient resolution of preexisting mitral valve (MV) prolapse during acute cardiac dysfunction and the development of dynamic left ventricular (LV) outflow tract obstruction. The patient presented with lightheadedness, chest pain, and compromised hemodynamic status. echocardiography revealed akinesis and deformation of the LV anterior wall and apex, hyperdynamic activity in the bases, anterior MV leaflet systolic anterior motion without prolapse, and a dynamic outflow tract gradient. Myocardial function fully recovered over 1 month. Repeat ultrasonography showed posterior MV leaflet prolapse and no anterior MV leaflet systolic anterior motion. Elongated MV leaflets may have contributed to dynamic outflow tract obstruction and life-threatening hemodynamic compromise during LV conformational change.
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6/43. 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.
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7/43. coronary vasospasm inducing dynamic left ventricular outflow tract obstruction.

    An 80-year-old man was admitted to the emergency department of our institution due to acute, anterior-wall myocardial infarction and cardiogenic shock. Two-dimensional echocardiography revealed systolic anterior motion of the mitral leaflets with severe left ventricular outflow tract obstruction. Although coronary angiography showed normal coronary arteries, an ergonovine provocation test induced diffuse coronary constriction of the left coronary artery, with chest pain, and ST-T changes seen on the electrocardiogram. These clinical signs caused us to suspect coronary spasm. The present case serves as a reminder that coronary vasospasm may be a factor in the development of dynamic left ventricular outflow tract obstruction. Early detection and intensive efforts to relieve vasospasm, including emergency coronary angiography and intracoronary injection of nitroglycerin, are essential.
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8/43. Obstruction of the left ventricular outflow tract after mitral valvuloplasty with implantation of Gregori's ring.

    Mitral valvuloplasty is efficient for repairing mitral valve disease with few complications. In some cases, obstruction of the left ventricular outflow tract may occur due to systolic anterior motion of the mitral valve. We report the case of a patient with this complication and a pressure gradient between the left ventricle and the aorta of 130 mm Hg after mitral valvuloplasty with implantation of a Gregori's ring. The management was clinical with suspension of the vasoactive drugs and introduction of a beta-blocker. Two years after the surgery, the patient is asymptomatic and has a normal life.
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9/43. Dynamic left ventricular outflow tract obstruction in the setting of acute anterior myocardial infarction: a serious and potentially fatal complication?

    Dynamic left ventricular outflow tract (LVOT) obstruction was thought to be a hallmark of hypertrophic obstructive cardiomyopathy, especially in those cases with isolated asymmetric septal hypertrophy and systolic anterior motion (SAM) of the mitral valve. Recently, several authors described the occurrence of a dynamic LVOT obstruction during acute coronary insufficiency in ventricles without significant myocardial hypertrophy. The LVOT gradient was reported to disappear following resolution of the ischemic syndrome. Furthermore, it was reported that LVOT obstruction in the setting of acute myocardial infarction could predispose to cardiac rupture. We describe four cases with acute anterior myocardial infarction complicated with a dynamic LVOT obstruction documented by transthoracic Doppler echocardiogram. The detection of the dynamic LVOT obstruction allowed us to optimize the pharmacological treatment in each case. In spite of therapy, two of our patients worsened progressively to fatal cardiogenic shock and cardiac rupture. In conclusion, the development of a LVOT obstruction during acute anterior myocardial infarction has to be considered a serious and potentially fatal complication.
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10/43. Modified septal myectomy and repair of mitral valve apparatus for the treatment of hypertrophic cardiomyopathy.

    A modified technique is described of septal myectomy using a resectoscope and mitral valve repair to relieve left ventricular outflow tract obstruction and correct mitral regurgitation in a young patient with hypertrophic cardiomyopathy. This modification offers better control over the extent of septal tissue excision and may reduce the incidence of iatrogenic ventricular septal defect. Using this procedure it was possible to correct systolic anterior motion of the anterior mitral leaflet while obviating the need to replace the mitral valve.
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