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1/7. 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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2/7. Emergency correction of coagulation for mitral valve replacement in an orally anticoagulated 17-year-old patient with pronounced hepatic dysfunction.

    A 17-year-old patient with Shone's disease had to be readmitted to the hospital 3 months after implantation of an artificial aortic valve because of extreme mitral insufficiency with consecutive pulmonary edema and hepatic dysfunction. He had been orally anticoagulated and presented with a high international normalized ratio of 6.7. Emergency replacement of the mitral valve was possible only after administration of prothrombin-complex concentrate, as vitamin k(1) and fresh frozen plasma did not correct the hemostatic defect sufficiently.
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3/7. Dynamic left ventricular outflow tract obstruction: an unusual mechanism mimicking anterior myocardial infarction with cardiogenic shock.

    Cardiogenic shock is a frequent and threatening complication in the course of acute myocardial infarction. Besides the well known causes (left ventricular failure, acquired interventricular defect, papillary muscle rupture, free wall rupture) other less frequent mechanisms recognize a functional substrate. The recognition of such mechanisms makes us to revert to the treatments with completely different prognostic implications. In our Coronary Care Unit we encountered, in a period of 12 months, 4 patients who presented clinical, electrocardiographic and/or echographic signs and symptoms of acute myocardial infarction, with different degrees of heart failure up to cardiogenic shock. Only 1 patient showed a severe stenosis of the left anterior descending coronary artery and a significant creatine kinase reduction. Left ventriculography, performed at admission, was unable to disclose the true mechanism of clinical presentation. Only a thorough echographic examination disclosed the presence of a dynamic left ventricular outflow tract obstruction as the cause of heart failure culminating in cardiogenic shock. Once recognized, pathophysiological treatment (administration of beta-blockers and withdrawal of vasodilators, inotropic drugs and intra-aortic balloon pump) led to a dramatic improvement, with an almost complete left ventricular function recovery. Left ventricular outflow tract obstruction is a mechanism that can lead to severe heart failure as a complication of an acute myocardial infarction. Conversely such a mechanism can be precipitated by other causes (hypotension, hypovolemia, especially in hypertensive patients) and can mimic an acute myocardial infarction. Its incidence is not negligible: in our Coronary Care Unit it accounted for about 15% of all cases of myocardial infarction requiring inotropic support. An accurate echocardiographic examination is mandatory even after coronary angiography, and always permits the physician to select the appropriate therapy.
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4/7. Failure of disopyramide to improve right ventricular outflow tract obstruction after living-donor lobar lung transplantation.

    Right ventricular (RV) outflow tract obstruction (RVOTO) is an uncommon complication of lung transplantation in patients with pulmonary hypertension (PH) and both medical management and surgical intervention are required. A 28-year-old female with primary PH was referred and because she did not respond to medical treatment, living-donor lobar lung transplantation was performed. The operation was successful, but dyspnea and exercise intolerance developed during rehabilitation and transthoracic echocardiography revealed RVOTO. Intravenous disopyramide during cardiac catheterization reduced the pressure gradient from 35 mmHg to 16 mmHg without decreasing RV systolic pressure. However, electrical and hemodynamic parameters were adversely affected by disopyramide and thus, after cardiac catheterization, administration of fluid and a low dose of atenolol was started, and her symptoms improved. Transthoracic echocardiography showed improvement in the RVOTO. This case suggests that disopyramide should be avoided for patients with RVOTO following lung transplantation and that other negative inotropic agents, such as beta-blockers, are more effective for relief of RVOTO.
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5/7. Catecholamine therapy inducing dynamic left ventricular outflow tract obstruction.

    Hypertrophic obstructive cardiomyopathy with significant hypertrophy of the basal septum is the most frequently reported cause of left ventricular outflow tract (LVOT) obstruction. Additionally, other conditions such as dehydration, sepsis, vasodilatation, or mitral valve repair have been associated with LVOT obstruction. In this report, we present a case of a patient without hypertrophy who developed severe dynamic left ventricular outflow tract obstruction during catecholamine stimulation for shock that complicated severe pancreatitis. The present case serves as a reminder that hypovolemia together with a hyperdynamic state resulting from catecholamine administration may result in the development of dynamic LVOT obstruction even if baseline cardiac evaluation is unremarkable. Early detection and intensive efforts to reverse the underlying conditions, including cessation of catecholamine therapy and correction of hypovolemia are essential.
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6/7. Relief of left ventricular outflow obstruction by cibenzoline in a patient with Fabry's disease--a case report.

    A 46-year-old man was admitted for further evaluation of exertional chest discomfort. One family member had experienced sudden death, and 2 others had died of heart failure, including 1 known to have had Fabry's disease. The patient was also diagnosed with Fabry's disease, based on reduced leukocyte alpha-galactosidase A activity, 2.0 nmol/mg protein/hour, as well as endomyocardial biopsy findings of marked sarcoplasmic vacuolization of cardiac muscle cells by light microscopy and lamellated "zebra bodies'' in the cytoplasm shown by electron microscopy. echocardiography disclosed marked left ventricular hypertrophy and systolic anterior motion of the mitral leaflets. On cardiac catheterization, a left ventricular peak systolic outflow gradient of 50 mm Hg was noted; this decreased to 10 mm Hg following intravenous administration of 100 mg of cibenzoline. It is imperative to recognize the existence of cases with Fabry's disease associated with left ventricular outflow obstruction.
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7/7. Inspiratory right ventricular outflow obstruction in a patient with hypertrophic cardiomyopathy.

    A patient with familial hypertrophic cardiomyopathy with exertional near syncope is reported. Intra-right ventricular obstruction was demonstrated by hemodynamic studies during inspiration and the valsalva maneuver with systemic hypotension. Improvement occurred following the administration of propranolol. It was suggested that syncope might be precipitated by hemodynamic changes such as a high output state and a depressed cardiac volume in relation to intra-right ventricular obstruction in patients with hypertrophic cardiomyopathy.
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