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1/6. Iterative atrial tachycardia originating from the coronary sinus musculature.

    A case of iterative atrial tachycardia leading to dilated cardiomyopathy is reported. During electrophysiologic study, the tachycardia showed a markedly irregular cycle length associated with changes in atrial activation breakthrough as demonstrated by coronary sinus (CS) recordings and frequently degenerated into self-terminating atrial fibrillation. Left atrial transseptal mapping demonstrated the earliest endocardial atrial activation close to the posterolateral mitral annulus, but this was invariably later than that recorded within the CS, where low-energy radiofrequency applications eliminated the tachycardia. No acute vessel damage was observed at postablation CS angiography. In accordance with previously published experimental data, we hypothesized that the muscular sleeves surrounding the CS might be involved in the genesis of this tachycardia. During 6-month follow-up, the patient remained asymptomatic without tachycardia recurrences and with complete recovery of left ventricular function, confirming the reversible nature of the tachycardia-induced cardiomyopathy.
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2/6. chylous ascites as the main manifestation of left ventricular dysfunction: a case report.

    BACKGROUND: Ascites is one of the most common complications of liver diseases, even though in 15% of the cases it is related to extrahepatic diseases; 3% are of cardiac nature and they appear associated with signs and symptoms of heart failure. CASE PRESENTATION: A 70 year old man was admitted with more than one year history of abdominal distension and a weight gain of 10 kilograms. He is asymptomatic and walks 2000-3000 meters a day without angor or dyspnea. The physical examination shows moderate abdominal distension, with no hepatosplenomegaly or edema, and there is mild jugular vein distension. The studies performed (complete laboratory work up, paracentesis, liver biopsy, echocardiogram, intrahepatic pressure measurements, etc.) showed a chylous ascites related to portal hypertension, and left ventricular dysfunction was the only probable cause found. CONCLUSION: Asymptomatic heart dysfunction can mimic liver disease and should be kept in mind as a cause of chylous ascites.
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3/6. hydroxychloroquine-induced cardiotoxicity in a 39-year-old woman with systemic lupus erythematosus and systolic dysfunction.

    A 39-year-old woman with a history of systemic lupus erythematosus developed chest pain and conduction abnormalities. An echocardiographic examination revealed systolic dysfunction and ventricular thickening. Because of the unclear nature of her cardiac disease, right ventricular endomyocardial biopsy was performed. light microscopy showed diffuse myocyte vacuolization without myocarditis, and transmission electron microscopy demonstrated sarcoplasmic myelinoid and curvilinear bodies, diagnostic of hydroxychloroquine toxicity. Among patients with autoimmune connective disorders, cardiac dysfunction may be a result of the disease or occasionally of its treatment. Although the nature of the cardiac disease (myocardial, valvular, or pericardial) can generally be evaluated echocardiographically, endomyocardial biopsy may be indicated to rule out diseases with a specific microscopic appearance, such as myocarditis or hydroxychloroquine cardiotoxicity.
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4/6. Live three-dimensional transthoracic contrast echocardiographic assessment of apical hypertrophic cardiomyopathy.

    We describe an adult patient in whom live three-dimensional transthoracic echocardiography combined with intravenous use of an echo contrast agent was useful in making a definitive diagnosis of apical hypertrophic cardiomyopathy and in characterizing the nature and full extent of the hypertrophy.
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5/6. Acute respiratory distress syndrome with transiently impaired left ventricular function and torsades de pointes arrhythmia unmasking congenital long qt syndrome in a 25-yr-old woman.

    We report a case of recurrent episodes of torsades de pointes arrhythmia in the setting of transiently impaired left ventricular ejection fraction, acute respiratory distress syndrome, transient hypokalaemia and QT-prolonging drugs, in a previously healthy 25-yr-old female patient. In the course of the clinical and genetic work-up this patient was newly diagnosed with a mutation in KCNH2 encoding the alpha-subunit of the human repolarizing potassium channel I(Kr). This case report illustrates the multivariate nature of long-QT syndrome, and emphasizes the usefulness of a pharmacological test for repolarization abnormalities.
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6/6. Transthoracic real-time three-dimensional echocardiography in the diagnosis and description of noncompaction of ventricular myocardium.

    BACKGROUND: Numerous modalities have been used to diagnose and characterize noncompaction of ventricular myocardium (NCVM) including magnetic resonance imaging, two-dimensional echocardiography (2DE), contrast-enhanced 2DE, and angiography. The current case series examines the use of real-time three-dimensional transthoracic echocardiography (RT-3DE) in four such cases of NCVM. methods: From December 2003 to March 2004, we performed RT-3DE using a Philips Sonos 7500 echocardiographic scanner equipped with a 2-4 MHz 3D matrix array transthoracic probe, to evaluate four patients with NCVM. The real-time 3D transthoracic probe allows for dataset acquisition in an ultrasound wedge, which can be manipulated instantaneously. In addition, complete 3D volume rendering is acquired, allowing for volumetric imaging. RESULTS: The age range of the patients was 2 months to 42 years. One patient had the codiagnoses of coarctation of the aorta and bicuspid aortic valve. In all four patients, RT-3DE enabled diagnosis and provided detailed characterization of the affected myocardium. Entire trabecular projections and intertrabecular recesses were easily visualized simultaneously, and endocardial borders were clearly demarcated. Wall motion abnormalities of the affected myocardium were clearly visualized. The compacted and noncompacted portions of the myocardium could be differentiated well. CONCLUSIONS: Our study provides preliminary data highlighting the utility and feasibility of RT-3DE in the clinical characterization of NCVM. The complex 3D nature of this disorder and the endocardial hypertrabeculation were more readily visualized than with 2DE.
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