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1/10. Echocardiographic diagnosis of sinus Valsalva aneurysm rupture in two pediatric patients.

    Sinus Valsalva aneurysm rupture (SVAR) is a rare cardiac abnormality that requires surgical correction when diagnosed. Previously, cardiac catheterization and angiography were thought to be necessary for its diagnosis. We present two pediatric cases of SVAR with subarterial ventricular septal defect (VSD) diagnosed noninvasively by echocardiography; surgical findings confirmed the diagnosis. In both of our cases the origin of SVAR was the right coronary sinus. The first case was ruptured into the right ventricular cavity; the second was ruptured into the right ventricular outflow tract. Continuous murmurs heard during follow-up of children with VSD must alert the physician to this pathology. Combined two-dimensional, Doppler and color-Doppler echocardiography is an accurate, noninvasive method for diagnosis of SVAR.
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2/10. Transient left ventricular aneurysm and hypertrophy accompanied by polymorphic ventricular tachycardia in a patient suspected of acute myocarditis.

    A 75-year-old woman presented with recurrent ventricular tachycardia (VT) compatible with torsades de pointes (TdP) based on sinus bradycardia and QT prolongation. Previously she had received pirmenol, at a serum concentration within therapeutic range, for her paroxysmal atrial fibrillation. Emergent cardiac catheterization identified a ventricular aneurysm of the anteroapical and inferior wall along with angiographically normal coronary arteries. A right ventricular endomyocardial biopsy revealed postmyocarditic change. The left ventricular contraction improved after 5 weeks of conservative treatment. A follow-up echocardiogram revealed transient thickening of partial left ventricular wall consistent with the segment of the aneurysm. Several months later, almost all abnormal findings had improved except for sustained deep negative T waves in precordial leads. Acute myocarditis was primarily suspected as the cause of her clinical presentation.
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3/10. Regional cardiac sympathetic denervation and systolic compression of a septal perforator branch in a sudden death survivor with hypertrophic cardiomyopathy.

    Hypertrophic cardiomyopathy is a heterogeneous primary cardiac disease with a broad clinical spectrum, including a high risk for sudden death in a certain subgroup of patients. However, the precise criteria for identifying the subgroup at high risk have not been established. The authors describe a 41-year-old man with hypertrophic cardiomyopathy who was examined after an episode of aborted sudden death. Extensive invasive and noninvasive investigations did not reveal a cardiac abnormality other than severe sympathetic denervation in the midbasal septal wall on iodine-123 MIBG SPECT imaging and concentric left ventricular hypertrophy on echocardiography. A retrospective review of the cardiac catheterization findings, however, revealed systolic compression of a septal perforator artery. The aborted sudden death of the patient was most likely associated with systolic compression of a septal branch or sympathetic denervation, which may have precipitated a ventricular arrhythmia in this patient.
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4/10. Chronic mitral regurgitation secondary to a perivalvular leak from remote trauma.

    We report a rare case of mitral regurgitation as a result of perivalvular leak in a 49-year-old man with a history of blunt trauma. He presented with a 2-month history of progressive exertional dyspnea, angina, and heart failure. Preoperative transesophageal echocardiography demonstrated severe mitral regurgitation as a result of a perivalvular leak of the mitral valve that was not evident on transthoracic echocardiography and cardiac catheterization.
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5/10. early diagnosis of stress-induced apical ballooning syndrome based on classic echocardiographic findings and correlation with cardiac catheterization.

    Stress-induced apical ballooning has been described as a reversible condition involving the apical left ventricular wall, sparing the base, and causing a ballooning appearance of the left ventricle during systole despite normal coronaries. We are presenting 4 cases of apical ballooning seen at our institution with echocardiographic correlation. echocardiography showed similar anatomical apical ballooning of the left ventricular apex. The diagnosis of apical ballooning syndrome was suspected based on echocardiography in conjunction with clinical data before cardiac catheterization was performed. In one case, in addition to classic left ventricular apical ballooning, marked right ventricular apical akinesia was present on the initial echocardiographic examination. This makes diagnosis of apical ballooning syndrome most likely in this patient before cardiac catheterization. Therefore, we suggest using echocardiography more often for the early diagnosis of this disease, based on careful anatomic evaluation in conjunction with clinical data. Wall motion analysis should reveal an apical ballooning appearance involving many coronary territories. Furthermore, the additional presence of right ventricular apical akinesia during echocardiographic examination makes the diagnosis of this syndrome more likely.
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keywords = catheterization
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6/10. Intercoronary connection with bidirectional blood flow and concentric left ventricular hypertrophy.

    cardiac catheterization in a 55-year-old man, with a 6-month history of atypical chest pain and Q-waves in DII, DIII and AVF, showed concentric left ventricular (LV) hypertrophy and a large intercoronary connection between right coronary artery (RCA) and circumflex artery (CX), with bidirectional blood flow. Although the RCA and CX were normal, selective injection of CX filled RCA retrogradely and in the same way selective injection of RCA filled CX. Possible mechanisms and literature are reviewed.
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7/10. Complete heart block complicating retrograde left ventricular catheterization: case report and review.

    Complete heart block (CHB) following retrograde left ventricular catheterization is uncommon. We report a case of transient CHB in a patient with severe aortic stenosis and bifascicular block on the baseline surface electrocardiogram during retrograde left ventricular catheterization. The block resolved spontaneously without sequelae. A review of the literature using medline was performed and recommendations for temporary and permanent pacing are outlined.
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ranking = 6
keywords = catheterization
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8/10. A case report of siblings with hypertrophic cardiomyopathy that progressed to dilated cardiomyopathy--case reports.

    The authors encountered 2 brothers with hypertrophic cardiomyopathy (HCM), both of whom were later found to have progressed to dilated cardiomyopathy (DCM). Case No. 1 (younger brother): A diagnosis of HCM without left ventricular (LV) dilatation was made at age two by cardiac catheterization and echocardiogram. However, he had been well until age thirteen when dyspnea developed. Echocardiograms showed LV wall thickening (septum 20 mm, and posterior 18 mm) and LV dilatation (left ventricular diastolic diameter [LVDd] 60 mm) accompanied by LV dysfunction (ejection fraction [EF] 18%). At autopsy, the heart weighed 875 g and showed LV dilatation with thickened walls. Case No. 2 (elder brother): At age twelve, he was found to have an increased heart size on x-ray when his family was evaluated owing to his brother's condition. cardiac catheterization and echocardiogram showed HCM without LV dilatation. He remained asymptomatic until age twenty when dyspnea developed, at which echocardiography showed a dilated LV. The authors speculate that the HCM with progression to DCM is an atypical type of familial HCM and that progression to DCM is genetically determined.
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ranking = 2
keywords = catheterization
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9/10. Right ventricular aneurysm associated with advanced hypertrophic cardiomyopathy.

    A previously undescribed case of right ventricular aneurysm (RVA) associated with hypertrophic cardiomyopathy in an advanced stage is reported. The diagnosis was established by noninvasive (cardiac two-dimensional echocardiogram and nuclear MRI) and invasive (cardiac catheterization, angiography, and biventricular endomyocardial biopsy) cardiac examinations, which documented hypertrophied, dilated and hypokinetic biventricular chambers associated with typical histologic findings (histologic hypertrophic cardiomyopathy index of 66%). A prominent narrowing of myocardial arterioles, extended to the right ventricular myocardium, has been identified and has been hypothesized as being responsible for RVA formation.
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keywords = catheterization
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10/10. Reversal of dilated to hypertrophic cardiomyopathy after alcohol abstinence.

    Left ventricular dilation and systolic dysfunction develop in 14-16% of patients with hypertrophic cardiomyopathy. Such findings may easily be misdiagnosed as dilated cardiomyopathy. It is unknown whether left ventricular dilatation and systolic dysfunction in patients with hypertrophic cardiomyopathy are reversible. A 35-year-old man had been a heavy drinker for 13 years and was abstinent for 1 year. Five years previously he suffered cardiac arrest and, based on echocardiographic, radionuclide, and cardiac catheterization findings, the diagnosis of alcohol-induced dilated cardiomyopathy was established. At presentation the heart was of normal size, with concentric left ventricular hypertrophy and only slightly reduced systolic function. Hypertrophic cardiomyopathy was diagnosed since no other cause for left ventricular hypertrophy could be detected. In hypertrophic cardiomyopathy, alcohol may induce reversible systolic dysfunction and left ventricular dilatation.
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