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11/67. An infant with subvalvar and valvar aortic stenosis, subvalvar and valvar pulmonary stenosis, severe biventricular hypertrophy and pulmonary hemorrhage.

    An infant with subvalvar and valvar pulmonary stenosis, subvalvar, and valvar aortic stenosis and hypertrophic cardiomyopathy, who presented with pulmonary hemorrhage, is reported. He had right ventricular hypertrophy, thickened pulmonary valve leaflets, severe asymmetric left ventricular hypertrophy with outflow tract obstruction, and a thickened and dysplastic aortic valve.
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ranking = 1
keywords = cardiomyopathy
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12/67. Isolated left ventricular noncompaction: an unclassified cardiomyopathy with severe prognosis in adults.

    Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy, which appears to represent an arrest in intrauterine endomyocardial morphogenesis. It is diagnosed both in children and adults. Its common presentation involves heart failure symptoms, ventricular tachyarrhythmias and thromboembolic events, but the age of onset varies widely. The diagnosis is made by the combined appearance of numerous, excessively prominent trabeculations and multiple deep intertrabecular recesses perfused from the ventricular cavity, commonly involving the apical and midventricular segments of the left ventricle. Although the peculiar echocardiographic picture may possibly lead to the correct diagnosis, this condition may be often misdiagnosed or unrecognized since it is not widely known.
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ranking = 5
keywords = cardiomyopathy
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13/67. Isolated non-compaction of the ventricular myocardium: prenatal diagnosis and natural history.

    Isolated non-compaction of the ventricular myocardium (NCVM) is a rare cardiomyopathy characterized by the persistence of numerous marked ventricular trabeculations and deep intertrabecular recesses with direct vascular supply by the ventricular cavities. We report two cases diagnosed by fetal echocardiography at 27 and 30 weeks' gestation, respectively. Postnatal echocardiography verified the presence of the NCVM seen prenatally. Diagnosis was confirmed at postmortem following neonatal demise in the first case. Surgical intervention for exomphalos and extrahepatic biliary atresia was required in the second case, but there is no clinical abnormality of the cardiovascular system a year after delivery. The uncertainty of prognosis and the familial recurrence described elsewhere indicate the difficulty of counseling and the value of prenatal diagnosis, which is feasible using currently available ultrasonographic equipment.
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ranking = 1
keywords = cardiomyopathy
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14/67. Batista operation with aortic valve replacement for valvular cardiomyopathy.

    We performed a successful Batista operation with aortic valve replacement (AVR), graft replacement of the ascending aorta, and tricuspid annuloplasty for a patient with valvular cardiomyopathy with severe aortic stenosis, an ascending aortic aneurysm, and grade 3 tricuspid regurgitation.
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ranking = 5
keywords = cardiomyopathy
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15/67. Left ventricular dysfunction resulting from frequent unifocal ventricular ectopics with resolution following radiofrequency ablation.

    A case is presented, in which asymptomatic but persistent right ventricular outflow tract (RVOT) ectopics resulted in left ventricular (LV) dilatation and systolic dysfunction. The patient underwent extensive investigation with no other cause for the cardiomyopathy being found. Successful ablation of the RVOT ectopic focus resulted in normalization of LV size and function. This case suggests that frequent ventricular ectopy should be considered as a potentially remediable cause of LV dysfunction.
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ranking = 1
keywords = cardiomyopathy
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16/67. Multiple coronary artery-left ventricular microfistulae in a patient with apical hypertrophic cardiomyopathy: a demonstration by transthoracic color Doppler echocardiography.

    Among the congenital coronary artery fistulae, multiple coronary artery microfistulae arising from the left and right coronary artery and emptying into the left ventricle are very rare and little is known of their anatomic and clinical features, especially in apical hypertrophic cardiomyopathy. A 67-year- old woman was referred for the evaluation of chest pain at exertion, and shortness of breath. Electrocardiographic and echocardiographic findings were typical of apical hypertrophic cardiomyopathy. Coronary arteriography showed normal epicardial coronary arteries, but multiple coronary artery-left ventricular microfistulae arising from the left and right coronary arteries. Transthoracic color Doppler echocardiography, using a high frequency transducer with a low Nyquist limit, demonstrated multiple coronary artery-left ventricular microfistulae just beneath the apical impulse window.
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ranking = 6
keywords = cardiomyopathy
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17/67. Hypertrophic non-obstructive cardiomyopathy with aortic and mitral regurgitation: an unusual presentation for surgical option.

    The case is described of a pediatric patient with hypertrophic non-obstructive cardiomyopathy (HNCM) presenting with aortic and mitral regurgitation. This 17-year-old boy had been followed for HNCM since he was aged 4 years. Serial echocardiography had been showing the emergence and progression of aortic regurgitation and mitral regurgitation since the patient was aged 6 years. He started to have syncope attacks at 16 years of age and fell into congestive heart failure at 17 years. He underwent successful mitral valve repair by means of double-orifice technique, and aortic valve repair with raphe triangular resection, cusp plication and subvalvular annuloplasty.
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ranking = 5
keywords = cardiomyopathy
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18/67. Myocardial disarray in noonan syndrome.

    OBJECTIVE--To characterise the histopathology of the left ventricular hypertrophy commonly associated with noonan syndrome by assessing the extent of myocyte disarray and therefore to define one aspect of the relation between this disease and idiopathic hypertrophic cardiomyopathy. DESIGN--Blinded histological analysis. SETTING--Hospital medical school. patients--Six hearts of children with the Noonan phenotype and isolated ventricular hypertrophy were compared with age and sex matched controls. methods--Histological analysis was performed with an image analyser under light microscopy. Representative sections from the entire left ventricular free wall were examined. Results were expressed as the percentage of fields showing disarray related to the number of fields evaluated: 100 fields were examined for each patient. RESULTS--In the patients with noonan syndrome myocardial disarray was present in the ventricular septum in 24 (5.7)% (mean (SD)) of fields and in the free wall in 22.2 (6.8)%. In the controls disarray was present in the septum in 3.8 (2.3)% of fields and in the free wall in 2.4 (2.8)%. In both regions the extent of disarray was significantly greater in patients with noonan syndrome (p < 0.0005; 95% confidence interval 14 to 26.3 for the septum: p < 0.005, 95% confidence interval 11.4 to 28.2 for the free wall). CONCLUSIONS--The ventricular hypertrophy associated with noonan syndrome is histologically similar to hypertrophic cardiomyopathy but whether the two diseases are the expression of the same genetic defect remains to be determined.
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ranking = 2
keywords = cardiomyopathy
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19/67. Improvement of ventricular arrhythmia by octreotide treatment in acromegalic cardiomyopathy.

    We report a case of acromegalic cardiomyopathy in a 46-year-old Japanese man with pituitary adenoma. Increased secretion of growth hormone and insulin-like growth factor I were detected. He had left ventricular hypertrophy, impaired cardiac function, and frequent ventricular premature complexes. After 2-month treatment with octreotide, a long-acting somatostatin analogue, levels of both hormones were decreased. At the same time, left ventricular hypertrophy (intraventricular septal thickness: 22.5 to 17.8 mm), cardiac function (ejection fraction: 38 to 50%), and frequency of ventricular premature complexes (17,249 to 2,882 beats a day) were improved. Transsphenoidal surgery was then safely performed. Treatment with octreotide is thought to have some effect on improvement of ventricular arrhythmia in acromegalic heart.
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ranking = 5
keywords = cardiomyopathy
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20/67. Noncompaction of the ventricular myocardium associated with mitral regurgitation and preserved ventricular systolic function.

    Noncompaction of the ventricular myocardium is an embryonic cardiomyopathy that is increasingly being recognized. Mitral regurgitation, when present, is usually a result of the associated left ventricular systolic dysfunction. We report 4 patients with noncompaction of the ventricular myocardium in whom ventricular systolic function was preserved. Mitral regurgitation was associated with changes in the mitral valve leaflets and an abnormal coaptation pattern. This association of noncompaction of the ventricular myocardium with mitral regurgitation has not, to our knowledge, been reported.
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ranking = 1
keywords = cardiomyopathy
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