471/907. siblings with left ventricular diverticulum and hypertrophic cardiomyopathy. This report describes very rare siblings who had a left ventricular muscular diverticulum and hypertrophic cardiomyopathy. The first case is a 16-year-old male. On two-dimensional echocardiography, a left ventricular muscular diverticulum in the posterior wall and mitral valve prolapse were detected. The former was verified by left ventriculography. Endomyocardial biopsy showed findings compatible with hypertrophic cardiomyopathy. The second case is a 13-year-old female. Two-dimensional echocardiography revealed a left ventricular muscular diverticulum in the same location as that of the first case, and mitral valve prolapse as well. The former was confirmed by left ventriculography. The endomyocardial biopsy findings were compatible with hypertrophic cardiomyopathy. The coronary angiograms were normal in both cases. To our knowledge, familial appearance of a left ventricular diverticulum has not yet been reported, and a left ventricular diverticulum at the posterior wall in cases with hypertrophic cardiomyopathy is very rare. ( info) |
| An 8 month old girl presented with undiagnosed non-anatomic congenital cardiomyopathy with massive cardiomegaly on chest x-ray film. Her older sibling had died suddenly at 6 months of age from what appeared to be a similar abnormality. Utilizing phosphorus-31 nuclear magnetic resonance (P-31 NMR) surface coil spectroscopy, a metabolic disorder was demonstrated in both her myocardium and skeletal muscle, revealing a phosphocreatine (PCr) to inorganic phosphate (Pi) ratio of half of that for a normal control infant. Manipulation of serum substrate availability indicated that medium chain triglycerides alone did not improve myocardial metabolism, but that intravenous glucose or oral carbohydrate loading raised the myocardial PCr/Pi ratio from 1.0 /- 0.05 to 1.8 /- 0.1 (p less than 0.01) without significantly affecting the PCr/Pi value of her resting skeletal muscle. This study demonstrates the feasibility of using P-31 nuclear magnetic resonance to evaluate the biochemistry of the human myocardium in vivo and to diagnose a metabolic abnormality. phosphorus-31 nuclear magnetic resonance can thus be used to optimize therapy for human disease. ( info) |
473/907. Three autopsy cases of progression to left ventricular dilatation in patients with hypertrophic cardiomyopathy. The hearts of three cases of congestive heart failure with dilated left ventricles developing in patients with symptomatic hypertrophic cardiomyopathy (HCM) were morphologically investigated. The results showed that disproportionate hypertrophy and dilatation of the left ventricles, accompanied by massive fibrosis and myocardial disarray, were present in the three patients. The mean percent area of fibrosis of the left ventricle was 34.7% and 47.4% at the upper third and lower third levels, respectively, and was much more frequently associated with disarray (84.4 /- 12.3%). Moreover, the fibrosis was most extensive in the lateral wall of the left ventricle, followed by the posterior, anterior, and interventricular walls. The fibrosis was also diffuse regardless of the subendocardial or subepicardial region of the heart. The findings in the present study suggest that the disarray in this particular series of HCM might be responsible for the mechanism of the fibrosis leading to dilatation of the left ventricle. ( info) |
474/907. Unusual distribution of left ventricular hypertrophy in obstructive hypertrophic cardiomyopathy: localized posterobasal free wall thickening in two patients. Two patients with hypertrophic cardiomyopathy and a previously unreported distribution of left ventricular hypertrophy are described. Each patient showed substantial wall thickening that was confined to the posterobasal segment of the left ventricular free wall, as well as evidence of dynamic subaortic obstruction. This observation emphasizes the fact that hypertrophic cardiomyopathy is characterized by a broad morphologic spectrum. Furthermore, it demonstrates a unique circumstance in which left ventricular outflow tract obstruction may occur in the absence of anterior and basal septal hypertrophy. ( info) |
475/907. Large opaque hemithorax due to cardiomegaly and atelectasis. We studied the clinical and radiologic features of five infants with a large opaque left hemithorax due to the combination of cardiomegaly and atelectasis. The causes of cardiomegaly were cardiomyopathy (two patients), congestive heart failure due to fluid overload, congenital mitral insufficiency, and idiopathic hypertrophic subaortic stenosis. In all five patients, there was initial total opacity of the left hemithorax with shift of the heart and mediastinum to the right due to the combination of cardiac enlargement and total lung collapse. The correct diagnosis may be suggested from conventional chest roentgenograms. Noninvasive supplementary studies, such as high-kilovolt roentgenography, fluoroscopy, esophagography, echocardiography, and radionuclide angiography, should be done for clarification. ( info) |
476/907. thallium-201 imaging in a patient with mid-ventricular hypertrophic obstructive cardiomyopathy. Findings specific to mid-ventricular hypertrophic obstructive cardiomyopathy were obtained in a patient by means of 201Tl planar myocardial scintigraphy. Namely, a myocardial band-like image dividing the left ventricle into two chambers was clearly shown. This was identified as hypertrophic muscle with sphincter-like muscular stenosis at the mid portion of the left ventricle. ( info) |
477/907. Mid-cavity obstruction in apical hypertrophy: Doppler evidence of diastolic intraventricular gradient with higher apical pressure. Three cases of apical left ventricular hypertrophy demonstrating a sequestered small left ventricular apical cavity with complete systolic and partial diastolic intraventricular obstruction are described. Doppler studies revealed that flow from the apical chamber is aborted during early systole with further emptying during diastole through a narrow intraventricular channel, where a diastolic high-velocity jet (greater than or equal to 2.5 m/sec) directed from apex to base was localized. This indicated a significantly higher pressure in early diastole in the sequestered apical cavity. Filling of the apical chamber occurred late in diastole and during isovolumic ventricular contraction. The observations by Doppler of complete intraventricular systolic and partial diastolic obstruction in apical hypertrophy are new and may have significant clinical implications. ( info) |
478/907. Familial complete heart block in hypertrophic cardiomyopathy. We report the finding of complete heart block in two siblings (brother and sister) with hypertrophic cardiomyopathy. Its relationship to the disease is discussed and the relevant literature is reviewed. ( info) |
479/907. aneurysm of the mitral valve in a patient with hypertrophic cardiomyopathy. We report a case of mitral valve aneurysm and severe mitral regurgitation complicating infective endocarditis in a patient with hypertrophic cardiomyopathy. 2-dimensional echocardiography revealed a saccular structure in the anterior mitral leaflet that bulged into the left atrium throughout the cardiac cycle. pathology of the excised valve showed inflammation, early repair and perforation of the aneurysm. Concurrent mitral insufficiency or trauma resulting from septal-anterior mitral leaflet contact may predispose to valvular infection. The repair process of this damaged focus and the loss of the elastic properties of the valve may contribute to aneurysm formation. The aneurysm in this case formed in less than 5 weeks. ( info) |
| We report a case of apical hypertrophic cardiomyopathy associated with acute myocardial infarction with significant coronary stenosis. The present case demonstrates that coronary artery stenosis can occur in apical hypertrophic cardiomyopathy. ( info) |