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Cardiac catheterization, angiocardiography and ventricular muscle biopsy were performed in forty patients with idiopathic cardiomyopathy and included 21 cases of hypertrophic cardiomyopathy and 19 cases of congestive cardiomyopathy. 1) Cardiac catheterization revealed normal cardiac index and stroke index in both types, although there was a slight tendency toward decrease in cases of CCM. HCM showed slightly elevated right ventricular end-diastolic pressure and left ventricular end-diastolic pressure with a high incidence of atrial kick. CCM showed an elevated mean pulmonary artery, mean pulmonary wedge and left ventricular end-diastolic pressure. 2) Angiocardiographic findings revealed that in HCM left ventricular end-diastolic volume as well as left ventricular end-systolic volume, ejection fraction, meanVcf and MNSER were within normal range, and left ventricular anterior wall thickness, left ventricular mass and shortening of short axis in systole were increased. In CCM left ventricular end-diastolic volume and end-systolic volume increased, and ejection fraction, meanVcf, MNSER were decreased. The left ventricular anterior wall thickness was normal, and the left ventricular mass was smaller compared to the volume. The shortening of long and short axes in systole was slight. Left ventricular asynergy and mitral regurgitation occurred frequently. Coronary cineangiograms revealed normal patterns in both types. 3) Histological findings revealed hypertrophy of myofibers, degenerative changes, i.e. scarcity of myofibrils, deformity of nucleus and vacuolization of myocardial fibers, and collagen proliferation in both types. 4) No definite relationship was seen between parameters of left ventricular function and the findings of biopsied left ventricular muscle except for increase in wall thickness which might be apparently due to hypertrophy of the myocardial fibers.
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PMID:Angiocardiograms and hemodynamics in idiopathic cardiomyopathy, with reference to histology of biopsied ventricular myocardium. 15 67

Deletions and point mutations of mitochondrial DNA (mtDNA) of patients with dilated or hypertrophic cardiomyopathy were analyzed using the polymerase chain reaction and fluorescence-based direct sequencing. The patients included are with hypertrophic cardiomyopathy associated with left ventricular dilatation, a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), and a patient with fatal infantile cardiomyopathy. Deletions were frequently seen in mtDNA in patients with dilated cardiomyopathy. The mtDNA was sequenced and the direct repeat at each edge of deletion was identified as (5'-CATCAACAACCG-3') which was located in the ATPase6 gene and in the D-loop region. In a patient with hypertrophic cardiomyopathy associated with left ventricular dilatation, another mutant mtDNA was found not to have directly repeated sequence, and was revealed to jump from nucleotide position 8,992 to position 16,072 of mtDNA resulting in a 7,079 bp deletion. This patient had unique point mutation in the tRNA genes. A G-to-A transition in the tRNA(Cys) gene (nucleotide position 5,821) at the aminoacyl acceptor stem and an A-to-G transition in the tRNA(Thr) gene (nucleotide position 15,951) were identified. In a patient with MELAS, an A-to-G transition in the tRNA(Leu)(UUR) gene (nucleotide position 3,243) was observed. This mutation was located at the 5' end of the dihydrouridine loop of this tRNA molecule, and would disturb its function. In a patient with hypertrophic cardiomyopathy associated with lactic acidosis, mutations of mtDNA should be suspected.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Mitochondrial DNA mutations in cardiomyopathy. 143 21

The long-term results of patients undergoing myotomy/myectomy of the ventricular septum for obstructive hypertrophic cardiomyopathy are documented in 31 patients (15 women, 16 men, age range 21 to 80 years [mean 55]) with mean New York Heart Association functional class III to IV congestive heart failure, who underwent radical myotomy/myectomy at the Brigham and Women's Hospital from 1972 to 1991. Preoperative gradients by catheterization or echocardiography ranged from 26 to 240 mm Hg (average 96). There were no operative deaths. Two patients developed early postoperative complete heart block requiring a transvenous pacemaker. Clinical follow-up was 1 to 14 years (mean 6.5). All surviving patients were restudied by echocardiography and clinical examination. The mean postoperative functional class was II. Postoperative gradients ranged from 0 to 30 mm Hg (mean 4.5) (p less than 0.001 compared with preoperative values). There were 5 late deaths (low cardiac output in 2, stroke in 2, and acute respiratory failure in 1); 4 of 5 deaths occurred in patients with concomitant coronary artery disease. Survival at 10 years was 86 +/- 9%. There were no reoperations for subaortic obstruction.
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PMID:Long-term follow-up of patients undergoing myotomy/myectomy for obstructive hypertrophic cardiomyopathy. 151 16

Abnormalities of left ventricular (LV) systolic performance develop during exercise in patients with coronary artery disease (CAD) as a result of ischemia-induced regional wall motion abnormalities. Like patients with hypertension and those with hypertrophic cardiomyopathy, patients with CAD display abnormalities of LV diastolic performance under basal conditions in the absence of ischemia. The purpose of these studies was to compare the effects of bepridil versus those of propranolol or diltiazem in patients with exertional angina pectoris. LV systolic and diastolic performance were assessed at rest and during peak upright bicycle exercise by first-pass radionuclide ventriculography. Compared with propranolol, bepridil increased exercise capacity, cardiac output, and stroke volume and decreased systemic vascular resistance. Compared with diltiazem, bepridil increased exercise capacity, peak filling rate, and early diastolic filling fraction and decreased systemic vascular resistance, heart rate, time to peak filling rate, and atrial filling volume. Bepridil therapy is associated with improved exercise capacity and decreased anginal frequency and nitroglycerin consumption. In addition, its use is accompanied by favorable changes in LV systolic and diastolic function at rest and during exercise. These changes are consistent with benefits resulting from resolution of myocardial ischemia as well as from positive lusitropic effects of bepridil on the ventricular myocardium.
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PMID:Effects of antianginal therapy on left ventricular systolic and diastolic performance: comparison of the response to bepridil, propranolol, and diltiazem. 155 88

To assess the behavior of the subvalvular pressure gradient under physical exercise, 13 patients with obstructive hypertrophic cardiomyopathy were examined during upright bicycle ergometry by means of Doppler echocardiography. Additionally, right-sided cardiac catheterization was performed within 7 days. In 10 patients adequate Doppler tracings could be obtained during exercise. The Doppler-derived systolic pressure gradient increased from 75 +/- 24 to 140 +/- 42 mm Hg (p less than 0.0005). This was associated with an increase in the duration of the systolic mitral-septal contact from 59 +/- 21 to 136 +/- 28 ms (p less than 0.0005). Correlation between the pressure gradient and the duration of mitral-septal contact at rest and during exercise was good (r = 0.86), whereas correlation between the resting and exercise pressure gradient (r = 0.34) did not reach statistical significance. The increase in stroke volume during exercise, from 90 +/- 18 to 95 +/- 24 ml, was significant (p less than 0.05) but minimal. Therefore, only a moderate increase in systolic flow, from 205 +/- 54 to 268 +/- 78 ml/s (p less than 0.0005), was observed. Outflow tract resistance, defined as the ratio of the pressure gradient to systolic flow, increased from 0.38 +/- 0.11 to 0.57 +/- 0.24 mm Hg.s/ml (p less than 0.01). Thus, in a selected group of patients with hypertrophic cardiomyopathy a substantial increase in the maximal pressure gradient during upright bicycle ergometry was demonstrated in most patients. Exercise Doppler echocardiography may be valuable to assess the hemodynamic significance of obstruction in individual patients in a physiologic setting and has a potential to monitor the effect of therapeutic interventions.
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PMID:Doppler echocardiographic assessment of the pressure gradient during bicycle ergometry in hypertrophic cardiomyopathy. 843 Jun 67

Total and regional (by 7 zones) contractility of left ventricular functions in 25 patients with hypertrophic cardiomyopathy (HCMP) were characterized by a different combination of normal and elevated values of total and regional ejection fractions, rate indices of systolic expulsion. Normal or slightly changed values of the end-diastolic volume were combined with normal or elevated values of the stroke volume. Comparative analysis of the values characterizing cardiodynamics, led to working out criteria of differential diagnosis in HCMP patients with heart pain and in CHD patients with myocardial hypertrophy (predominance of the signs of left ventricular hyperfunction in the former and contractility hypofunction and asynergy in the latter). Changes in the diastolic filling of the left ventricle indicated its disturbed diastolic function in both groups of patients.
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PMID:[Radionuclide ventriculography in the diagnosis of hypertrophic cardiomyopathy]. 165 88

In order to investigate the relation between exercise intolerance and cardiac output (CO) increase during exercise in patients with hypertrophic cardiomyopathy (HCM), submaximal treadmill exercise (Sheffield protocol) with simultaneous measurement of CO and oxygen uptake (VO2) by acetylene rebreathing method were performed in twelve patients with HCM who had no demonstrable left ventricular outflow obstruction and fifteen control men. The measurements were made at rest, during exercise and recovery. 1) At rest cardiac index (CI) in patients with HCM was significantly less than in control subjects but the difference was small (HCM: 1.9 +/- 0.35 l/min/m2 vs control: 2.2 +/- 0.38, p less than 0.05), and was almost the same during light exercise (stage 1-3), but clearly increased during middle to submaximal stages (stage 4 and 5) (stage 5: 4.5 +/- 1.25 vs 8.0 +/- 1.30, p less than 0.01). 2) Heart rate was not different in both groups at rest, during exercise and recovery, which was considered to explain that the difference of CI was derived from that of stroke index (SI). 3) The change of VO2 showed the same tendency as that of CI. The difference increased considerably during the middle to submaximal stages (Stage 5: 15.3 +/- 1.42 ml/min/kg vs 25.4 +/- 4.42, p less than 0.01). 4) During recovery, CI and VO2 in patients with HCM indicated a delay of beginning to decrease toward the rest level, compared with control subjects. 5) VO2 was closely related to CI (r = 0.90, p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Changes in cardiac output during exercise in patients with hypertrophic cardiomyopathy: the study of these influences on exercise intolerance]. 174 68

Thrombus formation in the left atrium and left ventricle is primarily due to stasis of blood which causes activation of the coagulation system. Migration of thrombotic material into the circulation depends on the dynamic forces of the circulation. Atrial fibrillation is the commonest underlying cardiac disorder predisposing to thromboembolism. Rheumatic mitral stenosis, left atrial enlargement, prior myocardial infarction, hypertension, and echocardiographic left ventricular hypertrophy are risk factors for thromboembolic stroke in elderly patients with chronic atrial fibrillation. Non-valvular atrial fibrillation accounts for 45% of cardiac sources of thromboembolic stroke and includes patients with ischemic heart disease, hypertension, thyrotoxic heart disease, hypertrophic cardiomyopathy, chronic sinoatrial disorder, and idiopathic atrial fibrillation. 15% of cardiac sources of thromboembolic stroke are associated with acute myocardial infarction, 10% with left ventricular aneurysm and mural thrombi remote from an acute myocardial infarction, 10% with rheumatic valvular heart disease, and 10% with prosthetic cardiac valves. Mitral valve prolapse, mitral annular calcium, nonischemic cardiomyopathies, infective endocarditis, nonbacterial thrombotic endocarditis, left atrial myxoma, paradoxical embolism associated with congenital heart disease, calcific aortic stenosis, and complex atherosclerotic plaque within the proximal aorta also contribute to thromboembolism.
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PMID:Etiology and pathogenesis of thromboembolism. 176 43

Twenty patients with different types of muscular dystrophy (MD) were included in a cross-sectional study by means of electrocardiography and ultrasound cardiography. A manifest cardiomyopathy was detected in 8 patients; a latent cardiomyopathy was found in 4. A hypertrophic cardiomyopathy was especially frequent in facioscapulohumeral MD, a congestive cardiomyopathy in Becker-Kiener MD. The ECG showed a reduction in the QT interval and frequent block formers in the X-chromosomal inherited forms and the trunc-girdle form. Bradycardia and a prolonged QT interval were frequent in myotonic dystrophy and facioscapulohumeral MD. Signs of cardiac infarction in the ECG were most frequent in the trunc-girdle forms. A high cardiac output per minute in conjunction with increased left ventricular volume was frequent in Becker-Kiener and Landouzy MD. A left ventricular dysfunction with reduced ejection was characteristic of myotonic dystrophy and trunc-girdle MD. A mitral valve prolapse was more frequent with increasing severity of the muscle disease and was particularly frequent in myotonic dystrophic and Landouzy MD. The cardiac output per minute and the stroke volume were significantly lower (P less than or equal to 0.03) where a mitral valve prolapse was present.
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PMID:The heart in muscular dystrophy: an electrocardiographic and ultrasound study of 20 patients. 179 Jan 64

As many as 40 men suffering from essential hypertension (EH) and left ventricular hypertrophy (LVH) or hypertrophic cardiomyopathy (HCMP) were examined. All the patients exercised on a treadmill according to the Cornell protocol taking into consideration the ST/HR slope and the ST/HR index, underwent echocardiography with measurements of the left ventricular mass (LVM), and coronary ventriculography. Coronary insufficiency was revealed in all the patients. Of these, 11 patients suffered from it due to associated EH and coronary heart disease (CHD), 31 had relative coronary insufficiency in the presence of associated EH and LVH phenomena with no stenosis of coronary vessels, and 7 patients showed up relative coronary insufficiency in the presence of HCMP. The ST/HR slope and the ST/HR index correlated well with the LVM and the asymmetry index of the left ventricle but in patients with associated relative coronary insufficiency and EH. In patients with associated EH and CHD, the ST-dependent parameters correlated well neither with the degree of atherosclerosis spreading nor with the LVM. This may indicate that both factors influence the gravity of coronary insufficiency at a time. In case a patient suffering from associated EH and coronary insufficiency phenomena has the ST/HR slope greater than or equal to greater than or equal to 4.5 microV/stroke/min and/or the ST/HR index greater than or equal to greater than or equal to 2.5 microV/stroke/min, it is more likely that myocardial ischemia is provoked by concomitant atherosclerosis of coronary arteries (sensitivity 28%, specificity 71%).
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PMID:[The ST-segment-dependent indices of patients with left ventricular hypertrophy and coronary failure]. 183


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