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PubMed1968

Left ventricular function in patients with chronic obstructive pulmonary disease.

Left ventricular function was assessed in six patients with essentially normal cardiopulmonary function, in five patients with primary myocardial disease, and in 16 patients with chronic obstructive pulmonary disease by determining the response of the ventricle to an increased resistance to ejection. Studies were performed at the time of cardiac catheterization and increased resistance to left ventricular ejection was produced by the intravenous infusion of methoxamine. In the control patients, methoxamine produced an increase in stroke volume index (SVI), in stroke work index (SWI), and stroke power index (SPI), whereas left ventricular end-diastolic pressure (LVEDP) increased only moderately. In contrast SVI, SWI, and SPI fell, whereas LVEDP increased inordinately in the patients with myocardiopathy. The patients with chronic obstructive pulmonary disease responded to the infusion with an increase in SVI, SWI, SPI, and LVEDP comparable to the control patients. Furthermore, in this latter group of patients, a quantitatively similar response was observed in those with essentially normal resting hemodynamics, in those with resting pulmonary hypertension, and in those whose disease had progressed to the stage of right ventricular failure. This study provides no evidence that chronic obstructive pulmonary disease results in chronic impairment of left ventricular function, but on the contrary, has demonstrated that the left ventricle responds normally to an increased pressure load in these patients.

Adult↗

PubMed1985

Cardiovascular diseases due to viruses.

In acute myocardiopathy and pericardiopathy, Coxsackie virus B infections are increasingly recognized as a cause of primary myocardial disease and may cause chronic cardiovascular disease. With other viruses, such as cytomegaloviruses and rubella virus, transplacental infection during pregnancy may occur, and this can cause the congenital rubella syndrome, which involves heart abnormalities. Other viruses are now under study. Myocarditis was observed in a newborn infant infected with Coxsackie virus B3, which was isolated by tissue culture methods. Experimental infection of Coxsackie virus A and B was studied in suckling mice and the histopathologic changes in heart muscle were observed. Laboratory findings of viral infection are very useful for clinical diagnosis, however care needs to be taken with respect to the obtaining of specimens, diagnostic procedures, and the assessment of results.

Animals↗

PubMed1976

[RI angiocardiography of idiopathic cardiomyopathy (author's transl)].

This study was aimed to evaluate the noninvasive method of radioisotopic angiocardiography for detecting a disproportionate thickning of the interventricular septum relative to the left ventricular free wall in the patient with idiopathic hypertrophic cardiomyopathy. The experimental study was performed to compare the imaged size of myocardial wall obtained my filling up with technetium 99 m labeled sponge in biventricular cavities, with the actually measured thickness of the heart specimen. The resultant correlation between actual and imaged size of the left ventricular wall was excellent (r = 0.95). In the clinical application of this method, a gated cardiac blood pool scan was performed after the intravenous administration of technetium 99 m labeled human serum albumin in a dose of 10 mCi. The cardiac images were obtained in anterior, 30, 35, 40 and 45 degree left anterior oblique projection, and evaluated by visual inspection and semi-quantitatively by tracing the myocardial borders. As a result, the imaged asymmetric septal hypertrophy was evident in all four patients with PMD, with a ratio of septum to left ventricular free wall of 1.35 compared to 0.76 in five patients with left ventricular hypertrophy of known heart disease, and 0.88 in twenty-one miscellaneous cardiac patients without left ventricular hypertrophy. Thus, RI angiocardiography provides a safe, repeatable method for detection and evaluation of Primary Myocardial Disease.

Cardiomegaly↗

PubMed1978

[Myocardial infarction "with angiographically normal coronary arteries (author's transl)].

Within a cohort of 121 patients with clinical picture and electrocardiographic pattern of myocardial infarction four cases (3%) had normal coronary arteried demonstrated by selective coronary angiography. Two patients revealed no risk factors, two patients were heavy cigarette smokers. Mean age of patients was 34 years. Exact analysis of clinical, hemodynamic and angiographis data confirmed assumption of regional myoaggressive myocarditis as the underlying disease in one patient and suggested regional myocarditis with high probability in another case. The two other patients however could not be classified as having coronary or primary myocardial disease. The investigation suggests that some cases with the clinical and electrocardiographic pattern of "myocardial infarction" with normal coronary arteries may be due to regional myoagressive myocarditis.

Adult↗

PubMed2000

Inherited infantile dilated cardiomyopathy in dogs: genetic, clinical, biochemical, and morphologic findings.

Dilated cardiomyopathy, a lethal disease characterized by left ventricular dilation and systolic dysfunction, is relatively common in humans and other mammals. Idiopathic dilated cardiomyopathy (IDCM) is a primary myocardial disease of unknown cause and can be a familial disorder. This report describes autosomal recessive IDCM in dogs. It occurs in Portuguese Water Dog (PWD) pups and is manifested by acute, vague clinical signs and sudden death. Affected pups have progressive reduction of fractional shortening that can be demonstrated by echocardiography prior to the development of clinical signs. Furthermore, these pups have low plasma taurine levels when consuming certain diets. Affected pups had dilation of the left ventricle and alterations in the sarcomere appearance, while immunohistochemical and biochemical studies demonstrate an increase in desmin, a cytoskeleton protein. The clinical and morphologic findings of IDCM in PWDs are distinct from those reported in adult IDCM. Finally, the clinical and echocardiographic manifestations were reversible in some pups following oral taurine supplementation for 2 months. These results suggest that IDCM in PWDs is correlated with low plasma taurine levels.

Animals↗

PubMed1991

Functional description of the left ventricle in patients with volume overload, pressure overload, and myocardial disease using cine magnetic resonance imaging.

Cine magnetic resonance imaging (MRI) is a three-dimensional imaging technique with sufficient temporal resolution to provide quantitation of left ventricular (LV) dimensions and function. It can be used to describe the three-dimensional geometrical and functional characteristics of various cardiac disease states. Accordingly, the purpose of the study was to noninvasively characterize LV function with cine MRI using LV mass, systolic wall stress, ejection fraction, and fractional wall thickening, and to compare these functional determinants among three major cardiac disease states. Patients were selected from echocardiographic criteria and specific history of disease and divided into the following groups: compensated moderate to severe aortic regurgitation; compensated LV hypertrophy caused by systemic hypertension; and dilated congestive cardiomyopathy. Normal subjects without evidence of heart disease were studied for comparison. Cine MRI was performed at 1.5 Tesla in the short-axis plane. Wall stress was calculated from MRI, along with blood pressure recordings and carotid pulse tracings, according to the Laplacian expression. The end-diastolic volume to LV mass ratio distinguished between concentric and eccentric LV hypertrophy. Peak-systolic wall stress was increased in volume overload lesions and myocardial disease and significantly different (P less than .01) compared with pressure overload lesions and normal subjects. Ejection fraction was significantly decreased (P less than .01) in myocardial disease compared with volume and pressure overload lesions and normal subjects. Among these disease states, LV mass was not different but was significantly higher in all (P less than .01) compared with normal subjects. Fractional wall thickening was increased in pressure overload lesions. Thus, the increase in LV mass in volume overload lesions and primary myocardial disease is not adequate to prevent an increase in wall stress, whereas LV mass increased sufficiently in pressure overload lesions to normalize systolic wall stress. Cine MRI can be used to noninvasively describe the three-dimensional geometry and functional state of the heart in various pathological conditions and might be used to monitor therapeutic effects.

Adult↗

PubMed1987

Hypertrophic cardiomyopathy: current views on etiology, pathophysiology, and management.

Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease of unknown cause that is characterized by a hypertrophied, nondilated, hypercontractile left ventricle. Its etiology and pathogenesis remain undefined but the three principal factors implicated are a genetic predisposition, a hypersensitivity to catecholamines, and an abnormal calcium metabolism. The hypertrophy typically involves the intraventricular septum to varying degrees, but may also involve the apex or free wall and even be concentric. The disease occurs in either an obstructive or a nonobstructive form depending on whether an intraventricular pressure gradient can be demonstrated at rest or on provocation. The gradient and obstruction to outflow is usually seen in patients with asymmetric septal hypertrophy (ASH) and anterior motion of the mitral valve during systole (SAM). Abnormal left ventricular diastolic function characterized by inadequate filling and impaired relaxation has been shown to be very important in both the obstructive and nonobstructive forms of the disease. In addition, inadequate coronary vasodilator reserve as a result of small vessel disease, microvascular spasm, and/or low capillary density per unit myocardial mass has been implicated as an important cause of ischemia in patients without coronary artery disease. HCM is a disease of young adulthood with relatively slow progression; young patients are often asymptomatic, whereas older patients are more limited by dyspnea, angina, dizziness, or syncope. Supraventricular tachyarrhythmias occur in 30% of patients, and high-grade ventricular arrhythmias occur in over 75%. The annual mortality is 3-5%. The common mode of demise is sudden cardiac death. Therefore, the primary objectives of treatment are the amelioration of symptoms, the control of arrhythmias, and the prevention of sudden death. Beta-adrenoreceptor blocking agents decrease myocardial contractility and oxygen demands and increase ventricular volume; therefore, they are most useful in patients with the obstructive form of HCM. Calcium channel antagonists enhance left ventricular relaxation, relieve microvascular spasm, and improve coronary filling and therefore are the agents of choice in patients with diastolic dysfunction. The ability of the calcium channel antagonists to decrease contractility makes them valuable in patients with obstructive HCM. Arterial vasodilators, diuretics, nitrates, and inotropic agents should be avoided because they can increase the intraventricular gradient. Myomyectomy is reserved for those patients with the obstructive form of HCM whose symptoms are refractory to medical therapy.(ABSTRACT TRUNCATED AT 400 WORDS)

Cardiomyopathy, Hypertrophic↗

PubMed1989

Transient ST-segment elevation in subarachnoid hemorrhage.

Electrocardiographic manifestation mimicking the hyperacute phase of myocardial infarction and the electrical alternans of the elevated ST-segment in association with subarachnoid hemorrhage were reported in two patients with no evidence of heart disease. In both cases the ST-segment changes were transient and there were no persistent changes suggestive of underlying myocardial damage or ischemia. These findings suggested that the electrocardiographic changes were probably secondary to subarachnoid hemorrhage and not an expression of primary myocardial disease. The electrocardiographic abnormalities could be explained by altered autonomic activity to coronary arteries or directly to the myocardium.

Diagnosis, Differential↗

PubMed1976

Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction.

The nature, prevalence, functional significance, and indeed existence of myocardial disease in progressive systemic sclerosis (PSS) has been debated. In this study the clinical and pathological features of 52 autopsied patients were analyzed in an attempt to resolve these questions. A distinctive focal myocardial lesion ranging from contraction band necrosis to replacement fibrosis throughout both ventricular walls was present in 23 patients who had widely patent extramural coronary arteries. There were no morphologic abnormalities of the intramyocardial coronary arteries to account for these lesions. Comparing those patients having severe (13), mild (10), or no (24) PSS myocardial lesions, and patent extramural coronary arteries, there were no major differences in age, sex, frequency and severity of pulmonary, renal or hypertensive disease which could account for the myocardial necrosis and fibrosis. The three groups did differ, however, with regard to clinical cardiac abnormalities: ventricular arrhythmias and conduction disturbances were six and two times as frequent, respectively, in those with severe myocardial PSS compared to the other two groups. A pattern of primary myocardial disease with intractable congestive heart failure resulted from severe myocardial PSS in four patients, angina pectoris with normal coronary arteries was associated with the severe myocardial lesion in three patients, and sudden death in five. The occurrence of contraction band necrosis suggests that the myocardial damage in PSS might be due to intermittent vascular spasm of the type recognized in the digits and possibly kidneys and lungs, i.e., an intramyocardial Raynaud's phenomenon. The findings in our patients clearly show that myocardial progressive systemic sclerosis is a distinct entity with relatively frequent occurrence which may lead to arrhythmias, congestive heart failure, angina pectoris with normal coronary arteries and sudden death.

Adolescent↗

PubMed1993

Discordance in degree of right and left ventricular dilation in patients with dilated cardiomyopathy: recognition and clinical implications.

OBJECTIVES: The purpose of the present study was to assess the influence of variations in the relative degree of dilation of left and right ventricular chambers on the clinical outcome of patients with dilated cardiomyopathy. BACKGROUND: Dilated cardiomyopathy, a primary myocardial disease characterized by ventricular dilation and systolic dysfunction, is generally associated with a poor prognosis. However, considerable variability has been observed in the clinical course and the morphologic and hemodynamic features in individual patients. METHODS: We evaluated 67 consecutive patients with dilated cardiomyopathy and without evidence of ischemic or primary valvular heart disease. On the basis of diastolic ventricular chamber area measurements obtained by echocardiography, patients were classified into two groups: 38 patients with a relatively equal degree of left and right ventricular dilation (LV congruent to RV) and 29 patients with predominant and disproportionate dilation of the left ventricle (LV > RV). RESULTS: The 67 patients ranged in age from 19 to 81 years (mean 56); 49 (73%) were male. The two subsets of patients with dilated cardiomyopathy did not differ with regard to age, left ventricular diastolic dimension, wall thickness and mass or ejection fraction. However, patients in the LV congruent to RV group showed more severe mitral and tricuspid regurgitation by Doppler echocardiography than did those in the LV > RV group (p = 0.01 for mitral and 0.004 for tricuspid regurgitation). Over the follow-up period of 2 to 60 months (mean 28), there were 19 deaths. Survival in the LV > RV group was significantly better than in the LV congruent to RV group (p = 0.03). CONCLUSIONS: Patients with dilated cardiomyopathy represent a heterogeneous group with regard to both clinical outcome and the relative degree of left and right ventricular chamber dilation. Patients in the LV > RV subset appear to have better overall survival and less severe mitral and tricuspid regurgitation than do patients in the LV congruent to RV subset. Longitudinal studies are needed to determine whether these morphologic subsets in fact represent a continuum within the disease spectrum of dilated cardiomyopathy.

Cardiomyopathy, Dilated↗

PubMed1991

[Cardiac mast cells in myocardial diseases].

Mast cells play a role in inflammation and immunological reactions. Cardiac mast cells with their granules are demonstrated easily by toluidine blue staining. We evaluated cardiac mast cells in endomyocardial biopsy specimens in patients with acute myocarditis (n = 17), idiopathic dilated cardiomyopathy (n = 17), idiopathic hypertrophic cardiomyopathy (n = 17) and control subjects (n = 12). Cardiac mast cells increase to an extreme degree in patients with acute myocarditis (2.4 counts/mm2) and increase relatively in patients with idiopathic dilated cardiomyopathy (1.4 counts/mm2). Patients with idiopathic hypertrophic cardiomyopathy (0.4 counts/mm2) were similar to control subjects (0.5 counts/mm2). Cardiac mast cells increased in accord with the severity of cellular infiltration and of interstitial fibrosis except for some cases with very severe fibrosis. In the acute myocarditis group, cardiac mast cells were well demonstrated in the early stage of the illness. But degranulations of mast cells did not give us any significant information in this study. Evaluation of cardiac mast cells will provide us with a new aspect in studying primary myocardial diseases.

Adolescent↗

PubMed1989

Relation of third and fourth heart sounds to blood velocity during left ventricular filling.

To investigate the relation between changes in left ventricular inflow velocity and the timing of third and fourth heart sounds, simultaneous phonocardiograms and continuous wave Doppler traces were recorded in 48 patients (aged 17-78) with heart disease and in 21 normal children. The onset of the first vibration of the third heart sound coincided with peak left ventricular inflow blood velocity to within 5 ms in all but two of the patients. The mean (SD) difference between the two events was 5 (5) ms, which did not differ significantly from zero. The relation was similar in patients with primary myocardial disease (11), and in those with valve disease (26), hypertension (five), and coronary artery disease (four). In the normal children, the mean interval was 2.5 (5) ms--not significantly different from zero. By contrast, the first deflection of the fourth heart sound consistently preceded the timing of peak atrial inflow velocity by 55 (10) ms. Agreement was much closer between the onset of atrial flow and the onset of the atrial sound (mean difference 1 (5) ms, not significantly different from zero). Gallop sounds seem to be closely related to changes in ventricular inflow velocity, and thus to the effects of forces acting on blood flow. The forces underlying the third sound seem to arise within the ventricle and are responsible for sudden deceleration of flow during rapid ventricular filling. The fourth sound, occurring at the onset of the "a" wave, is more likely to arise from dissipation of forces causing acceleration of blood flow--that is, atrial systole itself.

Adolescent↗

PubMed1983

Selective coronary arteriography: a clinical comparison of two contrast agents.

The radiopacity and complications of meglumine iothalamate 52% and sodium iothalamate 26% (Vascoray) were compared with those of meglumine diatrizoate 66% and sodium diatrizoate 10% (Renografin -76) in 2258 patients with and without cardiac disease. There was no difference in radiopacity and the type and incidence of adverse reactions were similar, but the frequency was significantly higher (p less than 0.05) with Vascoray in patients with constrictive pericarditis, dissecting aortic aneurysm, and primary pulmonary hypertension. The difference in the frequency of hypotension, sinus bradycardia, and transient asystole in the Renografin -76 and Vascoray groups was statistically significant. Ventricular arrhythmias occurred in 6% of the patients with primary myocardial disease compared to an average of 0.7% in those without this cardiac abnormality (p less than 0.01), but there was no significant difference in the frequency in the two contrast agent groups. All reactions were treated and the studies were performed without mortality. Results of this study show that iothalamate formulation with sodium to meglumine ratio of 1:2 containing 410 mEq/L of sodium (Vascoray) is suitable and safe for clinical use for roentgenographic studies of the heart, and coronary artery circulation.

Adult↗

PubMedDate not supplied

Evolution of heart disease in utero.

Cardiac embyogenesis occurs in the first 6 to 7 weeks of human development. Although it is during this time that many of the major cardiovascular defects develop, many of these lesions continue to evolve and others develop in the latter half of gestation. There may be development or progression of ventricular inflow or outflow tract and arch obstruction, and ventricular or great artery hypoplasia. There may be progressive antrioventricular or semi-lunar valve regurgitation which can compromise the fetal circulation. There may be development of dysrhythmias, primary myocardial disease and heart failure. The fetal shunts, the foramen ovale and ductus arteriosus, may change in form and function. Finally, cardiac tumors may develop, grow, or regress. Knowledge of the mechanisms of and potential for progression in fetal heart disease is critical for counseling regarding prognosis and for planning of prenatal and neonatal management.

Disease Progression↗

PubMedDate not supplied

Doppler echocardiographic assessment of left ventricular diastolic function in myotonic dystrophy.

We utilized Doppler echocardiography to characterize left ventricular diastolic function in 42 patients with myotonic dystrophy (mean age 37 +/- 12 years, 64% male) who had no symptoms of heart failure and had normal left ventricular systolic function. Data were compared with those in 41 normal control subjects of similar age and gender. Heart rate, systemic blood pressure, and cardiac dimensions (wall thickness, left atrial and left ventricular cavity dimensions) were similar and not significantly different in patients and controls. As a group, patients showed significantly increased deceleration time and decreased rate of decline of flow velocity in early diastole (p < 0.0001 and p < 0.01, respectively) when compared to controls. Individual patient analysis showed that 10 (24%) of the 42 patients with myotonic dystrophy had 2 or more abnormal Doppler indexes of diastolic function consistent with a pattern of impaired left ventricular relaxation. The most common abnormalities were increased deceleration time (> 224 ms; 9 patients), prolonged isovolumic relaxation time (> 103 ms; 8 patients) and reduced rate of decline of flow velocity in early diastole (< 2.1 m/s2; 5 patients). In addition, peak early diastolic flow velocity was reduced (< 43 cm/s) in 3 patients and early to atrial peak flow velocity ratio was reduced (< 1) in 2 patients. Comparison of subgroups of patients with and without abnormal Doppler indexes showed no significant differences with regard to age, gender, heart rate, systemic blood pressure, severity of neuromuscular disease, and cardiac dimensions. After study, patients were clinically followed up for a mean period of 20 +/- 7 months (range 12-35). During observation no patients died and none experienced symptoms of heart failure. This Doppler echocardiographic analysis demonstrates that diastolic abnormalities may be present in patients with myotonic dystrophy, even in the absence of symptoms of cardiac failure or left ventricular systolic dysfunction. These diastolic abnormalities suggest an intrinsic myocardial abnormality in patients with myotonic dystrophy; however, whether they represent a preclinical phase of myocardial involvement or an intrinsic feature of the primary myocardial disease process in myotonic dystrophy remains to be elucidated.

Adolescent↗

PubMed1987

Specific heart muscle disease in diabetes mellitus--a functional structural correlation.

We report the morphology of diabetic myocardium obtained by endomyocardial biopsy in 16 diabetics. The material was divided into three groups. The first comprised six patients with unexplained cardiomegaly and obscure congestive cardiac failure. The second group, also of six patients, had no cardiac signs and symptoms but exhibited abnormal systolic time intervals. The third group, of 4 patients, was without any cardiac symptoms or signs and had normal systolic time intervals. The vascular and extravascular changes observed were more pronounced in the symptomatic group, intermediate in the asymptomatic patients with abnormal intervals and least in those without symptoms and normal intervals. This provides supporting evidence for the existence of a specific primary myocardial disease in diabetes with good functional structural correlation.

Adolescent↗

PubMed1983

Systolic time intervals in children with heart disease.

Of the systolic time intervals, the preejection period is known to correlate well with invasively measured isometric contraction time, and increase of the quotient preejection period/left ventricular ejection time (PEP/LVET) is considered to be of a good indicator of left ventricular failure. The different systolic time intervals have been recorded from the carotid pulse curve from 40 normal children, 20 aged five and 20 aged ten years. Their PEP/LVET was 0.31 +/- 0.04. Seventy-eight children with different heart diseases were then investigated. In patients with congenital total heart block or extrasystoles, there was a negative correlation between PEP/LVET and the R-R interval, in accordance with the Frank-Starling law. In patients with ventricular septal defects PEP/LVET differentiates between small and large shunts; the increased PEP/LVET of the latter normalizes after operation. The increased PEP/RVET of children with transposition of the great arteries is an expression of the inadequacy of the right ventricle as a systemic chamber. In aortic stenosis "normalization" of a previously decreased PEP/LVET may indicate early left ventricular failure. In primary myocardial disease registration of the systolic time intervals enables us to follow the left ventricular function more closely than is possible with invasive techniques.

Cardiomyopathies↗

PubMed1990

Current perspectives in hypertrophic cardiomyopathy: diagnosis, clinical management, and prevention of disability and sudden cardiac death.

Hypertrophic cardiomyopathy is a rare primary myocardial disease known for its dramatic morphologic and clinical manifestations. Sudden cardiac death and functional cardiac symptoms are common. However, differing pathologic mechanisms may be responsible for similar clinical symptoms and make a unified approach to therapy impossible. This review will discuss the genetics, criteria for diagnosis, relationship among pathophysiologic abnormalities and clinical symptoms, and management of hypertrophic cardiomyopathy.

Cardiomyopathy, Hypertrophic↗