Radiological patterns of obstructive cardiomyopathy of the left ventricle in childhood

The authors report 9 cases of obstructive cardiomyopathy of the left ventricle in childhood. Angiograms performed by the Seldinger technique showed the characteristic radiological features of the disease which may be either idiopathic in origin or associated with an aortic stenosis.     

Acute myocardial infarction with diminutive right coronary artery and obstructive hypertrophic cardiomyopathy without significant coronary stenoses

Focal tissue abnormalities consistent with regional ischemia have been reported in patients with hypertrophic cardiomyopathy (HCM). Coronary microvascular dysfunction has been also reported to be present in patients with HCM despite normal epicardial coronary arteries. Moreover, it has been demonstrated that in the case of HCM and idiopathic left ventricular hypertrophy, hypoplastic coronary arteries as diminutive vessels are present and that obstructive hypertrophic cardiomyopathy is associated with enhanced thrombin generation and platelet activation. Previously, it has been described an acute myocardial infarction in a young athlete with non-obstructive hypertrophic cardiomyopathy and normal coronary arteries. We present a case of an acute myocardial infarction with diminutive right coronary artery and obstructive hypertrophic cardiomyopathy without significant coronary stenoses. To our knowledge, this is the first report of an acute myocardial infarction with diminutive right coronary artery and obstructive hypertrophic cardiomyopathy without significant coronary stenoses.     

Beneficial effect of cibenzoline in a patient with hypertrophic obstructive cardiomyopathy complicated with idiopathic interstitial pneumonia

A 72-year-old woman who had idiopathic interstitial pneumonia was admitted due to general fatigue. Echocardiography revealed asymmetric septal hypertrophy and systolic anterior movement of the mitral valve. In addition, Doppler echocardiography revealed a pressure gradient of 52 mmHg in the left ventricular outflow tract. Hypertrophic obstructive cardiomyopathy was diagnosed. Because she had a respiratory disease, she was treated with cibenzoline instead of beta-blockers. After treatment her pressure gradient decreased to 10 mmHg, but respiratory symptom remained unchanged. This finding suggests that cibenzoline is useful for patients with hypertrophic obstructive cardiomyopathy complicated with respiratory disease.     

Transient Hypertrophic Obstructive Cardiomyopathy During Pregnancy

Hypertrophic obstructive cardiomyopathy (HOCM) is a primary myocardial disease with typical echocardiography features of asymmetric septal hypertrophy and evidence of left ventricular outflow tract obstruction. The natural history of HOCM is characterized by a slow progression of symptoms and a significant annual mortality rate. We report an unusual case of transient HOCM during pregnancy with evidence of total resolution postpartum.
hypertrophic cardiomyopathy, pregnancy, idiopathic hypertrophic subaortic stenosis, two-dimensional echocardiography, Doppler echocardiography     

Drug treatment of hypertrophic cardiomyopathy

Both, beta-receptor blocking agents and calcium-antagonists have been proven to be very effective in the treatment of idiopathic hypertrophic cardiomyopathy. In the vast majority of patients they lead to a significant reduction of the predominant clinical symptoms--angina and dyspnea--or even abolish them. Since beta-blockers primarily act on the contractile behaviour of the left ventricle they are especially suited in the obstructive form of the disease where they reduce outflow tract obstruction. In contrast, calcium-antagonists seem to mainly influence diastolic muscle mechanics by increasing diastolic distensibility, and thus may be indicated in the nonobstructive forms of hypertrophic cardiomyopathy. In cases with severe, life-threatening arrhythmias especially if associated with syncope, an antiarrhythmic therapy is mandatory.     

Resting heart rate and cardiac function in dilated cardiomyopathy

We hypothesized that, within the normal range of resting heart rate, heart rate and left ventricular ejection fraction would be inversely correlated and heart rate and left ventricular filling would be correlated in patients with dilated cardiomyopathy and not correlated in patients with normal cardiac function. At rest, heart rate, left ventricular ejection fraction, and three measures of diastolic filling (time to peak filling rate, peak filling rate, and first half filling fraction) were recorded using radionuclide ventriculography in subjects with no cardiac disease, patients with idiopathic dilated cardiomyopathy, and patients with dilated cardiomyopathy associated with ischemic heart disease. Heart rate had significant inverse correlations with left ventricular ejection fraction (r=-0.55, P=0.0007) and time to peak filling rate (r=-0.47, P=0.005) and a positive correlation with peak filling rate (r=0.73, P<0.0001) in patients with idiopathic dilated cardiomyopathy; heart rate was correlated only weakly with these measures in the absence of cardiac disease and essentially was not correlated in dilated cardiomyopathy due to ischemic heart disease. The change in resting heart rate with left ventricular ejection fraction and time to peak filling rate were significantly (P<0.05) different between patients with no cardiac disease and those with idiopathic dilated cardiomyopathy. Thus, resting heart rate correlated significantly with left ventricular ejection fraction and diastolic filling in patients with idiopathic dilated cardiomyopathy.     

Significance of coronary angiography, left heart catheterization, and endomyocardial biopsy for the diagnosis of idiopathic dilated cardiomyopathy.

Many physicians assume that a reliable diagnosis of idiopathic dilated cardiomyopathy can be made by noninvasive methods, mainly echocardiography. On the other hand, use of endomyocardial biopsy in those patients who have undergone left heart catheterization and who demonstrate left ventricular dysfunction of unknown origin is increasing. Therefore the purpose of this study was to investigate the yield of that diagnostic strategy in patients with the tentative diagnosis of idiopathic dilated cardiomyopathy. Between 1980 and 1988, 3.2% of our 15,442 patients were diagnosed as having idiopathic dilated cardiomyopathy on the basis of left heart catheterizations and coronary angiograms. Idiopathic dilated cardiomyopathy was diagnosed in 444 patients on the basis of clinical data and results of noninvasive tests before catheterization, but in only 295 of these cases was the diagnosis confirmed by means of coronary angiography and left heart catheterization (predictive value of noninvasive tests is 66%). The remaining 34% of patients demonstrated extensive coronary artery disease (13%), significant valvular heart disease (11%), and other or no heart disease (10%). The diagnostic sensitivity of noninvasive tools (patient history, ECG, stress test, echocardiography) was only 59%. Left heart catheterization can easily be combined with endomyocardial biopsy. With the use of histologic techniques, specific heart muscle diseases were detectable in 3.5% of 209 patients, but in only 1% could therapeutic consequences be determined. Thus coronary angiography and left heart catheterization are mandatory for the correct diagnosis of idiopathic dilated cardiomyopathy.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of hypertrophic cardiomyopathy with right ventricular outflow obstruction manifested during three-year follow-up study

A case is reported of a 55-year-old female with idiopathic hypertrophic cardiomyopathy, which was accompanied with outflow obstruction in the right ventricle developed during the previous 3 years without lesion of the left ventricle. In 1984, she was admitted to our hospital to be examined for cardiac murmurs and abnormal electrocardiogram including ST depression and inverted T. The findings of echocardiography and cardiac catheterization revealed non-obstructive hypertrophic cardiomyopathy. She had been treated with sympathetic beta-blockade and calcium antagonist for 3 years until she complained of dyspnea on exertion, and she was readmitted to our hospital in 1987. Echocardiographic findings showed protrusion of the ventricular septum toward the right ventricle and systolic turbulent flow along the right ventricular outflow tract (by pulsed Doppler technique). A pressure gradient of 20 mmHg across the protrusion was detected by the examination of the cardiac catheter. However, neither protrusion nor pressure gradient was observed in the left ventricular outflow tract as well as that in 1984. Idiopathic hypertrophic cardiomyopathy has been described as involving both ventricles, and outflow obstruction is the usual hemodynamic finding in the left ventricle. However, right ventricular outflow obstruction is the usual hemodynamic finding in the left ventricle. However, right ventricular outflow obstruction with the left ventricular outflow tract intact has been very rare. In addition, in this case, the change of hemodynamic characteristics from non-obstructive to obstructive hypertrophic cardiomyopathy, and the development of these changes only in the right ventricle were observed during the last 3 years.     

Echocardiographic mitral systolic motion in left ventricular aneurysm.

An abnormal, convex systolic anterior motion of the mitral valve, somewhat similar to that seen in idiopathic hypertrophic subaortic stenosis (hypertrophic obstructive cardiomyopathy) and occasionally in atrial septal defect, was found on the echocardiogram in 8 out of 9 patients with angiographically (7 patients) and/or clinically (2 patients) diagnosed left ventricular aneurysm secondary to myocardial infarction. The only patient whose echocardiogram failed to demonstrate convex systolic anterior motion was the only patient who had an inferior wall aneurysm; the rest of the group had anterior and/or apical aneurysms. In contrast to patients with typical idiopathic hypertrophic subaortic stenosis, the convex systolic anterior motion of the mitral valve observed in our patients tended to 'peak' rather than 'plateau'. Through the basis for this abnormal septal motion in our patients is uncertain, alterations in left vantricular configuration, plus a relatively vigorously contracting posterior left ventricular wall in the presence of abnormal interventricular septal motion, are probable contributory factors. Though non specific, in the proper clinical and echocardiographic setting convex systolic anterior motion of the mitral valve may be another sign of left ventricular aneurysm.     

Echocardiographic mitral systolic motion in left ventricular aneurysm.

An abnormal, convex systolic anterior motion of the mitral valve, somewhat similar to that seen in idiopathic hypertrophic subaortic stenosis (hypertrophic obstructive cardiomyopathy) and occasionally in atrial septal defect, was found on the echocardiogram in 8 out of 9 patients with angiographically (7 patients) and/or clinically (2 patients) diagnosed left ventricular aneurysm secondary to myocardial infarction. The only patient whose echocardiogram failed to demonstrate convex systolic anterior motion was the only patient who had an inferior wall aneurysm; the rest of the group had anterior and/or apical aneurysms. In contrast to patients with typical idiopathic hypertrophic subaortic stenosis, the convex systolic anterior motion of the mitral valve observed in our patients tended to 'peak' rather than 'plateau'. Through the basis for this abnormal septal motion in our patients is uncertain, alterations in left vantricular configuration, plus a relatively vigorously contracting posterior left ventricular wall in the presence of abnormal interventricular septal motion, are probable contributory factors. Though non specific, in the proper clinical and echocardiographic setting convex systolic anterior motion of the mitral valve may be another sign of left ventricular aneurysm.     

Impact of percutaneous transluminal septal myocardial ablation on refractory paroxysmal atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy

Atrial fibrillation is commonly observed in patients with hypertrophic obstructive cardiomyopathy. Episodes of paroxysmal atrial fibrillation are often torturous and limit the quality of life by causing congestive heart failure, transient hypotension, or bradycardia. Control of paroxysmal atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy is considered to be important for symptomatic improvement and prevention of the development to chronic atrial fibrillation. The authors report on 3 patients with hypertrophic obstructive cardiomyopathy who suffered from paroxysmal atrial fibrillation despite receiving medical treatment using antiarrhythmic agents. However, after undergoing percutaneous transluminal septal myocardial ablation, the incidence of episodes became significantly less frequent. Percutaneous transluminal septal myocardial ablation is normally performed for attenuating left ventricular obstruction by reducing the systolic anterior motion of the mitral leaflet. However, in these patients, this procedure was also effective in preventing supraventricular arrhythmia, probably by improving left ventricular diastolic dysfunction, smooth blood inflow into the left ventricular, and decreasing the pressure stress against the left atrial wall.     

Mid-ventricular hypertrophic obstructive cardiomyopathy presenting with acute myocardial infarction

Mid-ventricular hypertrophic obstructive cardiomyopathy is a rare type of cardiomyopathy that can be accompanied by apical aneurysm. We report the case of a patient who presented with ventricular fibrillation, ST-segment elevation on electrocardiography, and cardiac-enzyme elevation, in the presence of normal coronary arteries. Echocardiography and magnetic resonance imaging showed an hourglass appearance of the left ventricle with an aneurysm in the apex. Left-heart catheterization and continuous-wave Doppler echocardiography revealed a pressure gradient between the apical and basal chambers of the left ventricle. Impaired coronary artery circulation might play a role in the development of mid-ventricular obstruction in patients with mid-ventricular hypertrophic obstructive cardiomyopathy.     

Comparison of long-term outcome of alcoholic and idiopathic dilated cardiomyopathy.

AIMS: The outcome of alcoholic cardiomyopathy is thought to be better than idiopathic dilated cardiomyopathy if patients abstain from alcohol. The aim of this study was to compare the long-term clinical outcome of alcoholic and idiopathic dilated cardiomyopathy. METHODS AND RESULTS: Of 134 patients with dilated cardiomyopathy and normal coronary angiography, 50 had alcoholic cardiomyopathy; they were compared serially to 84 patients with idiopathic dilated cardiomyopathy. Left ventricular end-diastolic diameter, left ventricular ejection fraction and cardiac index, severity of ventricular arrhythmias, measurement of heart rate variability and results of signal-averaged ECG were similar in both groups. Although alcohol withdrawal was strongly recommended but observed in only 70% of patients with alcoholic cardiomyopathy, both groups had similar outcome in terms of cardiac death after follow-up treatment of 47+/-40 months. Multivariate analysis in the entire cohort demonstrated that increased pulmonary capillary wedge pressure (P=0. 003), alcoholism and lack of abstinence during follow-up (P=0.006) and decreased standard deviation of all normal-to-normal RR intervals (P=0.02) were independent predictors of cardiac death. CONCLUSION: In contrast with previous studies, patients with alcoholic cardiomyopathy did not have a better outcome than patients with idiopathic dilated cardiomyopathy. Alcoholism without abstinence was a strong predictor of cardiac death. This suggests that a more aggressive approach to alcohol cessation is needed in these patients.     

Significant X-ray patterns in cardiomyopathy--an approach improving noninvasive diagnosis

To identify their characteristic patterns, the X-ray dorso-ventral chest images of 118 patients with cardiomyopathy (63--dilated cardiomyopathy, 25--hypertrophic obstructive cardiomyopathy, 30--hypertrophic nonobstructive cardiomyopathy) were analyzed and compared with those of 22 patients without cardiac abnormalities. All 140 patients underwent comprehensive invasive cardiac examinations. In dilated and hypertrophic nonobstructive cardiomyopathy transvasal endomyocardial biopsy was performed. Irrespective of the type and the aetiology of cardiomyopathy, 4 characteristic patterns of X-ray findings could be identified: a class of left heart abnormality (class L) and 3 types of bilateral cardiac abnormalities with various stages of left heart failure (class L + R). An association between the image patterns and haemodynamic parameters (left ventricular end-diastolic pressure, wall mass index, pulmonary mean pressure) was demonstrated which was most obvious in dilated and less evident in hypertrophic obstructive cardiomyopathy. Mitral insufficiency in all types and classes frequently corresponded with the X-ray findings of left atrial enlargement. The X-ray classification method proved to be an efficient approach and superior to the heart-to-lung ratio.     

Echocardiography in the diagnosis of hypertrophic obstructive cardiomyopathy

The role of echocardiography in the diagnosis of hypertrophic obstructive cardiomyopathy is reviewed. Salient features include a narrowed left ventricular outflow space, the increased thickness of the interventricular septum and the systolic anterior motion of the anterior mitral leaflet. The last may be present at rest, or it may be provoked with appropriate maneuvers. When all three features are demonstrated, a diagnosis of hypertrophic obstructive cardiomyopathy can be made with confidence. In the absence of resting or provocable systolic anterior motion, a diagnosis of nonobstructive cardiomyopathy may be entertained. An additional important echocardiographic feature of hypertrophic obstructive cardiomyopathy consists of an interventricular septal to posterior wall ratio in excess of 1.5, suggesting the asymmetric distribution of hypertrophy. Inert interventricular septum, aortic valve preclosure and a reduced diastolic slope of the anterior mitral leaflet are frequently present, but not specific for the condition. When hypertrophic obstructive cardiomyopathy is associated with other unrelated diseases, the index of suspicion may be raised even in the absence of all the “classic” echo signs.     

Comparison of echocardiographic features of noncompaction of the left ventricle in adults versus idiopathic dilated cardiomyopathy in adults

Noncompaction of left ventricular myocardium (NCLV), or "spongy myocardium," in adults represents an arrest in endomyocardial morphogenesis and occurs as an isolated cardiomyopathy. Because NCLV can be readily mistaken for idiopathic dilated cardiomyopathy, echocardiographic features other than the structural features of the myocardial wall need to be carefully defined for distinguishing the 2 conditions. This study was therefore designed to characterize the echocardiographic features that could be useful for differentiating NCLV from idiopathic dilated cardiomyopathy.     

Acute mitral regurgitation due to ruptured chordae tendineae in a patient with hypertrophic obstructive cardiomyopathy: a case report

A 63-year-old woman had been followed up for hypertrophic obstructive cardiomyopathy with 85 mmHg of left ventricular outflow tract pressure gradient over 7 years. She was hospitalized because of acute dyspnea and syncope. On admission, echocardiography revealed severe mitral regurgitation with ruptured chordae tendineae at the medial scallop of the posterior mitral leaflet. Mitral valve replacement was successfully performed and her symptoms improved to 28 mmHg of left ventricular outflow tract pressure gradient. In patients with hypertrophic obstructive cardiomyopathy, elevated left ventricular systolic pressure and systolic anterior motion of the mitral leaflets may lead to mucoid degeneration in the chordae tendineae. Rupture of the mitral chordae tendineae should be considered in the differential diagnosis of acutely deteriorated mitral regurgitation in patients with hypertrophic obstructive cardiomyopathy, because this is a rare but critical complication.     

Felty's and Sj?gren's syndromes and hypertrophic obstructive cardiomyopathy (case report)

Our findings refer to a female patient with Felty's and Sj?gren's syndromes and hypertrophic obstructive cardiomyopathy. The rheumatoid granuloma found in the left ventricle at autopsy and the absence of other conditions which may be manifested by hypertrophic obstructive cardiomyopathy suggest that the case studied, probably represents an extraarticular manifestation of Felty's and Sj?gren's syndromes.     

Left ventricular structural adaptations to obstructive sleep apnea in dilated cardiomyopathy

RATIONALE AND OBJECTIVES: Obstructive sleep apnea is common among patients with heart failure and exposes the left ventricle to trophic mechanical and adrenergic stimuli. We hypothesized that in heart failure patients with nonischemic dilated cardiomyopathy (a condition characterized by eccentric hypertrophy), those with obstructive sleep apnea would have a higher prevalence of left ventricular hypertrophy by wall thickness criteria (> or = 12 mm), and greater septal thickness than those without obstructive sleep apnea. METHODS AND RESULTS: We performed echocardiography and polysomnography in 47 patients with nonischemic dilated cardiomyopathy. Obstructive sleep apnea was present in 45% of these patients. The prevalence of left ventricular hypertrophy was greater in those with than in those without obstructive sleep apnea (47.6 vs. 15.4%, p = 0.016). Interventricular septal thickness (p < 0.001) and relative wall thickness (p = 0.011) were significantly greater in those with than in those without obstructive sleep apnea. However, there was no significant difference in posterior wall thickness between the groups. The frequency of obstructive apneas and hypopneas during sleep was the only significant independent correlate of septal thickness (p = 0.001). CONCLUSIONS: In patients with nonischemic dilated cardiomyopathy, the presence of obstructive sleep apnea is associated with an increased prevalence of left ventricular hypertrophy. The higher relative wall thickness and interventricular septal thickness in patients with obstructive sleep apnea indicate that the left ventricle is relatively less eccentric than in patients without obstructive sleep apnea, and that such remodeling affects mainly the septum. These structural adaptations may reflect unique nocturnal mechanical and adrenergic stimuli associated with obstructive sleep apnea.     

Sleep-disordered breathing in patients with idiopathic cardiomyopathy.

BACKGROUND: Sleep-disordered breathing may adversely affect heart function, and thereby contribute to the progression of heart failure. A study was undertaken in patients with idiopathic cardiomyopathy to document the characteristics of sleep-disordered breathing. METHODS AND RESULTS: Thirty-five patients with a diagnosis of idiopathic cardiomyopathy, comprising 20 patients with dilated cardiomyopathy (DCM) and 15 patients with hypertrophic cardiomyopathy (HCM), underwent overnight polysomnography. Of these 35, 16 (80%) of the DCM patients and 7 (47%) of the HCM patients had sleep-disordered breathing. Central sleep apnea-hypopnea syndrome (CSAHS) was seen in 10 DCM patients, but not in the HCM patients, and obstructive sleep apnea-hypopnea syndrome (OSAHS) was seen in 6 DCM patients and 7 HCM patients. CSAHS was seen in DCM patients with a low left ventricular ejection fraction. HCM patients with OSAHS had a significantly greater body mass index (BMI) than those without OSAHS and CSAHS (27.6 +/- 3.8 vs 22.0 +/- 4.0 kg/m2, p<0.05). DCM patients with OSAHS had a larger BMI than those with CSAHS (29.3 +/- 5.8 vs 24.2 +/- 4.0 kg/m2, p<0.05) and those without OSAHS and CSAHS (29.3 +/- 5.8 vs 21.3 +/- 3.1 kg/m2, p<0.05). CONCLUSIONS: Sleep-disordered breathing is common in patients with idiopathic cardiomyopathy; half of DCM patients had CSAHS, which was closely associated with obesity.