Congenital left ventricular diverticulum diagnosed by 64-detector CT imaging

A congenital left ventricular (LV) diverticulum or aneurysm is a rare cardiac malformation. It is a developmental anomaly that occurs during embryogenesis. Most congenital LV aneurysms and diverticula are asymptomatic or may cause systemic embolization, heart failure, valvular regurgitation, ventricular wall rupture, ventricular tachycardia or sudden cardiac death. Diagnosis is established by imaging studies such as echocardiography, magnetic resonance imaging or left ventriculography, visualizing the structural changes. We report the case of a 28-year-old male referred for the evaluation of atypical chest pain who was found to have an LV diverticulum affecting the inferoposterior wall of his LV.     

Multimodality imaging of a left ventricular aneurysm in a patient with normal coronary arteries: Unusual localization

Left ventricular aneurysm (LVA) is rare but potentially fatal complication of myocardial infarction. It has been strictly defined as a distinct area of abnormal left ventricular diastolic contour with systolic dyskinesia or paradoxical bulging. It may be asymptomatic. However, complications including thromboembolism, heart failure, valvular regurgitation, arrhythmia, and rarely rupture may be the initial presentation. Diagnosis may be established by transthoracic echocardiography, left ventriculography, computed tomographic angiography, and cardiac magnetic resonance imaging. Here, we report a case of uncommonly located LVA in an adult patient with normal coronary arteries, which was diagnosed with multimodality imaging.     

Left ventricular aneurysm as a coronary risk factor independent of overall left ventricular function

Patients with left ventricular aneurysm (LVA) have been shown to have a higher mortality rate than those with normal left ventricular function. The purpose of this study was to determine whether or not LVA, in patients with coronary artery disease, is a risk factor independent of left ventricular function. Thirty-nine patients with angiographically demonstrated segmental dyskinesis (LVA group) were retrospectively compared to 28 patients with segmental akinesis and ejection fraction less than 60% (control group). There was no significant difference in age, ejection fraction, severity of coronary artery disease, cardiac index, or frequency of cardiac surgery between the two groups. Compared to control subjects, the LVA group had a significantly higher left ventricular end-diastolic pressure and greater tendency to have apical involvement. Although electrocardiography, echocardiography, and radioventriculography were each highly specific, their sensitivities were only 40% to 60%. Follow-up data were available for a mean of 33 months after catheterization. No significant benefit from aneurysmectomy could be demonstrated. There was an insignificant trend in the LVA group toward more severe congestive heart failure and less angina. There was no significant difference in the reinfarction rate, incidence of ventricular tachycardia, or embolism. Mortality rate was 38% in the LVA group and 32% in the control group (p = 0.59). We conclude that LVA is not an independent risk factor for congestive heart failure, angina, ventricular tachycardia, reinfarction, embolism, or death.     

Echocardiographic findings in systemic amyloidosis: spectrum of cardiac involvement and relation to survival

One hundred thirty-two patients with biopsy-proven systemic amyloidosis underwent echocardiographic examination to define the spectrum of cardiac involvement. Echocardiographic abnormalities were then correlated with clinical variables and survival at follow-up. Patients were subgrouped by left ventricular wall thickness: Group I, mean wall thickness 12 mm or less; Group II, mean wall thickness greater than 12 mm but less than 15 mm; Group III, mean wall thickness 15 mm or greater; or Group IV, atypical features such as wall motion abnormalities or left ventricular dilation. Patients with greater wall thickness had a higher frequency of associated echocardiographic abnormalities such as left atrial enlargement or granular sparkling appearance on two-dimensional examination and, more commonly, reduced systolic function. The occurrence of clinical congestive heart failure was strongly correlated with greater wall thickness and multiple other echocardiographic abnormalities. Survival was negatively influenced both by greater wall thickness and reduced systolic function. The median survival of the entire group was 1.1 years. Echocardiographic examination is an important tool for establishing the presence of cardiac amyloid involvement and may be useful in estimating prognosis in such patients.     

Echocardiographic abnormalities in chronic alcoholics with and without overt congestive heart failure

To assess the type and prevalence of cardiac abnormalities in heavy drinkers with and without overt congestive heart failure, M mode echocardiography was performed in 11 symptomatic chronic alcoholics with dilated (congestive) cardiomyopathy and in 22 asymptomatic chronic alcoholics. Echocardiographic data in both groups were adjusted for age and body surface area using previously derived regression equations. All 11 symptomatic patients had a significantly decreased left ventricular percent fractional shortening (mean 14 percent, normal range 28 to 44) along with significant increases in left ventricular systolic and diastolic dimensions (mean increases of 105 and 48 percent above normal, respectively), left atrial dimension (mean increase 21 percent) and estimated left ventricular mass (mean increase 105 percent). Among the 22 asymptomatic patients, 15 (68 percent) demonstrated significant increases in at least one of the following echocardiographic variables: left ventricular mass, left ventricular dimensions, septal and left ventricular wall thicknesses, and left atrial dimension. Asymptomatic patients could be classified into two subgroups: (1) those with a left ventricular diastolic dimension less than 10 percent above the normal predicted value and an increased left ventricular wall thickness to radius ratio (mean increase 16 percent above normal) and upper normal percent fractional shortening, and (2) those with a left ventricular diastolic dimension 10 to 24 percent above normal and a slightly subnormal thickness to radius ratio and lower normal percent fractional shortening. Echocardiographic abnormalities in asymptomatic chronic alcoholics did not correlate with the presence or absence of auscultatory abnormalities on physical examination and appear to reflect an earlier stage in the spectrum of alcoholic disease before the development of dilated cardiomyopathy.     

Arrhythmic manifestations in patients with congenital left ventricular aneurysms and diverticula

Congenital left ventricular aneurysms and diverticula (LVA/Ds) are rare cardiac malformations that can be detected using echocardiography or other imaging techniques. Some of these patients present with ventricular arrhythmias. This study investigated clinical characteristics of patients with congenital LVA/D presenting with arrhythmic manifestations. Over the previous 20 years 250 patients were diagnosed to have congenital LVA/D at our institution. Diagnosis was made using echocardiography after exclusion of coronary artery disease, local cardiac inflammatory processes, traumatic causes, or cardiomyopathies. At initial presentation 32 of the 250 patients (13%, average age 45 years, range 25 to 65, 21 men and 11 women) exhibited arrhythmias. At least 2 LVA/Ds were present in 6 of these patients. LVA/Ds were localized at the posterobasal, apical, anteroseptal, and anterolateral walls in 12, 11, 4, and 5 patients, respectively. The most common complaints at presentation were syncope or presyncope in 18 patients and palpitations in 11 patients. One patient had survived sudden cardiac death. Long-term electrocardiographic recordings showed ventricular tachycardia (VT) or ventricular fibrillation in 17 patients (53%). Twelve patients underwent electrophysiologic testing. Nine patients had inducible ventricular tachyarrhythmia, whereas induced tachycardia was similar to that during spontaneous arrhythmia in 7 patients. In conclusion, patients with congenital LVA/Ds who present with arrhythmic manifestations commonly have VT. Electrophysiologic testing can reproduce clinical VT in most of these patients.     

Tropenerkrankungen des Herzens

American trypanosomiasis is a parasitosis found through Latin America; it is transmitted through contact with fecal excretions of triatomine bugs or through blood transfusion. Up to 30% of the infected individuals develop a chronic form of Chagas disease, mainly affecting the heart, digestive tract or central nervous system. Clinical syndromes compatible with Chagas disease include conduction system abnormalities, apical aneurysms, disturbances of wall motion without signs of coronary heart disease, bradyarrhythmias or tachyarrhythmias, and dilated cardiomyopathy. Cardiac involvement is reported in up to 2% of echinococcosis patients. Hydatid cysts of the interventricular septum cause conduction system abnormalities. Intrapericardial rupture of a cyst can lead to pericarditis and cardiac tamponade, while the consequences of intracardiac rupture may be embolization to the pulmonary or systemic circulation. An anaphylactic reaction and death may follow intracavitary rupture.     

Postinfarction left ventricular pseudoaneurysm

Left ventricular pseudoaneurysm is a rare complication of heart rupture as a result of acute myocardial infarction, tumour infiltration or infective pericarditis. This pathology is often diagnosed accidentally because of non-specific clinical manifestations such as congestive heart failure or no symptoms at all. Diagnosis of pseudoaneurysm should result in an urgent surgical treatment as the risk of sudden death due to aneurysm rupture is high. In this report we present a patient who underwent successful surgical treatment of left ventricular pseudoaneurysm as a complication of myocardial infarction.     

Coronary artery disease detection-limitations of stress testing in left ventricular dysfunction

Incidental diagnosis of left ventricular systolic dysfunction(LVD) is common in clinical practice. The prevalence of asymptomatic LVD(Ejection Fraction,EF 50%) is 6.0% in men and 0.8% in women and is twice as common as symptomatic LVD. The timely and definitive exclusion of an ischemic etiology is central to optimizing care and reducing mortality in LVD. Advances in cardiovascular imaging provide many options for imaging of patients with left ventricular dysfunction. Clinician experience,patient endurance,imaging modality characteristics,cost and safety determine the choice of testing. In this review,we have compared the diagnostic utility of established tests-nuclear and echocardiographic stress testing with newer techniques like coronary computerized tomography and cardiac magnetic resonance imaging and highlight their inherent limitations in patients with underlying left ventricular dysfunction.     

Prevalence and spectrum of coronary artery anomalies in patients with an isolated congenital left ventricular aneurysm or diverticulum

Background:

Congenital left ventricular aneurysm (LVA) and diverticulum (LVD) are rare cardiac anomalies and frequently associated with other cardiac anomalies. The objective of our study was to investigate the prevalence and the spectrum of coronary anomalies in such patients.

Hypothesis:

The incidence of coronary anomalies is increased in patients with LVA or LVD.

Methods:

We assessed 117 patients with isolated LVA or LVD for the prevalence of coronary anomalies and compared the findings with an age‐ and sex‐matched control group (n = 117) without the diagnosis of LVA or LVD.

Results:

Coronary anomalies were present in 58.1% of the study population (68 of 117). The median age of affected patients was 64 years, and 45 (38.5%) were male. Coronary anomalies were more prevalent in patients with LVA or LVD (58.1% vs 6.8%, P < 0.001), male patients (89% vs 57%, P = 0.0002), and in patients with nonapical location of LVA or LVD (24% vs 45%, P = 0.02) compared with control, whereas age and type (LVA vs LVD) had no influence (57% vs 57.6%, P = 0.4; and 58.8% vs 57.6%, P = 0.87, respectively). The number of adverse cardiac events was similar in both groups during a 4.2‐year follow‐up period (29% vs 19%, P = 0.09). None of the patients in our series had major coronary anomalies with potential lethal consequences.

Conclusions:

This large single‐center study suggests that the prevalence of abnormal coronary‐artery anatomy in patients with isolated LVA or LVD is as high as 58.1%. However, we did not identify major coronary anomalies with potential lethal consequences, and the clinical course during follow‐up was not influenced by the presence or absence of coronary anomalies. © 2011 Wiley Periodicals, Inc. The authors have no funding, financial relationships, or conflicts of interest to disclose.     

Clinical utility of gated cardiac blood pool imaging in congestive left heart failure

Our experience with gated cardiac blood pool imaging in the evaluation of congestive left-sided heart failure was reviewed in 82 patients. Ventricular contraction patterns, right and left ventricular size, and regional wall motion were evaluated from technetium-99m-albumin gated blood pool scans obtained in anterior and left anterior oblique projections. Patterns of ventricular function shown by scan were classified as follows: normal right and left ventricular size and contraction, normal left ventricular size with right ventricular enlargement, left ventricular volume overload, diffuse left ventricular hypokinesis, regional left ventricular asynergy, left ventricular aneurysm and hypertrophic cardiomyopathy. In 34 of 36 patients who underwent cardiac catheterization, the pattern of left ventricular dysfunction revealed by scan agreed with the findings on left ventriculography. Left ventricular end-diastolic diameters were significantly (p < 0.001) increased in patients with heart failure due to previous myocardial infarction, congestive cardiomyopathy, left ventricular volume overload and left ventricular pressure overload. Right ventricular diameters were increased predominantly among patients with congestive cardiomyopathy and mitral stenosis. Clinically, gated cardiac imaging was useful for (1) diagnostic screening prior to cardiac catheterization; (2) determination of the potential for improvement with surgical operation; and (3) prognostication from the severity of left ventricular dysfunction.     

Obesity and the heart

Obesity has been identified as an independent risk factor for coronary heart disease and congestive heart failure. Although congestive heart failure can be secondary to coronary heart disease, in morbid obesity these conditions can be independent. Cardiac structure and function can be altered even in the absence of systemic hypertension and underlying organic heart disease. In obese patients total blood volume increases and creates a high cardiac output state that may cause ventricular dilatation and ultimately eccentric hypertrophy of the left (and possibly the right) ventricle. Eccentric left ventricular hypertrophy produces diastolic dysfunction. Systolic dysfunction may ensue due to excessive wall stress if wall thickening fails to keep pace with dilatation. This disorder is referred to as obesity cardiomyopathy. The frequent coexistence of systemic hypertension in obese individuals facilitates development of left ventricular dilatation and hypertrophy. Congestive heart failure may occur and may be attributable to left ventricular diastolic dysfunction or to combined diastolic and systolic dysfunction. The risk of coronary heart disease seems to be more strictly correlated to central obesity than to increased body mass index. Insulin resistance seems to be the key factor that links obesity and ischaemic heart disease. In such a condition the so called Syndrome X appears. It is characterized by: obesity, systemic hypertension, diabetes mellitus, hypertriglyceridaemia and reduced HDL cholesterol levels. Considering that left ventricular hypertrophy is often present, many risk factors coexist in obese patients. Weight loss is very useful in obese patients. It may reduce mortality and morbidity for coronary heart disease and delay or avoid the appearance of congestive heart failure. It is proved that after weight loss, blood pressure, glucose, cholesterol, triglycerides and left ventricular mass decrease.     

Cardiac involvement in systemic sclerosis

Systemic sclerosis (SS) can involve the pericardium, myocardium, conduction system, and cardiac valves. The presence of overt clinical signs of cardiac disease is a poor prognostic sign. Clinical manifestations include dyspnea, palpitations, chest pain, syncope, and symptoms of right heart failure. Prevalence of clinically symptomatic pericardial disease is 5-16%. However, ecocardiographic prevalence is 5.4- 41% and at autopsy is 33-77.5%. Patchy fibrosis is the characteristic myocardial finding in SS. Contraction band necrosis is the typical pathological finding. Important complications of fibrosis include left ventricular hypertrophy, as well as systolic and diastolic dysfunction of both ventricles. Early detection of these abnormalities is very important, mainly of the diastolic dysfunction, since it occurs before the systolic dysfunction and can predict important cardiac damage. Association of skeletal myositis with myocardial disease has been described. Patients with skeletal myositis are more likely to develop congestive heart failure, sustained symptomatic arrythmias, and cardiac sudden death. Coronary arteries are normal in systemic sclerosis, but there is no endomyocardial vessel involvement. There is an increased prevalence of arrhytmias, mainly premature atrial and ventricular contractions, as well as conduction system disease. Cardiac valvular involvement is minor in systemic sclerosis; mitral valve is the most frequently affected. Other abnormalities described in this disease include peripheral large vessels stiffness and secondary cardiac involvement due to pulmonary and systemic arterial hypertension. Cardiac involvement confers a high morbi-mortality risk in systemic sclerosis.     

Obesity cardiomyopathy: pathophysiology and evolution of the clinical syndrome

Obesity produces an increase in total blood volume and cardiac output because of the high metabolic activity of excessive fat. In moderate to severe cases of obesity, this may lead to left ventricular dilation, increased left ventricular wall stress, compensatory (eccentric) left ventricular hypertrophy, and left ventricular diastolic dysfunction. Left ventricular systolic dysfunction may occur if wall stress remains high because of inadequate hypertrophy. Right ventricular structure and function may be similarly affected by the aforementioned morphologic and hemodynamic alterations and by pulmonary hypertension related to the sleep apnea/ obesity hypoventilation syndrome. The term obesity cardiomyopathy is applied when these cardiac structural and hemodynamic changes result in congestive heart failure. Obesity cardiomyopathy typically occurs in persons with severe and long-standing obesity. The predominant causes of death in those with obesity cardiomyopathy are progressive congestive heart failure and sudden cardiac death.     

Diagnosis and Management of Cardiac Sarcoidosis

Cardiac sarcoidosis is an underdiagnosed disease that may be present in as many as 25% of patients with systemic sarcoidosis. Although most commonly recognized in patients with other manifestations of sarcoidosis, it may occur in isolation and its presence is often not appreciated. Cardiac sarcoidosis may present as asymptomatic left ventricular dysfunction, congestive heart failure, atrioventricular block, atrial or ventricular arrhythmia and sudden death. Although untested in clinical trials, early use of high-dose steroid therapy may halt or reverse cardiac damage. This article reviews the clinical manifestations, diagnosis and treatment of sarcoidosis, with an emphasis on new imaging techniques and therapies.     

Age-adjusted heart rate variability as an index of the severity and prognosis of heart failure

The age-adjusted, heart rate variability (HRV) was evaluated as a parameter for the severity of heart failure and its prognosis. HRV was obtained by 24-h Holter monitoring in patients with left ventricular dysfunction (LVD). New York Heart Association (NYHA) functional classification, echocardiography, radioisotope ventriculography, and blood examination were performed, and compared between patients and normal subjects. The evaluation was repeated during the follow-up period. Finally, using the lower limit of HRV, patients were divided into either normal or abnormal group for each low-frequency power (LF) and high-frequency power (HF) (age-adjusted HRV). Other parameters of heart failure and prognosis were compared between these 2 groups. HRV tended to be lower in patients with LVD. HF decreased at the early stage of heart failure, but did not decrease progressively. LF decreased progressively. HRV change paralleled the change of NYHA. The abnormal HRV group showed a poor prognosis for cardiac death, but not for sudden cardiac death. In patients with LVD, HRV was decreased compared with the normal subjects. Change in HRV correlated with the change in NYHA classification. Age-adjusted HRV correlated with cardiac-death prognosis, but not for sudden cardiac death.     

The cardiovascular manifestations of the hypereosinophilic syndrome. Prospective study of 26 patients, with review of the literature.

The major cause of morbidity and mortality in patients with the hypereosinophilic syndrome is cardiac dysfunction. A review of 65 cases from the literature (historic series) revealed the following cardiovascular manifestations to be most common: dyspnea (60 per cent), signs of congestive heart failure (75 per cent), murmur of mitral regurgitation (49 per cent), cardiomegaly (37 per cent), T wave inversions on electrocardiogram (37 per cent) and pathologic findings of endocardial fibrosis, myocardial inflammation and mural thrombus formation (57 per cent). We have prospectively followed 26 patients with the hypereosinophilic syndrome for up to nine years (average follow-up prospectively was 3.3 years, retrospectively 5.7 years). Common cardiac findings in our 26 patients were dyspnea (42 per cent), chest pain (27 per cent), signs of congestive heart failure (38 per cent), murmur of mitral regurgitation (42 per cent), cardiomegaly (35 per cent) and T wave inversions (35 per cent). Thus, these patients demonstrated cardiovascular manifestations similar to those in the historic series, although the literature review showed a higher incidence of overt congestive heart failure.Of 22 patients having echocardiograms, 55 per cent demonstrated some clinical, roentgenographic or electrocardiographic evidence of cardiac involvement, but 82 per cent had echocardiographic abnormalities. This suggests that the echocardiogram is a sensitive and perhaps early indicator of cardiac involvement in this disease. Common echocardiographic findings included increased left ventricular wall thickness (68 per cent), left ventricular mass (73 per cent) and left atrial size (37 per cent). Prospective echocardiographic follow-up of 18 patients (for up to four and a half years) revealed that seven of eight untreated or inadequately treated patients had increases in left ventricular wall thickness, whereas all 10 adequately treated patients had decreases (eight of 10) or no change (two of 10) in left ventricular wall thickness. This suggests that adequate antihypereosinophilic therapy (with prednisone and/or hydroxyurea) may stabilize and, in some cases, reverse the cardiac manifestations of the hypereosinophilic syndrome.In previous studies, congestive heart failure due to eosinophilic cardiomyopathy has been reported to be very resistant to therapy. In our patients with congestive heart failure, treatment has been almost invariably effective when digitalis and diuretics were combined with adequate antihypereosinophilic therapy.     

Hypertrophic cardiomyopathy in infants: clinical features and natural history

The clinical and morphologic features of hypertrophic cardiomyopathy in 20 patients recognized as having cardiac disease in the first year of life are described. Fourteen of these 20 infants were initially suspected of having heart disease solely because a heart murmur was identified. However, the infants showed a variety of clinical findings, including signs of marked congestive heart failure (in the presence of non-dilated ventricular cavities and normal or increased left ventricular contractility) and substantial cardiac enlargement on chest radiograph. Other findings were markedly different from those usually present in older children and adults with hypertrophic cardiomyopathy (e.g., right ventricular hypertrophy on the ECG and cyanosis). Consequently, in 14 infants, the initial clinical diagnosis was congenital cardiac malformation other than hypertrophic cardiomyopathy. Twelve of the 14 infants who underwent left-heart catheterization showed substantial obstruction to left ventricular outflow (peak systolic pressure gradient greater than or equal to 35 mm Hg). However, unlike older patients with hypertrophic cardiomyopathy, infants with this condition commonly had marked obstruction to right ventricular outflow (35-106 mm Hg) (nine patients); in six patients, the magnitude of obstruction to right ventricular outflow was at least as great as that to left ventricular outflow. Asymmetric hypertrophy of the ventricular septum relative to the left ventricular free wall was present in the 16 patients who had echocardiographic or necropsy examination. Ventricular septal thickening was substantial in patients studied both before and after 6 months of age (mean 16 mm), indicating that in patients with hypertrophic cardiomyopathy, marked left ventricular hypertrophy may be present early in life and is probably congenital. The clinical course was variable in these patients, but the onset of marked congestive heart failure in the first year of life appeared to be an unfavorable prognostic sign; nine of the 11 infants with congestive heart failure died within the first year of life. In infants with hypertrophic cardiomyopathy, unlike older children and adults with this condition, sudden death was less common (two patients) than death due to progressive congestive heart failure.