Multimodality imaging showing complete cardiovascular involvement by Erdheim-Chester disease.

Erdheim-Chester disease (ECD) is a multisystem non-Langerhans form of cell histiocytosis. Histiocytic infiltration leads to xanthogranulomatous infiltrates of multiple organ systems. Erdheim-Chester disease was first reported in 1930, only 320 cases reported in the literature. Cardiac involvement in ECD carries worst prognosis beside the central nervous system. We report the first case with pan-cardiac involvement diagnosed with multimodality imaging.

Evaluation of left ventricular rotation in obese subjects by velocity vector imaging.

AIMS: Obesity is a well-known risk factor in the development of cardiovascular disease. We hypothesize that early left ventricular (LV) dysfunction secondary to obesity could be signalled by abnormal LV rotation. METHODS AND RESULTS: This prospective study examined 60 subjects divided into two groups: obese group with body mass index (BMI) >or=30 and control group with BMI <25. The peak rotation, twist, and torsion of the left ventricle were studied in obese and control subjects, using velocity vector imaging. Age and gender were comparable between the two groups. Obese subjects had higher BMI, waist circumference, fasting glucose, triglycerides, systolic and diastolic blood pressure, low-density lipoprotein cholesterol, and lower high-density lipoprotein cholesterol (P < 0.05). In obese subjects, LV mass and LV mass index were increased, and the ratio of mitral early and late diastolic filling velocity was decreased (P < 0.05). In obese subjects, the peak twist and torsion of the left ventricle displayed a lower trend, and the peak rotation of the left ventricle apex decreased significantly (3.81 +/- 2.09 degrees vs. 5.77 +/- 3.27 degrees , P < 0.001). CONCLUSION: Obesity was associated with changes in LV rotation. Velocity vector imaging is a feasible and reproducible echocardiographic technique for the detection of early subclinical LV dysfunction.

Unicuspid aortic valve in adults: a systematic review.

BACKGROUND AND AIM OF THE STUDY: The natural history of the unicuspid aortic valve (UAV) is poorly described in the literature. In order to study the association between UAV with any other cardiac or extra cardiac abnormalities, an evidence-based systematic review was carried out. METHODS: A computerized search was carried out of the medical literature published between 1st January 1966 and 1st September 2008 of the following databases: MEDLINE; EMBASE; Web of Science; and the Cochrane Database. RESULTS: A total of 231 cases of adult UAV was identified in 38 articles. The mean patient age was 42 years, and the most common presenting symptoms reported (in 52 cases) included dyspnea (44%; n=23), angina (21%; n=11), and dizziness or syncope (8%; n=4). The most common lesion in UAV was isolated aortic stenosis (AS) (41%; n=95) and AS with or without aortic regurgitation (28%; n=64). The preoperative diagnosis of UAV is rare, and 139 cases (60%) of UAV were reported at autopsy or by examination of surgically excised valves. Aortic valve replacement was performed in 166 cases (82%). Concomitant aortic surgery was performed in 47 of the UAV cases (23%), either for a dilated or aneurysmal aorta. CONCLUSION: UAV shares many of the features of bicuspid aortic valve, including valvular dysfunction, aortic dilatation, aortic dissection, and dystrophic calcification, although these conditions develop at an earlier age and progress at a faster pace in UAV. Further investigations are warranted regarding the possibility of a familial incidence, associated histopathological changes in the aorta, preoperative diagnostic tools, ideal follow up and surgical intervention.

Impact of pericardial adhesions on diastolic function as assessed by vortex formation time, a parameter of transmitral flow efficiency.

BACKGROUND: Pericardial adhesions are a pathophysiological marker of constrictive pericarditis (CP), which impairs cardiac filling by limiting the total cardiac volume compliance and diastolic filling function. We studied diastolic transmitral flow efficiency as a new parameter of filling function in a pericardial adhesion animal model. We hypothesized that vortex formation time (VFT), an index of optimal efficient diastolic transmitral flow, is altered by patchy pericardial-epicardial adhesions. METHODS: In 8 open-chest pigs, the heart was exposed while preserving the pericardium. We experimentally simulated early pericardial constriction and patchy adhesions by instilling instant glue into the pericardial space and using pericardial-epicardial stitches. We studied left ventricular (LV) function and characterized intraventricular blood flow with conventional and Doppler echocardiography at baseline and following the experimental intervention. RESULTS: Significant decreases in end-diastolic volume, ejection fraction, stroke volume, and late diastolic filling velocity reflected the effects of the pericardial adhesions. The mean VFT value decreased from 3.61 ± 0.47 to 2.26 ± 0.45 (P = 0.0002). Hemodynamic variables indicated the inhibiting effect of pericardial adhesion on both contraction (decrease in systolic blood pressure and +dP/dt decreased) and relaxation (decrease in the magnitude of -dP/dt and prolongation of Tau) function. CONCLUSION: Patchy pericardial adhesions not only negatively impact LV mechanical functioning but the decrease of VFT from normal to suboptimal value suggests impairment of transmitral flow efficiency.

Fatal migratory pulmonary thromboembolism following successful pericardiocentesis.

Echocardiography-guided pericardiocentesis is relatively safe with minimal risk in experienced hands. However, complications can occur because of the procedure. This report describes a unique case of an 84-year-old man with unanticipated fatal pulmonary thromboembolism following a successful pericardiocentesis.

Contrast-enhanced right atrial mass: tumour or thrombus?

We present a case of a 61-year-old female diagnosed with soft-tissue fibrosarcoma of the shoulder with a right atrial mass on transthoracic echocardiogram. To further assess the mass, contrast transoesophageal echo was performed. The mass was highly considered to be a thrombus due to the presence of multiple risk factors: atrial fibrillation, catheter use, and pulmonary embolism. However, a metastatic fibrosarcoma could not be excluded, given its enhancement with echo contrast. Finally, it was proved to be a thrombus with its rapid resolution while on anticoagulation. Therefore, it should be noted that in rare circumstances, thrombus may show enhancement with echo contrast and one needs to integrate all of the patient information to arrive at the correct diagnosis.

Selective echocardiographic analysis of epicardial and endocardial left ventricular rotational mechanics in an animal model of pericardial adhesions.

AIMS: Diagnosis of pericardial adhesions is challenging. Twisting of the left ventricle (LV) is essential for normal LV functioning. We experimentally characterized the impact of pericardial adhesions on epicardial and endocardial LV rotational mechanics with velocity vector imaging (VVI). METHODS AND RESULTS: In nine open-chest pigs, the heart was exposed while preserving the pericardium. Early-stage pericardial adhesions were simulated by instilling tissue glue to pericardial space. Using VVI, LV rotational mechanics was quantitatively assessed endocardially and epicardially along with haemodynamic data at baseline and following the experimental intervention. End-diastolic volume, ejection fraction, stroke volume, late diastolic filling velocity, and LV endocardial torsion decreased significantly. LV epicardial torsion showed only a trend towards decrease (P = 0.141). Endocardial twist and torsion decreased significantly (P = 0.007) from 8.6 +/- 2.2 degree and 1.497 +/- 0.397 degree/cm to 5.3 +/- 1.8 degree and 0.97 +/- 0.38 degree/cm, respectively; epicardial twist showed a trend towards a decrease in its magnitude. Gradients of endocardial/epicardial twist and torsion did not significantly change. CONCLUSION: The model suggests that early-stage pericardial adhesions reduce both epicardial and endocardial LV twist and torsion without a significant alteration in their transmural gradient. Selective endocardial/epicardial analysis of LV twisting mechanics may have a diagnostic role in detection of early formation of pericardial adhesions.

Increase in the late diastolic filling force is associated with impaired transmitral flow efficiency in acute moderate elevation of left ventricular afterload.

OBJECTIVE: Analysis of intraventricular flow force and efficiency is a novel concept of quantitatively assessing left ventricular (LV) hemodynamic performance. We have parametrically characterized diastolic filling flow by early inflow force, late inflow force, and total inflow force and by vortex formation time (VFT), a fundamental parameter of fluid transport efficiency. The purpose of this study was to determine what changes in inflow forces characterize a decrease in diastolic blood transport efficiency in acute moderate elevation of LV afterload. METHODS: In 8 open-chested pigs, the flow force and VFT parameters were calculated from conventional and flow Doppler echocardiographic measurements at baseline and during a brief (3-minute) moderate elevation of afterload induced by increasing the systolic blood pressure to 130% of the baseline value. RESULTS: Systolic LV function decreased significantly during elevated afterload. Early inflow force did not significantly change, whereas late inflow force increased from 5,822.09 +/- 1,656.5 (mean +/- SD) to 13,948.25 +/- 9,773.96 dyne (P = .049), and total inflow force increased from 13,783.35 +/- 4,816.58 to 21,836.67 +/- 8,635.33 dyne (P = .031). Vortex formation time decreased from 4.09 +/- 0.29 to 2.79 +/- 1.1 (P = .0068), confirming suboptimal flow transport efficiency. CONCLUSIONS: Even a brief moderate increase of LV afterload causes a significant increase in late diastolic filling force and impairs transmitral flow efficiency.

Risk of adverse events after coronary artery bypass graft and subsequent noncardiac surgery.

AIMS: Coronary artery bypass grafts (CABGs) are increasingly performed in elderly patients. Risk factors and outcomes are poorly described for those undergoing noncardiac surgery within 1 year after CABG. Our objectives were to assess the risk and predictors of major adverse events associated with noncardiac surgery within 1 year after CABG. METHODS: In a retrospective review of medical records at Mayo Clinic (Rochester, MN, USA), over a period of 5 years, we identified patients who underwent noncardiac procedures within 1 year post-CABG. All events that occurred within 30 days after noncardiac surgery and deaths within 1 year after noncardiac surgery were considered to be related to CABG. RESULTS: We identified 211 patients; of these, 21 patients had 24 adverse events. Within 1 year, 11 died, and within the first 30 days, three myocardial infarctions, six acute congestive heart failure episodes, three cerebrovascular accidents and one deep vein thrombosis episode had occurred. Predictors of an adverse event included emergency operation (odds ratio: 6.8), ejection fraction less than 45% (p < 0.001) and elevated right ventricular systolic pressure by 40 mmHg or more (p = 0.03). After the noncardiac procedure, patients requiring dialysis (p = 0.02), ventilatory support (p = 0.03) and longer hospital stay (p = 0.03) had greater rates of adverse outcomes. CONCLUSION: Post-CABG, preoperative ejection fraction less than 45%, right ventricular systolic pressure of 40 mmHg or more, as well as emergent noncardiac surgery, were predictors of adverse outcomes after the noncardiac procedure. Longer postoperative hospital stay, dialysis, as well as ventilatory support, were predictors of adverse outcomes after CABG.

The left ventricle responds to acute graded elevation of right ventricular afterload by augmentation of twist magnitude and untwist rate.

BACKGROUND: The right and left ventricles share the interventricular septum, which mechanically transmits pressure gradients. The aim of this study was to investigate how acute mild or moderate right ventricular (RV) afterload affects left ventricular (LV) function. METHODS: In 14 open-chest pigs (mean weight, 43 ± 4 kg) with preserved pericardium, acute mild (>35 and ≤50 mm Hg) and moderate (>50 and ≤60 mm Hg) RV pressure loading conditions were induced by constriction of the pulmonary artery. Hemodynamic parameters and LV twist and untwist were evaluated under each condition. RESULTS: From baseline to mild and moderate RV afterload, the mean RV systolic pressure increased from 31.0 ± 4.3 to 41.1 ± 2.7 and 52.7 ± 3.4 mm Hg (P < .001), while LV twist magnitudes increased from 15.4 ± 5.1° to 18.5 ± 3.1° and 19.8 ± 5.0° (P = .004), respectively. Absolute values of LV untwist rate increased from -116.9 ± 64.9°/sec to -160.0 ± 53.3°/sec and -169.1 ± 47.0°/sec, respectively (P = .001). After adjusting for all variables, only the ratio of the early and atrial components of mitral inflow and RV outflow tract acceleration time was significantly associated with the LV twist magnitude and LV untwist rate. CONCLUSIONS: In an acute setting, the left ventricle responds to suddenly elevated RV afterload and decreased RV stroke volume by promptly increasing its twist magnitude and untwist rate.

Epicardial fat and its association with cardiovascular risk: a cross-sectional observational study.

BACKGROUND: The association between visceral obesity and cardiovascular risk has been well described. Some studies show a proportional relationship between the presence of visceral obesity and epicardial fat. Measuring the amount of epicardial adipose tissue (EAT) can be a novel parameter that is inexpensive and easy to obtain and may be helpful in cardiovascular risk stratification. However, the relationship between epicardial fat and cardiac function and that between epicardial fat and cardiac risk factors is less well described. OBJECTIVES: To evaluate the association between echocardiographic epicardial fat and the morphologic and physiologic changes observed at echocardiography and to evaluate the association between epicardial fat and cardiac risk factors. A cross-sectional study of 97 echocardiographic studies (females, n = 42) was conducted. Two groups were identified: epicardial fat ≥ 5 mm (group I) and <5 mm (group II). RESULTS: Epicardial fat >5 mm was associated with LA enlargement, with lower ejection fraction, increased left ventricular mass, and abnormal diastolic function. On a multivariable regression analysis, all these parameters also correlated individually with EAT thickness independent of age. Hyperglycemia (DM), systolic hypertension, and lipid parameters for metabolic syndrome showed a trend for positive association, but this was not statistically significant. The association was not significant even for higher cutoff limits of EAT thickness. CONCLUSION: Epicardial fat >5 mm is associated with cardiac abnormalities on echocardiography. This is a sensitive assessment of body fat distribution, is easily available at echocardiography, and is simple to acquire at no added cost. Further studies looking at the appropriate cut-off thickness of EAT and the sites of measurement to be used are needed. Comparison of this simple and inexpensive measure with other measures of obesity, such as waist-hip ratio, body mass index, Dexa scan of visceral fat, and magnetic resonance imaging of visceral, are needed.

Extensive cardiac metastases secondary to uterine leiomyosarcoma.

Uterine leiomyosarcoma metastatic to multiple cardiac chambers occurs infrequently. Over the past 4 decades, there have been only 11 reported cases of cardiac metastasis secondary to uterine leiomyosarcoma. Metastatic cardiac tumors are commonly diagnosed postmortem, although it may be possible to diagnose them during life. The authors report a rare case of a 48-year-old woman with uterine leiomyosarcoma who was diagnosed with extensive cardiac metastases (in the left atrium, right and left ventricles, pulmonary artery, and pulmonary vein). Although extensive cardiac metastases are suggestive of imminent death, aggressive treatment resulted in survival for >1 year for this patient, which underscores the importance of treatment in select patients.

Quantification of left ventricular twisting mechanics by velocity vector imaging in an animal model of pericardial adhesions.

Diagnosis of constrictive pericarditis remains clinically challenging. Untwisting of the left ventricle (LV) is essential for normal LV diastolic function. Echocardiography is able to measure LV twisting mechanics. We designed an animal model of constrictive pericarditis to determine how pericardial-epicardial adhesions impair LV twisting mechanics. In eight open-chest pigs, the heart was exposed while preserving the pericardium. We simulated early constrictive pericarditis by pericardial constriction and patchy adhesions induced with instant glue and pericardial-epicardial stitches. Using Velocity Vector Imaging (VVI), LV magnitudes of twisting and untwisting were measured along with hemodynamic data at baseline and after the experimental intervention. Significant decreases in end-diastolic volume, ejection fraction, stroke volume, and late diastolic filling velocity reflected the effects of the pericardial adhesions. Magnitude of LV untwisting rate decreased from -80+/-23 degrees /s to -26+/-10 degrees /s (p=0.0009). LV twisting rate dropped from 78+/-20 degrees /s to 40+/-8 degrees /s (p=0.0039) and LV twist magnitude decreased from 9+/-2 degrees to 5+/-2 degrees (p=0.0081). Patchy pericardial adhesions are associated with reductions in LV untwisting rate and twisting magnitude, consistent with a negative impact of constrictive pericarditis on systolic and diastolic function. Impairments in LV twisting mechanics may have a diagnostic role in the detection of early stages of constrictive pericarditis.

Right ventricular free wall circumferential strain reflects graded elevation in acute right ventricular afterload.

This study was designed to evaluate the concurrent changes in the right ventricular free wall (RVFW) movement in experimentally induced, acute mild, moderate, and severe right ventricle (RV) afterload conditions. In 14 open-chest pigs (weight 43 +/- 4 kg) with preserved pericardia, acute mild (>35 and <50 mmHg), moderate (> or =50 and < or =60 mmHg), and severe (>60 mmHg) increases in RV systolic pressure (RVSP) were induced by constriction of the pulmonary artery. At each step, longitudinal, radial, and circumferential strains and strain rates were measured in both the RVFW and the interventricular septum. The mean RVSPs were 31.0 +/- 4.3 mmHg at baseline and 41.1 +/- 2.7 mmHg during mild, 52.7 +/- 3.4 mmHg during moderate, and 61.7 +/- 1.6 mmHg during severe afterload conditions. The RVFW circumferential strains showed significant differences among baseline, mild, moderate, and severe afterload conditions (-10.5 +/- 3.9, -8.3 +/- 3.3, -5.4 +/- 2.7, and -7.5 +/- 5.3%, respectively, P = 0.008) and had significant linear correlation with RVSP (r = 0.636, P < 0.001) if the severe condition was excluded. Decrease of the RVFW circumferential strain magnitude is the most distinct response in acute mild and moderate RV afterload that could aid in detection of clinical conditions associated with acutely increasing RV afterload.

Correlation of automated function imaging (AFI) to conventional strain analyses of regional and global right ventricular function.

BACKGROUND: Automated function imaging is a software tool available to facilitate the efficiency of workflow when analyzing left ventricular strain. In this study, automated function imaging was compared with a conventional approach for the analysis of right ventricular strain in normal and pressure-overloaded right ventricles. METHODS: Twelve pigs were subjected to graded acute right ventricular systolic pressure overload. Intraclass and interclass correlation coefficients (ICCs) with 95% confidence intervals were used for statistical evaluation, with grading based on the kappa statistic as follows: ICC >0.75 = excellent, 0.4 to 0.75 = good, and <0.40 = poor. RESULTS: Intraobserver and interobserver variability for both regional and global strains consistently ranged from good to excellent (ICC, 0.50-0.99), with good agreement between the conventional and automated methods. CONCLUSION: Automated function imaging correlates well with conventional strain analysis of the right ventricle. Automated function imaging is a practical tool for measuring regional and global longitudinal strain in both normal and pressure-overloaded right ventricles.

Impact of acute moderate elevation in left ventricular afterload on diastolic transmitral flow efficiency: analysis by vortex formation time.

BACKGROUND: The formation of a vortex alongside a diastolic jet signifies an efficient blood transport mechanism. Vortex formation time (VFT) is an index of the optimal conditions for vortex formation. It was hypothesized that left ventricular (LV) afterload impairs diastolic transmitral flow efficiency and therefore shifts the VFT out of its optimal range. METHODS: In 9 open-chest pigs, LV afterload was elevated by externally constricting the ascending aorta and increasing systolic blood pressure to 130% of baseline value for 3 minutes. RESULTS: Systolic LV function decreased, diastolic filling velocity increased only during the late (atrial) phase from 0.46 +/- 0.06 to 0.63 +/- 0.19 m/s (P = .0231), and end-diastolic LV volume and heart rate remained unchanged. VFT decreased from 4.09 +/- 0.27 to 2.78 +/- 1.03 (P = .0046). CONCLUSION: An acute, moderate elevation in LV afterload worsens conditions for diastolic vortex formation, suggesting impaired blood transport efficiency.

Echocardiographic quantitation of mitral regurgitation.

Mitral valve regurgitation is a common valvular problem, particularly in developing nations. It causes significant morbidity and mortality, especially if the severity of valve regurgitation is underestimated. Echocardiography plays a significant role in the diagnoses, serial follow-up and management of patients with valvular heart disease. However, precise quantitation of the severity of mitral regurgitation is a crucial element in the therapeutic decisions for managing mitral regurgitation. An accurate assessment of the severity of mitral regurgitation allows for optimal timing of surgical intervention, culminating in improved patient outcomes. This review provides a systematic approach to the quantitation of mitral regurgitation using the echocardiography and Doppler methodologies that are available in the modern noninvasive imaging and hemodynamic laboratory. Additional, novel and evolving noninvasive imaging modalities are reviewed briefly.

The metabolic syndrome and cardiovascular disease: part 2.

In part 1 of this overview, the authors reviewed the epidemiology, definitions, pathophysiology, and interaction of inflammatory markers that are elaborated from the very active paracrine adipocyte. In part 2, they focus on the management of the metabolic syndrome. Management of this syndrome includes identification of individuals in the early stages of the syndrome and emphasizes the risk of developing diabetes mellitus and the progression to cardiovascular disease. This can be accomplished by simple physical examination and an assessment of routine laboratory findings, which should include measurement of fasting glucose, a lipid profile, and body morphometrics. A 10-year risk assessment is needed in patients who have a diagnosis of the metabolic syndrome. The most important therapeutic intervention in the metabolic syndrome is lifestyle change, with a focus on weight reduction and regular leisure time physical activity.