Right ventricular ejection fraction derived from intraoperative three-dimensional transesophageal echocardiography versus cardiac magnetic resonance imaging.

PURPOSE: Right ventricle (RV) assessment is critical during cardiac surgery. Traditional assessment consists of visual estimation and measurement of validated parameters. Cardiac magnetic resonance imaging (cMRI) is the gold standard for RV analysis, and transthoracic three-dimensional (3D) echocardiography is validated against this. We aimed to show that intraoperative 3D transesophageal echocardiography (TEE) RV assessment is feasible and can produce results that correlate with cMRI. METHODS: We recruited cardiac surgery patients who underwent cMRI within the preceding twelve preoperative months. An anesthetic protocol was followed pre-sternotomy and a 3D RV data set was acquired. We used TOMTEC 4D RV-Function to derive RV end-diastolic volume (EDV), end-systolic volume (ESV), and ejection fraction (EF). We compared these data with the corresponding MRI values. RESULTS: Twenty-five patients were included. Transesophageal echocardiography EDV and ESV differed from MRI measurements with a mean bias of -53 mL (95% confidence interval [CI], -80 to 26) and -21 mL (95% CI, -34 to -9). Transesophageal echocardiography EF did not differ significantly, with a mean bias of -4% (95% CI, -8 to 1). Results were unchanged after excluding MRIs older than 180 days. Correlation coefficients for EDV, ESV, and EF were r = 0.85, 0.91, and 0.80, respectively. Interclass correlation coefficients for EDV, ESV, and EF were 0.86, 0.89, and 0.96, respectively. CONCLUSIONS: Intraoperative TEE RV, EDV, and ESV are underestimated relative to cMRI because of analysis, anesthetic, and ventilation factors. The EF showed a low mean difference, and all values showed strong correlation with MRI. Reproducibility and feasibility were excellent and increased use in clinical practice should be considered.

RéSUMé: OBJECTIF: L'évaluation du ventricule droit (VD) est essentielle pendant la chirurgie cardiaque. L'évaluation traditionnelle consiste en une estimation visuelle et une mesure de paramètres validés. L'imagerie par résonance magnétique cardiaque (IRMc) est l'étalon-or pour l'analyse du VD, et l'échocardiographie transthoracique tridimensionnelle (3D) est validée par rapport cette modalité. Notre objectif était de démontrer que l'évaluation peropératoire du VD par l'échocardiographie transœsophagienne (ETO) était faisable et pouvait générer des résultats en corrélation avec l'IRMc. MéTHODE: Nous avons recruté des patient·es de chirurgie cardiaque ayant bénéficié d'une IRMc au cours des douze mois préopératoires précédents. Un protocole anesthésique a été suivi avant la sternotomie et un ensemble de données 3D sur le VD a été acquis. Nous avons utilisé le système TOMTEC 4D RV-Function pour calculer le volume télédiastolique (VTD), le volume télésystolique (VTS) et la fraction d'éjection (FE). Nous avons comparé ces données avec les valeurs correspondantes obtenues à l'IRM. RéSULTATS: Vingt-cinq personnes ont été incluses. Les valeurs de VTD et VTS obtenues à l'échocardiographie transœsophagienne différaient des mesures obtenues par IRM avec un biais moyen de ­53 mL (intervalle de confiance [IC] à 95 %, ­80 à 26) et ­21 mL (IC 95 %, ­34 à ­9). La FE obtenue par échocardiographie transœsophagienne ne différait pas significativement, avec un biais moyen de ­4 % (IC 95 %, ­8 à 1). Les résultats étaient inchangés après l'exclusion des IRM réalisés plus de 180 jours auparavant. Les coefficients de corrélation pour le VTD, le VTS et la FE étaient r = 0,85, 0,91 et 0,80, respectivement. Les coefficients de corrélation interclasse pour le VTD, le VTS et la FE étaient de 0,86, 0,89 et 0,96, respectivement. CONCLUSION: L'ETO peropératoire sous-estime les mesures du VD, du VTD et du VTS par rapport à l'IRMc en raison de facteurs d'analyse, d'anesthésie et de ventilation. La FE a montré une faible différence moyenne, et toutes les valeurs ont montré une forte corrélation avec l'IRM. La reproductibilité et la faisabilité étaient excellentes et une utilisation accrue dans la pratique clinique devrait être envisagée.

Does cardiac imaging surveillance strategy influence outcomes in patients with early breast cancer?

Background: Many patients with breast cancer receive therapies with the potential to cause cardiotoxicity. Echocardiography and multiple-gated acquisition (MUGA) scans are the most used modalities to assess cardiac function during treatment in high-risk patients; however, the optimal imaging strategy and the impact on outcome are unknown. Methods: Consecutive patients with stage 0-3 breast cancer undergoing pre-treatment echocardiography or MUGA were identified from a tertiary care cancer center from 2010-2019. Demographics, medical history, imaging data and clinical events were collected from hospital charts and administrative databases. The primary outcome is a composite of all-cause death or heart failure event. Clinical and imaging predictors of outcome were evaluated on univariable and multivariable analyses. Results: 1028 patients underwent pre-treatment MUGA and 1032 underwent echocardiography. The groups were well matched for most clinical characteristics except patients undergoing MUGA were younger, had more stage 3 breast cancer and more HER2 over-expressing and triple negative cases. Routine follow-up cardiac imaging scan was obtained in 39.3% of patients with MUGA and 38.0% with echocardiography. During a median follow-up of 2448 (1489, 3160) days, there were 194 deaths, including 7 cardiovascular deaths, and 28 heart failure events with no difference in events between the MUGA and echocardiography groups. There were no imaging predictors of the primary composite outcome or cardiac events. Patients without follow-up imaging had similar adjusted risk for the composite outcome compared to those with imaging follow-up, hazard ratio 0.8 (95% confidence interval 0.5,1.3), p=0.457. Conclusion: The selection of pretreatment echocardiography or MUGA did not influence the risk of death or heart failure in patients with early breast cancer. Many patients did not have any follow-up cardiac imaging and did not suffer worse outcomes. Cardiovascular deaths and heart failure event rates were low and the value of long-term cardiac imaging surveillance should be further evaluated.

A Contemporary Review of the Effects of Exercise Training on Cardiac Structure and Function and Cardiovascular Risk Profile: Insights From Imaging.

Exercise is a commonly prescribed therapy for patients with established cardiovascular disease or those at high risk for de novo disease. Exercise-based, multidisciplinary programs have been associated with improved clinical outcomes post myocardial infarction and is now recommended for patients with cancer at elevated risk for cardiovascular complications. Imaging studies have documented numerous beneficial effects of exercise on cardiac structure and function, vascular function and more recently on the cardiovascular risk profile. In this contemporary review, we will discuss the effects of exercise training on imaging-derived cardiovascular outcomes. For cardiac imaging via echocardiography or magnetic resonance, we will review the effects of exercise on left ventricular function and remodeling in patients with established or at risk for cardiac disease (myocardial infarction, heart failure, cancer survivors), and the potential utility of exercise stress to assess cardiac reserve. Exercise training also has salient effects on vascular function and health including the attenuation of age-associated arterial stiffness and thickening as assessed by Doppler ultrasound. Finally, we will review recent data on the relationship between exercise training and regional adipose tissue deposition, an emerging marker of cardiovascular risk. Imaging provides comprehensive and accurate quantification of cardiac, vascular and cardiometabolic health, and may allow refinement of risk stratification in select patient populations. Future studies are needed to evaluate the clinical utility of novel imaging metrics following exercise training.

Right Ventricular Remodeling in Hypoplastic Left Heart Syndrome is Minimally Impacted by Cardiopulmonary Bypass: A Comparison of Norwood vs. Hybrid.

Right ventricular (RV) remodeling in hypoplastic left heart syndrome (HLHS) begins prenatally and continues through staged palliations. However, it is unclear if the most marked observed remodeling post-Norwood is secondary to cardiopulmonary bypass (CPB) exposure or if it is an adaptation intrinsic to the systemic RV. This study aims to determine the impact of CPB on RV remodeling in HLHS. Echocardiograms of HLHS survivors undergoing stage 1 Norwood (n = 26) or Hybrid (n = 20) were analyzed at pre- and post-stage 1, pre- and post-bidirectional cavo-pulmonary anastomosis (BCPA), and pre-Fontan. RV fractional area change (FAC), vector velocity imaging for longitudinal & derived circumferential deformation (global radial shortening (GRS) = peak radial displacement/end-diastolic diameter), and deformation ratio (longitudinal/ circumferential) were assessed. Both groups had similar age, clinical status and functional parameters pre-stage 1. No difference in RV size and sphericity at any stage between groups. RVFAC was normal (> 35%) throughout for both groups. Both Norwood and Hybrid patients had increased GRS (p = 0.0001) post-stage 1 and corresponding unchanged longitudinal strain, resulting in decreased deformation ratio (greater relative RV circumferential contraction), p = 0.0001. Deformation ratio remained decreased in both groups in subsequent stages. Irrespective of timing of the first CPB exposure, both Norwood and Hybrid patients underwent similar RV remodeling, with relative increase in circumferential to longitudinal contraction soon after stage 1 palliation. The observed RV remodeling in HLHS survivors were minimally impacted by CPB.

Rapid atrophy of cardiac left ventricular mass in patients with non-small cell carcinoma of the lung.

BACKGROUND: Cancer is a systemic catabolic condition affecting skeletal muscle and fat. We aimed to determine whether cardiac atrophy occurs in this condition and assess its association with cardiac function, symptoms, and clinical outcomes. METHODS: Treatment naïve metastatic non-small cell lung cancer patients (n = 50) were assessed prior to and 4 months after commencement of carboplatin-based palliative chemotherapy. Methods included echocardiography for left ventricular mass (LVM) and LV function [LV ejection fraction, global longitudinal strain (GLS), diastolic function], computed tomography to quantify skeletal muscle and total adipose tissue, Eastern Cooperative Oncology Group Performance Status (ECOG-PS), validated questionnaires for dyspnoea and fatigue, plasma biomarkers, tumour response to therapy, and overall survival. RESULTS: During 112 ± 6 days, the median change in LVM was -8.9% [95% confidence interval (95% CI) -10.8 to -4.8, P < 0.001]. Quartiles of LVM loss were -20.1%, -12.9%, -4.8%, and +5.5%. Losses of muscle, adipose tissue, and LVM were frequently concurrent; LVM loss > median value was associated with loss of skeletal muscle [odds ratio (OR) = 4.5, 95% CI: 1.4-14.8, P=0.01] and loss of total adipose tissue (OR = 10.0, 95% CI: 2.7-36.7, P < 0.001). LVM loss was associated with decreased GLS (OR = 6.6, 95% CI: 1.9-22.7, P=0.003) but not with LV ejection fraction or diastolic function. In the population overall, plasma levels of C-reactive protein (P=0.008), high sensitivity troponin T (hs-TnT) (P=0.03), and galectin-3 (P=0.02) increased over time, while N-terminal pro B-type natriuretic peptide and hs-cTnI did not change over time. C-reactive protein was the only biomarker associated with LVM loss but at the univariate level only. Independent predictors of LVM loss were prior weight loss (adjusted OR = 10.2, 95% CI: 2.2-46.9, P=0.003) and tumour progression (adjusted OR = 14.6, 95% CI: 1.4-153.9, P=0.02). LVM loss was associated with exacerbations of fatigue (OR = 6.6, 95% CI: 1.9-22.7, P=0.003), dyspnoea (OR = 9.3, 95% CI: 2.4-35.8, P<0.001), and deterioration of performance status (OR = 4.8, 95% CI: 1.3-18.3,P=0.02). Patients with concurrent loss of LVM, skeletal muscle, and fat were more likely to deteriorate in all three symptom domains and to have reduced survival (P=0.05). CONCLUSIONS: Intense LVM atrophy is associated with non-small cell lung cancer-induced cachexia. Loss of LVM was associated with emerging alterations of GLS, indicating subtle changes in left ventricular function. Longer term studies are needed to assess the full scope of cardiac atrophy and its impact. LVM atrophy arises in conjunction with losses of fat and skeletal muscle and is temporally associated with meaningful declines in performance status, worsening of fatigue, and dyspnoea, as well as poorer tumour response and decreased survival. The specific contribution of LVM atrophy to these outcomes requires further study.

Undiagnosed cardiac deficits in non-small cell carcinoma patients in the candidate population for anti-cachexia clinical trials.

PURPOSE: Currently, there is no approved therapy for cancer cachexia. According to European and American regulatory agencies, physical function improvements would be approvable co-primary endpoints of new anti-cachexia medications. As physical functioning is in part dependent on cardiac functioning, we aimed to explore the cardiac status of a group of patients meeting current criteria for inclusion in cachexia clinical trials. METHODS: Seventy treatment-naive patients with metastatic NSCLC [36 (51.4%) male; 96% ECOG 0-1; eligible for carboplatin-based therapy and meeting eligibility criteria for cachexia clinical trials] were recruited before the start of first-line carboplatin-based chemotherapy. Patients were evaluated by echocardiography, electrocardiography, and scales for fatigue and dyspnea. Computed tomography cross-sectional images were utilized for body composition analysis. RESULTS: In 9/70 patients (12.8%), echocardiography allowed discovery of clinically relevant cardiac disorders [seven patients with left ventricular ejection fraction (LVEF) 32%-47%; one patient with severe right ventricular dilation and severe pulmonary hypertension and one patient with severe pericardial effusion warranted hospitalization and drainage]. Another 10/70 (14.3%) patients had diastolic dysfunction with preserved LVEF. The cardiac conditions were associated with aggravated fatigue (p < 0.05), dyspnea (p < 0.05), and anemia (p = 0.06). Five out of seven patients with LVEF < 50% were sarcopenic and one was borderline sarcopenic. CONCLUSION: Baseline cardiac status of the metastatic NSCLC patients adds potential heterogeneity for anti-cachexia clinical trials. Detailed cardiac screening data might be useful for inclusion/exclusion criteria, randomization, and post hoc analysis.

Ultrasound assessment of carotid arteries: Current concepts, methodologies, diagnostic criteria, and technological advancements.

Following cardiac disease and cancer, stroke continues to be the third leading cause of death and disability due to chronic disease in the developed world. Appropriate screening tools are integral to early detection and prevention of major cardiovascular events. In a carotid artery, the presence of increased intima-media thickness, plaque, or stenosis is associated with increased risk of a transient ischemic attack or a stroke. Carotid artery ultrasound remains a long-standing and reliable tool in the current armamentarium of diagnostic modalities used to assess vascular morbidity at an early stage. The procedure has, over the last two decades, undergone considerable upgrades in technology, approach, and utility. This review examines in detail the current state and usage of this integrally important means of extracranial cerebrovascular assessment.

What is the minimum change in left ventricular ejection fraction, which can be measured with contrast echocardiography?

BACKGROUND: There are limited data on what is the minimum change that can be detected in cancer patients undergoing treatment with cardiotoxic drugs and are referred for monitoring left ventricular (LV) function. OBJECTIVE: To assess the variability in the measurement of LV volumes and ejection fraction (EF) in contrast echocardiography and to determine the minimum detectable difference (MDD) between two EF measurements that can be deemed significant. METHODS: A total of 150 patients were divided into three groups according to EF (EF <53, 53-60, and >60%). Each group consisted of 50 randomly selected cancer patients who underwent contrast echocardiography between July 2010 and May 2014. Repeated measurements of LV volumes and EF were performed offline by a sonographer and a cardiologist. Inter-observer variability was assessed by analysis of variance. Measurement error was estimated by standard error of measurement and MDD. RESULTS: The 95% confidence interval with a single measurement of EF was 2 percentage points in the groups of patients with EF <53% and EF >60%, and 2.5 percentage points for patients with EF 53-60%. The MDD for EF, end-diastolic volume and end-systolic volume that could be recognized with 95% confidence interval were 4 percentage points, 7 mL and 4 mL, respectively. CONCLUSION: Contrast echocardiography is a reliable tool for serial measurements of EF to monitor cardiotoxicity due to chemotherapy. In a high-volume echocardiography laboratory with experienced staff, the MDD for EF of 4 percentage points on a good-quality recording demonstrates the high reproducibility of the Simpson's method using contrast echocardiography.

Variability of left ventricular volume and ejection fraction measurements using contrast echocardiography: The influence of the left ventricular length measurements in a large cohort of patients during monitoring cardiotoxic effects of chemotherapy.

OBJECTIVE: To investigate the influence of length difference in left ventricular (LV) long axis between the apical four-chamber and two-chamber views on measurements of LV volumes and ejection fraction (EF). METHODS: Seven hundred consecutive cancer patients underwent contrast echocardiography from July 2010 to May 2014. All patients received the echocardiographic contrast agent Definity. Recordings of apical views were analyzed by a sonographer and then by a cardiologist. The end-diastolic and end-systolic LV volumes (EDV and ESV), and LV lengths as well as EF, were measured using the biplane Simpson's method. Inter-observer variability was assessed using relative mean error (RME) and Bland-Altman analysis. RESULTS: Six hundred ninety-two patients had contrast echocardiograms with complete endocardial definition. The LV length difference of the long axis measured by the cardiologist was ≤1 mm in 284 studies (41%), 2 mm in 146 studies (21%), 3 mm in 103 studies (15%), and ≥4 mm in 159 studies (23%). The limits of agreement (LOA) and RME increase with the increasing length difference. Compared to the groups with length difference <4 mm, the RME of the measurements of indexed EDV, indexed ESV, and EF was significantly greater in the group with length difference ≥4 mm (P < .05). CONCLUSION: These results highlight the need for reviewing the LV long axis length measurements in order to provide reproducible LV volumes and EF measurements and may be used as benchmarks for quality control. A length difference of ≤3 mm can be achieved in most of our patients and is associated with an excellent inter-observer agreement.

High fat mass associates with occurrence of targeted therapy-induced left ventricular ejection fraction reduction in patients with renal cell carcinoma.

BACKGROUND & AIMS: Recent research suggests that variations of skeletal muscle (SM) and fat predict the severity of chemotherapy-induced toxicities in patients with renal cell carcinoma (RCC). Cardio-toxicity has not been evaluated in this context. METHODS: In this study we considered 47 RCC patients who participated in randomized clinical trials of sorafenib or sunitinib (i.e., targeted therapy). To capture cardio-toxicity, multi gated acquisition (MUGA) scan-defined left ventricular ejection fraction (LVEF) tests (at least 3 tests over 1 year of treatment) were abstracted. Computed tomography (CT) cross-sectional images were analyzed before start of targeted therapy and at 1 year to define SM and fat at baseline and changes over time concurrent with MUGA-defined LVEF measurement. RESULTS: MUGA-defined cardio-toxicity (usually fall in LVEF >10% to an absolute LVEF<55%) occurred in 8/47 (17%) patients over 1 year of targeted therapy (all were male). Percentage of patients with high fat mass (baseline CT-defined total adipose tissue/indexed by height2 greater than the gender-specific median value) was higher among patients with cardio-toxicity versus patients without cardio-toxicity [7 (87.5%) versus 16 (41.0%); p = 0.02]. The percentage of SM loss in patients with cardio-toxicity was higher than the patients without cardio-toxicity [median of loss (%) -7 versus 0 respectively; p = 0.04]. CONCLUSION: Cardio-toxicity in RCC patients might be associated with high fat mass. This finding is distinct from prior observations that low body weight and sarcopenia associated with non-cardiac toxicities of targeted therapies. Concurrence of SM loss over time and development of cardio-toxicity is reported for the first time.

The Role of Cardio-Oncology in the Interprofessional Care of Adult Patients Receiving Cancer Therapy.

OBJECTIVE: To discuss the toxic effects of therapy to the structure and function of the cardiovascular system and the role of the cardio-oncology team in the interprofessional care of adult patients, including current approaches, research findings, and future endeavors DATA SOURCE: Published articles and international cardiology and oncology association guidance documents. CONCLUSION: Although a new field of study, cardio-oncology is a rapidly expanding area of great clinical need. Evidence is only now accumulating, with most guidelines based on opinion or extrapolated from cardiovascular literature. Oncology care providers face complex decisions on a daily basis, whether before, during, or following definitive cancer treatments. IMPLICATIONS FOR NURSING PRACTICE: In the era of both traditional and targeted cancer therapies, the long-term side effects to the cardiovascular system and, consequently, the needs of cancer survivors are increasingly complex. Accordingly, oncology nurses must not only be aware of such potential effects, but should conduct careful serial symptom review and consider risk-reduction and cancer rehabilitation strategies across the disease trajectory.

Circulating levels of tumor necrosis factor-alpha receptor 2 are increased in heart failure with preserved ejection fraction relative to heart failure with reduced ejection fraction: evidence for a divergence in pathophysiology.

BACKGROUND: Various pathways have been implicated in the pathogenesis of heart failure (HF) with preserved ejection fraction (HFPEF). Inflammation in response to comorbid conditions, such as hypertension and diabetes, may play a proportionally larger role in HFPEF as compared to HF with reduced ejection fraction (HFREF). METHODS AND RESULTS: This study investigated inflammation mediated by the tumor necrosis factor-alpha (TNFα) axis in community-based cohorts of HFPEF patients (n = 100), HFREF patients (n = 100) and healthy controls (n = 50). Enzyme-linked immunosorbent assays were used to investigate levels of TNFα, its two receptors (TNFR1 and TNFR2), and a non-TNFα cytokine, interleukin-6 (IL-6), in plasma derived from peripheral blood samples. Plasma levels of TNFα and TNFR1 were significantly elevated in HFPEF relative to controls, while levels of TNFR2 were significantly higher in HFPEF than both controls and HFREF. TNFα, TNFR1 and TNFR2 were each significantly associated with at least two of the following: age, estimated glomerular filtration rate, hypertension, diabetes, smoking, peripheral vascular disease or history of atrial fibrillation. TNFR2 levels were also significantly associated with increasing grade of diastolic dysfunction and severity of symptoms in HFPEF. CONCLUSIONS: Inflammation mediated through TNFα and its receptors, TNFR1 and TNFR2, may represent an important component of a comorbidity-induced inflammatory response that partially drives the pathophysiology of HFPEF.

Concurrent evolution of cancer cachexia and heart failure: bilateral effects exist.

Cancer cachexia is defined as a multifactorial syndrome of involuntary weight loss characterized by an ongoing loss of skeletal muscle mass and progressive functional impairment. It is postulated that cardiac dysfunction/atrophy parallels skeletal muscle atrophy in cancer cachexia. Cardiotoxic chemotherapy may additionally result in cardiac dysfunction and heart failure in some cancer patients. Heart failure thus may be a consequence of either ongoing cachexia or chemotherapy-induced cardiotoxicity; at the same time, heart failure can result in cachexia, especially muscle wasting. Therefore, the subsequent heart failure and cardiac cachexia can exacerbate the existing cancer-induced cachexia. We discuss these bilateral effects between cancer cachexia and heart failure in cancer patients. Since cachectic patients are more susceptible to chemotherapy-induced toxicity overall, this may also include increased cardiotoxicity of antineoplastic agents. Patients with cachexia could thus be doubly unfortunate, with cachexia-related cardiac dysfunction/heart failure and increased susceptibility to cardiotoxicity during treatment. Cardiovascular risk factors as well as pre-existing heart failure seem to exacerbate cardiac susceptibility against cachexia and increase the rate of cardiac cachexia. Hence, chemotherapy-induced cardiotoxicity, cardiovascular risk factors, and pre-existing heart failure may accelerate the vicious cycle of cachexia-heart failure. The impact of cancer cachexia on cardiac dysfunction/heart failure in cancer patients has not been thoroughly studied. A combination of serial echocardiography for detection of cachexia-induced cardiac remodeling and computed tomography image analysis for detection of skeletal muscle wasting would appear a practical and non-invasive approach to develop an understanding of cardiac structural/functional alterations that are directly related to cachexia.

Smith predictor-based robot control for ultrasound-guided teleoperated beating-heart surgery.

Performing surgery on fast-moving heart structures while the heart is freely beating is next to impossible. Nevertheless, the ability to do this would greatly benefit patients. By controlling a teleoperated robot to continuously follow the heart's motion, the heart can be made to appear stationary. The surgeon will then be able to operate on a seemingly stationary heart when in reality it is freely beating. The heart's motion is measured from ultrasound images and thus involves a non-negligible delay due to image acquisition and processing, estimated to be 150 ms that, if not compensated for, can cause the teleoperated robot's end-effector (i.e., the surgical tool) to collide with and puncture the heart. This research proposes the use of a Smith predictor to compensate for this time delay in calculating the reference position for the teleoperated robot. The results suggest that heart motion tracking is improved as the introduction of the Smith predictor significantly decreases the mean absolute error, which is the error in making the distance between the robot's end-effector and the heart follow the surgeon's motion, and the mean integrated square error.

Systolic and diastolic function assessment in fabry disease patients using speckle-tracking imaging and comparison with conventional echocardiographic measurements.

BACKGROUND: Fabry cardiomyopathy is characterized by progressive left ventricular hypertrophy (LVH) associated with diastolic dysfunction and is the most common cause of death in Fabry disease (FD). However, LVH is not present in all subjects, particularly early in disease progression and in female patients. Direct assessment of myocardial deformation by strain and strain rate (SR) analysis may be sensitive to detect subclinical Fabry cardiomyopathy independent of the presence of LVH. METHODS: Systolic (longitudinal, circumferential, and radial systolic strain and SR) and diastolic (SR during isovolumic relaxation [SR(IVR)] and early diastole and strain at peak transmitral E wave) function was assessed in 16 patients with FD using two-dimensional speckle-tracking echocardiography. In addition, mean S' and E' mitral annular velocities by Doppler tissue imaging were measured. Diastolic filling indices, including E/SR(IVR) and E/E' ratios, were calculated. The patients were compared with 24 healthy age-matched and gender-matched controls. RESULTS: All 16 patients with FD had normal left ventricular ejection fractions, and nine patients had LVH. Compared with controls, patients with FD had reduced longitudinal systolic strain (P < .001) and systolic SR (P = .007), while there were no differences in circumferential systolic strain and S'. Diastolic function assessment showed reduced longitudinal early diastolic SR (P = .001), SR(IVR) (P < .001), and E/SR(IVR) (P < .001), while radial and circumferential diastolic function was not affected. Of the conventional diastolic function indices, reductions were seen in E (P = .006), E' (P = .021), and E/E' ratio (P < .001). After correcting for LVH, only SR(IVR) (P < .001) and E/SR(IVR) (P = .025) remained significantly different between patients with FD and controls, with sensitivity of 94% and specificity of 92% for SR(IVR) of 0.235 sec(-1) (area under the receiver operating characteristic curve, 0.953). CONCLUSIONS: Strain and SR analysis is useful in identifying patients with FD with reduced myocardial function, with longitudinal systolic strain and diastolic isovolumic SR being superior to the other echocardiographic measurements of myocardial contraction and relaxation and independent of LVH.

Adenosine stress myocardial contrast echocardiography for the detection of coronary artery disease: a comparison with coronary angiography and cardiac magnetic resonance.

OBJECTIVES: To evaluate the accuracy of adenosine myocardial contrast echocardiography (MCE) in diagnosing coronary artery disease (CAD). BACKGROUND: Adenosine stress echocardiography is not routinely used in the assessment of CAD. Since ultrasound microbubble contrast agents enable improved wall motion analysis and simultaneous assessment of myocardial perfusion, we sought to evaluate the diagnostic performance of combined wall motion/perfusion imaging with adenosine MCE in patients with suspected CAD. We evaluated the accuracy of adenosine MCE in identifying 1) the presence of anatomic disease, as defined by X-ray angiography, and 2) the functional significance of CAD, as determined by high field-strength (3-T), multiparametric cardiac magnetic resonance (CMR) imaging. METHODS: Sixty-five patients with suspected CAD were studied before angiography with MCE and CMR, at stress (140 µg/kg/min intravenous adenosine) and at rest. For MCE, 2-, 3- and 4-chamber long-axis images were acquired during intravenous sulfur hexafluoride infusion. For CMR, short-axis first-pass perfusion and delayed enhancement images were acquired following intravenous gadolinium-diethylenetriaminepentaacetic acid bolus injections (0.05 mmol/kg). Quantitative coronary angiography served as a reference standard for anatomic disease (significant CAD defined as ≥ 50% reference diameter in vessels with diameter ≥ 2 mm). RESULTS: Compared with X-ray angiography, MCE provided diagnostic accuracy of 82%, sensitivity of 85%, and specificity of 76% for detecting significant coronary stenosis. Disease location was also identified with reasonable accuracy (diagnostic accuracy 81% for left anterior descending disease, 77% for left circumflex artery disease, and 84% for right coronary artery disease). With CMR as the reference standard for functional assessment, MCE provided diagnostic accuracy of 79%, sensitivity of 85%, and specificity of 74%. Interobserver agreement for MCE was 79% (95% confidence interval: 67% to 88%). CONCLUSIONS: Adenosine MCE achieved favorable diagnostic performance in identifying the presence and functional significance of coronary stenosis. Adenosine MCE may be useful in the clinical setting for evaluating patients with suspected CAD.

Valvular heart disease and the use of cabergoline for the treatment of prolactinoma.

OBJECTIVE: The use of high doses of the ergot-derived dopamine agonist cabergoline (> 3 mg/day), especially with cumulative doses > 4000 mg, has been associated with an increase in cardiac valvular thickening and significant (moderate to severe) regurgitation. Whether lower doses commonly used in the treatment of prolactinomas (0.25-3 mg/week) are also associated with significant valvulopathy is controversial. The mitral valve tenting area, a subclinical index of leaflet stiffening, has also been correlated with the cumulative dose of cabergoline and severity of valvular regurgitation. DESIGN/PATIENTS/MEASUREMENTS: We performed transthoracic echocardiography (TTE) on 50 prolactinoma patients (48% macroprolactinomas, 52% microprolactinomas, 30 male, 20 female, age 51.2 +/- 2.2 years, mean +/- SEM) who had been taking cabergoline for 6.6 +/- 0.5 years (range 1-13 years) with cumulative doses of 443 +/- 53 mg, to determine the prevalence of significant valvular thickening (> 0.5 cm) and regurgitation, and measured the mitral valve tenting area and height. The results were compared to those from age- and sex-matched controls with normal left ventricular function. RESULTS: No significant valvular thickening or regurgitation of any valve was detected in the prolactinoma group and the prevalence of mild valvular regurgitation was not higher than in the case-control group. The mitral valve tenting area and height were not significantly greater than in the control group. There was no correlation between tenting area or height and cumulative cabergoline dose. CONCLUSIONS: We found no evidence of increased mitral valve tenting area/height, valvular thickening or significant regurgitation with the long-term administration of the commonly used doses of cabergoline to treat prolactinoma.