The corrected left ventricular ejection fraction: a potential new measure of ventricular function.

Left ventricular ejection fraction (LVEF) has a limited role in predicting outlook in heart diseases including heart failure. We quantified the independent geometric factors that determine LVEF using cardiac MRI and sought to provide an improved measure of ventricular function by adjusting for such independent variables. A mathematical model was used to analyse the independent effects of structural variables and myocardial shortening on LVEF. These results informed analysis of cardiac MRI data from 183 patients (53 idiopathic dilated cardiomyopathy (DCM), 36 amyloidosis, 55 hypertensives and 39 healthy controls). Left ventricular volumes, LVEF, wall thickness, internal dimensions and longitudinal and midwall fractional shortening were measured. The modelling demonstrated LVEF increased in a curvilinear manner with increasing mFS and longitudinal shortening and wall thickness but decreased with increasing internal diameter. Controls in the clinical cohort had a mean LVEF 64  ±  7%, hypertensives 66  ±  8%, amyloid 49 ±  16% and DCM 30  ±  11%. The mean end-diastolic wall thickness in controls was 8  ±  1 mm, DCM 8  ±  1 mm, hypertensives 11  ±  3 mm and amyloid 14  ±  3 mm, P < 0.0001). LVEF correlated with absolute wall thickening relative to ventricular size (R2 = 0.766). A regression equation was derived from raw MRI data (R2 = 0.856) and used to 'correct' LVEF (EFc) by adjusting the wall thickness and ventricular size to the mean of the control group. Improved quantification of the effects of geometric changes and strain significantly enhances understanding the myocardial mechanics. The EFc resulted in reclassification of a 'ventricular function' in some individuals and may provide an improved measure of myocardial performance especially in thick-walled, low-volume ventricles.

Repaired coarctation of the aorta, persistent arterial hypertension and the selfish brain.

BACKGROUND: It has been estimated that 20-30% of repaired aortic coarctation (CoA) patients develop hypertension, with significant cardiovascular morbidity and mortality. Vertebral artery hypoplasia (VAH) with an incomplete posterior circle of Willis (ipCoW; VAH + ipCoW) is associated with increased cerebrovascular resistance before the onset of increased sympathetic nerve activity in borderline hypertensive humans, suggesting brainstem hypoperfusion may evoke hypertension to maintain cerebral blood flow: the "selfish brain" hypothesis. We now assess the "selfish brain" in hypertension post-CoA repair. METHODS: Time-of-flight cardiovascular magnetic resonance angiography from 127 repaired CoA patients (34 ± 14 years, 61% male, systolic blood pressure (SBP) 138 ± 19 mmHg, diastolic blood pressure (DBP) 76 ± 11 mmHg) was compared with 33 normotensive controls (42 ± 14 years, 48% male, SBP 124 ± 10 mmHg, DBP 76 ± 8 mmHg). VAH was defined as < 2 mm and ipCoW as hypoplasia of one or both posterior communicating arteries. RESULTS: VAH + ipCoW was more prevalent in repaired CoA than controls (odds ratio: 5.8 [1.6-20.8], p = 0.007), after controlling for age, sex and body mass index (BMI). VAH + ipCoW was an independent predictor of hypertension (odds ratio: 2.5 [1.2-5.2], p = 0.017), after controlling for age, gender and BMI. Repaired CoA subjects with VAH + ipCoW were more likely to have difficult to treat hypertension (odds ratio: 3.3 [1.01-10.7], p = 0.049). Neither age at time of CoA repair nor any specific repair type were significant predictors of VAH + ipCoW in univariate regression analysis. CONCLUSIONS: VAH + ipCoW predicts arterial hypertension and difficult to treat hypertension in repaired CoA. It is unrelated to age at time of repair or repair type. CoA appears to be a marker of wider congenital cerebrovascular problems. Understanding the "selfish brain" in post-CoA repair may help guide management. JOURNAL SUBJECT CODES: High Blood Pressure; Hypertension; Magnetic Resonance Imaging (MRI); Cardiovascular Surgery; Cerebrovascular Malformations.

Computed tomography in congenital heart disease: how generic principles can be applied to create bespoke protocols in the Fontan circuit.

Cardiac computed tomography (CCT) has become an invaluable cross-sectional imaging modality in congenital heart disease (CHD) patients. However, altered anatomical connections and cardiovascular physiology makes CHD arguably the most challenging area in CCT imaging, which remains a complimentary modality to cardiac magnetic resonance and echocardiography. A bespoke CT protocol is often required to achieve a diagnostic examination; this can be achieved through careful consideration of the basic principles of image acquisition and contrast administration. This article reviews these principles and demonstrates how they can be applied to CCT in CHD using the Fontan circulation as an example.

An unusual flare of anti-synthetase syndrome during concurrent trastuzumab therapy given for recurrent breast cancer.

We present the case of a patient with relapsing anti-synthetase syndrome (ASS) that may have been triggered by monoclonal antibody trastuzumab therapy given for breast cancer. A 52-year-old female with a history of anti-Jo1-associated ASS went into remission with glucocorticoids and mycophenolate mofetil. Her past history included invasive ductal carcinoma of the right breast that was fully treated six years prior to the onset of ASS. She subsequently developed recurrent right-sided breast cancer that was treated with right mastectomy and six cycles of cyclophosphamide-docetaxel chemotherapy. She commenced adjuvant trastuzumab and letrozole therapy, and following the sixth injection of trastuzumab, she was admitted with clinical features consistent with a flare of ASS, which included swinging fever, interstitial lung disease, myositis, and possible subclinical myocarditis, despite recent treatment with cyclophosphamide. She responded to intravenous IV methylprednisolone followed by increased doses of oral glucocorticoids, and she remains stable on immunomodulatory treatment and letrozole monotherapy given for breast cancer. This report provides a concise overview of ASS along with other cases of cardiac and pulmonary diseases attributed to trastuzumab therapy reported in the medical literature.

Mitral Valve Diverticulum Presenting with Severe Mitral Stenosis: Case Report.

The case is presented of a non-infectious anterior mitral valve leaflet diverticulum, which appeared as symptomatic mitral stenosis. Unlike previous reports, there was no histological myxomatous degeneration of the valve. To the authors' knowledge, this is the first time a mitral valve diverticulum resulting in severe mitral stenosis has been reported in the literature.

The effect of reducing spatial resolution by in-plane partial volume averaging on peak velocity measurements in phase contrast magnetic resonance angiography.

BACKGROUND: The aim of this study was to quantify the degree of the effect of in-plane partial volume averaging on recorded peak velocity in phase contrast magnetic resonance angiography (PCMRA). METHODS: Using cardiac optimized 1.5 Tesla MRI scanners (Siemens Symphony and Avanto), 145 flow measurements (14 anatomical locations; ventricular outlets, aortic valve (AorV), aorta (5 sites), pulmonary arteries (3 sites), pulmonary veins, superior and inferior vena cava)- in 37 subjects (consisting of healthy volunteers, congenital and acquired heart disease patients) were analyzed by Siemens Argus default voxel averaging technique (where peak velocity = mean of highest velocity voxel and four neighbouring voxels) and by single voxel technique (1.3×1.3×5 or 1.7×1.7×5.5 mm3) (where peak velocity = highest velocity voxel only). The effect of scan protocol (breath hold versus free breathing) and scanner type (Siemens Symphony versus Siemens Avanto) were also assessed. Statistical significance was defined as P<0.05. RESULTS: There was a significant mean increase in peak velocity of 7.1% when single voxel technique was used compared to voxel averaging (P<0.0001). Significant increases in peak velocity were observed by single voxel technique compared to voxel averaging regardless of subject type, anatomical flow location, scanner type and breathing command. Disabling voxel averaging did not affect the volume of flow recorded. CONCLUSIONS: Reducing spatial resolution by the use of voxel averaging produces a significant underestimation of peak velocity. While this is of itself not surprising this is the first report to quantify the size of the effect. When PCMRA is used to assess peak velocity recording pixel averaging should be disabled.

Extra-cardiac findings in cardiovascular magnetic resonance: what the imaging cardiologist needs to know.

Cardiovascular magnetic resonance (CMR) is an established non-invasive technique to comprehensively assess cardiovascular structure and function in a variety of acquired and inherited cardiac conditions. A significant amount of the neck, thorax and upper abdomen are imaged at the time of routine clinical CMR, particularly in the initial multi-slice axial and coronal images. The discovery of unsuspected disease at the time of imaging has ethical, financial and medico-legal implications. Extra-cardiac findings at the time of CMR are common, can be important and can change clinical management. Certain patient groups undergoing CMR are at particular risk of important extra-cardiac findings as several of the cardiovascular risk factors for atherosclerosis are also risk factors for malignancy. Furthermore, the presence of certain extra-cardiac findings may contribute to the interpretation of the primary cardiac pathology as some cardiac conditions have multi-systemic extra-cardiac involvement. The aim of this review is to give an overview of the type of extra-cardiac findings that may become apparent on CMR, subdivided by anatomical location. We focus on normal variant anatomy that may mimic disease, common incidental extra-cardiac findings and important imaging signs that help distinguish sinister pathology from benign disease. We also aim to provide a framework to the approach and potential further diagnostic work-up of incidental extra-cardiac findings discovered at the time of CMR. However, it is beyond the scope of this review to discuss and determine the clinical significance of extracardiac findings at CMR.

Endovascular closure of thoracic aortic pseudoaneurysms: A combined device occlusion and coil embolization technique in patients unsuitable for surgery or stenting.

OBJECTIVES: Our aim was to retrospectively evaluate non-stent graft closure of ascending aortic pseudoaneurysms at our center over a 10-year period, and describe a combined device occlusion and coil embolization technique. BACKGROUND: Aortic pseudoaneurysms (APAs) are a rare complication post cardiothoracic surgery, but can have fatal complications. There is increasing use of percutaneous interventional techniques for occlusion of aortic pseudoaneurysms in patients who are considered unsuitable for surgery. Stent graft deployment may not be possible depending on the specific anatomy and pathology. METHODS AND RESULTS: Retrospective evaluation of the catheter laboratory database was performed at our center and anonymized data was obtained for patients who had nonstent endovascular treatment of APAs. Twelve patients were identified with a mean age of 63 ± 16 years. Seven patients had the combined occlusion and embolization technique, only 1/7 (14.3%) died from complications related to APAs. Five patients had occlusion device only, 3/5 (60%) died of complications related to their APA. The mean survival for the patients who had a combination procedure was 33.2±.22.6 months (range, 1 - 60 months), compared to 2.7 ± 2.6 months with device closure only (note 2 patients had short follow up of <3 months). CONCLUSIONS: We evaluate non-stent graft percutaneous closure of APAs in a high-risk patient group and provide data on the use of a novel combined occlusion device and coil embolization technique. We feel this is a viable approach to APA closure in this population but this will require larger clinical studies in the future. © 2016 Wiley Periodicals, Inc.

A Novel Cause of Acute Coronary Syndrome Due to Dynamic Extrinsic Coronary Artery Compression by a Rib Exostosis: Multimodality Imaging Diagnosis.

We report a case of acute coronary syndrome secondary to intermittent extrinsic compression of the left anterior descending coronary artery by inward-pointing rib exostosis in an 18-year-old woman during forceful repeated expiration in labour. The diagnosis was achieved using multimodality noninvasive cardiac imaging. In particular, we demonstrated the novel role of expiratory-phase cardiac computed tomography in confirming the anatomical relationship of the bony exostosis to the left anterior descending coronary artery. The case reminds us the heart and mediastinum move dynamically, relative to the bony thorax, throughout the respiratory cycle, and that changes in cardiac physiology in pregnancy may become pathological.

Optimising the imaging plane for right ventricular magnetic resonance volume analysis in adult patients referred for assessment of right ventricular structure and function.

INTRODUCTION: Our aim was to evaluate the reproducibility and accuracy of using short-axis and axial (transaxial) plane for magnetic resonance imaging analysis in adult patients referred for assessment of right ventricular (RV) structure and function. METHODS: Twenty consecutive subjects (10 male, 10 female, mean age 32.2 ± 14.8 years) who were referred for RV assessment and had cardiac magnetic resonance imaging were retrospectively selected. Axial and short-axis manual contouring was performed using cine steady-state free precession sequences by three experienced imaging specialists. The reproducibility of end diastolic volumes, end systolic volumes and ejection fraction was assessed with intraclass correlation coefficients (ICCs) and paired t-tests. Left ventricular stroke volume (LVSV) and RV stroke volumes (RVSV) were compared with concordance correlation coefficients (CCCs) and t-tests to determine accuracy. RESULTS: The concordance between the RVSV and LVSV was good using both methods (axial RVSV CCC = 0.93, short-axis RVSV CCC = 0.86). Paired t-test and analysis of variance showed that the LV/RV stroke volume differences were not significant (p = 0.17). There was slight improvement in interobserver reliability with end systolic volume measurements (axial ICC = 0.92, short-axis ICC = 0.81) but this failed to reach statistical significance (p = 0.37). There was excellent intraobserver variability (ICC > 0.9). CONCLUSION: This study shows that there is no statistically significant difference in reproducibility or accuracy using the short-axis or axial orientations in RV volume analysis in adult patients being referred for RV assessment.

Computed tomographic coronary angiography - is it ready as a screening tool for coronary artery disease?

Currently, there are no formal screening programmes for coronary artery disease (CAD). Computed tomographic coronary angiography (CTCA) has been suggested as a non-invasive and reliable method of atherosclerotic plaque assessment, with the potential for use in screening programmes. In this article, we briefly present the current understanding of atherosclerotic plaque formation, explain key technological aspects of CTCA and critique this method in the light of World Health Organisation (WHO) criteria for devising a screening programme. Current evolving and future insights are also considered. Overall, in our view, there is currently insufficient evidence to support the formal use of CTCA in a screening programme for CAD, although this viewpoint will undoubtedly evolve.

Hypereosinophilic syndrome masquerading as a myocardial infarction causing decompensated heart failure.

BACKGROUND: An 81 year old female patient diagnosed with a chronic low grade hypereosinophilic syndrome presented with angina and dyspnoea. CASE PRESENTATION: She was managed for a non-ST elevated myocardial infarction since her troponin levels were elevated. On day 5, she suffered an acute clinical deterioration with type I respiratory failure and cardiogenic shock, accompanied by deterioration in left ventricular systolic function demonstrated on echocardiography, and this coincided with a marked rise in eosinophil count. Secondary causes of eosinophilia were excluded permitting a diagnosis of Hypereosinophilic Syndrome (HES) to be made. Coronary angiography revealed unobstructed arteries. Supportive treatment for heart failure included diuretic and inotropes but she dramatically improved both clinically and echocardiographically upon commencement of high dose steroids and hydroxycarbamide. Cardiac magnetic resonance imaging (CMR) demonstrated diffuse, shallow endomyocardial enhancement with late gadolinium, consistent with a diagnosis of eosinophilic myocarditis. CONCLUSION: Hypereosinophilic Syndrome can masquerade as a myocardial infarction causing decompensated heart failure. Early recognition and treatment with steroids can improve outcome.

Diagnosing cardiac involvement in the hypereosinophilic syndrome by cardiac magnetic resonance.

Hypereosinophilic syndrome is characterized by unexplained hypereosinophilia involving different organ systems. The investigators present a patient diagnosed with hypereosinophilic syndrome in which cardiac magnetic resonance was pivotal in establishing the presence of cardiac involvement.

Systemic septic embolisation secondary to an atrial myxoma in a young woman.

We present a case of a left atrial myxoma infected with Staphylococcus aureus in a 35-year-old woman who was found to have a retained tampon. Multiple systemic septic emboli were seen on computed tomography imaging of the brain, spleen and kidneys. She was successfully treated by surgical excision of the myxoma and 4 weeks of antibiotic therapy. We postulate that the source of this patient's S. aureus infection was tampon use, leading to a toxic shock syndrome and causing infection of an undiagnosed left atrial myxoma, which led to the embolisation. As far as the authors are aware, this is the first reported case of such an entity. In those with an underlying cardiac predisposition, tampon use may represent a risk of infection with S. aureus, and we intend to heighten clinical awareness of this potentially life-threatening association. We also discuss the diagnosis, complications and treatment of infected atrial myxoma and illustrate the imaging findings.

Popliteal pseudoaneurysm secondary to a tibial osteochondroma: diagnosis with multi-detector row computed tomographic angiography.

A 17-year-old male presented with right knee pain following mild trauma whilst playing badminton. He subsequently developed a popliteal pseudoaneurysm secondary to trauma from a tibial osteochondroma. This is a recognised though very rare occurrence. Its appearance is reported for the first time using multidetector row computed tomographic angiography (MDCTA). The clinical presentation and management of the popliteal pseudoaneurysm are outlined and the imaging findings are illustrated. There is increasing usefulness of MDCTA as an accessible, accurate, noninvasive clinical tool in the emergency diagnostic setting. Its use in the management of this unusual condition is demonstrated with emphasis on 3D, multi-planar reconstruction post-processing techniques.

Thrombolysis for central venous occlusion causing bilateral chylothorax in a patient with down syndrome.

A 20-year-old woman with Down syndrome (trisomy 21) and acute lymphoblastic leukemia presented with severe respiratory compromise secondary to bilateral chylothorax as a result of central venous thrombosis and extensive upper-limb deep venous thrombosis. The chylothorax was successfully managed by catheter-directed thrombolysis and angioplasty of the venous occlusions. The development of venous thrombosis was likely to have been multifactorial. It is recognized that there is an increased incidence of congenital lymphatic anomalies in Down syndrome, which may have been a contributing factor in the development of chylothorax in this patient. This report illustrates the angiographic findings, demonstrates the successful vascular recanalization, and discusses the etiology and management of central venous thrombosis and chylothorax. The case is also presented to contribute to the expanding evidence in support of catheter-directed venous thrombolysis in selected clinical circumstances.