Reliability of pediatric ventricular function analysis by short-axis "single-cycle-stack-advance" single-shot compressed-sensing cines in minimal breath-hold time.

OBJECTIVES: Cardiovascular magnetic resonance (CMR) cine imaging by compressed sensing (CS) is promising for patients unable to tolerate long breath-holding. However, the need for a steady-state free-precession (SSFP) preparation cardiac cycle for each slice extends the breath-hold duration (e.g. for 10 slices, 20 cardiac cycles) to an impractical length. We investigated a method reducing breath-hold duration by half and assessed its reliability for biventricular volume analysis in a pediatric population. METHODS: Fifty-five consecutive pediatric patients (median age 12 years, range 7-17) referred for assessment of congenital heart disease or cardiomyopathy were included. Conventional multiple breath-hold SSFP short-axis (SAX) stack cines served as the reference. Real-time CS SSFP cines were applied without the steady-state preparation cycle preceding each SAX cine slice, accepting the limitation of omitting late diastole. The total acquisition time was 1 RR interval/slice. Volumetric analysis was performed for conventional and "single-cycle-stack-advance" (SCSA) cine stacks. RESULTS: Bland-Altman analyses [bias (limits of agreement)] showed good agreement in left ventricular (LV) end-diastolic volume (EDV) [3.6 mL (- 5.8, 12.9)], LV end-systolic volume (ESV) [1.3 mL (- 6.0, 8.6)], LV ejection fraction (EF) [0.1% (- 4.9, 5.1)], right ventricular (RV) EDV [3.5 mL (- 3.34, 10.0)], RV ESV [- 0.23 mL (- 7.4, 6.9)], and RV EF [1.70%, (- 3.7, 7.1)] with a trend toward underestimating LV and RV EDVs with the SCSA method. Image quality was comparable for both methods (p = 0.37). CONCLUSIONS: LV and RV volumetric parameters agreed well between the SCSA and the conventional sequences. The SCSA method halves the breath-hold duration of the commercially available CS sequence and is a reliable alternative for volumetric analysis in a pediatric population. KEY POINTS: • Compressed sensing is a promising accelerated cardiovascular magnetic resonance imaging technique. • We omitted the steady-state preparation cardiac cycle preceding each cine slice in compressed sensing and achieved an acquisition speed of 1 RR interval/slice. • This modification called "single-cycle-stack-advance" enabled the acquisition of an entire short-axis cine stack in a single short breath hold. • When tested in a pediatric patient group, the left and right ventricular volumetric parameters agreed well between the "single-cycle-stack-advance" and the conventional sequences.

Personalised external aortic root support (PEARS) to stabilise an aortic root aneurysm.

Patients with congenitally determined aortic root aneurysms are at risk of aortic valve regurgitation, aortic dissection, rupture and death. Personalised external aortic root support (PEARS) may provide an alternative to aortic root replacement. This was a multi-centre, prospective cohort of all consecutive patients who received ExoVasc mesh implants for a dilated aortic root between 2004 and 2017. Baseline and peri-operative characteristics, as well as early postoperative outcomes are described, and time-related survival and re-operation free survival are estimated using the Kaplan-Meier method. From 2004 through 2017, 117 consecutive patients have received ExoVasc mesh implants for aortic root aneurysm. The inclusion criteria were an aortic root/sinus of Valsalva and ascending aorta with asymptomatic dilatation of between 40 and 50 mm in diameter in patients aged 16 years or more. Patients with more than mild aortic regurgitation were excluded. There was one early death. The length of stay was within seven days in 75% of patients. In conclusion, the operation achieves the objectives of valve-sparing root replacement. PEARS may be seen as a low-risk conservative operation, which can be applied earlier on in the disease process, and which is complementary to more invasive procedures, such as valve-sparing root replacement or total root replacement.

Comparison of 3 T and 1.5 T for T2* magnetic resonance of tissue iron.

BACKGROUND: T2* magnetic resonance of tissue iron concentration has improved the outcome of transfusion dependant anaemia patients. Clinical evaluation is performed at 1.5 T but scanners operating at 3 T are increasing in numbers. There is a paucity of data on the relative merits of iron quantification at 3 T vs 1.5 T. METHODS: A total of 104 transfusion dependent anaemia patients and 20 normal volunteers were prospectively recruited to undergo cardiac and liver T2* assessment at both 1.5 T and 3 T. Intra-observer, inter-observer and inter-study reproducibility analysis were performed on 20 randomly selected patients for cardiac and liver T2*. RESULTS: Association between heart and liver T2* at 1.5 T and 3 T was non-linear with good fit (R (2) = 0.954, p < 0.001 for heart white-blood (WB) imaging; R (2) = 0.931, p < 0.001 for heart black-blood (BB) imaging; R (2) = 0.993, p < 0.001 for liver imaging). R2* approximately doubled between 1.5 T and 3 T with linear fits for both heart and liver (94, 94 and 105 % respectively). Coefficients of variation for intra- and inter-observer reproducibility, as well as inter-study reproducibility trended to be less good at 3 T (3.5 to 6.5 %) than at 1.5 T (1.4 to 5.7 %) for both heart and liver T2*. Artefact scores for the heart were significantly worse with the 3 T BB sequence (median 4, IQR 2-5) compared with the 1.5 T BB sequence (4 [3-5], p = 0.007). CONCLUSION: Heart and liver T2* and R2* at 3 T show close association with 1.5 T values, but there were more artefacts at 3 T and trends to lower reproducibility causing difficulty in quantifying low T2* values with high tissue iron. Therefore T2* imaging at 1.5 T remains the gold standard for clinical practice. However, in centres where only 3 T is available, equivalent values at 1.5 T may be approximated by halving the 3 T tissue R2* with subsequent conversion to T2*.

Validation of T2* in-line analysis for tissue iron quantification at 1.5 T.

BACKGROUND: There is a need for improved worldwide access to tissue iron quantification using T2* cardiovascular magnetic resonance (CMR). One route to facilitate this would be simple in-line T2* analysis widely available on MR scanners. We therefore compared our clinically validated and established T2* method at Royal Brompton Hospital (RBH T2*) against a novel work-in-progress (WIP) sequence with in-line T2* measurement from Siemens (WIP T2*). METHODS: Healthy volunteers (n = 22) and patients with iron overload (n = 78) were recruited (53 males, median age 34 years). A 1.5 T study (Magnetom Avanto, Siemens) was performed on all subjects. The same mid-ventricular short axis cardiac slice and transaxial slice through the liver were used to acquire both RBH T2* images and WIP T2* maps for each participant. Cardiac white blood (WB) and black blood (BB) sequences were acquired. Intraobserver, interobserver and interstudy reproducibility were measured on the same data from a subset of 20 participants. RESULTS: Liver T2* values ranged from 0.8 to 35.7 ms (median 5.1 ms) and cardiac T2* values from 6.0 to 52.3 ms (median 31 ms). The coefficient of variance (CoV) values for direct comparison of T2* values by RBH and WIP were 6.1-7.8 % across techniques. Accurate delineation of the septum was difficult on some WIP T2* maps due to artefacts. The inability to manually correct for noise by truncation of erroneous later echo times led to some overestimation of T2* using WIP T2* compared with the RBH T2*. Reproducibility CoV results for RBH T2* ranged from 1.5 to 5.7 % which were better than the reproducibility of WIP T2* values of 4.1-16.6 %. CONCLUSIONS: Iron estimation using the T2* CMR sequence in combination with Siemens' in-line data processing is generally satisfactory and may help facilitate global access to tissue iron assessment. The current automated T2* map technique is less good for tissue iron assessment with noisy data at low T2* values.

Effect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients.

OBJECTIVE: Personalized external aortic root support (PEARS) is a novel surgical approach with the aim of stabilizing the aortic root size and decreasing risk of dissection in Marfan syndrome patients. A bespoke polymer mesh tailored to each patient's individual aorta shape is produced by modeling and then surgically implanted. The aim of this study is to assess the mechanical effects of PEARS on the aortic root systolic downward motion (an important determinant of aortic wall stress), aortic root distension and on the left ventricle (LV). METHODS/RESULTS: A cohort of 27 Marfan patients had a prophylactic PEARS surgery between 2004 and 2012 with 24 having preoperative and follow-up cardiovascular magnetic resonance imaging studies. Systolic downward aortic root motion, aortic root distension, LV volumes/mass and mitral annular systolic excursion before the operation and in the latest follow-up were measured randomly and blinded. After a median follow-up of 50.5 (IQR 25.5-72) months following implantation of PEARS, systolic downward motion of aortic root was significantly decreased (12.6±3.6mm pre-operation vs 7.9±2.9mm latest follow-up, p<0.00001). There was a tendency for a decrease in systolic aortic root distension but this was not significant (median 4.5% vs 2%, p=0.35). There was no significant change in LV volumes, ejection fraction, mass and mitral annular systolic excursion in follow-up. CONCLUSIONS: PEARS surgery decreases systolic downward aortic root motion which is an important determinant of longitudinal aortic wall stress. Aortic wall distension and Windkessel function are not significantly impaired in the follow-up after implantation of the mesh which is also supported by the lack of deterioration of LV volumes or mass.

An unusual mass of tricuspid valve in an adult patient: blood-filled cyst.

Blood-filled cysts (BFC) within the heart are common findings at postmortem examinations of fetuses and infants. However, such cysts are very rare entities in adolescents and adults. We report here an adult case of BFC attached to the posterior leaflet of the tricuspid valve, demonstrating the importance of multimodal diagnostic imaging combining both echocardiography and magnetic resonance imaging.

Anomalous origin and interarterial course of right coronary artery associated with angina and proven ischemia.

Clinical significance of coronary arteries with anomalous origin and/or course is highly heterogeneous. Anomalies with the origin from the opposite sinus and interarterial course can be associated with angina, syncope, and sudden cardiac death. However, there are no clear guidelines for diagnosis and treatment of such cases. We present the case of a young lady who presented with typical angina, and later proved to have an anomalous right coronary artery (RCA) originating from the left sinus of Valsalva coursing between the aorta and pulmonary artery. This was associated with demonstrable stress ischemia with nuclear perfusion scan. The patient underwent surgery with a bypass graft to the anomalous RCA with complete relief of her angina.

[Comparison of psychosocial risk factors between patients who experience acute myocardial infarction before and after 40 years of age]. / Kirk yas altinda ve üzerinde akut miyokart enfarktüsü geçiren hastalarda psikososyal risk faktörlerinin karsilastirilmasi

OBJECTIVES: Several studies have shown that psychosocial risk factors such as stress and depression make substantial contribution to the pathogenesis of coronary artery disease. This study aimed to investigate acute stress factors prior to acute myocardial infarction (AMI), and stress, depression, and anxiety levels during the subacute period in AMI patients aged ≤ 40 years, in comparison with AMI patients aged >40 years. STUDY DESIGN: The study included 200 first-time AMI patients aged ≤ 40 years (n=100; mean age 35 ± 4 years) and >40 years (n=100; mean age 54 ± 9 years). The DASS 21 scale (Depression Anxiety Stress Scales) was administered via face-to-face interviews in the early recovery period of AMI. The patients were also questioned whether they had experienced acute stress factors such as severe emotional or physical stressful events within two hours before the onset of chest pain. In addition, coronary angiography results were assessed based on the two age groups. RESULTS: Comparison of the two age groups showed significantly higher frequencies of family history of CAD and smoking in the younger group, and significantly higher frequencies of hypertension, diabetes mellitus, and dyslipidemia in the older group (p<0.05). History of acute stress factors was significantly more common (52% vs. 20%, p<0.01) and stress, depression, and anxiety scores of the DASS 21 scale were all significantly higher in the younger group (p<0.01). On coronary angiography, younger patients predominantly had normal coronary arteries and single-vessel disease, whereas multi-vessel disease was more prevalent in the older age group (p<0.01). CONCLUSION: Triggers of acute stress and psychosocial risk factors may contribute to the occurrence of AMI in individuals younger than 40 years.

Treatment of prosthetic valve thrombosis: rationale for a prospective randomized clinical trial.

BACKGROUND AND AIM OF THE STUDY: Prosthetic valve thrombosis (PVT) is a serious complication that may cause significant morbidity and mortality. Despite advances in valve technology, patient education and adequate anticoagulation, the risk of PVT persists indefinitely. Certain clinical and prothrombotic states, including atrial fibrillation, insufficient anticoagulation and pregnancy, predispose these patients to develop PVT. The optimal treatment of the condition is controversial, and to date no randomized clinical trials have been conducted to assist in decision making. Consequently, the guidelines lack definitive Class I recommendations, have significant disparities, and--in most cases--leave the decision to the clinician's experience. METHODS: A review of the studies, guidelines, and expert opinions on the treatment of PVT was conducted, and treatment recommendations made for obstructive and non-obstructive thrombotic cases, based on the results of the recent reports. RESULTS: Although surgical therapy has been the traditional therapeutic approach, studies with low-dose and slow-infusion rates of thrombolytic agents have revealed excellent results in these patients. Surgery should be considered first in patients with left atrial thrombus, active bleeding, and within the first four days after valve replacement. Patients with nonobstructive PVT can initially be managed with an intensification of anticoagulation. CONCLUSION: The management of PVT remains controversial. Clearly, a prospective randomized clinical trial is required, and this could be achieved by developing an international database for patient enrollment and randomization into available treatment strategies.

Severe obstructive hypertrophic cardiomyopathy occurring secondary to mitochondrial disease.

Mitochondrial disorders have been recognized as important secondary causes of cardiomyopathies. Differentiation of these cases from primary cardiomyopathies is important since the pathogenesis, accompanying systemic manifestations, and prognosis may be different. The typical cardiac manifestation of mitochondrial disorders is hypertrophic cardiomyopathy. We report on an 11-year-old girl with severe obstructive hypertrophic cardiomyopathy and mild myopathy of the lower extremities. Surgical left ventricular septal myectomy was performed and ragged red fibers typical of mitochondrial disorders were detected on histological examination of the resected myocardial sample. Subsequent electron microscopic examination revealed ultrastructurally abnormal mitochondria in the skeletal muscle biopsy, though respiratory chain enzyme analysis was normal. Cardiomyopathy may be the presenting or the sole manifestation of a mitochondrial disorder. Nonobstructive hypertrophic cardiomyopathy has been considered to be the typical cardiac phenotype of mitochondrial disorders, and cases with left ventricular outflow tract obstruction have only rarely been reported.

Mitral valve resistance as a determinant of resting and stress pulmonary artery pressure in patients with mitral stenosis: a dobutamine stress study.

BACKGROUND: Severity of mitral stenosis (MS) is assessed by means of mitral valve area and mean transmitral gradient. However, these conventional stenosis indexes poorly reflect the major hemodynamic consequence of MS, which is increase in pulmonary artery pressure (PAP). Valve resistance (VR) is a physiologic expression of stenosis because it incorporates both the pressure gradient and flow data. Previously, in patients with aortic stenosis, hemodynamic burden on the left ventricle has been shown to be closely related to aortic VR but not to aortic valve area. Accordingly, we hypothesized that mitral VR may also better reflect the hemodynamic burden of MS and, hence, be an important determinant of PAP in patients with MS. This study sought to evaluate the relation between several echocardiographic parameters of MS severity, in particular mitral VR and the resting and stress PAP in patients with MS. Determinants of exercise capacity were also assessed. METHODS: Twenty patients with pure MS were studied by Doppler echocardiography. Mitral valve area, mean transmitral gradient, mitral VR, net atrioventricular compliance, and left atrial diameter were derived from resting Doppler echocardiographic examination as possible determinants of resting and stress PAP. PAP was measured by Doppler echocardiography at rest and during dobutamine-induced stress. Patients completed a symptom-limited exercise test to determine exercise capacity. Determinants of resting and stress PAP and exercise capacity were analyzed. RESULTS: Systolic PAP increased significantly from 39.2 +/- 9.4 mm Hg at rest to 59.5 +/- 18.4 mm Hg during dobutamine-induced stress. Mitral VR was the most closely correlated stenosis index with the resting and stress PAP (r = 0.80, P < .001 and r = 0.93, P < .001, respectively) and it was an independent predictor for both with multivariate analysis. Exercise capacity was mostly and equally correlated with stress PAP (r = -0.62, P = .004) and mitral VR (r = -0.62, P = .004). Multivariate analysis revealed stress PAP as the only significant independent predictor of exercise capacity. CONCLUSION: Mitral VR is the strongest and the independent predictor of both resting and stress PAP in patients with MS and by this aspect it is superior to mitral valve area and mean transmitral gradient in the expression of stenosis severity. These results underline the importance of mitral VR as a severity index in patients with MS.

Caseous calcification and liquefaction of the mitral annulus: a diagnostic confounder.

Caseous calcification of the mitral annulus is a rare form of periannular calcification that has a distinct appearance. It generally appears as a large spheric mass like calcification with a central echolucent area that may lead to diagnostic errors. Cardiac imagers should be familiar with this rare form of periannular calcification. We report the case of a 62-year-old woman in whom a suspicious spheric mass like calcification was detected with multislice computed tomography which was performed for coronary artery calcium scoring. Echocardiography displayed the typical findings of caseous calcification of the mitral annulus with central liquefaction.

Serum anti-p53 antibodies do not occur in patients with heart failure due to idiopathic dilated and ischemic cardiomyopathies.

BACKGROUND: P53 is a key protein which controls cell cycle arrest and apoptosis in response to DNA damage. Auto-antibodies against p53 have been detected in some cancer patients and also in patients with autoimmune diseases. In these patients, the main cause of anti-p53 antibody occurrence was considered to be increased intracellular p53 protein in cancer cells and autoreactive lymphocytes, respectively. Intracellular p53 also increases with cardiomyocyte apoptosis during heart failure and autoreactive lymphocytes play a role in the course of idiopathic dilated cardiomyopathy (IDC) and ischemic cardiomyopathy (ICM). Based on these observations, we hypothesized that anti-p53 antibody response may also occur in patients with heart failure due to ICM and IDC. AIM: The aim of this study was to evaluate anti-p53 antibodies in the serum of patients with heart failure due to IDC and ICM. METHODS: 70 eligible patients with heart failure and severe left ventricular systolic dysfunction (mean fractional shortening 12.03 +/- 3.93%) were included in the study. The aetiology of heart failure was IDC in 26 patients and ICM in 44 patients, according to the angiographic and echocardiographic findings. RESULTS: Anti-p53 antibodies were not detected in any of the patients. CONCLUSION: Anti-p53 antibodies do not occur in patients with heart failure due to IDC and ICM, possible explanations are discussed in the text.