Hypertrophy Regression With N-Acetylcysteine in Hypertrophic Cardiomyopathy (HALT-HCM): A Randomized, Placebo-Controlled, Double-Blind Pilot Study.

RATIONALE: Hypertrophic cardiomyopathy (HCM) is a genetic paradigm of cardiac hypertrophy. Cardiac hypertrophy and interstitial fibrosis are important risk factors for sudden death and morbidity in HCM. Oxidative stress is implicated in the pathogenesis of cardiac hypertrophy and fibrosis. Treatment with antioxidant N-acetylcysteine (NAC) reverses cardiac hypertrophy and fibrosis in animal models of HCM. OBJECTIVE: To determine effect sizes of NAC on indices of cardiac hypertrophy and fibrosis in patients with established HCM. METHODS AND RESULTS: HALT-HCM (Hypertrophy Regression With N-Acetylcysteine in Hypertrophic Cardiomyopathy) is a double-blind, randomized, sex-matched, placebo-controlled single-center pilot study in patients with HCM. Patients with HCM, who had a left ventricular wall thickness of ≥15 mm, were randomized either to a placebo or to NAC (1:2 ratio, respectively). NAC was titrated ≤2.4 g per day. Clinical evaluation, blood chemistry, and 6-minute walk test were performed every 3 months, and electrocardiography, echocardiography, and cardiac magnetic resonance imaging, the latter whenever not contraindicated, before and after 12 months of treatment. Eighty-five of 232 screened patients met the eligibility criteria, 42 agreed to participate; 29 were randomized to NAC and 13 to placebo groups. Demographic, echocardiographic, and cardiac magnetic resonance imaging phenotypes at the baseline between the 2 groups were similar. WSE in 38 patients identified a spectrum of 42 pathogenic variants in genes implicated in HCM in 26 participants. Twenty-four patients in the NAC group and 11 in the placebo group completed the study. Six severe adverse events occurred in the NAC group but were considered unrelated to NAC. The effect sizes of NAC on the clinical phenotype, echocardiographic, and cardiac magnetic resonance imaging indices of cardiac hypertrophy, function, and extent of late gadolinium enhancement-a surrogate for fibrosis-were small. CONCLUSIONS: Treatment with NAC for 12 months had small effect sizes on indices of cardiac hypertrophy or fibrosis. The small sample size of the HALT-HCM study hinders from making firm conclusions about efficacy of NAC in HCM. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT01537926.

Two-center clinical validation and quantitative assessment of respiratory triggered retrospectively cardiac gated balanced-SSFP cine cardiovascular magnetic resonance imaging in adults.

BACKGROUND: Breath-hold (BH) requirement remains the limiting factor on the spatio-temporal resolution and coverage of the cine balanced steady-state free precession (bSSFP) cardiovascular magnetic resonance (CMR) imaging. In this prospective two-center clinical trial, we validated the performance of a respiratory triggered (RT) bSSFP cine sequence for evaluation of biventricular function. METHODS: Our study included 23 asymptomatic healthy subjects and 60 consecutive patients from Institute A (n = 39) and Institute B (n = 21) referred for a clinically indicated CMR study. We implemented a RT sequence with a respiratory synchronized drive to steady state (SS) of bSSFP signal, before the commencement of image data acquisition with prospective cardiac arrhythmia rejection and retrospectively cardiac gated reconstruction in real-time. Left (LV) and right (RV) ventricular function and LV mass were evaluated by using RT-bSSFP and conventional BH-bSSFP sequences with one cardiac cycle for SS preparation keeping all the imaging parameters identical. The performance of the sequences was evaluated by using quantitative and semi-quantitative metrics. RESULTS: Global LV and RV functional parameters and LV mass obtained from the RT-bSSFP and BH-bSSFP sequences were in good agreement. Quantitative metrics designed to capture fluctuation in SS signal intensity showed no significant difference between sequences. In addition, blood-to-myocardial contrast was nearly identical between sequences. The combined clinical score for image quality was excellent or good for 100% of cases with the BH-bSSFP and 83% of cases with the RT-bSSFP sequence. The de facto image acquisition time for RT-bSSFP was statistically significantly longer than that for conventional BH-bSSFP (7.9 ± 3.4 min vs. 5.1 ± 2.6 min). CONCLUSIONS: Cine RT-bSSFP is an alternative for evaluating global biventricular function with contrast and spatio-temporal resolutions that are similar to those attained by using the BH-bSSFP sequence, albeit with a modest time penalty and a small reduction in image quality.

Myocardial Stress Perfusion MRI: Experience in Pediatric and Young-Adult Patients Following Arterial Switch Operation Utilizing Regadenoson.

Dextro-transposition of the great arteries (D-TGA) is one of the most common cyanotic heart lesions. The arterial switch operation (ASO) is the preferred surgical palliation for D-TGA. One of the primary concerns following the ASO is complications arising from the coronary artery transfer. There is a need for myocardial perfusion assessment within ASO patients. There is no report on the utility of regadenoson as a stress agent in children following ASO. Our objective was to observe the safety and feasibility of regadenoson as a pharmacologic stressor for perfusion cardiac MR in a pilot cohort of pediatric and young-adult patients who have undergone ASO. We reviewed our initial experience with regadenoson stress cardiac MR in 36 pediatric and young-adult patients 15.1 ± 4.5 years (range 0.2-22 years) with history of ASO. The weight was 61.6 ± 21.5 kg (range 3.8-93 kg). All patients underwent cardiac MR because of concern for ischemia. Subjects' heart rate and blood pressure were monitored and pharmacologic stress was induced by injection of regadenoson. We evaluated their hemodynamic response and adverse effects using changes in vital signs and onset of symptoms. A pediatric cardiologist and radiologist qualitatively assessed myocardial perfusion and viability images. All stress cardiac MR examinations were completed without adverse events. Resting heart rate was 72 ± 13 beats per minute (bpm) and rose to peak of 120 ± 17 bpm (95 ± 50% increase, p < 0.005) with regadenoson. Image quality was considered good or diagnostic in all cases. A total of 11/36 (31%) patients had a perfusion defect on the stress FPP images. 14 of the 36 patients (39%) underwent cardiac catheterization within 6 months of the CMR and the findings showed excellent agreement. Regadenoson may be a useful coronary hyperemia agent to utilize for pediatric patients following arterial switch procedure when there is concern for ischemia. The ability to administer as a single bolus with one IV makes it advantageous in pediatrics. In a limited number of cases, regadenoson stress perfusion showed excellent agreement with cardiac catheterization.

Identification of established arrhythmogenic right ventricular cardiomyopathy mutation in a patient with the contrasting phenotype of hypertrophic cardiomyopathy.

BACKGROUND: Advances in the nucleic acid sequencing technologies have ushered in the era of genetic-based "precision medicine". Applications of the genetic discoveries to practice of medicine, however, are hindered by phenotypic variability of the genetic variants. The report illustrates extreme pleiotropic phenotypes associated with an established causal mutation for hereditary cardiomyopathy. CASE PRESENTATION: We report a 61-year old white female who presented with syncope and echocardiographic and cardiac magnetic resonance (CMR) imaging findings consistent with the diagnosis of hypertrophic cardiomyopathy (HCM). The electrocardiogram, however, showed a QRS pattern resembling an Epsilon wave, a feature of arrhythmogenic right ventricular cardiomyopathy (ARVC). Whole exome sequencing (mean depth of coverage of exons 178X) analysis did not identify a pathogenic variant in the known HCM genes but identified an established causal mutation for ARVC. The mutation involves a canonical splice accepter site (c.2146-1G > C) in the PKP2 gene, which encodes plakophillin 2. Sanger sequencing confirmed the mutation. PKP2 is the most common causal gene for ARVC but has not been implicated in HCM. Findings on echocardiography and CMR during the course of 4-year follow up showed septal hypertrophy and a hyperdynamic left ventricle, consistent with the diagnosis of HCM. However, neither baseline nor follow up echocardiography and CMR studies showed evidence of ARVC. The right ventricle was normal in size, thickness, and function and there was no evidence of fibro-fatty infiltration in the myocardium. CONCLUSIONS: The patient carries an established pathogenic mutation for ARVC and a subtle finding of ARVC but exhibits the classic phenotype of HCM, a contrasting phenotype to ARVC. The case illustrates the need for detailed phenotypic characterization for patients with hereditary cardiomyopathies as well as the challenges physicians face in applying the genetic discoveries in practicing genetic-based "precision medicine".

Comparison of Low-Dose Higher-Relaxivity and Standard-Dose Lower-Relaxivity Contrast Media for Delayed-Enhancement MRI: A Blinded Randomized Crossover Study.

OBJECTIVE: The gadolinium-based MRI contrast agent gadobenate dimeglumine has nearly twice the MR relaxivity of gadopentetate dimeglumine at 1.5 T. The purpose of this study was to determine whether a lower dose (0.1 mmol/kg) of gadobenate dimeglumine can be used to obtain delayed-enhancement MR images comparable to those obtained with a standard dose (0.2 mmol/kg) of gadopentetate dimeglumine. SUBJECTS AND METHODS: In this blinded randomized crossover study, 20 patients with known myocardial infarction underwent two separate delayed-enhancement MRI examinations after receiving 0.1 mmol/kg gadobenate dimeglumine and 0.2 mmol/kg gadopentetate dimeglumine (random administration). The conspicuity of lesion enhancement 5, 10, and 20 minutes after contrast administration was quantified as relative enhancement ratio (RER). RESULTS: With either gadolinium-based contrast agent, damaged myocardium had higher signal intensity than normal remote myocardium (RER > 4) on delayed-enhancement MR images, and the blood RER declined over time after contrast administration. The blood RER was not significantly higher for gadobenate dimeglumine than for gadopentetate dimeglumine at 5 and 10 minutes. Nevertheless, there was a larger reduction in blood RER for gadobenate dimeglumine than for gadopentetate dimeglumine between 5 and 10 minutes and between 10 and 20 minutes. The volumes of enhancement were similar for gadobenate dimeglumine (13.6 ± 8.8 cm(3)) and gadopentetate dimeglumine (13.5 ± 8.9 cm(3)) (p = 0.98). The mean difference in Bland-Altman analysis for delayed-enhancement volume between the agents was 0.1 cm(3). CONCLUSION: Qualitatively and quantitatively, delayed-enhancement MR images of ischemic myocardium obtained with 0.1 mmol/kg gadobenate dimeglumine are comparable to those obtained with 0.2 mmol/kg gadopentetate dimeglumine 5, 10, and 20 minutes after contrast administration.

Gadolinium-Based Contrast Agents: Updates and Answers to Typical Questions Regarding Gadolinium Use.

Gadolinium-based contrast agents have expanded the diagnostic usefulness and capability of magnetic resonance imaging. Despite their highly favorable safety profile, these agents have been associated with nephrogenic systemic fibrosis in a small number of patients who have advanced kidney disease. Recently, trace amounts of gadolinium deposition in the brain and other organs have been reported after contrast exposure, even in patients with normal renal function. In this review, we provide a brief overview of recent updates and discuss typical clinical situations related to the use of gadolinium-based contrast agents.

A randomized study of transendocardial injection of autologous bone marrow mononuclear cells and cell function analysis in ischemic heart failure (FOCUS-HF).

BACKGROUND: Autologous bone marrow mononuclear cell (ABMMNC) therapy has shown promise in patients with heart failure (HF). Cell function analysis may be important in interpreting trial results. METHODS: In this prospective study, we evaluated the safety and efficacy of the transendocardial delivery of ABMMNCs in no-option patients with chronic HF. Efficacy was assessed by maximal myocardial oxygen consumption, single photon emission computed tomography, 2-dimensional echocardiography, and quality-of-life assessment (Minnesota Living with Heart Failure and Short Form 36). We also characterized patients' bone marrow cells by flow cytometry, colony-forming unit, and proliferative assays. RESULTS: Cell-treated (n = 20) and control patients (n = 10) were similar at baseline. The procedure was safe; adverse events were similar in both groups. Canadian Cardiovascular Society angina score improved significantly (P = .001) in cell-treated patients, but function was not affected. Quality-of-life scores improved significantly at 6 months (P = .009 Minnesota Living with Heart Failure and P = .002 physical component of Short Form 36) over baseline in cell-treated but not control patients. Single photon emission computed tomography data suggested a trend toward improved perfusion in cell-treated patients. The proportion of fixed defects significantly increased in control (P = .02) but not in treated patients (P = .16). Function of patients' bone marrow mononuclear cells was severely impaired. Stratifying cell results by age showed that younger patients (≤60 years) had significantly more mesenchymal progenitor cells (colony-forming unit fibroblasts) than patients >60 years (20.16 ± 14.6 vs 10.92 ± 7.8, P = .04). Furthermore, cell-treated younger patients had significantly improved maximal myocardial oxygen consumption (15 ± 5.8, 18.6 ± 2.7, and 17 ± 3.7 mL/kg per minute at baseline, 3 months, and 6 months, respectively) compared with similarly aged control patients (14.3 ± 2.5, 13.7 ± 3.7, and 14.6 ± 4.7 mL/kg per minute, P = .04). CONCLUSIONS: ABMMNC therapy is safe and improves symptoms, quality of life, and possibly perfusion in patients with chronic HF.

Ultrafast Computation of Left Ventricular Ejection Fraction by Using Temporal Intensity Variation in Cine Cardiac Magnetic Resonance.

Cardiac magnetic resonance enables comprehensive cardiac evaluation; however, intense time and labor requirements for data acquisition and processing have discouraged many clinicians from using it. We have developed an alternative image-processing algorithm that requires minimal user interaction: an ultrafast algorithm that computes left ventricular ejection fraction (LVEF) by using temporal intensity variation in cine balanced steady-state free precession (bSSFP) short-axis images, with or without contrast medium. We evaluated the algorithm's performance against an expert observer's analysis for segmenting the LV cavity in 65 study participants (LVEF range, 12%-70%). In 12 instances, contrast medium was administered before cine imaging. Bland-Altman analysis revealed quantitative effects of LV basal, midcavity, and apical morphologic variation on the algorithm's accuracy. Total computation time for the LV stack was <2.5 seconds. The algorithm accurately delineated endocardial boundaries in 1,132 of 1,216 slices (93%). When contours in the extreme basal and apical slices were not adequate, they were replaced with manually drawn contours. The Bland-Altman mean differences were <1.2 mL (0.8%) for end-diastolic volume, <5 mL (6%) for end-systolic volume, and <3% for LVEF. Standard deviation of the difference was ≤4.1% of LV volume for all sections except the midcavity in end-systole (8.3% of end-systolic volume). We conclude that temporal intensity variation-based ultrafast LVEF computation is clinically accurate across a range of LV shapes and wall motions and is suitable for postcontrast cine SSFP imaging. Our algorithm enables real-time processing of cine bSSFP images on a commercial scanner console within 3 seconds in an unobtrusive automated process.

Prognostic significance of delayed-enhancement magnetic resonance imaging: survival of 857 patients with and without left ventricular dysfunction.

BACKGROUND: Left ventricular ejection fraction is a powerful independent predictor of survival in cardiac patients, especially those with coronary artery disease. Delayed-enhancement magnetic resonance imaging (DE-MRI) can accurately identify irreversible myocardial injury with high spatial and contrast resolution. To date, relatively limited data are available on the prognostic value of DE-MRI, so we sought to determine whether DE-MRI findings independently predict survival. METHODS AND RESULTS: The medical records of 857 consecutive patients who had complete cine and DE-MRI evaluation at a tertiary care center were reviewed regardless of whether the patients had coronary artery disease. The presence and extent of myocardial scar were evaluated qualitatively by a single experienced observer. The primary, composite end point was all-cause mortality or cardiac transplantation. Survival data were obtained from the Social Security Death Index. The median follow-up was 4.4 years; 252 patients (29%) reached one of the end points. Independent predictors of mortality or transplantation included congestive heart failure, ejection fraction, and age (P<0.0001 for each), as well as scar index (hazard ratio, 1.26; 95% confidence interval, 1.02 to 1.55; P=0.033). Similarly, in subsets of patients with or without coronary artery disease, scar index also independently predicted mortality or transplantation (hazard ratio, 1.33; 95% confidence interval, 1.05 to 1.68; P=0.018; and hazard ratio, 5.65; 95% confidence interval, 1.74 to 18.3; P=0.004, respectively). Cox regression analysis showed worse outcome in patients with any DE in addition to depressed left ventricular ejection fraction (<50%). CONCLUSIONS: The degree of DE detected by DE-MRI appears to strongly predict all-cause mortality or cardiac transplantation after adjustment for traditional, well-known prognosticators.

Left Ventricular Noncompaction Detected by Cardiac Magnetic Resonance Screening: A Reexamination of Diagnostic Criteria.

In a previous cross-sectional screening study of 5,169 middle and high school students (mean age, 13.1 ± 1.78 yr) in which we estimated the prevalence of high-risk cardiovascular conditions associated with sudden cardiac death, we incidentally detected by cardiac magnetic resonance (CMR) 959 cases (18.6%) of left ventricular noncompaction (LVNC) that met the Petersen diagnostic criterion (noncompaction:compaction ratio >2.3). Short-axis CMR images were available for 511 of these cases (the Short-Axis Study Set). To determine how many of those cases were truly abnormal, we analyzed the short-axis images in terms of LV structural and functional variables and applied 3 published diagnostic criteria besides the Petersen criterion to our findings. The estimated prevalences were 17.5% based on trabeculated LV mass (Jacquier criterion), 7.4% based on trabeculated LV volume (Choi criterion), and 1.3% based on trabeculated LV mass and distribution (Grothoff criterion). Absent longitudinal clinical outcomes data or accepted diagnostic standards, our analysis of the screening data from the Short-Axis Study Set did not definitively differentiate normal from pathologic cases. However, it does suggest that many of the cases might be normal anatomic variants. It also suggests that cases marked by pathologically excessive LV trabeculation, even if asymptomatic, might involve unsustainable physiologic disadvantages that increase the risk of LV dysfunction, pathologic remodeling, arrhythmias, or mural thrombi. These disadvantages may escape detection, particularly in children developing from prepubescence through adolescence. Longitudinal follow-up of suspected LVNC cases to ascertain their natural history and clinical outcome is warranted.

Mitral Valve Flow and Myocardial Motion Assessed with Dual-Echo Dual-Velocity Cardiac MRI.

PURPOSE: To develop a dual-echo phase-contrast (DEPC) MRI approach with which each echo is acquired by using a different velocity sensitivity within one repetition time (TR) and demonstrate the feasibility of this approach to measure transmitral blood flow (E) and myocardial tissue (E m) velocities. MATERIALS AND METHODS: The flow across tubes of known diameter was measured by using the proposed DEPC method and compared with flowmeter measurements and theoretic predictions. Then, with both the DEPC MRI sequence and the conventional single-echo phase-contrast (SEPC) MRI sequence, E, E m, and E/E m were measured in six healthy volunteers (mean age, 49 years ± 13 [standard deviation]) and eight patients (mean age, 54 years ± 15) being evaluated for cardiac disease. Differences between the DEPC and conventional SEPC MRI methods were assessed by percent error, Pearson correlation, and Bland-Altman analyses. RESULTS: Velocities measured in vitro and in vivo by using the SEPC and DEPC MRI approaches were well correlated (r 2 > 0.97), with negligible bias (<0.5 cm/sec) and comparable velocity-to-noise ratios. Imaging times were approximately 19% shorter with the DEPC method (TR, 5.7 msec) than with the SEPC method (TR, 2.8 msec ± 4.2) (P < .05). CONCLUSION: The proposed DEPC method was sensitive to two velocity regimes within a single TR, resulting in a shorter imaging time compared with the imaging time in conventional SEPC MRI. Preliminary human study results suggest the feasibility of using this approach to estimate E/E m.Supplemental material is available for this article.© RSNA, 2020.

Multidetector Computed Tomography (MDCT) Angiography in the Pre-Procedural Assessment of Patients Undergoing Transcatheter Aortic Valve Replacement.

Transcatheter aortic valve replacement (TAVR) initially emerged as an alternative option to surgical aortic valve replacement (SAVR) for patients with severe aortic stenosis who were considered either inoperable or high-risk for surgery. However, since its advent the role of TAVR has been continuously evolving on the basis of clinical trials which showed that TAVR is non-inferior to SAVR in patients with moderate as well as low-risk for surgery. Because of recent technological advances, multidetector computer tomography (MDCT) is inherently suitable for the pre-procedural assessment of patients being considered for TAVR within a very short imaging time, MDCT can measure the diameter of the aortic annulus, provide detailed information regarding the status of the entire thoracoabdominal aorta, and assess the caliber of the peripheral vasculature used for transcatheter heart valve delivery. This information helps interventionists make optimal pre-procedural decisions and avoid complications. To familiarize non-imaging specialists with the role of MDCT in TAVR, we provide a concise overview of our approach to using this modality for the pre-procedural assessment of TAVR candidates.

Use of magnetic resonance imaging to assess myocardial perfusion after transmyocardial laser revascularization.

BACKGROUND: Transmyocardial laser revascularization (TMLR) is an alternative treatment modality for patients with refractory angina who are not candidates for conventional surgical or percutaneous revascularization. Clinical studies of TMLR have not shown one-to-one correlation between increased myocardial perfusion and improved clinical status. METHODS: Three patients (51, 53, and 70 years old) with severe, diffuse coronary artery disease not amenable to conventional surgical revascularization and with angina (Canadian Cardiovascular Society [CCS] class 3-4) refractory to maximal medical therapy underwent TMLR with a CO2 laser. Preoperative and postoperative cardiac magnetic resonance imaging (MRI) were performed to assess left ventricular perfusion and wall-motion changes in the laser-treated areas. Postoperative MRIs were performed within 6 months of TMLR and at 12 months. Angina status was assessed with the Seattle Angina Questionnaire. RESULTS: Postoperative adenosine stress myocardial perfusion imaging with MRI revealed improved overall perfusion and a reduction in subendocardial hypoperfused areas when compared to preoperative images. In all patients, an improvement in CCS class was consistent with an improvement in perfusion. CONCLUSIONS: Cardiac MRI can be used to assess improved subendocardial perfusion after TMLR treatment. In our study, we found that endocardial perfusion was maintained over a 12-month period.

Labeling Stem Cells with a New Hybrid Bismuth/Carbon Nanotube Contrast Agent for X-Ray Imaging.

The poor retention and survival of cells after transplantation to solid tissue represent a major obstacle for the effectiveness of stem cell-based therapies. The ability to track stem cells in vivo can lead to a better understanding of the biodistribution of transplanted cells, in addition to improving the analysis of stem cell therapies' outcomes. Here, we described the use of a carbon nanotube-based contrast agent (CA) for X-ray computed tomography (CT) imaging as an intracellular CA to label bone marrow-derived mesenchymal stem cells (MSCs). Porcine MSCs were labeled without observed cytotoxicity. The CA consists of a hybrid material containing ultra-short single-walled carbon nanotubes (20-80 nm in length, US-tubes) and Bi(III) oxo-salicylate clusters which contain four Bi3+ ions per cluster (Bi4C). The CA is thus abbreviated as Bi4C@US-tubes.

Acute type I aortic dissection with or without antegrade stent delivery: Mid-term outcomes.

OBJECTIVE: We determined the effect of antegrade stent delivery in the descending thoracic aorta on short- and mid-term clinical and imaging outcomes for patients who underwent repair of acute DeBakey type I aortic dissection. METHODS: Outcomes were evaluated for 178 patients who underwent acute type I aortic dissection between 2005 and 2016 (standard repair, n = 115 [64.6%]; antegrade stent delivery, n = 63 [35.4%]). Propensity score match and multivariable analyses were performed to assess outcomes. RESULTS: The stent and standard repair groups had similar rates of operative mortality (30-day or in-hospital) (12.7% vs 17.4%, P = .41), persistent stroke (6.3% vs 5.3%, P = .75), and persistent paraplegia/paraparesis (1.6% vs 0.9%, P = 1.0). Propensity score match analysis indicated that the operative mortality rate was higher in the standard repair group (P = .059), which the multivariable analysis confirmed. The persistent stroke rate was nonsignificantly higher in the stent group (P = .66). Persistent paraplegia/paraparesis rates were similar in both groups (P = 1.0), and the overall rates of spinal cord ischemia were nonsignificantly higher in the stent group (P = .18). During follow-up (mean duration, 4.6 ± 3.6 y), computed tomography showed that stented patients more often had remodeling of the descending thoracic aorta (P = .0002) and somewhat more often had remodeling of the thoracoabdominal aorta (P = .13). Stented patients also had fewer subsequent procedures (P = .25). The 3- and 5-year survivals were 73.3% ± 6.9% and 49.9% ± 7.6% in the matched stented group and 66.3% ± 9.4% and 41.6% ± 7.7% in the matched standard group, respectively (P = .015 for overall survival). CONCLUSIONS: In the short term, antegrade stent delivery was associated with less operative mortality. In the mid-term, promising remodeling of the false lumen was seen in stented patients, as were (nonsignificantly) lower rates of subsequent procedures in the thoracoabdominal aorta. Mid-term survival was also greater in the stented patients.

High-Risk Cardiovascular Conditions in Sports-Related Sudden Death: Prevalence in 5,169 Schoolchildren Screened via Cardiac Magnetic Resonance.

Improving preparticipation screening of candidates for sports necessitates establishing the prevalence of high-risk cardiovascular conditions (hr-CVC) that predispose young people to sudden cardiac death (SCD). Our accurate, novel protocol chiefly involved the use of cardiac magnetic resonance (CMR) to estimate this prevalence. Middle and high school students from a general United States population were screened by means of questionnaires, resting electrocardiograms, and CMR to determine the prevalence of 3 types of hr-CVC: electrocardiographic abnormalities, cardiomyopathies, and anomalous coronary artery origin from the opposite sinus with intramural coronary course (ACAOS-IM). We examined the range of normal left ventricular size and function in the main study cohort (schoolchildren 11-14 yr old). We defined diagnostic criteria for hr-CVC and compared the cardiac measurements of these younger participants with those of older children whom we examined (age, 15-18 yr). From 5,169 completed diagnostic studies (mean participant age, 13.06 ± 1.78 yr), CMR results revealed 76 previously undiagnosed cases of hr-CVC (1.47% of the total cohort): 11 of dilated cardiomyopathy (14.5%), 3 of nonobstructive hypertrophic cardiomyopathy (3.9%), 23 ACAOS-IM cases (30.3%; 6 left-ACAOS and 17 right-ACAOS), 4 Wolff-Parkinson-White patterns (5.3%), 34 prolonged QT intervals (44.7%), and 1 Brugada pattern (1.3%). Cardiomyopathies were significantly more prevalent in the older children. Of note, we identified 959 cases (18.5%) of left ventricular noncompaction. If our estimate is accurate, only 1.47% of school-age sports participants will need focused secondary evaluations; the rest can probably be reassured about their cardiac health after one 30-minute screening study.

High-Performance Hybrid Bismuth-Carbon Nanotube Based Contrast Agent for X-ray CT Imaging.

Carbon nanotubes (CNTs) have been used for a plethora of biomedical applications, including their use as delivery vehicles for drugs, imaging agents, proteins, DNA, and other materials. Here, we describe the synthesis and characterization of a new CNT-based contrast agent (CA) for X-ray computed tomography (CT) imaging. The CA is a hybrid material derived from ultrashort single-walled carbon nanotubes (20-80 nm long, US-tubes) and Bi(III) oxo-salicylate clusters with four Bi(III) ions per cluster (Bi4C). The element bismuth was chosen over iodine, which is the conventional element used for CT CAs in the clinic today due to its high X-ray attenuation capability and its low toxicity, which makes bismuth a more-promising element for new CT CA design. The new CA contains 20% by weight bismuth with no detectable release of bismuth after a 48 h challenge by various biological media at 37 °C, demonstrating the presence of a strong interaction between the two components of the hybrid material. The performance of the new Bi4C@US-tubes solid material as a CT CA has been assessed using a clinical scanner and found to possess an X-ray attenuation ability of >2000 Hounsfield units (HU).

Inflammation and Rupture of a Congenital Pericardial Cyst Manifesting Itself as an Acute Chest Pain Syndrome.

We present the case of a 63-year-old woman with a remote history of supraventricular tachycardia and hyperlipidemia, who presented with recurrent episodes of acute-onset chest pain. An electrocardiogram showed no evidence of acute coronary syndrome. A chest radiograph revealed a prominent right-sided heart border. A suspected congenital pericardial cyst was identified on a computed tomographic chest scan, and stranding was noted around the cyst. The patient was treated with nonsteroidal anti-inflammatory drugs, and the pain initially abated. Another flare-up was treated similarly. Cardiac magnetic resonance imaging was then performed after symptoms had resolved, and no evidence of the cyst was seen. The suspected cause of the patient's chest pain was acute inflammation of a congenital pericardial cyst with subsequent rupture and resolution of symptoms.

Retrograde Ascending Aortic Dissection After Thoracic Endovascular Aortic Repair for Distal Aortic Dissection or With Zone 0 Landing: Association, Risk Factors, and True Incidence.

BACKGROUND: We attempted to determine the true incidence of retrograde ascending aortic dissection (rAAD) and to challenge its reported association with distal aortic dissection or zone 0 deployment. METHODS: From January 2005 to August 2014, 305 patients who were at risk for rAAD underwent thoracic endovascular aortic repair. We excluded patients with prior ascending, hemiarch, or traditional or frozen elephant trunk grafts, or who required concomitant ascending graft placement. Patients in group A (n = 111, 36.4%) had distal aortic dissection or hematoma (n = 75, 67.6%) or required landing of the endograft in zone 0 of the native ascending aorta (n = 36, 32.4%). Patients in group B (n = 194, 63.6%) had nondissected descending or distal arch aneurysm (n = 172), penetrating ulcer (n = 9), coarctation (n = 6), endoleak not caused by dissection (n = 3), aortobronchial fistula (n = 3), or transection (n = 1). RESULTS: The incidence of rAAD was 1.3% overall (n = 4), 0.9% in group A (n = 1, Cook Zenith TX2), and 1.5% in group B (n = 3; 1 Talent Captivia, 2 Cook Zenith TX2; p = 0.64). No zone 0-treated patient had rAAD. Two patients from group B died, and 1 was treated nonoperatively. The median interval between thoracic endovascular aortic repair and rAAD was 11 days (range, 0 to 90 days). CONCLUSIONS: Post-thoracic endovascular aortic repair rAAD is a rare but lethal complication. Operator experience is crucial for prompt recognition and prevention. It does not appear that rAAD is specifically associated with distal aortic dissection or landing in zone 0. To our knowledge, this is one of the few studies to report the true incidence of rAAD in at-risk patients.

Endovascular repair of the ascending aorta: when and how to implement the current technology.

BACKGROUND: The purpose of our study was to examine when and how to implement the current endoluminal stent graft technology to treat ascending aortic disease. METHODS: During a 7-year period (March 2006 through July 2013), 7 consecutive patients (median age, 69 years; range, 61.5 to 80.5 years) with multiple comorbidities underwent endoluminal repair of the ascending aorta. Six had an ascending aortic pseudoaneurysm, and 1 had iatrogenic coarctation. The median number of prior sternotomies was 2 (range, 1 to 4). RESULTS: Technical success was achieved in all but 1 patient, with 1 death (14.3%) at 30 days. The endoluminal technology used included the Gore TAG (W.L. Gore and Associates, Flagstaff, AZ) thoracic graft (including the new C-TAG) in 6 patients, the Talent stent graft (Medtronic, Santa Rosa, CA) in 1, an Excluder cuff (W.L. Gore) in 2, and an Amplatzer occluder (AGA Medical Corp, Plymouth, MN) in 1. More than 1 stent was placed in 4 patients. Three patients required innominate artery stenting, and 1 required additional left common carotid artery stenting. One patient (14.3%) required intraoperative conversion to open surgical repair. Median follow-up was 14.4 months (interquartile [25th to 75th percentile] range, 5.5 to 22.6 months) with 66.6% overall survival. No aortic-related death was reported during the follow-up period. CONCLUSIONS: Stent grafting of the ascending aorta is feasible but limited and is reserved for high-risk individuals. Technical expertise is essential, and follow-up is mandatory. Technical points, tips, and challenges of the current endovascular technology to effectively treat the ascending aorta are described.