Cardiovasc Magn Reson Research Articles

A tale of 2 conversions: Dichotomous decision in the spectrum of Shone syndrome is still a dilemma

A tale of 2 conversions: Dichotomous decision in the spectrum of Shone syndrome is still a dilemma

MO034A SUBGROUP ANALYSIS OF FEMALE PATIENTS IN A PHASE 3 OPEN-LABEL STUDY TO ASSESS THE SAFETY AND EFFICACY OF PEGUNIGALSIDASE ALFA IN PATIENTS WITH FABRY DISEASE PREVIOUSLY TREATED WITH AGALSIDASE ALFA

Abstract Background and Aims Females with Fabry disease (FD) often develop symptoms and disease complications later in life than males. However, they can experience significant health declines, including renal function impairment. Pegunigalsidase alfa is a novel PEGylated alpha-galactosidase A enzyme in development for the treatment of patients with FD with potential pharmacokinetic benefits. We previously reported that males with FD showed improvements in several parameters including median (minimum, maximum) estimated glomerular filtration rate (eGFR) slope from -4.6 (-20.5, 4.8) to -1.1 (-18.6, 14.2) mL/min/1.73m2/year after treatment with pegunigalsidase alfa.(Tondel et al. ASN 2020. PO0562. www.asn.scientificposters.com) Here we report a subgroup analysis of the safety and efficacy of pegunigalsidase alfa treatment in females with FD. Method BRIDGE (PB-102-F30; NCT03018730) is a phase 3, open-label, switch-over study designed to assess the safety and efficacy of pegunigalsidase alfa in adults with FD previously treated with agalsidase alfa for at least 2 years. Patients received intravenous pegunigalsidase alfa at 1 mg/kg every other week for 12 months. Results Twenty-two patients were enrolled in the study; of the 20 patients who completed 12 months of study treatment, 7 were female. Females had a mean age of 46.7 years (range: 26–59 years), and had the following median (minimum, maximum) baseline measurements: residual enzymatic activity in leucocytes of 23.7% (16, 46) of the normal laboratory mean; plasma lyso-Gb3 of 12.9 (7.4, 23.2) nmol/L; eGFR of 87.7 (55.3, 109.2) mL/min/1.73m2; and an annualized eGFR slope of −3.7 (-11.2, 1.5) mL/min/1.73m2/year. After 12 months of pegunigalsidase alfa treatment, the annualized eGFR slope was 1.4 (-6.3, 4.1) mL/min/1.73m2/year, indicating an improvement from baseline of 5.9 mL/min/1.73m2/year. In addition, plasma lyso-Gb3 had a reduction of 23.3% (-45.7, -17.3). Although all females had baseline mean residual enzyme activity > 5% and were previously treated with agalsidase alfa, only 2 had stable kidney disease (eGFR slope ≥ -3 mL/min/1.73m2/year), while 2 had moderately progressing kidney disease (eGFR slope between ≥-5 and < -3 mL/min/1.73m2/year), and 3 had fast progressing kidney disease (eGFR slope < -5 mL/min/1.73 m²/year).( Wanner et al. 2018 Mol Genet Metab 124:189-203) After treatment all but 1 patient experienced categorical improvement or remained stable; this patient had a decline of < 3 mL/min/1.73m2/year and remained in the fast progressing disease category. Mean left ventricular mass index in females increased from 66.9 g/m2 at baseline to 74.1 g/m2 at month 12, but remained within normal ranges(47–77 g/m2).(Kawel-Boehm et al. 2015 J Cardiovasc Magn Reson 17:29) All females had at least 1 treatment-emergent adverse event (TEAE), and all TEAEs were mild or moderate. The most common TEAEs reported in female were nasopharyngitis (n=2), oropharyngeal pain (n=2), and headache (n=2). None of these TEAEs were considered related to treatment. However, 2 females had injection site reactions and 2 developed transient, non-neutralizing anti-drug antibodies to pegunigalsidase alfa treatment. Conclusion The current study included females with symptoms of Fabry disease comparable to the disease presentation of males enrolled in this study. At baseline most females had eGFR decline characterizing progressive or rapidly progressive kidney disease. Most females showed improvements in disease status following 12 months of pegunigalsidase alfa treatment, as previously reported for males enrolled in this study. This long-term, controlled study suggests a potential benefit and a favorable safety profile for pegunigalsidase alfa on renal function in females with FD previously treated with agalsidase alfa. While this subgroup analysis should be interpreted with caution due to the small number of patients, these findings may provide valuable insight for future studies.

Minimally invasive pulmonary valve replacement via left anterior minithoracotomy.

ABSTRACT Pulmonary valve replacement is one of the most common indications for reoperation in adults with congenital heart disease. Finding an alternative to repeat median sternotomy may facilitate recovery and shorten hospital stay. We present our technique of left anterior minithoracotomy for pulmonary valve replacement.

57-Year-Old Man With Atypical Chest Pain

57-Year-Old Man With Atypical Chest Pain

CMR in Phenotyping the Arrhythmic Substrate

The purpose of this review is to explore the role of cardiac magnetic resonance imaging (CMR) in the evaluation of myocardial disorders that can present with significant cardiac arrhythmias. In addition, we explore recent developments in the field of CMR in the evaluation of such conditions, such as T1 and T2 mapping techniques. The importance of CMR as a diagnostic tool in the evaluation of such conditions lies in the fact that in routine clinical practice these conditions are encountered relatively frequently, and often important clinical decisions are made based on the findings of cardiac investigations such as CMR, e.g., decisions surrounding revascularization or implantable cardioverter defibrillator (ICD) therapy. In fact, CMR is now included in many practice guidelines (American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines et al. in J Thorac Cardiovasc Surg, 142(6):e153–203, 2011; Authors/Task Force members et al. in Eur Heart J, 35(39):2733–79, 2014; Epstein et al. in Heart Rhythm, 5(6):934–55, 2008; Maron et al. in J Am Coll Cardiol, 42(9):1687–713, 2003) and is routinely used in the evaluation of cardiomyopathic and ischemic myocardial conditions in many institutions. Although late gadolinium enhancement (LGE)-CMR allows a very sensitive and reproducible qualitative assessment of myocardial replacement fibrosis, it is limited in regards to absolute quantification of myocardial fibrosis, and also, the assessment of diffuse fibrosis by CMR can be technically challenging. As a result, in recent years, T1 and T2 mapping techniques have been welcomed by the CMR community, as potential methods of quantitating myocardial fibrosis accurately. Areas of diffuse myocardial fibrosis have greater T1 values (by about 10–20%) than normal tissue, before intravenous Gd is given. Post-Gd administration, T1 values are lower than normal in diffuse myocardial fibrosis. Thus, using this technique maps can be generated that explore the myocardium in detail and can detect early myocardial fibrosis, often before the presence of LGE. T2-weighted CMR identifies myocardial edema before the onset of irreversible ischemic injury and has shown to have value in risk-stratifying patients with chest pain (Eitel and Friedrich in J Cardiovasc Magn Reson, 13:13, 2011). Clinical acceptance of T2-weighted CMR has, however, been limited by well-known technical problems associated with existing techniques. T2 quantification using T2 mapping in the future should overcome these problems. Both T1 and T2 mapping techniques are discussed in further detail below. The assessment of infarct heterogeneity, as measured by peri-infarct zone (PIZ) mass and percentage PIZ (%PIZ), as compared to normal myocardium, is a valuable CMR tool for the assessment of tissue characteristics in the peri-infarct region (Morgan and Kwong in Curr Treat Options Cardiovasc Med, 17(11):53, 2015). It has recently been shown to have useful prognostic information (Watanabe et al. in Circ Cardiovasc Imaging, 7(6):887–94, 2014) in patients with coronary artery disease (CAD) and left ventricular (LV) dysfunction referred for CMR. This is discussed in further detail below. A recent paper by Neilan et al. (JACC Cardiovasc Imaging, 8(4):414–23, (2015)) studied 137 patients who underwent CMR post-resuscitated cardiac arrest and found that in cases where the cause of sudden cardiac death (SCD) was not initially clear, the use of CMR provided a diagnosis in 104 patients (76%). In a multivariable analysis, the strongest predictors of recurrent events were the presence and the extent of LGE (p < 0.001). In summary, this paper, through recent evidence and clinical examples, explores those myocardial conditions which pose a significant clinical issue in the form of potentially dangerous cardiac dysrhythmias. Early detection of predictive myocardial characteristics beyond the traditional risk factors has been welcomed by cardiology and cardiac imaging communities. Much work is in progress to make new methods such as T1 and T2 mapping more clinically available and applicable and to ensure that imaging time is not lengthened beyond that which is comfortable for the patient.

006 Role of T1 mapping as a complementary tool to T2* for non-invasive cardiac iron overload assessment

AbstractBackground Iron overload-related heart failure is the principal cause of death in transfused Thalassemia Major1–2 and other iron overload patients. Linking cardiac siderosis measured by T2* to therapy improves outcome in Thalassemia Major. Aim of our study is to compare T1 mapping (Modified Lock Locker Inversion recovery, MOLLI) to dark (DB) and bright (BB) blood T2*3–4 in cardiac iron overload and to support the hypothesis that T1 mapping has higher sensibility to T2* for small amount of iron, which would make it a complementary tool to T2* in borderline iron overload patients.5–6 Methods In a prospectively large single centre study of 138 Thalassemia Major patients and 32 healthy controls, we compared MOLLI to DB and BB T2* acquired on an Avanto 1.5T scanner (Siemens Healthcare, Erlangen, Germany). Linear regression analysis was used to assess the association between DBT2* and either BBT2* and MOLLI, and the determination coefficient was computed in a log-log scale with moving windows to detect the point where this association decreases.Results The relationship between T2* (here DB) and MOLLI is described by a log-log linear regression, which can be split in three different slopes: 1) T2* low, <20ms: r2=0.92; 2) T2*=20–28 ms: r2=0.80; 3) T2*>28 ms, no relationship. All subjects with T2*<20 ms had low T1; of those with T2*>20 ms, 38% had low T1.Conclusions These data support the former proposal that T1 detects missed iron 1 in 3 subjects with normal T2* and that T1 mapping is a complementary tool for non-invasive assessment of cardiac iron. The clinical significance of a low T1, normal T2* should be further investigated. A trend toward LV end diastolic volume increase was observed in the patients with low T1 and normal T2* at 24 months, but the sample was too small to be analysed (n=9).References. Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiovasc Magn Reson2008;10:42.. Carpenter JP, Pennell DJ. On T2* Magnetic Resonance and Cardiac Iron. Circulation2011;14:1519–28.. Wood JC, Otto-Duessel M, Aguilar M, et al. Cardiac Iron Determines Cardiac T2*, T2, and T1 in the Gerbil Model of Iron Cardiomyopathy. Circulation2005;112(4):535–543.. Carpenter JP, He T, Kirk P, et al. Calibration of myocardial T2 and T1 against iron concentration. J Cardiovasc Magn Reson2014;16:62.. Alam MH, Auger D, Smith GC, et al. T1 at 1.5T and 3T compared with conventional T2* at 1.5T for cardiac siderosis. J Cardiovasc Magn Reson. 2015;17:102.. Messroghli DR, Radjenovic A, Kozerke S, Higgins DM, Sivananthan MU, Ridgway JP. Modified Look-Locker inversion recovery (MOLLI) for high-resolution T1 mapping of the heart. Magn Reson Med2004;52(1):141–6.. Alam MH, Auger D, Smith GC, et al. T1 at 1.5T and 3T compared with conventional T2* at 1.5T for cardiac siderosis. J Cardiovasc Magn Reson2015;17(24):102.Download figure Open in new tab Download powerpoint Abstract 006 Figure 1

013 Free-breathing MOCO LGE leads to better image quality and faster scanning times in clinical practice

Objectives Late gadolinium enhancement (LGE) sequences have evolved. Free-breathing, motion-corrected LGE (MOCO-LGE)1,2 has several potential advantages over breath held LGE (bh-LGE)3 including minimal user input for the LGE short axis (SA) stack and no need for breath-holds. We hypothesised that the use of MOCO-LGE would be faster, cheaper and easier for clinical scanning, increasing throughput. Methods 200 consecutive clinical patients underwent bh-LGE or MOCO-LGE at 1.5T. Image quality (Figure 1), scan time, patient throughput (change-over time) and reader confidence were compared. LGE image quality was evaluated qualitatively (adaption of previously reported method)4 and quantitatively (assessing image texture heterogenity using grayscale lacunarity, λ). Results MOCO-LGE image quality was better than bh-LGE qualitatively (lower score better: 0.56±1.2 vs 1.93±0.83, p Conclusion MOCO-LGE is superior to bh-LGE in a clinical service, with better image quality, easier interpretation and faster scanning times. References . Maria JLedesma-Carbayo, Peter Kellman, Andrew EArai, et al. Motion corrected free-breathing delayed-enhancement imaging of myocardial infarction using nonrigid registration. JMRI Published Online First : 20 Jul 2007. 2. Peter Kellman, Andrew EArai. Cardiac imaging techniques for physicians: Late enhancement. JMRI Published Online First : 17 Aug 2012.3. Piehler KM, Wong TC, Puntil KS, et al. Free-breathing, motion-corrected late gadolinium enhancement is robust and extends risk stratification to vulnerable patients. Circ Cardiovasc Imaging 2013;6(3):423-32.4. Vincenzo Klinke, Stefano Muzzarelli, Nathalie Lauriers, et al. Quality assessment of cardiovascular magnetic resonance in the setting of the European CMR registry: description and validation of standardized criteria. J Cardiovasc Magn Reson 2013;15:55.

011 Adenosine stress T1 mapping: a novel contrast free method to assess myocardial perfusion and ischaemia in hypertrophic cardiomyopathy

Aim The aim of this study was to assess the ability of stress T1 mapping to detect perfusion abnormalities and inducible ischaemia in hypertrophic cardiomyopathy (HCM). Background Impaired perfusion reserve in HCM has been shown to be an independent predictor of adverse cardiovascular outcomes. CMR perfusion imaging currently requires the administration of gadolinium based contrast agents, which are contraindicated in allergy and renal failure, making non-contrast methods such as T1 mapping a safer and more affordable alternative. As adenosine stress T1 mapping has been shown to detect inducible ischaemia in patients with coronary disease, we hypothesised that stress T1 may be used to detect perfusion abnormalities in HCM with reasonable accuracy and T1 reactivity correlates with impaired myocardial blood flow (MBF) reserve and global longitudinal strain (GLS). Method 62 subjects with no known history of coronary disease (31 controls and 31 HCM patients) underwent CMR at 3T including cine imaging, tagging, rest and stress (adenosine 140 mcg/kg/min) blood oxygen level dependent imaging (BOLD), T1 mapping (ShMOLLI), first-pass perfusion imaging and late gadolinium imaging (LGE). Rest and stress T1 values from mid ventricular slice were derived and T1 reactivity determined. 1,2 MBF was estimated using Fermi function de-convolution method as previous described.3,4 Results Baseline characteristics are listed in Table 1. T1 reactivity was significantly reduced in regions of interest (ROI) with LGE and perfusion defects compared to the remaining ‘unaffected’ myocardium (Figure 1). Using a diagnostic threshold of 3.5% on ROC analysis for detecting impaired MBF in HCM, T1 reactivity had a sensitivity and specificity of 82% and 88% respectively (AUC 0.894, p Conclusions Stress T1 mapping is capable of distinguishing regions with perfusion defects versus normal perfusion in HCM without the need of contrast. Stress T1 reactivity, MBF reactivity, oxygenation and GLS were significantly reduced in HCM compared to controls. References . Liu A, Wijesurendra RS, Francis JM, et al. Adenosine Stress and Rest T1 Mapping Can Differentiate Between Ischaemic, Infarcted, Remote, and Normal Myocardium Without the Need for Gadolinium Contrast Agents. JACC Cardiovasc Imaging2016;9(1):27–36. Mahmod M, Piechnik SK, Levelt E, et al. Adenosine stress native T1 mapping in severe aortic stenosis: evidence for a role of the intravascular compartment on myocardial T1 values. J Cardiovasc Magn Reson 2014;16:92. Jerosch-Herold M. Quantification of myocardial perfusion by cardiovascular magnetic resonance. J Cardiov Magn Reson 2010;12(1):57. McCommis KS, Zhang H, Goldstein TA, et al. Myocardial Blood Volume Is Associated with Myocardial Oxygen Consumption: An Experimental Study with CMR in a Canine Model. JACC Cardiovascular imaging 2009;2(11):1313–1320.

Role of T1 Mapping As a Complementary Tool to T2* for Cardiac Iron Overload Assessment

Introduction. Iron overload-related heart failure is the principal cause of death in transfused Thalassemia Major (TM) (Modell B, Cardiovasc Magn Reson 2008;10:42-48). Iron toxicity is dose dependent so a strategy of chelation therapy titration (Kirk P, Circ 2009;120:1961-1968) before the onset of left ventricle (LV) impairment changes outcomes (Alpendurada F, Eur Heart J. 2010; 31:1648-54). The presence of iron in tissue detectably changes the magnetic properties of water, T1, T2 and T2*, as validated against tissue in animal and human models (Carpenter JP, Circ 2011;14:1519-28). T2*, the most used technique, is susceptible to non-iron influence (susceptibility artefact) and has low accuracy for high and low iron levels (Carpenter JP, J Cardiovasc Magn Reson. 2014;12:16-62). T1 mapping could complement T2* as it appears to have superior reproducibility and to detect mild iron missed by T2* (Abdel-Gadir A. J Cardiovasc Magn Reson. 2015;17(Suppl1):P312. Sado DM, J Magn Reson Imaging. 2015;41:1505-11), but studies to date have been small and not using state-of-the-art sequences.Methods. In a prospectively single centre study of 138 TM patients and 32 healthy volunteers (HV) (no known medical conditions, normal CMR scan), we compared T1 mapping (Modifier Lock Locker Inversion sequence - MOLLI - Siemens Works in progress 448B) to the gold-standard dark (DB) and bright (BB) blood T2*, acquired on an Avanto 1.5T (Siemens Healthcare, Erlangen, Germany). For both T2* sequences, a single 10mm mid-ventricular short axis slice was imaged at 8 echo-times (2.58ms to 18.19ms, increment 2.23ms), flip angle=20¡, FOV read/phase=400mm/56,3%. The same slice was used for T1 images (thickness 6mm, distance factor=67%, FOV read/phase=360/75%, TR=740, TE=1.13, with motion correction for the in-line map generation).Results and discussion.All participants provided informed consent.Table1 illustrates patients' and HV's details.T2* was defined normal under the cutpoint value of 20ms. T1 normal range, defined by the HV cohort was 918-1015ms (the 2.5-97.5 quantiles with CI 95%). For DBT2*<20ms, both BBT2* and T1 mapping were broadly indistinguishable from DBT2* (DBT2* vs BBT2* R2=0.95; DBT2* vs T1 R2=0.92; all p<0.001). All subjects with low DBT2* (n=24, 17.4%) had low T1; 52 patients had normal DBT2* but low T1 mapping, i.e. 38% patients were reclassified from normal to iron loaded by T1. The relationship between DBT2* and MOLLI was described by a log-log linear regression (R2=0.80, p<0.001). Upper panel of Fig1 shows T1 vs DBT2* correlation over a 20ms window as the window moves by 1ms at a time on X-axis (so at X-axis point ’n’, the Y-value is the R2 of the correlation of DBT2* vs T1 over the range n-to-n+20ms). As shown by lower panel of Fig1, three domains can be observed: strong relationship in the T2*=0-20ms range (R2=0.92, p<0.001); good relationship in the 21-28ms range, where the curve depicts a plateau (R2=0.80-0.77, p<0.001) and no relationship above 28ms. Given the conservative approach used to set T2* normality as above 20ms (Carpenter JP, Circ 2011;14:1519-28), the evidence that T2* SD values increase even for borderline T2* mean values (~20ms) (Anderson LJ, Eur Heart J. 2001;22:2171-9), and that 39% of normal T2* subjects have a low T1, we support prior suggestions that T1 is detecting mild iron in most of the subjects with DBT2*=20-28ms, missed by T2* as the threshold has had to be set too low for sensitivity reasons (Sado DM, J Magn Reson Imaging. 2015;41:1505-11). T1 mapping is thus a useful complementary tool to T2* both for clinical and research purposes. The reported reproducibilities of T1 square for power calculations and would translate into 6.25 to 49 times more power in studies to detect iron change (Alam MH, J Cardiovasc Magn Reson. 2015;24:17-102). Colour maps make iron instantly visible and add a confirmation step. Whether mild iron missed by T2* is important is unclear. In our cohort, 24-months follow-up was available for 9 patients with normal DBT2* and low T1. Although no statistical consideration is possible due to the small number, in those patients an increase in LV end diastolic volume was observed (from 78±18ml to 84±15ml), suggesting possible cardiotoxicity of even mild amount of iron. Further work is needed, especially in frail cohorts like children starting chelation and around pregnancy. [Display omitted] [Display omitted] DisclosuresMoon:gsk: Consultancy; Genzyme: Research Funding; Shire: Membership on an entity's Board of Directors or advisory committees. Cappellini:Novartis: Membership on an entity's Board of Directors or advisory committees; Genzyme-Sanofi: Membership on an entity's Board of Directors or advisory committees; Celgene: Membership on an entity's Board of Directors or advisory committees.

87 Dilatation of the Thoracic Aorta and Increased Arterial Stiffness is Common in Patients with Giant Cell Arteritis – Preliminary Findings from a Cardiac Magnetic Resonance Study

Background Giant cell arteritis (GCA) is the commonest primary systemic vasculitis affecting older people. Dilatation of the aorta may occur as a late complication and is believed to arise from damage to the aortic wall from inflammation. Objectives To determine the prevalence of thoracic aortic dilatation and assess aortic stiffness by CMR in patients with GCA diagnosed at least 2 years previously. Method Consecutive patients recruited to the UK GCA Consortium study were invited. 49 patients (median disease duration 4.5 years) underwent CMR at 3.0T (Philips Achieva TX). Cine images were acquired to measure the diameter of the ascending aorta (AsAo) and descending aorta (DsAo) at the level of the main pulmonary artery (MPA) and aortic arch from luminal edge-to-edge. Aortic stiffness was assessed by aortic distensibility (AD) and pulse-wave velocity (PWV). For AD, cine images (50 phases) were acquired in a plane transverse to AsAo at the level of MPA. Aortic contours were drawn manually at the times of minimal/maximal distension. For PWV, through-plane phase contrast velocity mapping was performed perpendicular to AsAo/DsAo at the level of MPA. Velocity-time curves were derived and the distance between the two locations measured to calculate PWV using the transit-time method. Results Patient characteristics: Mean age 73 ± 6 years, female gender 35 (71%), biopsy-positive 31 (63%), body surface area (BSA) 1.8 ± 0.2 m 2 , systolic blood pressure (BP) 148 ± 20 mmHg, pulse pressure 75 ± 21 mmHg. CMR measurements in Table 1. 30 (61%) patients had dilated thoracic aortas – corrected to BSA and applied to CMR nomograms [Davis et al . 2014. JCMR 16(1):9]. 5 (10%) patients had dilated AsAo at surgical intervention thresholds according to AHA/ACC guidelines. Aortic stiffness was increased with lower AD (median [IQR] 0.9 [0.8]10–3 mmHg -1 ) and higher PWV (11 ± 3 m/s) than normal ranges Aquaro et al . 1 Disease duration did not correlate with aortic measurements. 10/11 biopsy-negative and 17/31 biopsy-positive patients had dilated aortas. Age, body mass index (BMI) and BP were similar between dilated and non-dilated aorta groups. There was a female to male preponderance in the dilated group (26/35 vs. 4/30 respectively, P Conclusion Dilatation of the thoracic aorta and arterial stiffness are common in patients with GCA. There is female preponderance in dilatation without differences in basic demographics. In biopsy-negative patients, under-treatment and/or variability in phenotype could explain increased aortic dilatation. Further investigation will be required to evaluate the effect of severity, treatment length/type, disease duration and cardiovascular risk factors on aortic morphology and function. Reference Aquaro, et al .Observational study of regional aortic size referenced to body size: productionof a cardiovascular magnetic resonance nomogram Davis et al. J Cardiovasc MagnReson 2014; 16 :9, doi:10.1186/1532-429X-16-9

Pulse pressure affects the relationship between flow-mediated dilatation and cardiovascular risk

[1] Mewton N, Opdahl A, Choi EY, et al. Left ventricular global function index by magnetic resonance imaging—a novel marker for assessment of cardiac performance for the prediction of cardiovascular events: the multi-ethnic study of atherosclerosis. Hypertension 2013;61:770–8. [2] McComb C, Berry C. Prognostic importance of a newmeasure of global systolic heart function in healthy adults. Hypertension 2013;61:762–4. [3] Reinstadler SJ, Klug G, Feistritzer HJ, et al. Association of copeptin with myocardial infarct size and myocardial function after ST-segment elevation myocardial infarction. Heart 2013;99:1525–9. [4] Reinstadler S, Klug G, Feistritzer H, et al. Relation of plasma adiponectin levels and aortic stiffness after acute ST-segment elevation myocardial infarction. Eur Heart J Acute Cardiovasc Care 2014;3(1):10–7. [5] Klug G, Mayr A, Schenk S, et al. Prognostic value at 5 years of microvascular obstruction after acute myocardial infarction assessed by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2012;14:46. [6] Mayr A, Klug G, Schocke M, et al. Late microvascular obstruction after acute myocardial infarction: relation with cardiac and inflammatory markers. Int J Cardiol 2012;157:391–6. [7] Wu AH, Parsons L, Every NR, et al. Hospital outcomes in patients presenting with congestive heart failure complicating acute myocardial infarction: a report from the Second National Registry of Myocardial Infarction (NRMI-2). J Am Coll Cardiol 2002;40:1389–94. [8] Wu E, Ortiz JT, Tejedor P, et al. Infarct size by contrast enhanced cardiac magnetic resonance is a stronger predictor of outcomes than left ventricular ejection fraction or end-systolic volume index: prospective cohort study. Heart 2008;94:730–6. [9] Solomon SD, Anavekar N, Skali H, et al. Influence of ejection fraction on cardiovascular outcomes in a broad spectrum of heart failure patients. Circulation 2005;112:3738–44.

Long-term outcome of transitory “reversible” complete atrio-ventricular block unrelated to myocardial ischemia

fraction: comorbidities drive myocardial dysfunction and remodeling through coronarymicrovascular endothelial inflammation. J AmColl Cardiol 2013;62:263–71. [15] Sekhri V, Sanal S, DeLorenzo LJ, Aronow WS, Maguire GP. Cardiac sarcoidosis: a comprehensive review. Arch Med Sci 2011;7(4):546–54. [16] Hunt SA, Abraham WT, Chin MH, et al. 2009 focused update incorporated into the ACC/AHA 2005 Guidelines for the Diagnosis and Management of Heart Failure in Adults: a report of the American College of Cardiology Foundation/ American Heart Association Task Force on Practice Guidelines: developed in collaboration with the International Society for Heart and Lung Transplantation. Circulation 2009;119(14):e391–479. [17] Baughman RP, Teirstein AS, Judson MA, et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001;164:1885–9. [18] Quarta G, Holdright DR, Plant GT, et al. Cardiovascular magnetic resonance in cardiac sarcoidosis with MR conditional pacemaker in situ. J Cardiovasc Magn Reson 2011;13:26. [19] Fang L, Beale A, Ellims AH, et al. Associations between fibrocytes and postcontrast myocardial T1 times in hypertrophic cardiomyopathy. J Am Heart Assoc 2013;2(5): e000270.

Evaluation of epicardial and intra-myocardial fat in a patient with mitochondrial cardiomyopathy

Mitochondrial cardiomyopathy is one of the main manifestations in patients with primary mitochondrial respiratory chain disease characterized by mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and carries a similar prognosis. The phenotype frequently includes hypertrophic cardiomyopathy; dilated cardiomyopathy and left ventricular (LV) non-compaction are rarely present [ [1] Bates M.G. Bourke J.P. Giordano C. d'Amati G. Turnbull D.M. Taylor R.W. Cardiac involvement in mitochondrial DNA disease: clinical spectrum, diagnosis, and management. Eur Heart J. 2012; 33: 3023-3033 Crossref PubMed Scopus (147) Google Scholar ]. Cardiac magnetic resonance imaging (MRI) with gadolinium-based contrast agents has been widely used for evaluating the morphology and function of the left ventricle and injured myocardial tissue [ [2] Okayama S. Uemura S. Soeda T. et al. Clinical significance of papillary muscle late enhancement detected via cardiac magnetic resonance imaging in patients with single old myocardial infarction. Int J Cardiol. 2011; 146: 73-79 Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar ]. Cine imaging with a standard steady state free precession sequence has been validated for accurate evaluation of epicardial fat [ [3] Nelson A.J. Worthley M.I. Psaltis P.J. et al. Validation of cardiovascular magnetic resonance assessment of pericardial adipose tissue volume. J Cardiovasc Magn Reson. 2009; 5: 15 Crossref Scopus (81) Google Scholar ], which is associated with coronary atherosclerosis and LV systolic dysfunction [ [4] Khawaja T. Greer C. Chokshi A. et al. Epicardial fat volume in patients with left ventricular systolic dysfunction. Am J Cardiol. 2011; 108: 397-401 Abstract Full Text Full Text PDF PubMed Scopus (60) Google Scholar ]. Recent advances in magnetic resonance spectroscopy (MRS) have enabled noninvasive measurement of intra-myocardial triglyceride content [ [5] Nakae I. Mitsunami K. Yoshino T. et al. Clinical features of myocardial triglyceride in different types of cardiomyopathy assessed by proton magnetic resonance spectroscopy: comparison with myocardial creatine. J Card Fail. 2010; 16: 812-822 Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar ], which is increased and associated with LV diastolic dysfunction in patients with diabetes mellitus [ 6 McGavock J.M. Lingvay I. Zib I. et al. Cardiac steatosis in diabetes mellitus: a 1H-magnetic resonance spectroscopy study. Circulation. 2007; 116: 1170-1175 Crossref PubMed Scopus (464) Google Scholar , 7 Rijzewijk L.J. van der Meer R.W. Smit J.W. et al. Myocardial steatosis is an independent predictor of diastolic dysfunction in type 2 diabetes mellitus. J Am Coll Cardiol. 2008; 52: 1793-1799 Abstract Full Text Full Text PDF PubMed Scopus (423) Google Scholar ].

Serum soluble ST2 and interleukin-33 levels in patients with pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare disease characterized by a dramatic, progressive rise of pulmonary vascular resistance (PVR), leading to elevated pulmonary artery pressures, right ventricular (RV) overload, and, eventually, failure. Current evaluation of PAH is based on invasive hemodynamics (RHC), exercise capacity, echocardiography, and N-terminal pro brain natriuretic peptide (NT-proBNP) levels [ [1] Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC) European Respiratory Society (ERS) International Society of Heart and Lung Transplantation (ISHLT) et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009; 34: 1219-1263 Crossref PubMed Scopus (1091) Google Scholar ]; although published survival data support a strong clinical role for cardiac magnetic resonance imaging (CMR) of the RV, this technique is still underused [ [2] Bradlow W.M. Gibbs J.S. Mohiaddin R.H. Cardiovascular magnetic resonance in pulmonary hypertension. J Cardiovasc Magn Reson. 2012; 14: 6 Crossref PubMed Scopus (53) Google Scholar ]. Much research is currently focusing on the exploration of candidate soluble biomarkers of RV dysfunction and prognosis.

Invited Commentary

HomeRadioGraphicsVol. 32, No. 7 PreviousNext Cardiac ImagingInvited CommentaryLeena MammenLeena MammenAuthor AffiliationsMichigan State College of Human Medicine, Advanced Radiology Services Grand Rapids, MichiganLeena MammenPublished Online:Nov 2 2012https://doi.org/10.1148/Radiographics.32.7.125145MoreSectionsFull textPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In References1 Haghi D, Papavassiliu T, Kalmar Get al.. A hybrid approach for quantification of aortic valve stenosis using cardiac magnetic resonance imaging and echocardiography. J Cardiovasc Magn Reson 2005;7(3):581–586. Crossref, Medline, Google Scholar2 Vogel-Claussen J, Pannu H, Spevak PJ, Fishman EK, Bluemke DA. Cardiac valve assessment with MR imaging and 64-section multi-detector row CT. RadioGraphics 2006;26(6):1769–1784. Link, Google Scholar3 Zoghbi WA, Chambers JB, Dumesnil JGet al.. Recommendations for evaluation of prosthetic valves with echocardiography and Doppler ultrasound: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Task Force on Prosthetic Valves, developed in conjunction with the American College of Cardiology Cardiovascular Imaging Committee, Cardiac Imaging Committee of the American Heart Association, the European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography and the Canadian Society of Echocardiography, endorsed by the American College of Cardiology Foundation, American Heart Association, European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography, and Canadian Society of Echocardiography. J Am Soc Echocardiogr 2009;22(9):975–1014. Crossref, Medline, Google Scholar4 Zoghbi WA. New recommendations for evaluation of prosthetic valves with echocardiography and Doppler ultrasound. Methodist Debakey Cardiovasc J 2010;6(1):20–26. Crossref, Medline, Google ScholarArticle HistoryPublished online: Nov 2 2012Published in print: Nov 2012 FiguresReferencesRelatedDetailsRecommended Articles Imaging Spectrum of Valvular and Paravalvular Complications of Prosthetic Heart Valve at CT AngiographyRadiology: Cardiothoracic Imaging2021Volume: 3Issue: 4Multimodality Imaging of Complications of Cardiac Valve SurgeriesRadioGraphics2019Volume: 39Issue: 4pp. 932-956Role of Cardiac CT in Infective Endocarditis: Current Evidence, Opportunities, and ChallengesRadiology: Cardiothoracic Imaging2021Volume: 3Issue: 1Pre- and Postprocedure Imaging of Transcatheter Pulmonary Valve ImplantationRadioGraphics2022Volume: 42Issue: 4pp. 991-1011Transcatheter Mitral Valve Repair and Replacement: Current Evidence for Intervention and the Role of CT in Preprocedural Planning—A Review for Radiologists and Cardiologists AlikeRadiology: Cardiothoracic Imaging2020Volume: 2Issue: 1See More RSNA Education Exhibits Imaging of Transcather Aortic Valve Replacement Complications: A Pictorial ReviewDigital Posters2020Optimizing CT Protocols for Adult Congenital Heart Disease: A Primer for RadiologistsDigital Posters2019Multi-Modality Imaging of Congenital Semilunar Valvular and Perivalvular DiseaseDigital Posters2019 RSNA Case Collection Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA)RSNA Case Collection2020Pulmonary AtresiaRSNA Case Collection2022Papillary fibroelastomaRSNA Case Collection2020 Vol. 32, No. 7 Metrics Altmetric Score PDF download

Bicuspid aortic valve configuration and aortopathy pattern might represent different pathophysiologic substrates

The interindividual variability of pathologic bicuspid aortic valve (BAV) configuration is increasingly recognized. The 2007 classification system of Sievers and Schmidtke 1 describes this diversity of BAV cusp and commissure morphologic characteristics in an unambiguous fashion, thereby facilitating clear communication regarding pathologic BAV cusp configuration. There are numerous associated cardiovascular malformations, but the most common associated problems are ascending aortic and arch dilation. Four different clusters of BAV-associated aortopathy were identified by cluster analysis of computed tomographic or magnetic resonance thoracic aortic images and introduced by Fazel and associates 2 in 2008. The relationships between valvular configuration and pattern of BAV aortopathy, however, were unclear. Valve configuration and aortopathy pattern might be linked to hemodynamic conditions resulting in different distribution and extent of shear and/or stretch along the anterolateral aspect of the ascending aortic wall. Alternatively, there could be phenotypic variability related to specific gene mutations leading to various BAV cusp configuration types being associated with specific aortopathy patterns. Fazel and associates 2 did not identify an association between type of valve configuration and aortopathy pattern in 64 patients with a BAV. We updated their assessment using 127 BAV patients according to the Sievers BAV type and Stanford Fazel BAV aortopathy clusters (I-IV). The Sievers BAV type was determined intraoperatively, and aortopathy pattern was based on assessment of gated, thin-slice computed tomographic imaging with 3-dimensional reconstruction of the aortic root, tubular ascending aorta, and aortic arch by 2 independent observers. Inasmuch as both valve configuration and Fazel cluster data (Figure 1) failed normality tests (KolmogorovSmirnov, Shapiro-Wilk, P .05). When patients were assigned to different groups according to Sievers valve type, analysis of variance on ranks test showed no significant differences in aortopathy clusters (P ¼ .543). Comparison of groups according to Fazel aortopathy cluster using the same test revealed no significant difference when tested for Sievers cusp type (P ¼ .111). Subjectively, a distinctive fifth type of aortic morphologic disease not captured completely by our earlier quantitative, objective cluster analysis was noted frequently by both observers. This cluster included enlargement of the aortic root (sinuses of Valsalva) and the tubular ascending aortic segment (combination of Fazel clusters I and II). We describe this finding as cluster ‘‘Ia,’’ with preserved sinotubular junction and normal tubular ascending aortic segment (formerly, Fazel cluster I), and cluster ‘‘Ib,’’ in which the sinotubular junction is effaced and the tubularascending aorta is dilated (Figure 1). This impression requires validation in larger series of BAV patients. It should be noted that patientswithisolatedsingle-sinusaneurysmwithorwithout ascending aortic aneurysm are currently not captured by any of the Fazel clusters. Future studies should focus on this specific, interesting patient substrate. Debate continues about the impact of genetically determined intrinsic disease of the aortic wall, as opposed to altered hemodynamics increasing shear and stretch on the aortic wall, leading to progression of aortopathy. Hypothetically, hemodynamic sequelae of different BAV types might lead to different levels of aortic wall strain in different aortic regions. A significant association between Sievers type 1/L- R (‘‘majority type’’) cusp configuration and more severe degree of wall degeneration in the ascending aorta compared with Sievers 1/R-N (‘‘minority type’’) and 1/N-L valves has been described. 3 Valve function might be of importance, although not tested in detail in this study.

Quantitative assessment of right ventricular function in pectus excavatum

Pectus excavatum is a common congenital chest wall deformity. Underlying cardiac chambers, in particular the right atrium and right ventricle (RV), may be compressed between the vertebral column and the depressed sternum in patients with this thoracic deformity. Citing improvement in indices of cardiovascular function after corrective surgery, some investigators have suggested that the deformed chest may contribute to cardiopulmonary impairment. Although cardiopulmonary impairment certainly may contribute to symptoms in patients with pectus excavatum and guide the need for and timing of corrective surgery, guidelines for the assessment of resting cardiovascular function remain undefined. Furthermore, the qualitative echocardiographic assessment of RV function and volumes among patients with pectus excavatum may be technically difficult and limited by subjectivity. Quantitative echocardiographic parameters, such as tricuspid annular plane systolic excursion (TAPSE) and RV fractional area change, may offer a more accurate assessment of RV function in this anatomically distinct population. Cardiac magnetic resonance (CMR) imaging has emerged as the criterion standard in RV functional assessment and, more recently, has been validated in patients with pectus excavatum. We compared qualitative assessment of RV systolic impairment by visual echocardiographic inspection with quantitative assessment of RV function with CMR and echocardiographically derived TAPSE and RV fractional area change in a series of patients referred to our institution with mild to severe symptomatic pectus excavatum. Patients were being considered for corrective surgery. Our hypothesis was that the patients would be found to have normal RV function when assessed quantitatively, either through CMR or quantitative echocardiographic examination, despite impairment seen qualitatively.

Gadolinium-enhanced cardiovascular magnetic resonance for the detection and characterization of Loeffler endocarditis in patients with hypereosinophilic syndrome

Hypereosinophilic syndrome (HES) is a rare disorder characterized by a marked and persistent peripheral blood eosinophilia combined with organ-system dysfunction caused by eosinophil-associated tissue damage [ 1 Chusid M.J. Dale D.C. West B.C. Wolff S.M. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975; 54: 1-27 Crossref PubMed Scopus (1183) Google Scholar , 2 Klion A.D. Bochner B.S. Gleich G.J. et al. The Hypereosinophilic Syndromes Working G. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol. 2006; 117 (Epub 2006 May 3.): 1292-1302 Abstract Full Text Full Text PDF PubMed Scopus (303) Google Scholar ]. The clinical manifestations of HES are variable and depend on the target-organ affection by eosinophils. Cardiac involvement, originally described by Loeffler [ [3] Löffler W. Endocarditis parietalis fibroplastica mit Bluteosinophilie. Ein eigenartiges Krankheitsbild. Schweiz Med Wochenschr. 1936; 66: 817-820 Google Scholar ], is frequent and cardiac dysfunction is the major cause of morbidity and mortality in HES-patients [ [4] Weller P.F. Bubley G.J. The idiopathic hypereosinophilic syndrome. Blood. 1994; 83: 2759-2779 PubMed Google Scholar ]. The cardiac pathology of HES has been temporally divided into three stages: early acute necrotic, then thrombotic, and finally a late fibrotic stage [ [4] Weller P.F. Bubley G.J. The idiopathic hypereosinophilic syndrome. Blood. 1994; 83: 2759-2779 PubMed Google Scholar ]. The diagnosis of eosinophilic cardiac disease is based on the co-existence of endomyocardial eosinophils on myocardial biopsy and cardiovascular abnormalities for which other possible causes have been excluded. However, biopsy is invasive and most standard non-invasive diagnostic tools such as echocardiography are often unsatisfactory in detecting cardiac involvement. Contrast-enhanced cardiovascular magnetic resonance imaging (CE-CMR) can characterize different myocardial tissue abnormalities by typical patterns of late gadolinium enhancement (LGE), including necrosis, inflammation, and fibrosis [ [5] Pennell D.J. Cardiovascular magnetic resonance. Circulation. 2010; 121: 692-705 Crossref PubMed Scopus (211) Google Scholar ]. Thus, CE-CMR is an attractive technique to directly visualize the various cardiac pathologies that occur during the different stages of Loeffler endocarditis. So far, the usage of CMR in diagnosing HES associated heart disease has been described only in single case reports [ 6 Puvaneswary M. Joshua F. Ratnarajah S. Idiopathic hypereosinophilic syndrome: magnetic resonance imaging findings in endomyocardial fibrosis. Australas Radiol. 2001; 45: 524-527 Crossref PubMed Scopus (25) Google Scholar , 7 Syed I.S. Martinez M.W. Feng D.L. Glockner J.F. Cardiac magnetic resonance imaging of eosinophilic endomyocardial disease. Int J Cardiol. 2008; 126 (Epub 2007): e50-e52 Abstract Full Text Full Text PDF PubMed Scopus (63) Google Scholar , 8 Cheung S.C. Chan C.W. Insights of prognostication of Davies disease: what could we learn from serial magnetic resonance imaging studies?. Int J Cardiol. 2009; 12: 12 Google Scholar , 9 Chang S.A. Kim H.K. Park E.A. Kim Y.J. Sohn D.W. Images in cardiovascular medicine. Loeffler endocarditis mimicking apical hypertrophic cardiomyopathy. Circulation. 2009; 120: 82-85 Crossref PubMed Scopus (13) Google Scholar , 10 Deb K. Djavidani B. Buchner S. et al. Time course of eosinophilic myocarditis visualized by CMR. J Cardiovasc Magn Reson. 2008; 10: 21 Crossref PubMed Scopus (55) Google Scholar ] but no systematic evaluation has been reported. The purpose of this study was to evaluate the presence of cardiac involvement in patients with HES using CMR.

Cardiac Contusion in a Professional Soccer Player

We report here the case of a 30-year-old professional soccer player who presented to our hospital after sustaining a blunt chest trauma. To the best of our knowledge, this is the first reported case of cardiac contusion when acute myocardial edema and subsequent chronic myocardial injury were visualized with cardiac magnetic resonance (CMR) imaging. A professional soccer player presented to our hospital with persistent chest pain and fatigue after being hit in the chest by a soccer ball during a national league game. The hospital admission was 10 hours after the accident and onset of symptoms. The patient had no medical history and no recent history of infectious disease. The initial workup revealed elevated cardiac troponin T of 0.576 μg/L (normal range, 0.00 to 0.03 μg/L) and creatine kinase-MB levels of 25.4 U/L (normal range, 0 to 25 U/L); the markers of inflammation proved to be negative (C-reactive protein level, 0.9 mg/L [normal range, 0 to 5.0 mg/L]; white blood cell count, 7.0×103/μL [normal range, 4 to 10×103/μL]). The patient underwent a performance-enhancing drug screening 8 days before the event as part of the regular doping control protocol of professional soccer players, which proved to be negative. Furthermore, the toxicology panel testing for 7 drugs (phencyclidine, barbiturates, cannabinoids, amphetamines, benzodiazepines, …

Utility of Real-Time 3-Dimensional Echocardiography and Magnetic Resonance Imaging for Evaluation of Danon Disease

A 21-year-old man was admitted to the hospital with exertional dyspnea. At the age of 14, he was diagnosed with Danon disease by genetic analysis (a 2–base-pair deletion at positions 288 and 289 in exon 3 was identified in the lysosome-associated membrane protein-2 [LAMP2] gene of the patient, which led to a frameshift and resulted in a premature stop codon). A chest radiograph demonstrated moderate cardiomegaly (cardiothoracic ratio of 58%). The ECG exhibited normal sinus rhythm, wide and bifid P waves with a duration of 170 ms, complete left bundle-branch block with a QRS duration of 200 ms, and a leftward axis (−2°; Figure 1). Figure 1. Twelve-lead ECGs. Echocardiography revealed left ventricular (LV) dilatation with an end-diastolic internal dimension of 60 mm and diffusely hypokinetic LV wall motion with fractional shortening of 8% (Figure 2; Movie I of the online-only Data Supplement). …