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Dudley J Pennell

Publications and source records attributed to Dudley J Pennell.

At least 19 recordsLinked to original sources

Pangenomes aid accurate detection of large insertions and deletions from targeted sequencing: the case of cardiomyopathies.

BACKGROUND: Gene panels represent a widely used strategy for genetic testing in a vast range of Mendelian disorders. While this approach aids reliable bioinformatic detection of short coding variants, it often fails to detect many larger variants. Recent studies have recommended the adoption of pangenome references (as opposed to linear reference genomes like GRCh38) to augment detection of large variants from targeted sequencing, potentially providing diagnostic laboratories with the possibility to streamline diagnostic work-ups and reduce costs. METHODS: Here, we analyze 1969 cardiomyopathy cases and 1805 controls sequenced with the Illumina Trusight Cardio panel using a pangenome-based workflow (GRAF) and five conventional orthogonal methodologies (GATK HaplotypeCaller, GATK-gCNV, ExomeDepth, Manta and Lumpy-SV) to detect variants ≥ 20 bp in size. RESULTS: Following lab-based variant validation by means of PCR and Sanger sequencing, we show that GRAF conjugates higher precision and recall (F1 score 0.86) compared with other methods (F1 0-0.57) in detecting potentially pathogenic variants ≥ 20 bp from short-read panel data. Results were complemented by a comparison of the tools' performance in detecting ground truth variants on reference sample HG002 from Genome In A Bottle, which confirmed GRAF to outperform other tools also on exome sequencing (F1 0.97 vs. 0-0.94). Notably, in the HG002 benchmark dataset, GRAF also showed slightly improved performance compared to GATK HaplotypeCaller in the identification of small variants (1-19 bp; F1 0.975 vs. 0.968). CONCLUSIONS: Our results indicate that pangenome-based workflows aid improved detection of large variants from targeted sequencing data in the clinical context and suggest that they may contribute to more unified variant detection frameworks for all-size genetic variants in the future.

Humans↗

Reference right ventricular systolic and diastolic function normalized to age, gender and body surface area from steady-state free precession cardiovascular magnetic resonance.

AIMS: Recent advances in cardiovascular magnetic resonance (CMR) include improved image quality with steady-state free precession (SSFP) sequences and advanced post-processing of high temporal resolution ventricular function. We used these techniques to establish the reference values for right ventricular (RV) volumes and function. METHODS AND RESULTS: We studied 120 healthy subjects (60 men, 60 women; from 20 to 80 years) after exclusion of cardiovascular abnormality. Data were generated from SSFP cines, with three-dimensional modelling. Gender, body surface area (BSA), and age were independent predictors of several RV parameters. Normalized RV mass (RVM) and absolute and normalized RV volumes decreased significantly with age, whereas ejection fraction increased. For diastolic variables, absolute and normalized early peak filling rate (PFR(E)) decreased and absolute and normalized active peak filling rate (PFR(A)) in males increased, with decreased PFR(E)/PFR(A). Increasing BSA was associated with increased RVM, volumes, and PFR(E). Gender significantly influenced absolute and normalized mass and volumes, and absolute and normalized PFR(A). CONCLUSION: These data using state-of-the-art CMR show that normal values of RV systolic and diastolic parameters vary significantly by gender, BSA, and age. Appropriate reference ranges normalized to all three variables should be used in the determination of normality or severity of abnormality of RV dimensions and function.

Adult↗

The association of left ventricular mass with blood pressure, cigarette smoking and alcohol consumption; data from the LARGE Heart study.

BACKGROUND: Left ventricular mass is a risk factor for cardiovascular morbidity and mortality. Although factors associated with elevated left ventricular mass have been sought and studied extensively in elderly and in diseased subjects, few studies have examined the young and healthy. The aim of this study was to examine the possible influence of lifestyle on left ventricular mass in a large group of young men. METHODS: Left ventricular mass was assessed using cardiovascular magnetic resonance in 541 healthy Caucasian male army recruits. Anthropometric, lifestyle and blood pressure data were collected. RESULTS: Mean unadjusted left ventricular mass and left ventricular mass indexed to body surface area were 163.8+/-24.9 g and 86.6+/-10.2 g m(-2) respectively. In univariate analysis, age, height, weight, alcohol consumption, systolic blood pressure, diastolic blood pressure and indices of physical activity were positively associated with unadjusted left ventricular mass (all P<0.02). By contrast, smoking was associated with lower mean left ventricular mass; never smoked 167.5+/-25.8 g vs ex-smokers 159.1+/-25.2 g vs current smokers 161.0+/-23.1 g (P=0.007). Multivariate analysis revealed weight, systolic blood pressure, smoking status and indices of physical activity to be independent predictors of left ventricular mass. CONCLUSIONS: Our data confirm an association of age, body weight, height, physical activity, diastolic and systolic blood pressure with left ventricular mass. In addition, unexpectedly, we have found smoking is associated with lower left ventricular mass in a large sample of young healthy men. Although the latter association may result from confounding effects, such an interesting observation deserves further investigation.

Adolescent↗

Cardiovascular magnetic resonance in arrhythmogenic right ventricular cardiomyopathy revisited: comparison with task force criteria and genotype.

OBJECTIVES: We sought to assess the utility of cardiovascular magnetic resonance (CMR) in the evaluation of arrhythmogenic right ventricular cardiomyopathy (ARVC) in relation to diagnostic criteria and genotype. BACKGROUND: Timely diagnosis of ARVC is difficult as clinical findings may be subtle and nonspecific in early disease. The role of CMR is controversial owing to the absence of a standardized protocol, insufficient experience with the modality, and inherent difficulties in imaging the right ventricle. METHODS: Comprehensive CMR examination was performed in 232 patients undergoing evaluation for suspected ARVC. CMR outcomes were compared with: 1) prospective clinical diagnosis using Task Force guidelines, with and without the proposed modifications for familial ARVC; and 2) gene-carrier status in 35 individuals from genotyped families. RESULTS: CMR studies were positive in all 64 patients who prospectively fulfilled Task Force criteria, resulting in 100% sensitivity. Specificity in relation to Task Force criteria was low (29%). Of the 119 apparent false positives detected by CMR, however, 63 fulfilled modified diagnostic criteria for familial ARVC and 7 were obligate gene carriers, suggesting that CMR frequently identifies individuals with early disease, in whom Task Force criteria are relatively insensitive. This was borne out by evaluation of genotyped individuals (26 gene-positive and 9 gene-negative), in whom CMR had a sensitivity of 96% and a specificity of 78%. CONCLUSIONS: CMR is a valuable component of the diagnostic workup for ARVC when performed with a dedicated protocol by specialists with experience in analysis of volumes, right ventricular wall motion, and delayed-enhancement imaging.

Adolescent↗

Cardiovascular magnetic resonance, fibrosis, and prognosis in dilated cardiomyopathy.

OBJECTIVES: We studied the prognostic implications of midwall fibrosis in dilated cardiomyopathy (DCM) in a prospective longitudinal study. BACKGROUND: Risk stratification of patients with nonischemic DCM in the era of device implantation is problematic. Approximately 30% of patients with DCM have midwall fibrosis as detected by late gadolinium-enhancement (LGE) cardiovascular magnetic resonance (CMR), which may increase susceptibility to arrhythmia and progression of heart failure. METHODS: Consecutive DCM patients (n = 101) with the presence or absence of midwall fibrosis were followed up prospectively for 658 +/- 355 days for events. RESULTS: Midwall fibrosis was present in 35% of patients and was associated with a higher rate of the predefined primary combined end point of all-cause death and hospitalization for a cardiovascular event (hazard ratio 3.4, p = 0.01). Multivariate analysis showed midwall fibrosis as the sole significant predictor of death or hospitalization. However, there was no significant difference in all-cause mortality between the 2 groups. Midwall fibrosis also predicted secondary outcome measures of sudden cardiac death (SCD) or ventricular tachycardia (VT) (hazard ratio 5.2, p = 0.03). Midwall fibrosis remained predictive of SCD/VT after correction for baseline differences in left ventricular ejection fraction between the 2 groups. CONCLUSIONS: In DCM, midwall fibrosis determined by CMR is a predictor of the combined end point of all-cause mortality and cardiovascular hospitalization, which is independent of ventricular remodeling. In addition, midwall fibrosis by CMR predicts SCD/VT. This suggests a potential role for CMR in the risk stratification of patients with DCM, which may have value in determining the need for device therapy.

Adult↗

Comparison between myocardial contrast echocardiography and single-photon emission computed tomography for predicting transmurality of acute myocardial infarction.

Contrast-enhanced cardiovascular magnetic resonance imaging (CMR) has been shown to accurately assess transmural extent of infarction, which is an excellent predictor of long-term improvement in contractile function. We assessed the relative accuracy of myocardial contrast echocardiography (MCE) and single-photon emission computed tomography (SPECT) to predict transmural extent of infarction after acute myocardial infarction. MCE, SPECT, and CMR were performed in 40 patients with acute myocardial infarction 7 to 10 days after thrombolysis. CMR was used to divide the transmural extent of infarction into 5 groups: 0%, 1% to 25%, 26% to 50%, 51% to 75%, and 76% to 100% in dysfunctional segments. MCE and SPECT were compared with assessment grades of transmural extent of infarction. There was a significant relation (p<0.0001) between decreasing contrast intensity as assessed qualitatively by MCE and increasing transmural extent of infarction on CMR as was the case for SPECT. The accuracy of MCE (77%) to predict>50% transmural extent of infarction (nonviable myocardium) was significantly (p=0.02) superior to that of SPECT (70%). Absence of uptake on MCE and SPECT virtually ruled out 25% transmural extent of infarction (84% vs 76%, p=0.03). MCE and SPECT correlate well with the transmural extent of infarction. However, MCE is significantly more accurate in predicting >50% of the transmural extent of infarction and more sensitive in identifying <or=25% of the transmural extent of infarction than SPECT.

Cohort Studies↗

Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of fallot and its relationship to adverse markers of clinical outcome.

BACKGROUND: Late morbidity and mortality remain problematic after repair of tetralogy of Fallot (TOF). We hypothesized that fibrosis detected by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) would be present in adults with repaired TOF and would be related to adverse markers of outcome. METHOD AND RESULTS: LGE was scored in the right and left ventricles (RV and LV) of 92 adult patients who had undergone TOF repair. RV LGE was seen in all patients at surgical sites located in the outflow tract (99%) or the site of ventricular septal defect patching (98%) and in the inferior RV insertion point (79%) and trabeculated myocardium (24%). LV LGE (53%) was located at the apex consistent with apical vent insertion (49%), in the inferior or lateral wall consistent with infarction (5%), or in other areas (8%). Patients with supramedian RV LGE score were older (38 versus 27 years, P<0.001) and more symptomatic (38% versus 8% in New York Heart Association class II or greater, P=0.001), had increased levels of atrial natriuretic peptide (7.3 versus 4.9 pmol/L, P=0.041), and had a trend to higher brain natriuretic peptide (12.3 versus 7.2 pmol/L, P=0.086), exercise intolerance (maximum VO2 24 versus 28 mL.min(-1).kg(-1), P=0.021), RV dysfunction (RV end-systolic volume 61 versus 55 mL/m2, P=0.018; RV ejection fraction 50% versus 56%, P=0.007), and clinical arrhythmia (26% versus 10%, P=0.039). Non-apical vent LV LGE also correlated with markers of adverse outcome. In a multivariate model, RV LGE remained a predictor of arrhythmia. CONCLUSIONS: RV and LV LGE were common after TOF repair and were related to adverse clinical markers, including ventricular dysfunction, exercise intolerance, and neurohormonal activation. Furthermore, RV LGE was significantly associated with clinical arrhythmia.

Adolescent↗

Variation in the lipoprotein lipase gene influences exercise-induced left ventricular growth.

The adult heart relies predominantly on fatty acids (FA) for energy generation, and defects in FA catabolism cause dramatic left ventricular (LV) growth in early age. Since lipoprotein lipase (LPL) is the key enzyme in plasma triglyceride catabolism and is highly expressed in the myocardium, we investigated an association between the functional LPL gene serine 447 stop (S447X) variant and exercise-induced LV growth. The S447X variant was genotyped in 146 British Army recruits undergoing a 10-week exercise programme. Over the training period, X447 allele carriers showed less LV growth than S447 homozygotes (SS, 5.8+/-0.7%; SX, 2.2+/-1.5%; P=0.03) and a decrease in systolic blood pressure (DeltaSBP: SS, 1.9+/-1.3 mmHg; SX, -5.7+/-2.2 mmHg; P=0.015). Although LPL genotype did not significantly predict LV growth with DeltaSBP in statistical modelling (LPL, P=0.14; DeltaSBP, P=0.06), regression analysis indicated that LPL S447X genotype effect on DeltaSBP accounted for only 20% of the effect on LV growth. In multivariate analysis, LPL, peroxisome-proliferator-activated receptor alpha and angiotensin-converting enzyme genotypes were independent predictors of cardiac growth. Thus, LPL S447X genotype influenced exercise-induced changes in LV mass and SBP. Change in blood pressure accounted for a proportion of LV growth. These data suggest that increased myocardial FA availability may reduce exercise-induced LV growth.

Adult↗

Development of a novel optimized breathhold technique for myocardial T2 measurement in thalassemia.

PURPOSE: To develop a reproducible fast spin-echo (FSE) technique for accurate myocardial T2 measurement with application to iron overload assessment in thalassemia. MATERIALS AND METHODS: An FSE sequence was developed to permit acquisition of multiple TE images in one breathhold (BH-FSE). A dynamic black-blood scheme was introduced to better cancel blood signal. A nonselective refocusing train was also adopted to suppress stimulated echoes. The optimized technique was tested on phantoms and then applied to 10 normal volunteers and 10 thalassemia patients. Interstudy reproducibility was measured on all the 20 subjects. RESULTS: The mean difference in T2 values was 1.7% from phantom experiments between BH-FSE and the conventional spin-echo (SE) technique. High contrast BH-FSE images were acquired from human subjects, with minimal stimulated echoes and effective blood suppression (P = 0.0005). The coefficient of variation for interstudy reproducibility was 4.3%. T2 values from thalassemia patients were substantially lower than those from the normal subjects (45.2 +/- 26.1 msec vs. 56.9 +/- 8.4 ms, P = 0.02). CONCLUSION: The dynamic black-blood T2 sequence is a fast reproducible acquisition that compares favorably with conventional techniques, is robust to motion artifacts, and yields high blood-myocardium contrast. This technique may provide a useful tool in thalassemia and other scenarios requiring myocardial T2 quantification.

Adolescent↗

Evaluation of carotid artery wall volume measurement using novel semiautomated analysis software.

PURPOSE: To evaluate semiautomated analysis software for measuring the total carotid arterial wall volume (TWV) as a measure of atheroma burden. MATERIALS AND METHODS: Semiautomated-software and manual analyses of TWV measured by cardiovascular magnetic resonance (CMR) were compared in two phantom models, 10 subjects with no known carotid artery disease, and eight subjects with known carotid disease. The subjects were scanned twice for reproducibility. RESULTS: In subjects with no known carotid disease, semiautomated analysis of 98% of slices showed an improved interstudy coefficient of variation (COV) compared to manual analysis of 50% of slices (4.0% vs. 6.2%, P = 0.02). The proportion of matched cross-sectional slices usable for TWV measurement was superior (99% vs. 49%, P = 0.005) and the median analysis time was shorter (31 minutes vs. 90 minutes, P < 0.001) using the semiautomated software. In subjects with known carotid disease, semiautomated (99% of slices) and manual (56% of slices) analyses had comparable interstudy COVs (4.1% vs. 3.9%, P = 0.01). However, the proportion of matched cross-sectional slices usable for TWV measurement was greater using semiautomated contouring (96% vs. 56%, P = 0.01). CONCLUSION: Carotid CMR measurement of TWV using novel semiautomated analysis software shows good reproducibility, enables greater coverage of arterial vessel wall length, and is considerably faster compared to manual contouring.

Adult↗

Contrast echocardiography versus gated single photon emission computed tomography for the assessment of parameters of left ventricular remodeling after acute myocardial infarction.

BACKGROUND: Assessment of parameters of left ventricular (LV) remodeling after acute myocardial infarction (AMI) has both therapeutic and prognostic implication. Contrast echocardiography (CE) has the advantage of simultaneously assessing myocardial perfusion and LV remodeling. We aimed to evaluate the accuracy of CE to assess LV remodeling after AMI compared with technetium-99m sestamibi gated single photon emission computed tomography (SPECT). METHODS: Accordingly, 36 consecutive patients underwent gated SPECT, CE, and cardiovascular magnetic resonance imaging (CMR) 7 to 10 days after AMI. LV ejection fraction (LVEF), and LV end-systolic and end-diastolic volumes were assessed. RESULTS: Absolute differences for LVEF and LV end-diastolic volume between CMR and CE were significantly smaller than that between CMR and SPECT. CE estimate of LVEF more accurately classified patients into LVEF less than 35%, 35% to 45%, and greater than 45% (agreement = 83%, kappa = 0.66 with CMR) compared with SPECT (agreement = 61%, kappa = 0.36 with CMR). CONCLUSION: CE is more accurate than gated SPECT for the estimation of LV remodeling after AMI.

Echocardiography↗

The histological basis of late gadolinium enhancement cardiovascular magnetic resonance in a patient with Anderson-Fabry disease.

Anderson-Fabry Disease (AFD) is a storage disease that mimics hypertrophic cardiomyopathy. Late gadolinium enhancement (LGE) by cardiovascular magnetic resonance occurs in approximately 50% of patients in the basal inferolateral LV wall, but how an intracellular storage disease causes focal LGE is unknown. We present a whole-heart histological validation that LGE is caused by focal myocardial collagen scarring. This scarring may be the substrate for electrical re-entry and sudden arrhythmic death. The reasons for this distribution of fibrosis are unclear, but may reflect inhomogeneous left ventricular wall stress.

Analysis of Variance↗

Primary myocardial dysfunction in autosomal dominant EDMD. A tissue doppler and cardiovascular magnetic resonance study.

BACKGROUND: Emery-Dreifuss muscular dystrophy is a genetically heterogeneous form of muscular dystrophy. One form is inherited in an X-linked fashion and is secondary to mutations in the gene encoding the nuclear protein emerin. A more common variant is inherited in an autosomal dominant way (EDMD2) due to mutations affecting the nuclear lamina protein lamin A/C. Typical features of both conditions are relatively mild skeletal muscle weakness, but cardiac involvement develops almost invariably by adult age, including conduction defects, arrhythmias and cardiomyopathy. Thus, early detection of cardiac abnormalities may be important for planning early therapeutic intervention. AIM: In this study, we hypothesized that early myocardial dysfunction can be detected by tissue Doppler echocardiography and CMR in unselected patients with the autosomal dominant form of Emery-Dreifuss muscular dystrophy. This would suggest that fibrosis could be implicated in the pathogenesis of cardiac dysfunction in EDMD2. METHODS: Eight consecutive patients with genetically proven EDMD2 without pacemakers were enrolled in the study and compared to eight age-matched controls. All patients and controls first underwent a comprehensive echocardiographic-Doppler examination, followed by measurement of mitral annular velocities using pulsed tissue Doppler. Color M-mode tissue images were recorded from the parasternal long axis projections to derive Myocardial Velocity Gradients (MVG). Subsequently, all subjects underwent cardiovascular magnetic resonance (CMR) imaging for function, intrinsic myocardial tissue contrast using T1 and T2 weighted spin echo (TSE) for fat deposition and extrinsic contrast (Gadolinium-DTPA late fibrosis imaging). Strain measurements, using harmonic phase imaging (HARP) tagging were also derived. RESULTS: Cavity dimensions LV mass and fractional shortening were similar between patients and controls. The overall body mass index was less in patients than in controls (14.5 +/- 1.4 vs. 18.1 +/- 2.4 g/m2, p < 0.002). While systolic MVG were similar between groups, the early diastolic MVG was lower in patients than in controls (4 +/- 1.2 s-1 vs. 7.1 +/- 2.7, p < 0.02). On CMR, LA and LV, RV volumes were similar between patients and controls. CMR strain patterns, however, showed a significant reduction in inferior wall contractility in patients compared to controls (-0.06 +/- 0.02 vs -0.09 +/- 0.03, p < 0.05). No patient showed late gadolinium enhancement. CONCLUSION: Patients with EDMD2 have abnormal left ventricular function prior to developing any cardiac symptoms. The absence of myocardial fibrosis, however, by CMR suggests that this functional abnormality may not be secondary to scarring but could precede it. Tissue Doppler echocardiography and CMR are sensitive methods of assessing the presence of myocardial dysfunction prior to the development of any cardiovascular symptoms.

Adolescent↗

Severe endothelial dysfunction in young women with polycystic ovary syndrome is only partially explained by known cardiovascular risk factors.

OBJECTIVE: We aimed to assess whether metabolic abnormalities can explain endothelial dysfunction and associated cardiovascular disease risk (CVDr) in polycystic ovary syndrome (PCOS). Endothelial function, a recognized composite marker of CVDr, may be reduced in PCOS and can be precisely and noninvasively assessed by cardiovascular magnetic resonance (CMR). PATIENTS: Fourteen women with anovulatory PCOS (age [mean +/- SD] 33 +/- 4 years) and 13 controls (age: 33 +/- 6 years) with similar body mass index and regular menses. METHODS: Endothelium-dependent (flow-mediated dilatation - FMD) and -independent (glyceryl trinitrate - GTN) changes in the brachial artery area were measured using CMR in women with PCOS and controls. Arterial function was assessed twice, in the early follicular phase and mid cycle in controls and after an interval of 2 weeks in PCOS subjects. Fasting lipids, glucose, insulin and sex hormones were measured at the first visit. RESULTS: FMD was greatly reduced in women with PCOS compared to controls (-1%vs 5% and 2%vs 12%, P < 0.01) without differences in GTN responses. Risk factors were more prevalent in PCOS women and displayed significant linear relationships with FMD. PCOS status was the strongest predictor of FMD. Linear regression between PCOS and FMD remained significant after correction for all CVD risk markers linked to the metabolic syndrome. CONCLUSION: PCOS is associated with changes in CVD risk markers and pronounced endothelial dysfunction. However endothelial dysfunction with PCOS is only partly explained by recognized CVD risk markers.

Adult↗

Development of thalassaemic iron overload cardiomyopathy despite low liver iron levels and meticulous compliance to desferrioxamine.

It is believed that myocardial iron deposition and the resultant cardiomyopathy only occur in the presence of severe liver iron overload. Using cardiovascular magnetic resonance, it is now possible to assess myocardial and liver iron levels as well as cardiac function in the same scan, allowing this supposition to be examined. We describe a patient with progressive myocardial iron deposition and the development of early iron overload cardiomyopathy despite excellent compliance to standard subcutaneous desferrioxamine, minimal liver iron and well-controlled serum ferritin levels. These indirect markers remained far below the thresholds conventionally believed to be associated with increased cardiac risk.

Cardiomyopathies↗

Multi-center validation of the transferability of the magnetic resonance T2* technique for the quantification of tissue iron.

The transferability of the T2* technique for measurement of tissue iron between magnetic resonance (MR) scanners is unknown. Heart and liver multi-breath-hold T2* sequences were installed on MR scanners at six different sites. T2* was assessed locally in five or more patients with thalassemia major (n=39), and subjects were re-scanned at the standardization center in London. Inter-center reproducibility of T2* in heart and liver was 5.0% and 7.1%, with mean absolute differences in T2* of 1.3 ms and 0.45 ms, respectively. The MR multi-breath-hold T2* technique for tissue iron quantification is transferable between scanners with good reproducibility.

Humans↗

Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis.

Most deaths in beta-thalassemia major result from cardiac complications due to iron overload. Differential effects on myocardial siderosis may exist between different chelators. A randomized controlled trial was performed in 61 patients previously maintained on subcutaneous deferoxamine. The primary end point was the change in myocardial siderosis (myocardial T2(*)) over 1 year in patients maintained on subcutaneous deferoxamine or those switched to oral deferiprone monotherapy. The dose of deferiprone was 92 mg/kg/d and deferoxamine was 43 mg/kg for 5.7 d/wk. Compliance was 94% +/- 5.3% and 93% +/- 9.7% (P = .81), respectively. The improvement in myocardial T2(*) was significantly greater for deferiprone than deferoxamine (27% vs 13%; P = .023). Left ventricular ejection fraction increased significantly more in the deferiprone-treated group (3.1% vs 0.3% absolute units; P = .003). The changes in liver iron level (-0.93 mg/g dry weight vs -1.54 mg/g dry weight; P = .40) and serum ferritin level (-181 microg/L vs -466 microg/L; P = .16), respectively, were not significantly different between groups. The most frequent adverse events were transient gastrointestinal symptoms for deferiprone-treated patients and local reactions at the infusion site for deferoxamine. There were no episodes of agranulocytosis. Deferiprone monotherapy was significantly more effective than deferoxamine over 1 year in improving asymptomatic myocardial siderosis in beta-thalassemia major.

Adult↗

Late gadolinium enhancement cardiovascular magnetic resonance of the systemic right ventricle in adults with previous atrial redirection surgery for transposition of the great arteries.

BACKGROUND: Patients treated for transposition of the great arteries by atrial redirection surgery have a right ventricle (RV) that sustains systemic pressures long term. Late RV dysfunction occurs in these patients; the reasons for this are unclear, but myocardial fibrosis may be important. Myocardial fibrosis can be visualized by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR). We hypothesized that LGE would be present in the systemic RV and relate to adverse clinical features. METHODS AND RESULTS: We performed CMR on 36 consecutive adult patients (mean age, 27 years) after atrial redirection surgery for transposition of the great arteries. Late gadolinium RV enhancement was seen in 22 patients (61%) with various patterns. Patients with RV LGE were older (30 versus 22 years; P<0.001) and had increased RV end-systolic volume index (43 versus 35 mL/m2; P=0.03), decreased RV ejection fraction (57% versus 62%; P=0.02), increased QRS duration (108 versus 97 ms; P=0.01), and increased QT dispersion (93 versus 71 ms; P=0.002). The extent of LGE correlated with age (r=0.59, P<0.001) and QRS duration (r=0.67, P<0.001) and inversely with RV ejection fraction (r=-0.76, P<0.001). The incidence of documented arrhythmia and/or syncope (10 of 36) was significantly higher in the late gadolinium-positive group (9/22 versus 1/14; P=0.03). CONCLUSIONS: LGE CMR suggestive of myocardial fibrosis occurs in the systemic RV of patients after atrial redirection surgery. The extent of LGE correlates with age, ventricular dysfunction, electrophysiological parameters, and clinical events, suggesting prognostic importance that merits further investigation.

Adult↗