Stabilization of Early Duchenne Cardiomyopathy With Aldosterone Inhibition: Results of the Multicenter AIDMD Trial.

Background Duchenne muscular dystrophy incurs nearly universal dilated cardiomyopathy by the third decade of life, preceded by myocardial damage and impaired left ventricular strain by cardiac magnetic resonance. It has been shown that (1) mineralocorticoid receptor antagonist therapy with spironolactone attenuated damage while maintaining function when given early in a mouse model and (2) low-dose eplerenone stabilized left ventricular strain in boys with Duchenne muscular dystrophy and evident myocardial damage but preserved ejection fraction. We hypothesized that moderate-dose spironolactone versus eplerenone would provide similar cardioprotection in this first head-to-head randomized trial of available mineralocorticoid receptor antagonists, the AIDMD (Aldosterone Inhibition in Duchenne Muscular Dystrophy) trial. Methods and Results This was a multicenter, double-blind, randomized, noninferiority trial. Subjects were randomized to eplerenone, 50 mg, or spironolactone, 50 mg, orally once daily for 12 months. The primary outcome was change in left ventricular systolic strain at 12 months. Among 52 enrolled male subjects, aged 14 (interquartile range, 12-18) years, spironolactone was noninferior to eplerenone (∆strain, 0.4 [interquartile range, -0.4 to 0.6] versus 0.2 [interquartile range, -0.2 to 0.7]; P=0.542). Renal and pulmonary function remained stable in both groups, and no subjects experienced serious hyperkalemia. Infrequent adverse events included gynecomastia in one subject in the spironolactone arm and facial rash in one subject in the eplerenone arm. Conclusions In boys with Duchenne muscular dystrophy and preserved left ventricular ejection fraction, spironolactone added to background therapy is noninferior to eplerenone in preserving contractile function. These findings support early mineralocorticoid receptor antagonist therapy as effective and safe in a genetic disease with high cardiomyopathy risk. Clinical Trial Registration URL: http://www.clinicaltrials.gov. Unique identifier: NCT02354352.

Estimation of myocardial fibrosis in humans with dual energy CT.

BACKGROUND: The current clinical standard for in vivo imaging of myocardial fibrosis is contrast-enhanced cardiac magnetic resonance (CMR). We sought to validate a novel non-contrast dual energy computed tomography (DECT) method to estimate myocardial fibrosis in patients undergoing CMR with contrast. METHODS: All subjects underwent non-contrast, prospectively-triggered cardiac DECT on a single source scanner with interleaved acquisition between tube voltages of 80 and 140 kVp. Monochromatic images were reconstructed at 11 energies spanning 40-140 keV; a region of interest (ROI) was drawn in the mid-inferoseptal segment, recording mean attenuation value in the ROI, at each energy level. Comparison was made to data from single energy (70 keV) image data. Linear discriminant analysis (LDA) was performed to compare the predictive capability of single vs. multi-energy inferoseptal segment CT attenuation on myocardial fibrosis by both visually assessed LGE (absent/present fibrosis) and CMR T1 mapping-derived myocardial extracellular volume fraction (ECV). RESULTS: The multi-energy CT/LDA approach performed better than a single energy approach to discriminate among LGE-CMR classes of present/absence myocardial fibrosis severity, demonstrating correct classification rates of 89% and 71%, respectively. The multi-energy CT/LDA approach also performed better in correctly discriminating normal from elevated ECV, doing so in 89% of patients vs. correct distinction of normal/elevated ECV in only 70% using the single energy approach. CONCLUSIONS: Non-contrast cardiac DECT with multi-energy analysis better classifies myocardial fibrosis and extracellular volume compared to what is feasible with non-contrast single energy cardiac CT. These data support further evaluation of this approach to noninvasively assess myocardial fibrosis.

Eplerenone for early cardiomyopathy in Duchenne muscular dystrophy: results of a two-year open-label extension trial.

BACKGROUND: Cardiomyopathy is a leading cause of morbidity and mortality in boys with Duchenne muscular dystrophy (DMD). We recently showed in a 12-month double-blind randomized controlled trial that adding eplerenone to background medical therapy was cardioprotective in this population. The objective of this study was to evaluate the safety and efficacy of longer-term eplerenone therapy in boys with DMD. RESULTS: Eleven subjects (phase 1 baseline median [range] age: 13 [7 - 25] years) from the original 12-month trial at a single participating center were enrolled. Importantly, those who entered the extension study who had been on eplerenone previously were significantly older than those who had originally been on placebo (median age 10.5 vs. 18.0 years, p = 0.045). During an additional 24-month open-label extension study, all boys received eplerenone 25 mg orally once daily to treat preclinical DMD cardiomyopathy, defined as evident myocardial damage by late gadolinium enhancement cardiac magnetic resonance (LGE) with preserved ejection fraction (EF). The threshold for potassium level, the primary safety measure, was not exceeded in any non-hemolyzed blood sample. Over 24 months, left ventricular (LV) systolic strain, a more sensitive marker whose more negative values indicate greater contractility significantly improved (median change -4.4%, IQR -5.8 to -0.9%) in younger subjects whereas older subjects' strain remained stable without significant worsening or improvement (median change 0.2%, IQR -1.1 to 4.3%). EF and extent of myocardial damage by LGE remained stable in both groups over 2 years. CONCLUSIONS: Eplerenone offers effective and safe cardioprotection for boys with DMD, particularly when started at a younger age. Eplerenone is a useful clinical therapeutic option, particularly if treatment is initiated earlier in life when cardiac damage is minimal. TRIAL REGISTRATION: http://ClinicalTrials.gov identifier NCT01521546. Registered 26 January 2012.

Iron, inflammation and atherosclerosis risk in men vs. perimenopausal women.

OBJECTIVE: Age at first atherosclerotic event is typically older for women vs. men; monthly iron loss has been postulated to contribute to this advantage. We investigated the relationship between an MRI-based arterial wall biomarker and the serum inflammatory biomarker high-sensitivity C-reactive protein (hsCRP) in perimenopausal women vs. men. METHODS AND RESULTS: Women without evident atherosclerotic disease were prospectively enrolled and observed over 24 months of menopause transition, indicated by hormone levels and reduction in median number of menstrual cycles from 4 [3-6] per year to 0 [0-1] per year (P < 0.01). Higher hsCRP predicted shorter carotid artery wall T2* in women entering the menopause transition (r = -0.3139, P = 0.0014); this relationship weakened after 24 months of perimenopause in women (r = -0.1718, P = 0.0859) and was not significant in a cohort of men matched for age and cardiovascular risk category (r = -0.0310, P = 0.8362). Serum ferritin increased from baseline to 24-month follow-up during women's menopause transition (37 [20-79] to 67 [36-97] ng/mL, P < 0.01), but still remained lower compared to men (111 [45-220] ng/mL, P < 0.01). Circulating ferritin levels correlated with arterial wall T2* values in women at baseline (r = -0.3163, P = 0.0013) but not in women after 24 months (r = -0.0730, P = 0.4684) of menopause transition nor in men (r = 0.0862, P = 0.5644). CONCLUSIONS: An arterial wall iron-based imaging biomarker reflects degree of systemic inflammation in younger women, whereas this relationship is lost as women transition through menopause to become more similar to men. Iron homeostasis and inflammation in the arterial wall microenvironment warrants further investigation as a potential early target for interventions that mitigate atherosclerosis risk.

Eplerenone for early cardiomyopathy in Duchenne muscular dystrophy: a randomised, double-blind, placebo-controlled trial.

BACKGROUND: Cardiomyopathy is a leading cause of death in patients with Duchenne muscular dystrophy and myocardial damage precedes decline in left ventricular systolic function. We tested the efficacy of eplerenone on top of background therapy in patients with Duchenne muscular dystrophy with early myocardial disease. METHODS: In this randomised, double-blind, placebo-controlled trial, boys from three centres in the USA aged 7 years or older with Duchenne muscular dystrophy, myocardial damage by late gadolinium enhancement cardiac MRI and preserved ejection fraction received either eplerenone 25 mg or placebo orally, every other day for the first month and once daily thereafter, in addition to background clinician-directed therapy with either angiotensin-converting enzyme inhibitors (ACEI) or angiotensin receptor blockers (ARB). Computer-generated randomisation was done centrally using block sizes of four and six, and only the study statistician and the investigational pharmacy had the preset randomisation assignments. The primary outcome was change in left ventricular circumferential strain (Ecc) at 12 months, a measure of contractile dysfunction. Safety was established through serial serum potassium levels and measurement of cystatin C, a non-creatinine measure of kidney function. This trial is registered with ClinicalTrials.gov, number NCT01521546. FINDINGS: Between Jan 26, 2012, and July 3, 2013, 188 boys were screened and 42 were enrolled. 20 were randomly assigned to receive eplerenone and 22 to receive placebo, of whom 20 in the eplerenone group and 20 in the placebo group completed baseline, 6-month, and 12-month visits. After 12 months, decline in left ventricular circumferential strain was less in those who received eplerenone than in those who received placebo (median ΔEcc 1·0 [IQR 0·3-2·2] vs 2·2 [1·3-3·1]; p=0·020). Cystatin C concentrations remained normal in both groups, and all non-haemolysed blood samples showed normal potassium concentrations. One 23-year-old patient in the placebo group died of fat embolism, and another patient in the placebo group withdrew from the trial to address long-standing digestive issues. All other adverse events were mild: short-lived headaches coincident with seasonal allergies occurred in one patient given eplerenone, flushing occurred in one patient given placebo, and anxiety occurred in another patient given placebo. INTERPRETATION: In boys with Duchenne muscular dystrophy and preserved ejection fraction, addition of eplerenone to background ACEI or ARB therapy attenuates the progressive decline in left ventricular systolic function. Early use of available drugs warrants consideration in this population at high risk of cardiac death, but further studies are needed to determine the effect of combination cardioprotective therapy on event-free survival in Duchenne muscular dystrophy. FUNDING: BallouSkies, Parent Project for Muscular Dystrophy, US National Center for Advancing Translational Sciences, and US National Institutes of Health.

Cardiovascular magnetic resonance of cardiomyopathy in limb girdle muscular dystrophy 2B and 2I.

BACKGROUND: Limb girdle muscular dystrophies (LGMD) are inclusive of 7 autosomal dominant and 14 autosomal recessive disorders featuring progressive muscle weakness and atrophy. Studies of cardiac function have not yet been well-defined in deficiencies of dysferlin (LGMD2B) and fukutin related protein (LGMD2I). In this study of patients with these two forms of limb girdle muscular dystrophy, cardiovascular magnetic resonance (CMR) was used to more specifically define markers of cardiomyopathy including systolic dysfunction, myocardial fibrosis, and diastolic dysfunction. METHODS: Consecutive patients with genetically-proven LGMD types 2I (n = 7) and 2B (n = 9) and 8 control subjects were enrolled. All subjects underwent cardiac magnetic resonance (CMR) on a standard 1.5 Tesla clinical scanner with cine imaging for left ventricular (LV) volume and ejection fraction (EF) measurement, vector velocity analysis of cine data to calculate myocardial strain, and late post-gadolinium enhancement imaging (LGE) to assess for myocardial fibrosis. RESULTS: Sixteen LGMD patients (7 LGMD2I, 9 LGMD2B), and 8 control subjects completed CMR. All but one patient had normal LV size and systolic function; one (type 2I) had severe dilated cardiomyopathy. Of 15 LGMD patients with normal systolic function, LGE imaging revealed focal myocardial fibrosis in 7 (47%). Peak systolic circumferential strain rates were similar in patients vs. controls: εendo was -23.8 ± 8.5vs. -23.9 ± 4.2%, εepi was -11.5 ± 1.7% vs. -10.1 ± 4.2% (p = NS for all). Five of 7 LGE-positive patients had grade I diastolic dysfunction [2I (n = 2), 2B (n = 3)]. that was not present in any LGE-negative patients or controls. CONCLUSIONS: LGMD2I and LGMD2B generally result in mild structural and functional cardiac abnormalities, though severe dilated cardiomyopathy may occur. Long-term studies are warranted to evaluate the prognostic significance of subclinical fibrosis detected by CMR in these patients.

Impaired myocardial perfusion reserve and fibrosis in Friedreich ataxia: a mitochondrial cardiomyopathy with metabolic syndrome.

AIMS: Cardiomyopathy produces significant mortality in patients with Friedreich ataxia (FA), a genetic disorder that produces intra-mitochondrial iron accumulation. We sought to test the hypothesis that abnormal myocardial perfusion reserve and fibrosis represent early manifestations of cardiomyopathy. METHODS AND RESULTS: Twenty-six patients with genetically proven FA ages 36 ± 12 years without cardiomyopathy and eight controls underwent cardiac magnetic resonance with adenosine. Precontrast imaging for myocardial iron estimation was performed. Myocardial perfusion reserve index (MPRI) was quantified using the normalized upslope of myocardial enhancement during vasodilator stress vs. rest. Left ventricular (LV) mass and volumes were computed from short-axis cine images. Serologies included lipids, and platelets were isolated for iron quantification using inductively coupled plasma mass spectrometry. Left ventricular ejection fraction and mass averaged 64.1 ± 8.3% and 62.7 ± 16.7 g/m², respectively, indicating preserved systolic function and absence of significant hypertrophy. Myocardial perfusion reserve index quantification revealed significantly lower endocardial-to-epicardial perfusion reserve in patients vs. controls (0.80 ± 0.18 vs. 1.22 ± 0.36, P = 0.01). Lower MPRI was predicted by increased number of metabolic syndrome (met-S) features (P < 0.01). Worse concentric remodelling occurred with increased GAA repeat length (r = 0.64, P < 0.001). Peripheral platelet iron measurement showed no distinction between patients and controls (5.4 ± 8.5 × 10⁻7 vs. 5.5 ± 2.9 × 10⁻7 ng/platelet, P = 0.88), nor did myocardial T2* measures. CONCLUSIONS: Patients with FA have abnormal myocardial perfusion reserve that parallels met-S severity. Impaired perfusion reserve and fibrosis occur in the absence of significant hypertrophy and prior to clinical heart failure, providing potential therapeutic targets for stage B cardiomyopathy in FA and related myocardial diseases.

Design and Rationale for the Study of Changes in Iron and Atherosclerosis Risk in Perimenopause.

This study seeks to investigate changes in iron homeostasis and carotid arteries in women at risk of atherosclerosis, addressing a relatively unexplored hypothesis explaining why women have a 5-10 year lag in initial atherosclerotic events. Recent evidence points to hepcidin, the key regulator of macrophage iron uptake and release, as a potential mediator of risk. Furthermore, iron catalyzes the generation of free radicals that oxidize cholesterol stimulating atheroma formation. Magnetic resonance imaging (MRI) is ideally suited to study iron because of iron's local effects on magnetic susceptibility that can be quantified using a relaxation parameter called T2* ('T2-star'), as well as the ability to noninvasively characterize and quantify atherosclerotic plaque with MRI. This work outlines the rationale and study design to provide critical evidence related to the iron hypothesis, such that novel diagnostics and therapeutics to attenuate risk may be derived from a better understanding of iron's role in atherosclerosis.

Real-time cine and myocardial perfusion with treadmill exercise stress cardiovascular magnetic resonance in patients referred for stress SPECT.

BACKGROUND: To date, stress cardiovascular magnetic resonance (CMR) has relied on pharmacologic agents, and therefore lacked the physiologic information available only with exercise stress. METHODS: 43 patients age 25 to 81 years underwent a treadmill stress test incorporating both Tc99m SPECT and CMR. After rest Tc99m SPECT imaging, patients underwent resting cine CMR. Patients then underwent in-room exercise stress using a partially modified treadmill. 12-lead ECG monitoring was performed throughout. At peak stress, Tc99m was injected and patients rapidly returned to their prior position in the magnet for post-exercise cine and perfusion imaging. The patient table was pulled out of the magnet for recovery monitoring. The patient was sent back into the magnet for recovery cine and resting perfusion followed by delayed post-gadolinium imaging. Post-CMR, patients went to the adjacent SPECT lab to complete stress nuclear imaging. Each modality's images were reviewed blinded to the other's results. RESULTS: Patients completed on average 9.3 +/- 2.4 min of the Bruce protocol. Stress cine CMR was completed in 68 +/- 14 sec following termination of exercise, and stress perfusion CMR was completed in 88 +/- 8 sec. Agreement between SPECT and CMR was moderate (kappa = 0.58). Accuracy in eight patients who underwent coronary angiography was 7/8 for CMR and 5/8 for SPECT (p = 0.625). Follow-up at 6 months indicated freedom from cardiovascular events in 29/29 CMR-negative and 33/34 SPECT-negative patients. CONCLUSIONS: Exercise stress CMR including wall motion and perfusion is feasible in patients with suspected ischemic heart disease. Larger clinical trials are warranted based on the promising results of this pilot study to allow comparative effectiveness studies of this stress imaging system vs. other stress imaging modalities.

Continuous positive airway pressure therapy reduces right ventricular volume in patients with obstructive sleep apnea: a cardiovascular magnetic resonance study.

STUDY OBJECTIVES: There are few data on the effects of continuous positive airway pressure (CPAP) therapy on the structural and functional characteristics of the right heart in patients with obstructive sleep apnea (OSA). We sought to leverage the advantages of cardiac magnetic resonance imaging (CMR) and hypothesized that CPAP treatment would improve right ventricular (RV) function in a group of patients with OSA who were free of other comorbid conditions. METHODS: Patients with severe (apnea-hypopnea index > or = 30/h) untreated OSAwere prospectively enrolled. CMR included 3-dimensional measurement of biventricular size and function, and rest/stress myocardial perfusion and was performed at baseline and after 3 months of CPAP therapy. RESULTS: Fifteen patients with mild to moderate desaturation were enrolled; 2 could not undergo CMR due to claustrophobia and obesity. There were significant decreases in the Epworth Sleepiness Scale score (p < 0.0001) and RV end-systolic and RV end-diastolic volumes (p < 0.05) with CPAP. There was a trend toward improvement in RV ejection fraction, but the improvement did not reach statistical significance. Other measures such as left ventricular volumes, left ventricular ejection fraction, myocardial perfusion reserve index, and thickness of the interventricular septum and ventricular free wall did not change significantly. CONCLUSIONS: This preliminary study found that CPAP treatment decreases RV volumes in patients with severe OSA who are otherwise healthy. CMR offers a novel technique to determine the effects of CPAP on ventricular structure and function in patients with OSA. A randomized controlled study is needed to confirm the results of our study.

Dobutamine stress cardiac magnetic resonance imaging to detect myocardial ischemia in women.

This study sought to evaluate dobutamine stress cardiac magnetic resonance imaging (DCMRI) in women with abnormal stress nuclear testing results. Women with findings on stress nuclear exams, including electrocardiography and/or perfusion, thought to require further evaluation with invasive coronary angiography were prospectively enrolled. Multiplane cine imaging was obtained at rest and at each stage of inotropic stress with atropine as needed to achieve target heart rate. DCMRI results were compared with stress nuclear and invasive cardiac catheterization results. Of 23 patients enrolled successfully, 22 completed DCMRI examination without complications. In all cases, DCMRI imaging demonstrated appropriate stress response with no ischemia despite abnormalities on stress nuclear testing. In the 18 patients who also underwent invasive coronary angiography, no significant obstructive disease was identified. DCMRI may be a useful alternative to stress nuclear examination in women; larger studies are warranted to determine its potential to more accurately predict obstructive coronary artery disease.

In vivo atherosclerotic plaque characterization using magnetic susceptibility distinguishes symptom-producing plaques.

OBJECTIVES: We investigated the role of iron deposition in atherosclerotic plaque instability using a novel approach of in vivo plaque characterization by a noninvasive, noncontrast magnetic resonance-based T2* measurement. This approach was validated using ex vivo plaque analyses to establish that T2* accurately reflects intraplaque iron composition. BACKGROUND: Iron catalyzes free radical production, a key step for lipid peroxidation and atherosclerosis development. The parameter T2* measures tissue magnetic susceptibility, which historically has been used to quantify hepatic and myocardial iron. The T2* measurement has not been used for in vivo plaque characterization in patients with atherosclerosis. METHODS: Thirty-nine patients referred for carotid endarterectomy were prospectively enrolled to undergo preoperative carotid magnetic resonance imaging (MRI) and postoperative analysis of the explanted plaque. Clinical history of any symptoms attributable to each carotid lesion was recorded. We could not complete MRI in 4 subjects because of their claustrophobia, and 3 patients scanned before the institution of a neck stabilizer had motion artifact, precluding quantification. RESULTS: Symptomatic patients had significantly lower plaque T2* values (20.0 +/- 1.8 ms) compared with asymptomatic patients (34.4 +/- 2.7 ms, p < 0.001). Analytical methods demonstrated similar total iron (138.6 +/- 36.5 microg/g vs. 165.8 +/- 48.3 microg/g, p = NS) but less low molecular weight Fe(III) (7.3 +/- 3.8 microg/g vs. 17.7 +/- 4.0 microg/g, p < 0.05) in the explanted plaques of symptomatic versus asymptomatic patients, respectively, which is consistent with a shift in iron from Fe(III) to greater amounts of T2*-shortening forms of iron. Mass spectroscopy also showed significantly lower calcium (37.5 +/- 10.8 mg/g vs. 123.6 +/- 19.3 mg/g, p < 0.01) and greater copper (3.2 +/- 0.5 microg/g vs. 1.7 +/- 0.1 microg/g, p < 0.01) in plaques from symptomatic patients. CONCLUSIONS: In vivo measurement of intraplaque T2* using MRI is feasible and distinguishes symptom-producing from non-symptom-producing plaques in patients with carotid artery atherosclerosis. Symptom-producing plaques demonstrated characteristic changes in iron forms by ex vivo analysis, supporting the dynamic presence of iron in the microenvironment of atherosclerotic plaque.

Mid-myocardial fibrosis by cardiac magnetic resonance in patients with lamin A/C cardiomyopathy: possible substrate for diastolic dysfunction.

AIMS: We sought to identify patterns of myocardial fibrosis in vivo in patients with lamin cardiomyopathy, and to determine its functional significance. METHODS AND RESULTS: Eleven patients sharing the identical mutation in LMNA without contraindication to magnetic resonance were identified from a 1016-member pedigree. Eight autopsy hearts from deceased relatives were reviewed. Patients and age-matched controls underwent cardiac magnetic resonance that included measures of cardiac function and late gadolinium enhancement (LGE). LGE-CMR identified midmyocardial fibrosis of the basal interventricular septum in 5 of 11 LMNA patients that was identical to that seen in 6 autopsy specimens of related genotype-positive family members; this was not present in any of 11 controls. LGE-CMR was positive in the 5 oldest patients in the cohort, age 46 +/- 6 years compared to 24 +/- 10 years for LGE-negative subjects (p = 0.003). Systolic function was abnormal in 2 subjects, both with myocardial fibrosis. LGE-positivity distinguished patients with diastolic dysfunction by mitral inflow velocities from those with normal diastolic function; these patients also had significant left atrial enlargement compared to controls (p < 0.05). CONCLUSIONS: LGE-CMR can identify myocardial fibrosis under genetic control in vivo in patients with heritable cardiomyopathy similar in distribution to that observed at autopsy. Mid-myocardial fibrosis may form the substrate for diastolic dysfunction in these patients.

Multi-detector row cardiac computed tomography accurately quantifies right and left ventricular size and function compared with cardiac magnetic resonance.

BACKGROUND: Cardiac magnetic resonance (CMR) accurately quantifies right ventricular (RV) and left ventricular (LV) volumes and function. Limited availability of CMR and increasing use of MR-incompatible cardiovascular devices underscore the potential utility of cardiac computed tomography (CT) for ventricular quantification. This study quantified biventricular size and systolic function with multi-detector row CT compared with CMR imaging. METHODS: Twenty-six subjects prospectively underwent CT and CMR examinations on a 16-detector CT and 1.5 T MR scanner, respectively; claustrophobia in one and nongated CT imaging in another precluded complete imaging in 2 subjects. Contiguous multiphase short-axis images were generated from axial CT data, and steady-state free precession cine MR produced contiguous short-axis cines. Semiautomated software generated ventricular borders to calculate volume, mass, and ejection fraction (EF) from both sets of images. Blinded observers completed quantification and wall motion analyses of 23 CMR and CT data sets independently. RESULTS: All measures of LV size and function by cardiac CT correlated well with CMR over a wide range of LV function (LVEF 30%-72% by CMR), including end-diastolic volume (r = 0.97), end-systolic volume (r = 0.97), EF (r = 0.97), and mass (r = 0.95). Of 24 cases, 6 had inadequate contrast opacification of the RV precluding RV segmentation. In the remaining 18 CMR-CT data pairs, RVEF showed moderate agreement (r = 0.86), and RV volumes correlated well (r = 0.97 and 0.94 for RV end-diastolic volume and RV end-systolic volume, respectively). Ten percent of LV segments visualized by CT were inadequate for wall motion assessment due to motion artifact or inadequate contrast between myocardium and endocardium. For segments adequately visualized by both techniques, the mean kappa statistic was 0.88 (range 0.78-1.0), consistent with good agreement. CONCLUSION: Cardiac CT accurately quantifies LV size and function; RV quantification with cardiac CT requires optimized contrast opacification of the RV.

Usefulness of multidetector row computed tomography to quantify right ventricular size and function in adults with either tetralogy of Fallot or transposition of the great arteries.

An ever-increasing number of patients with congenital heart disease are reaching adulthood; follow-up often requires right ventricular (RV) quantification. Complex anatomy, acoustic window, and active implants such as pacemakers may limit modalities, such as echocardiography and cardiac magnetic resonance imaging (MRI), in this population. Recent advances in multidetector row cardiac computed tomography (MDCT) allow multiphase, multislice reconstructions in any plane but have not been applied to RV quantification. Fourteen patients with tetralogy of Fallot or transposition of the great arteries underwent cardiac MRI and MDCT examinations, with comparable quantification of RV volumes and ejection fractions and no systematic error by Bland-Altman analysis using MRI as the gold standard.