Gaucher disease type 3c: New patients with unique presentations and review of the literature.

Gaucher disease (GD) is the most prevalent lysosomal disorder caused by GBA mutations and abnormal glucocerebrosidase function, leading to glucocerebrosideaccumulation mainly in the liver, spleen, bone marrow, lungs, and occasionally in the central nervous system. Gaucher disease type 3c (GD3c) is a rare subtype of the subacute/chronic neuronopathic GD3, caused by homozygosity for the GBA p.Asp448His (D409H) mutation. GD3c is characterized mainly by cardiovascular and neuro-ophthalmological findings. In this paper, we describe four new GD3c patients exhibiting rare cardiovascular, pulmonary and psychiatric findings, as well as atypical disease courses. Review of the GD3c-related literature revealed clinical descriptions of 36 patients, presenting predominantly with cardiovascular calcifications; 15%, including Patient 1b in this study, had non-calcified lesions - fibrosis and atherosclerosis. Only 7.5% of patients have been described without heart disease, including Patient 3; however, Patient 2 had a fulminant coronary disease. Neurological findings in GD3c consist mainly of oculomotor apraxia (80%), which is absent in Patient 3, while other neurological findings are common (65%) but diverse. Patient 1b developed a psychiatric behavioral disorder, which has not been previously described in GD3c. Patient 1b also had interstitial lung disease, which was only described in one GD3c patient as pulmonary fibrosis. In view of these unique features, we recommend a revised surveillance protocol; however, further studies are required to establish the management of these patients and the role of GBA in the described pathologies.

Prognosis of stress-only SPECT myocardial perfusion imaging with prone imaging.

BACKGROUND: Stress-only single-photon emission computed tomography myocardial perfusion imaging (SO SPECT MPI) is associated with similarly benign prognosis as stress-rest SPECT MPI. However, previous studies have used attenuation correction rather than prone imaging to increase the rate of SO studies. OBJECTIVES: To assess the prognosis of SO SPECT MPI performed with prone imaging. METHODS: We performed a retrospective cohort analysis of all patients who underwent a Tc-99m gated SPECT MPI over a 58-month period. RESULTS: Two thousand four hundred and sixty five patients were followed up. Of them, 1114 (45.2%) patients had a SO supine test, 388 (15.7%) underwent a SO supine and prone test, and the remaining 963 (39.1%) patients underwent a full stress-rest SPECT MPI. There was a similar annual mortality rate between the SO supine/prone group (1.3%), the SO supine (1.5%), and the stress-rest (1.5%) group (P = 0.47). Patients in the stress-rest group were significantly more likely to suffer from myocardial infarction (MI) as compared to the other two groups with an annual rate of 0.7% as compared to 0.4% (P = 0.049). CONCLUSIONS: Normal supine-prone SO SPECT MPI is associated with a similarly benign prognosis as stress-rest SPECT MPI. The adjunction of prone imaging to the stress supine significantly increases the rate of SO SPECT MPI.

Yield of left ventricular dyssynchrony by gated SPECT MPI in patients with heart failure prior to implantable cardioverter-defibrillator or cardiac resynchronization therapy with a defibrillator: Characteristics and prediction of cardiac outcome.

BACKGROUND: Mechanical left ventricular dyssynchrony (MLVD) might contribute in the therapeutic decision-making in patients with heart failure (HF) prior to cardiac resynchronization therapy (CRT). Our aim was to assess MLVD in patients with HF prior to implantable cardioverter-defibrillator (ICD) compared to patients with CRT-D. METHODS: In a prospective study, patients with LVEF ≤ 35% who were scheduled for ICD or CRT-D, underwent gated SPECT myocardial perfusion imaging with technetium 99m sestamibi within 3 months prior procedure. MLVD was measured by phase analysis. RESULTS: The study cohort consisted of 143 patients, 71 with ICD and 72 with CRT-D. Age 68.3 ± 11 and LVEF 24 ± 6%. Phase standard deviation (SD) was 62.5 ± 18 and 59.7 ± 20 (P = NS), respectively. During follow-up of 23.7 ± 12.1 months, there were 10 vs 14 cardiac death in ICD and CRT-D, respectively (P = NS), hospitalization for HF, in 34 vs 53 (P < .001). In multivariate analysis, Phase SD was the independent predictor for cardiac death [HR 2.66 (95% CI 1.046-6.768), P = .04]. Kaplan-Meier curves of phase SD of 60° significantly identified ICD patients with and without cardiac deaths and hospitalization for HF exacerbation. CONCLUSIONS: MLVD by phase SD can identify patients with cardiac events and predict cardiac death in patients treated with ICD.

The effect of dabigatran and rivaroxaban on platelet reactivity and inflammatory markers.

The new oral anticoagulants (NOACs) reduce stroke and systemic embolism in patients with non-valvular atrial fibrillation (AF), but dabigatran may increase risk of coronary ischemic events for unclear reasons. Thus, this study assessed the effects of dabigatran and rivaroxaban on platelet reactivity and inflammatory markers in patients with non-valvular AF. Patients with non-valvular AF planned to begin treatment with NOACs were included. Seventeen patients were prescribed dabigatran and ten rivaroxaban. Platelet function (as assessed by multiple-electrode aggregometry, Impact-R shear-induced platelet deposition, P-selectin expression and plasma RANTES levels) and high-sensitivity C-reactive protein (hs-CRP) were measured at enrollment (prior to initiation of NOAC treatment) and at least 7 days into treatment with either dabigratran or rivaroxaban. Seventeen patients treated with dabigatran (mean age 69 ± 7 years, 35 % women, mean CHADS2 score 2.6 ± 1.2), and ten patients treated with rivaroxaban (mean age 73 ± 9 years, 20 % women, mean CHADS2 score 2.7 ± 1.6) completed the study. In both groups, there were no significant differences in platelet reactivity between the baseline and on-anticoagulant treatment time-points, as measured by each of the platelet-specific assays. There was a trend towards increased platelet reactivity in response to arachidonic acid from baseline to on-treatment in both groups, probably as a result of aspirin discontinuation in 33 % of patients. No significant differences were noted between baseline and on-treatment in hs-CRP in both anticoagulant groups. Treatment with dabigatran and rivaroxaban does not appear to be associated with changes in markers of platelet reactivity or systemic inflammation.

Prognostic value of left ventricular dyssynchrony by myocardial perfusion-gated SPECT in patients with normal and abnormal left ventricular functions.

BACKGROUND: Left ventricular (LV) dyssynchrony by phase analysis has been studied by myocardial perfusion imaging (MPI)-gated SPECT in patients with LV dysfunction in various clinical settings. We aimed to investigate the routine use of phase analysis with gated SPECT for predicting cardiac outcome. METHODS: Patients referred to a tertiary medical center in 2010-2011 prospectively underwent a gated SPECT and phase analysis, and follow-up for cardiac events. The values of clinical variables, MPI, LV function, and LV dyssynchrony in predicting cardiac events were tested by univariate and multivariate analyses. RESULTS: The study group included 787 patients (66.5 ± 11 years, 81% men) followed for a mean duration of 18.3 ± 6.2 months. There were 45 (6%) cardiac events defined as composite endpoint; cardiac death occurred in 26 patients, and the rest had new-onset or worsening heart failure and life-threatening arrhythmias. In multivariate analysis, it was shown that NYHA class, diabetes mellitus, and LVEF <50% were the independent predictors for composite endpoint. However, the independent predictors for cardiac mortality were NYHA class (for each increment in class) and phase standard deviation (SD) (for each 10° increment). CONCLUSION: Gated SPECT with phase analysis for the assessment of LV dyssynchrony can successfully predict cardiac death together with NYHA class, in patients with LV dysfunction.

Angiographic correlation of myocardial perfusion imaging with half the radiation dose using ordered-subset expectation maximization with resolution recovery software.

BACKGROUND: We previously described the feasibility of performing myocardial perfusion imaging (MPI) with half the technetium (Tc-99m) activity using ordered-subset expectation maximization with resolution recovery (OSEM-RR) processing. This study sought to assess the accuracy of this technique by correlating the findings with coronary angiography. METHODS: Of 290 patients who underwent MPI-gated SPECT using a half dose of Tc-99m sestamibi and OSEM-RR software in 2010-2012 at a tertiary medical center, 62 were referred for invasive coronary angiography within 90 days and formed the study group. Ischemia was defined as a summed difference score (SDS) of >3 on the MPI scan. Luminal stenosis of ≥70% on invasive coronary angiography served as the reference. RESULTS: Mean Tc-99m activity per study was 23.9 ± 11.5 mCi and mean effective radiation dose was 7.2 ± 3.4 mSv. MPI revealed no abnormalities in 10 patients (16.2%), myocardial infarction only in 8 patients (12.9%), and ischemia in 44 patients (71.1%). Overall sensitivity, specificity, and positive and negative predictive values for MPI compared to invasive angiography were 89.1%, 75.0%, 91.1%, and 70.5%, respectively. CONCLUSIONS: MPI SPECT performed with a half dose of Tc-99m and OSEM-RR image processing correlates well with invasive angiography. (J Nucl Cardiol 2013).

Feasibility of myocardial perfusion imaging with half the radiation dose in obese patients using ordered-subset expectation maximization with resolution recovery software.

BACKGROUND: We previously described the feasibility of myocardial perfusion imaging (MPI) with nearly half the radiation dose using ordered-subset expectation maximization with resolution recovery (OSEM-RR) processing. This study sought to determine if the findings can be expanded to obese patients. METHODS: Fifty obese patients (>100 kg) referred for MPI underwent stress-rest or rest-stress studies with a half dose of Tc-99m sestamibi in a 1-day protocol using OSEM-RR processing. Image quality and clinical results were compared with matched patients (by age, sex, weight, presence/probability of coronary artery disease) evaluated with standard "full-dose" Tc-99m sestamibi, mostly in a 2-day protocol. Dose activities were adjusted individually by weight. RESULTS: Mean Tc-99m activity was 33.4 ± 13.9 mCi in the half-dose group and 60 ± 10 mCi in the full-dose group (P < .0001). Respective mean effective doses per study were 10 ± 4 and 18 ± 3 mSv (P < .0001). Overall image quality was good-to-excellent in 94% of the half-dose group and 80% of the full-dose group (P < .045). There was no between-group difference in rate or size of ischemia or infarction, except for stress left ventricular ejection fraction. CONCLUSIONS: MPI with half the radiation dose is feasible in obese patients. Image quality is better than for full-dose MPI, and the procedure can be performed in 1 day.

Feasibility of myocardial perfusion imaging with half the radiation dose using ordered-subset expectation maximization with resolution recovery software.

OBJECTIVE: This study sought to assess the feasibility of performing myocardial perfusion imaging (MPI) with half the technetium activity using ordered-subset expectation maximization with resolution recovery (OSEM-RR), an iterative reconstruction software developed to improve count statistics and acquisition time. METHODS: Two hundred eighteen patients referred for MPI were randomly allocated to undergo stress-rest or rest-stress protocols with standard full-dose (FD) injections of technetium (Tc)-99m sestamibi or half-dose (HD) injections and OSEM-RR processing. Dose activities were adjusted individually by weight. The groups were compared for image quality and clinical results. RESULTS: The groups were similar for mean patient age, weight, and body mass index, sex distribution, pre-test probability of CAD and CAD prevalence. Mean Tc-99m activities for the low-dose and high-dose stages were as follows: FD group: 429 ± 85 MBq and 1132 ± 200 MBq; HD group: 263 ± 129 MBq and 629 ± 85 MBq (P < .0001 for both). Mean effective dose per study was 13.6 ± 1.4 mSv in the FD group and 7.7 ± 1.0 mSv in the HD group (P < .001). Over all image quality was good-to-excellent in 98% and 95% of the groups, respectively. However, when we analyzed the low-dose stage separately, image quality was slightly worse in the HD than the FD, though still within the good-to excellent range. CONCLUSIONS: MPI with nearly half the radiation dose is feasible with good image quality.

Identification of protective biologic factors in patients with high cardiovascular risk, but normal coronary arteries (NormCorn).

BACKGROUND: Endothelial progenitor cells (EPCs) have an important role in repair following vascular injury. Telomere length has been shown to be correlated with genome stability and overall cell health. We hypothesized that both EPCs and telomere size are related to protective mechanisms against coronary artery disease. Our aim was to evaluate the level and function of circulating EPCs and telomere length in patients with multiple cardiovascular risk factors and anatomically normal coronary arteries vs. matched controls. METHODS: We included 24 patients, with coronary CTA demonstrating normal coronaries and a high risk of CAD of >10% by ASCVD risk estimator. Control groups included 17 patients with similar cardiovascular profiles but with established CAD and a group of 20 healthy volunteers. Circulating EPCs levels were assessed by flow cytometry for expression of vascular endothelial growth factor receptor 2, CD34 and CD133. The capacity of the cells to form colony forming units (CFUs) was quantified after 1 week of culture. Telomere length was determined by the southern blotting technique. RESULTS: Patients with high risk for CVD and normal coronaries had augmented EPCs function, compared with the CAD group (1.1 vs. 0.22 CFU/f; P = 0.04) and longer telomeres compared with the CAD group (10.7 kb vs. 2.8 kb P = 0.015). These patients displayed a similar profile to the healthy group. CONCLUSION: Patients with a high risk for CAD, but normal coronary arteries have EPCs function and telomere length which resemble healthy volunteers, and augmented compared with patients with established CAD, which could serve as a protective mechanism against atherosclerosis development in these high-risk patients.

Assessment of the relationship between stenosis severity and distribution of coronary artery stenoses on multislice computed tomographic angiography and myocardial ischemia detected by single photon emission computed tomography.

BACKGROUND: The relationship between luminal stenosis measured by coronary CT angiography (CCTA) and severity of stress-induced ischemia seen on single photon emission computed tomographic myocardial perfusion imaging (SPECT-MPI) is not clearly defined. We sought to evaluate the relationship between stenosis severity assessed by CCTA and ischemia on SPECT-MPI. METHODS AND RESULTS: ECG-gated CCTA (64 slice dual source CT) and SPECT-MPI were performed within 6 months in 292 patients (ages 26-91, 73% male) with no prior history of coronary artery disease. Maximal coronary luminal narrowing, graded as 0, ≥25%, 50%, 70%, or 90% visual diameter reduction, was consensually assessed by two expert readers. Perfusion defect on SPECT-MPI was assessed by computer-assisted visual interpretation by an expert reader using the standard 17 segment, 5 point-scoring model (stress perfusion defect of ≥5% = abnormal). By SPECT-MPI, abnormal perfusion was seen in 46/292 patients. With increasing stenosis severity, positive predictive value (PPV) increased (42%, 51%, and 74%, P = .01) and negative predictive value was relatively unchanged (97%, 95%, and 91%) in detecting perfusion abnormalities on SPECT-MPI. In a receiver operator curve analysis, stenosis of 50% and 70% were equally effective in differentiating between the presence and absence of ischemia. In a multivariate analysis that included stenosis severity, multivessel disease, plaque composition, and presence of serial stenoses in a coronary artery, the strongest predictors of ischemia were stenosis of 50-89%, odds ratio (OR) 7.31, P = .001, stenosis ≥90%, OR 34.05, P = .0001, and serial stenosis ≥50% OR of 3.55, P = .006. CONCLUSIONS: The PPV of CCTA for ischemia by SPECT-MPI rises as stenosis severity increases. Luminal stenosis ≥90% on CCTA strongly predicts ischemia, while <50% stenosis strongly predicts the absence of ischemia. Serial stenosis of ≥50% in a vessel may offer incremental value in addition to stenosis severity in predicting ischemia.

Long-Term Outcomes of 560 Consecutive Patients Treated With Transcatheter Aortic Valve Implantation and Propensity Score-Matched Analysis of Early- Versus New-Generation Valves.

Transcatheter aortic valve implantation (TAVI) is an established treatment for severe aortic stenosis in patients at high or prohibitive surgical risk. Nevertheless, long-term clinical and echocardiographic data are still lacking. We carried out an analysis of 560 consecutive patients who underwent TAVI at our institution from 2008 to 2016 to evaluate temporal changes in TAVI characteristics, predictors of 1-year and long-term outcomes, and to compare the performance of the early- and new-generation valve systems. With time, we have adopted lower risk threshold for patient selection and have been using conscious sedation and transfemoral access preferentially (p <0.001 for all). The incidence of greater than mild PVL decreased from 16% to 7.6%, p = 0.029. Within 5 years, 47% of the patients died, the majority (78%) due to noncardiac causes. Independent predictors of 1-year death included periprocedural aspects (i.e., vascular complications, stroke, and PVL), whereas death occurring later than 1 year was solely related to baseline co-morbidities. Transvalvular gradients and residual regurgitation remained nonclinically significant for up to 5 years of follow-up. New-generation valves were associated with less PVL compared with propensity score-matched early-generation valves (p <0.001). In conclusion, TAVI utilization at our institution has progressed to include lower risk patients with transfemoral access becoming applicable in the great majority. Poor long-term survival is attributable to population factors rather than to procedural factors. Intermediate- and long-term hemodynamics are excellent. PVL has diminished significantly with the new-generation valves. Efforts to improve long- and short-term outcomes remain a therapeutic challenge.

Feasibility of myocardial perfusion SPECT with prone and half-time imaging.

BACKGROUND: Myocardial perfusion imaging with single-photon emission tomography (SPECT) is associated with reduced specificity due to tissue attenuation. This can be corrected by prone imaging while necessitating additional imaging time. Image processing with iterative reconstruction allows for a half-time (HT) acquisition. OBJECTIVE: To assess the feasibility of myocardial perfusion with SPECT using prone imaging with HT acquisition. METHODS: Ninety-one patients referred for SPECT myocardial perfusion imaging and weighing up to 90 kg were enrolled for HT supine and prone SPECT protocol. Patients with known myocardial infarction were excluded. HT prone imaging was performed when supine imaging was visually equivocal or abnormal. Image quality was compared for each patient between supine and prone imaging. RESULTS: Acquisition time was 17.9 ± 2.9 min in the HT group compared with 31.8 ± 5.8 min in patients imaged with full-time acquisition. Image quality was good or excellent in 85.7% of studies in a supine position and in 81.3% of studies in the prone position (P = 0.25). No study was considered as nondiagnostic. Prone imaging reduced the rate of equivocal scans from 40.7 to 15.4% and of ischemic studies from 34.1 to 7.7%. In the study population, 80% of inferior and septal defects were corrected by the prone position. CONCLUSION: In a selected population, HT prone and supine imaging is feasible and is associated with a good image quality in most studies whereas acquisition time is reduced almost by half.

Assessment of left ventricular regional wall motion and ejection fraction with low-radiation dose helical dual-source CT: comparison to two-dimensional echocardiography.

BACKGROUND: Electrocardiographic (ECG)-based tube current modulation during cardiac CT reduces radiation exposure but significantly increases noise in parts of the cardiac cycle where tube current is minimized. OBJECTIVE: We evaluated the effect of maximal ECG-based tube current reduction on left ventricular (LV) regional wall motion assessment and ejection fraction (EF) by comparing low-radiation helical dual-source CT (DSCT) to 2-dimensional transthoracic echocardiography (2D-TTE). METHODS: We studied 83 consecutive patients (15 with prior myocardial infarction) who underwent helically acquired DSCT coronary angiography with maximal ECG-based tube current modulation (low-radiation helical DSCT) and 2D-TTE within a 6-month period (median, 1 day), without any change in clinical status between the studies. In all patients, full tube current was applied only at 70% of the R-R interval, with minimal tube current (4% of maximum) in all other parts of the cardiac cycle. Reduced tube voltage (100 kVp) was combined with the maximal dose modulation in 34 patients. DSCT datasets were evaluated by a blinded, experienced cardiologist. Regional wall motion was assessed with the standard 17-segment model, with each segment scored as normal, hypokinetic, akinetic, and dyskinetic. RESULTS: Mean effective radiation dose for the low-radiation helical DSCT was 5.2 ± 1.7 mSv. Regional wall motion was evaluable in all segments on low-radiation helical DSCT. There was excellent agreement of wall motion scoring by low-radiation helical DSCT and 2D-TTE in 1382 of 1411 segments (98%; Cohen's κ value 0.83; 95% confidence interval, 0.76-0.89; P < 0.0001). Mean LVEF was 67.6% ± 10.3% on low-radiation helical DSCT and 61.8% ± 10.3% on 2D-TTE (P < 0.0001). CONCLUSION: Low-radiation dose helical coronary CT angiography with maximal ECG-based tube current modulation is comparable to 2D-TTE for regional wall motion and EF assessment.

Low-density lipoprotein and noncalcified coronary plaque composition in patients with newly diagnosed coronary artery disease on computed tomographic angiography.

We sought to determine significant relations between atherogenic lipoproteins and the contribution of calcified plaque (CP), mixed plaque (MP), and noncalcified plaque (NCP) to the total plaque (TP) burden in patients without previous coronary artery disease. From 823 adult patients without previously established coronary artery disease (52% receiving statin therapy, 34% asymptomatic) but with visible coronary plaque on coronary computed tomographic angiography, we obtained segmental CP, MP, NCP, and TP counts from contrast-enhanced, electrocardiographic-gated computed tomography. Multivariate linear regression analysis was used to determine the associations of clinical factors and lipoprotein levels to CP, MP, and NCP counts and CP/TP, MP/TP, and NCP/TP count ratios. Age, male gender, diabetes, smoking, and statin therapy were significantly associated with the CP count (p <0.001, p <0.001, p = 0.049, p = 0.016, and p = 0.003, respectively). Low-density lipoprotein (LDL) cholesterol was significantly associated with MP and NCP counts (all p values

Coronary artery calcium scoring using a reduced tube voltage and radiation dose protocol with dual-source computed tomography.

BACKGROUND: Technical advances to minimize radiation exposure because of imaging are in accord with the "as low as reasonably achievable" principle. OBJECTIVE: We aimed to determine whether coronary calcium scoring (CCS) by multidetector CT at a tube voltage of 100 kVp yields comparable results to the standard 120-kVp protocol while reducing radiation dose. METHODS: Sixty consecutive outpatients were scanned with a dual-source CT scanner with both the120- and 100-kVp protocols. The calcium threshold was 130 Hounsfield units (HUs) for 120 kVp and 147 HU for 100 kVp, as determined from phantom data. All 100-kVp scans were scored by an experienced reader blinded to 120-kVp data. RESULTS: Image quality was comparable for 100- and 120- kVp scans. Mean Agatston scores for 100 and 120 kVp were 189 +/- 484 and 189 +/- 498 (P = 0.92), with perfect correlation (r = 1.0; P < 0.0001; 95% limits of agreement, -36 to 37; bias, 0.6). Mean coronary calcium volume scores for 100 and 120 kVp were 143 +/- 370 mm(3) and 149 +/- 392 mm(3) (P = 0.26), with perfect correlation (r = 1.0; P < 0.0001; 95% limits of agreement, -35 to 32 mm(3); bias, -1.4 mm(3)). The mean absolute difference for Agatston scores between the protocols was 16.9, with excellent agreement (kappa = 0.95; P < 0.0001). Mean effective radiation dose for the 100-kVp protocol was significantly lower (1.17 mSv versus 1.70 mSv; P < 0.0001). CONCLUSION: A reduced tube current protocol using 100 kVp gives equivalent CCS results at reduced radiation exposure compared with a standard protocol at 120 kVp.

Moving beyond binary grading of coronary arterial stenoses on coronary computed tomographic angiography: insights for the imager and referring clinician.

OBJECTIVES: We evaluated the technical and clinical utility of visual 5-point coronary stenosis grading on coronary computed tomographic angiography (CCTA). BACKGROUND: The binary approach used to assess coronary stenoses on CCTA does not adequately describe borderline obstructive lesions and limits full expression of clinically useful information. METHODS: From 84 patients who underwent CCTA and invasive angiography, we identified 278 native coronary segments with > or =25% stenosis on CCTA after excluding all <25% stenotic, stented, and uninterpretable segments. Fifty <25% stenotic segments were randomly selected as controls. Segmental stenosis severity on CCTA was consensually graded using a 0 to 5 scale (grade 0 = none, grade 1 = 1% to 24%, grade 2 = 25% to 49%, grade 3 = 50% to 69%, grade 4 = 70% to 89%, grade 5 = 90% to 100%) by 2 readers, using visual inspection and computed tomography-based quantification (CTQCA). Invasive angiography-based stenosis quantification (IQCA) was performed for all segments, using the same 0 to 5 scale to score stenosis severity. RESULTS: On CCTA, 185 (56%) segments had intermediate stenoses (grade 2 or grade 3). Stenosis severity by IQCA increased significantly with each step-up in CCTA grade (p < 0.001). CTQCA did not perform better than visual inspection. Visual CCTA stenosis grading differed from IQCA by >1 grade in only 4% of grade 2 to grade 5 segments (10 of 278; 2% of CCTA grade 2 segments, 4% of grade 3, 8% of grade 4, 2% of grade 5). Overall quantitative correlation was strong (r = 0.82) with high variability in agreement between CTQCA and IQCA for individual segments (95% of differences between 27.2% and 34.6%). CONCLUSIONS: With current CCTA technology, experienced readers should consider adopting a visually based, multitiered grading approach to evaluate coronary stenoses. A < or =49% lesion on CCTA can be considered virtually exclusive of > or =70% stenosis by invasive angiography.

Dual-source coronary computed tomography angiography in patients with atrial fibrillation: initial experience.

BACKGROUND: Patients with atrial fibrillation (AF) are generally excluded from coronary CT angiography (CCTA) studies because of motion artifact resulting from irregular rhythm. The 83-millisecond temporal resolution of the dual-source CT (DSCT) may be sufficient to allow CCTA in patients with AF. OBJECTIVE: We examined the feasibility of DSCT in patients with AF referred for CCTA. METHODS: We compared results of CCTA with DSCT in 24 consecutive patients with AF with 119 control patients in sinus rhythm. Standard relative-delay phase reconstruction (40%-80% of cardiac cycle) was used, with additional absolute delay reconstruction performed when indicated. Image quality was scored both subjectively and objectively. RESULTS: Patients with AF were older (68.5 +/- 14.0 years versus 62.5 +/- 12.1 years; P = 0.03). Maximum heart rate during injection was 102.5 +/- 30.4 beats/min and 70.8 +/- 16.6 beats/min in the AF and control groups, respectively (P < 0.01). Mean (+/-SD) Agatston score was 321 +/- 366 (range, 0-1158) and 361 +/- 743 (range, 0-3948) in the AF and control groups, respectively (P = 0.8). No difference was observed in the proportion of uninterpretable segments between the 2 groups, 7 (2%) in the AF group and 12 (1%) in the control group (P = NS). Two (8%) of 24 studies in the AF group and 12 (10%) of 119 studies in the control group were nondiagnostic (P = NS). Image quality was good or excellent in 13 (54%) of 21 AF cases compared with 94 (79%) of 119 control cases (P = 0.01). Absolute delay reconstruction was needed in 9 (38%) of 24 AF cases. CONCLUSIONS: These preliminary data show that interpretable CCTA data can be obtained in patients with AF using DSCT. The need for absolute delay reconstruction is common.

Aortic size assessment by noncontrast cardiac computed tomography: normal limits by age, gender, and body surface area.

OBJECTIVES: To determine normal limits for ascending and descending thoracic aorta diameters in a large population of asymptomatic, low-risk adult subjects. BACKGROUND: Assessment of aortic size is possible from gated noncontrast computed tomography (CT) scans obtained for coronary calcium measurements. However, normal limits for aortic size by these studies have yet to be defined. METHODS: In 4,039 adult patients undergoing coronary artery calcium (CAC) scanning, systematic measurements of the ascending and descending thoracic aorta diameters were made at the level of the pulmonary artery bifurcation. Multiple linear regression analysis was used to detect risk factors independently associated with ascending and descending thoracic aorta diameter and exclude subjects with these parameters from the final analysis. The final analysis groups for ascending and descending thoracic aorta included 2,952 and 1,931 subjects, respectively. Subjects were then regrouped by gender, age, and body surface area (BSA) for ascending and descending aorta, separately, and for each group, the mean, standard deviation, and upper normal limit were calculated for aortic diameter as well as for the calculated cross-sectional aortic area. Also, linear regression models were used to create BSA versus aortic diameter nomograms by age groups, and a formula for calculating predicted aortic size by age, gender, and BSA was created. RESULTS: Age, BSA, gender, and hypertension were directly associated with thoracic aorta dimensions. Additionally, diabetes was associated with ascending aorta diameter, and smoking was associated with descending aorta diameter. The mean diameters for the final analysis group were 33 +/- 4 mm for the ascending and 24 +/- 3 mm for the descending thoracic aorta, respectively. The corresponding upper limits of normal diameters were 41 and 30 mm, respectively. CONCLUSIONS: Normal limits of ascending and descending aortic dimensions by noncontrast gated cardiac CT have been defined by age, gender, and BSA in a large, low-risk population of subjects undergoing CAC scanning.