Multimodality Assessment of Anomalous Aortic Origin of the Right Coronary Artery Presenting With Cardiac Arrest.

Anomalous aortic origin of coronary artery (AAOCA) can range from benign anatomic variants to those presenting with sudden cardiac arrest. This unique case of right AAOCA demonstrates detailed anatomic findings from cardiac computed tomography and the effects of transient acute coronary ischemia by cardiac magnetic resonance. (Level of Difficulty: Advanced.).

NHLBI-Sponsored Randomized Trial of Postconditioning During Primary Percutaneous Coronary Intervention for ST-Elevation Myocardial Infarction.

RATIONALE: Postconditioning at the time of primary percutaneous coronary intervention (PCI) for ST-segment-elevation myocardial infarction may reduce infarct size and improve myocardial salvage. However, clinical trials have shown inconsistent benefit. OBJECTIVE: We performed the first National Heart, Lung, and Blood Institute-sponsored trial of postconditioning in the United States using strict enrollment criteria to optimize the early benefits of postconditioning and assess its long-term effects on left ventricular (LV) function. METHODS AND RESULTS: We randomized 122 ST-segment-elevation myocardial infarction patients to postconditioning (4, 30 seconds PTCA [percutaneous transluminal coronary angioplasty] inflations/deflations)+PCI (n=65) versus routine PCI (n=57). All subjects had an occluded major epicardial artery (thrombolysis in myocardial infarction=0) with ischemic times between 1 and 6 hours with no evidence of preinfarction angina or collateral blood flow. Cardiac magnetic resonance imaging measured at 2 days post-PCI showed no difference between the postconditioning group and control in regards to infarct size (22.5±14.5 versus 24.0±18.5 g), myocardial salvage index (30.3±15.6% versus 31.5±23.6%), or mean LV ejection fraction. Magnetic resonance imaging at 12 months showed a significant recovery of LV ejection fraction in both groups (61.0±11.4% and 61.4±9.1%; P<0.01). Subjects randomized to postconditioning experienced more favorable remodeling over 1 year (LV end-diastolic volume =157±34 to 150±38 mL) compared with the control group (157±40 to 165±45 mL; P<0.03) and reduced microvascular obstruction ( P=0.05) on baseline magnetic resonance imaging and significantly less adverse LV remodeling compared with control subjects with microvascular obstruction ( P<0.05). No significant adverse events were associated with the postconditioning protocol and all patients but one (hemorrhagic stroke) survived through 1 year of follow-up. CONCLUSIONS: We found no early benefit of postconditioning on infarct size, myocardial salvage index, and LV function compared with routine PCI. However, postconditioning was associated with improved LV remodeling at 1 year of follow-up, especially in subjects with microvascular obstruction. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov . Unique identifier: NCT01324453.

Cardiovascular imaging trends in congenital heart disease: a single center experience.

BACKGROUND: Cardiac magnetic resonance imaging (MRI) and CT are available in the recent era at many pediatric cardiac centers. OBJECTIVE: The aim was to provide a contemporary description of diagnostic imaging trends for definition of congenital heart disease (CHD). METHODS: Echocardiography, cardiac catheterization, cardiac MRI, and cardiac CT use in patients with congenital heart disease at a single institution was retrospectively recorded (2005-2012). Surgical procedures were recorded. Total and modality-specific rates were estimated by Poisson regression and compared. The median age, studies in patients aged >17 years, and referral diagnosis were tabulated for the last year of review. RESULTS: An average of 11,940 cardiovascular diagnostic tests was performed annually. The number of total studies, echocardiograms, catheterizations, and surgical procedures, did not change significantly across time. Echocardiography comprised 95% to 97% of all studies performed during each year of review. The use of cardiac MRI (2%) and cardiac CT (1%) increased linearly (P < .001), and the use of diagnostic catheterization decreased (0.7%; P = .0005). The median age was 3 years for echocardiography, 15 years for MRI, 11 years for CT, and 3 years for catheterization. The percentage of patients aged >17 years was 9% for echocardiography, 33% for cardiac MRI, 29% for cardiac CT, and 8% for catheterization. Most patients undergoing CT, MRI, and diagnostic catheterization had moderate or complex CHD. CONCLUSION: Cardiac CT is used increasingly in the recent era for evaluation of CHD. The increased use of both cardiac CT and cardiac MRI are temporally associated with a decrease in diagnostic cardiac catheterization.

Safety and accuracy of dual-source coronary computed tomography angiography in the pediatric population.

BACKGROUND: High heart rates and radiation sensitivity have limited the use of coronary computed tomography angiography (CTA) in pediatric patients. OBJECTIVE: A contemporary evaluation of image quality and reduction in radiation exposure with dual-source CT technology has not been reported in a large cohort of pediatric patients undergoing coronary angiography. METHODS: Consecutive coronary CTA scans (n = 71) in 70 pediatric patients were retrospectively reviewed. Metoprolol was administered for heart rate control. Scans were divided by acquisition mode into 3 groups: retrospective electrocardiogram (ECG)-triggered spiral, prospective ECG-triggered, and prospective ECG-triggered high-pitch spiral scans. Heart rate, radiation dose, image quality, and diagnostic confidence were compared between groups. RESULTS: Median decrease in heart rate with metoprolol was 24% ± 14%. Median effective age-adjusted radiation dose for the entire group was 0.97 ± 1.20 mSv. Retrospective ECG-triggered scans had a median dose of 1.71 ± 1.4 mSv, prospectively ECG-triggered scans had a median dose of 0.9 ± 1.1 mSv, and prospectively ECG-triggered high-pitch spiral scans had a median effective dose of 0.27 ± 0.4 mSv. The difference between groups was statistically significant (P < 0.05). The contrast-to-noise ratio and the image quality score were similar between groups. CONCLUSION: Dual-source coronary CTA with a ß-blocker protocol uniformly achieves diagnostic coronary scans at a low radiation dose in pediatric patients. Image quality and diagnostic confidence are excellent for all scan modes in a wide spectrum of patients.

Assessment of an iterative reconstruction algorithm (SAFIRE) on image quality in pediatric cardiac CT datasets.

BACKGROUND: Pediatric cardiac patients often undergo repeat diagnostic testing, resulting in relatively high cumulative medical radiation exposure. Low-dose CT scanning techniques used to decrease radiation exposure may result in reduced image quality. OBJECTIVE: This study evaluates a prototype iterative reconstruction algorithm, sinogram-affirmed iterative reconstruction (SAFIRE), to determine the effect on qualitative and quantitative measures of image quality in pediatric cardiac CT datasets, compared with a standard weighted filtered back projection (wFBP) algorithm. METHODS: Seventy-four datasets obtained on a 128-slice dual-source CT system were evaluated for image quality using both the wFBP and the prototype iterative reconstruction algorithm. Contrast, noise, contrast-to-noise ratio, signal-to-noise ratio, and qualitative image quality were compared between groups. Data were analyzed as medians and 25th and 75th percentiles, and groups were compared with the use of the Wilcoxon singed-rank test or k sample equality of medians test. RESULTS: There was a 34% decrease in noise, a 41% increase in contrast-to-noise ratio, and a 56% increase in signal-to-noise ratio in the prototype iterative reconstruction, compared with wFBP. All differences were statistically significant (P < 0.001). Qualitative measures of image noise and noise texture were also improved in the iterative reconstruction group (P < 0.001 for both). Diagnostic confidence was similar between reconstruction techniques. Median scan dose length product was 15.5 mGy · cm. CONCLUSION: The prototype iterative reconstruction algorithm studied significantly reduces image noise and improves qualitative and quantitative measures of image quality in low-dose pediatric CT datasets, compared with standard wFBP.

Prevalence and clinical profile of myocardial crypts in hypertrophic cardiomyopathy.

BACKGROUND: In hypertrophic cardiomyopathy (HCM), cardiovascular MR can detect morphological abnormalities of the left ventricle (LV) not visualized with echocardiography. Although myocardial crypts (ie, narrow, blood-filled invaginations within the LV wall) have been recognized in HCM, all clinical implications of these structural abnormalities within the broad clinical HCM spectrum are not completely resolved. Therefore, we sought to characterize the prevalence and diagnostic significance of myocardial crypts in HCM patients. METHODS AND RESULTS: Cine and late gadolinium enhancement cardiovascular MR and 2-dimensional echocardiography were obtained in 292 consecutive patients with HCM including 31 genotype-positive/phenotype-negative family members without LV hypertrophy (28 ± 16 years; 51% male) and 261 patients with LV hypertrophy (46 ± 18 years; 60% male). Ninety-eight subjects without cardiovascular disease were controls. Myocardial crypts (1-6/patient) were identified only by cardiovascular MR in 19 of 31 genotype-positive/phenotype-negative patients (61%) compared with only 10 of 261 (4%) patients with HCM with LV hypertrophy (P<0.001) and were absent in control subjects. Twelve-lead electrocardiograms were normal in 10 (53%) of the genotype-positive/phenotype-negative patients with crypts. Crypts were confined to the basal LV, most commonly in the ventricular septum (n=21) or posterior LV free wall (n=4), and associated with normal LV contractility and absence of late gadolinium enhancement in all but one patient. CONCLUSIONS: LV myocardial crypts represent a distinctive morphological expression of HCM, occurring with different frequency in HCM patients with or without LV hypertrophy. Crypts are a novel cardiovascular MR imaging marker, which may identify individual HCM family members who should also be considered for diagnostic genetic testing. These data support an expanded role for cardiovascular MR in early evaluation of HCM families.

Accuracy and safety of high pitch computed tomography imaging in young children with complex congenital heart disease.

Multidetector computed tomographic angiography defines anatomy in complex congenital heart disease, but radiation exposure and general anesthesia requirements limit its application. The aim of this study was to compare radiation exposure, anesthesia use, and diagnostic accuracy between standard-pitch, single-source computed tomography and high-pitch, dual-source computed tomography for image quality and risk in a clinical pediatric population. Consecutive computed tomographic scans were evaluated in patients aged <2 years with complex congenital heart disease. Two groups were compared on the basis of standard- versus high-pitch scans. High-pitch scans were further divided into variable pitch (2.25 to 3.0) and highest pitch (3.4) groups. Image quality, radiation exposure, anesthesia use, and diagnostic confidence and accuracy were determined. Sixty-one scans were reviewed (29 at standard pitch, 32 at high pitch). Body surface area, scan length, and indications were similar. The median dose-length product for standard-pitch scans was 66 mGy · cm (range 29 to 372) compared to 7 mGy · cm (range 3 to 50) in all high-pitch scans. The median dose-length product was 28 mGy · cm (range 8 to 50) for variable high-pitch scans and 5 mGy · cm (range 3 to 12) for the highest fixed-pitch scans. Diagnostic confidence was similar, although high-pitch scans had higher image noise and lower contrast-to-noise ratios. All high-pitch scans were performed under sedation with free breathing, and all standard-pitch scans required general anesthesia. Diagnostic accuracy was 100% in the 2 groups, with 17 standard-pitch and 16 high-pitch patients undergoing procedural validation. In conclusion, high-pitch, dual-source computed tomography provides excellent diagnostic accuracy and markedly reduces radiation dose, although image quality is mildly reduced.

"Speckled" ventricular septum in hypertrophic cardiomyopathy revisited after 30 years.

We found a highly inconsistent relation between the granular and reflective ultrasound ("speckling") pattern frequently observed in the ventricular septum of patients with hypertrophic cardiomyopathy and evidence of myocardial fibrosis by contrast-enhanced cardiovascular magnetic resonance imaging. Therefore, this distinctive echocardiographic appearance of the myocardium does not accurately characterize left ventricular scarring and is most likely explained as an extraneous ultrasound signal pattern. In conclusion, myocardial fibrosis in patients with hypertrophic cardiomyopathy is most reliably identified using contrast-enhanced cardiovascular magnetic resonance imaging.

Novel hypertrophic cardiomyopathy phenotype: segmental hypertrophy isolated to the posterobasal left ventricular free wall.

Few other diseases show the degree of phenotypic heterogeneity expressed by HC. The two novel patients reported here with isolated posterobasal LV free wall hypertrophy (and mitral valve prolapse) extend this morphologic diversity even farther, now 3 decades after the introduction of contemporary 2-dimensional imaging.

Clinical utility of coronary CT angiography: coronary stenosis detection and prognosis in ambulatory patients.

INTRODUCTION: Multislice CT coronary angiography (MSCTA) accurately detects stenosis in patients undergoing coronary arteriography, but its accuracy in clinical outpatients is less certain. This study retrospectively analyzed MSCTA performance in a large outpatient cohort and examined 6-month clinical follow-up in those without invasive CA. METHODS: Patients underwent MSCTA for clinical indications including symptoms or noninvasive results being either equivocal or suspected as incorrect by referring clinicians. Standard 16-slice CT scanner techniques were used, and results were analyzed on the basis of both patient and vessel. Patients were treated medically or sent to invasive angiography on the basis of MSCTA results and judgment of referring clinicians. All invasive angiograms were analyzed using quantitative coronary angiography. Six-month clinical follow-up was determined in patients without CA. RESULTS: One thousand fifty-three consecutive patients were referred for MSCTA, resulting in 994 interpretable scans. Mean age was 58+/-13 years, 55% were male, 50% had prior noninvasive testing, and 90% had symptoms. Invasive angiography was performed in 160 patients, with significant stenoses present in 69%. MSCTA demonstrated 87% and 89% accuracy by patient- and vessel-based analysis, respectively, and was most accurate in the left main and right coronary arteries. Only two patients not referred for angiography had significant stenosis in those undergoing 6-month follow-up. CONCLUSIONS: MSCTA accurately detects obstructive coronary stenosis in clinical patients with possible cardiac symptoms, and effectively triages them for invasive angiography. Negative results are highly accurate in ruling out obstructive disease. Six-month prognosis is excellent in patients without significant disease determined by MSCT.

Acute and reversible cardiomyopathy provoked by stress in women from the United States.

BACKGROUND: A clinical entity characterized by acute but rapidly reversible left ventricular (LV) systolic dysfunction and triggered by psychological stress is emerging, with reports largely confined to Japan. METHODS AND RESULTS: Over a 32-month period, 22 consecutive patients with this novel cardiomyopathy were prospectively identified within a community-based practice in the Minneapolis-St. Paul, Minn, area. All patients were women aged 32 to 89 years old (mean 65+/-13 years); 21 (96%) were > or =50 years of age. The syndrome is characterized by (1) acute substernal chest pain with ST-segment elevation and/or T-wave inversion; (2) absence of significant coronary arterial narrowing by angiography; (3) systolic dysfunction (ejection fraction 29+/-9%), with abnormal wall motion of the mid and distal LV, ie, "apical ballooning"; and (4) profound psychological stress (eg, death of relatives, domestic abuse, arguments, catastrophic medical diagnoses, devastating financial or gambling losses) immediately preceding and triggering the cardiac events. A significant proportion of patients (37%) had hemodynamic compromise and required vasopressor agents and intra-aortic balloon counterpulsation. Each patient survived with normalized ejection fraction (63+/-6%; P<0.001) and rapid restoration to previous functional cardiovascular status within 6+/-3 days. In 95%, MRI identified diffusely distributed segmental wall-motion abnormalities that encompassed LV myocardium in multiple coronary arterial vascular territories. CONCLUSIONS: A reversible cardiomyopathy triggered by psychologically stressful events occurs in older women and may mimic evolving acute myocardial infarction or coronary syndrome. This condition is characterized by a distinctive form of systolic dysfunction that predominantly affects the distal LV chamber and a favorable outcome with appropriate medical therapy.

Treatment efficacy of noncontingent reinforcement during brief and extended application.

We evaluated the long-term therapeutic effects of noncontingent reinforcement (NCR). In Experiment 1, NCR effects were examined with 2 participants' arbitrary responses; in Experiment 2, NCR was used as treatment with 3 participants whose self-injurious behavior (SIB) was maintained by automatic reinforcement. In both experiments, NCR consisted of continuous access to a highly preferred leisure item and was implemented initially during 10-min and later during 120-min sessions. Varied reinforcers (leisure items) were subsequently introduced during 120-min sessions to determine if treatment effects might be extended. Finally (Experiment 2 only), NCR was implemented throughout the day in participants' homes. Results of Experiments 1 and 2 showed that reinforcers obtained through object manipulation can compete with those obtained automatically by engaging in SIB during brief NCR sessions. However, data from the 120-min sessions indicated that satiation to a specific leisure item might occur over periods of time more typical of those during which treatment would be implemented. Access to a variety of highly preferred leisure items extended the effectiveness of NCR for some individuals. When NCR was implemented throughout the day (Experiment 2), therapeutic effects were shown to be maintained for up to 1 year.

Response-restriction analysis: I. Assessment of activity preferences.

We used procedures based on response-restriction (RR) analysis to assess vocational and leisure activity preferences for 3 adults with developmental disabilities. To increase the efficiency of the analysis relative to that reported in previous research, we used criteria that allowed activities to be restricted at the earliest point at which a preference could be determined. Results obtained across two consecutive RR assessments showed some variability in overall preference rankings but a high degree of consistency for highly ranked items. Finally, we compared results of the RR assessment with those of an extended free-operant assessment and found that the RR assessment yielded (a) more differentiated patterns of preference and (b) more complete information about engagement with all of the target activities.

Response-restriction analysis: II. Alteration of activity preferences.

We used response-restriction (RR) assessments to identify the preferences of 7 individuals with mental retardation for a variety of vocational and leisure activities. We subsequently increased their engagement in nonpreferred activities using several procedures: response restriction per se versus a Premack-type contingency (Study 1), supplemental reinforcement for engagement in target activities (Study 2), and noncontingent pairing of reinforcers with nonpreferred activities (Study 3). Results indicated that preferences are not immutable and can be altered through a variety of relatively benign interventions and that the results of RR assessments may be helpful in determining which types of procedures may be most effective on an individual basis.