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Cardiac fibromas occur in 3% of patients with nevoid basal cell carcinoma syndrome (NBCCS), an autosomal dominant disorder characterized by multiple basal cell carcinomas, intracranial dural calcifications (Figure 1A), and odontogenic keratocysts (Figure 1B-C). Early diagnosis is essential for

Revascularization of coronary chronic total occlusion (CTO) are challenging as often the vessel course is unclear. Coronary computed tomography angiography (CTA) may overcome this shortcoming. A 70 year old man with a RCA CTO (~100mm) (figure 1(a)) and an occluded bypass graft to the RCA presented

Percutaneous treatment of tricuspid regurgitation using devices such as the MitraClip (Abbott) remains technically challenging. We sought to 3D print patient-specific right ventricle (RV) and tricuspid valve (TV) geometry, reconstructed from CT images, to assess the potential utility of such models

Objectives The aim of this study was to determine whether a normal stress-only single-photon emission computed tomographic myocardial perfusion tomography (SPECT) study confers the same prognosis as a normal SPECT on the basis of evaluation of stress and rest images. Background Current guidelines recommend stress and rest imaging to confirm that a SPECT study is normal. Methods We determined all-cause mortality in 16,854 consecutive patients who had a normal gated stress SPECT. Median follow-up was 4.5 years. A stress-only protocol was used in 8,034 patients (47.6%), whereas 8,820 (52.4%) had both stress and rest imaging. Results The overall unadjusted annual mortality rate in patients who had a normal SPECT with a stress-only protocol was lower than in those who required additional rest imaging (2.57% vs. 2.92%, p = 0.02). After adjustment for baseline clinical characteristics no significant differences in patient mortality were seen between the 2 imaging protocols, but the stress-only group received a 61% lower radiopharmaceutical dosage. Independent predictors of worse survival included increasing age, male sex, diabetes, history of coronary artery disease, and inability to exercise (all p Conclusions Patients determined to have a normal SPECT on the basis of stress imaging alone have a similar mortality rate as those who have a normal SPECT on the basis of evaluation of both stress and rest images. Our results support that additional rest imaging is not required in patients who have a normally appearing initial stress study. A significant reduction in radiation exposure can be achieved with such an approach.

Objectives The purpose of this paper is to examine the incidence and determinants of permanent complete heart block (CHB) after nonsurgical septal reduction therapy (NSRT), and to evaluate the clinical impact of permanent pacemaker (PPM) placement. Background Nonsurgical septal reduction therapy with ethanol improves the clinical and hemodynamic parameters in patients with symptomatic hypertrophic obstructive cardiomyopathy. Complete heart block is a common complication after NSRT. Methods The database of 261 consecutive patients who underwent NSRT at Baylor College of Medicine was reviewed. Clinical variables that were considered as possible determinants for CHB after NSRT were: age, gender, New York Heart Association (NYHA) functional class, left ventricular outflow tract (LVOT) gradient at rest or with provocation, septal thickness, and baseline exercise duration. For electrocardiographic (ECG) variables, the presence of first-degree atrioventricular (AV) block, bifascicular block, left bundle branch block, atrial fibrillation, and left ventricular hypertrophy were analyzed. In addition, the volume of ethanol injected, the method of administration of ethanol (i.e., bolus vs. slow injection [over 30 to 60 s]), number of septal arteries occluded, use of myocardial echocardiography, and infarct size as determined by peak creatine kinase level. Results Of 261 consecutive patients, 37 had PPM or automatic implantable cardiac defibrillator placed before NSRT. Of the remaining 224 patients, 31 (14%) developed CHB after the procedure. Multivariate logistic regression analysis showed that female gender (odds ratio [OR] 4.3; p = 0.02), bolus injection of ethanol (OR 51; p = 0.004), injecting more than one septal artery (OR 4.6; p = 0.016), the presence of left bundle branch block (OR 39; p = 0.002), and first-degree AV block (OR 14; p = 0.001) on the baseline ECG are independent predictors of CHB after NSRT. Patients requiring PPM placement had a similar improvement in their NYHA functional class, septal thickness reduction, LVOT gradient reduction, and improvement of exercise capacity when compared with patients who did not require pacing. Conclusions Multiple demographic, electrocardiographic, and technical factors seem to increase the risk of CHB after NSRT. Patients with CHB after NSRT derive similar clinical and hemodynamic benefit to patients who did not require permanent pacing.

Prediction of an optimal C–arm angle that aligns the coronary sinuses for valve deployment is critical during TAVR. Current solutions derive the deployment angle from multiple angiograms, reconstruction of multi–slice CT (MSCT) or C–arm CT rotational angiography (CTRA). We evaluated a method

The objective of this study was to investigate the predictive value of CACS obtained by noncontrast electrocardiogram-gated CT for incident ischemic stroke. We search Pubmed, Embase, Cochrane databases for prospective longitudinal studies of CACS which reported the incidence of ischemic stroke.

The aim of this study was to determine whether a normal stress-only single-photon emission computed tomographic myocardial perfusion tomography (SPECT) study confers the same prognosis as a normal SPECT on the basis of evaluation of stress and rest images.Current guidelines recommend stress and rest imaging to confirm that a SPECT study is normal.We determined all-cause mortality in 16,854 consecutive patients who had a normal gated stress SPECT. Median follow-up was 4.5 years. A stress-only protocol was used in 8,034 patients (47.6%), whereas 8,820 (52.4%) had both stress and rest imaging.The overall unadjusted annual mortality rate in patients who had a normal SPECT with a stress-only protocol was lower than in those who required additional rest imaging (2.57% vs. 2.92%, p = 0.02). After adjustment for baseline clinical characteristics no significant differences in patient mortality were seen between the 2 imaging protocols, but the stress-only group received a 61% lower radiopharmaceutical dosage. Independent predictors of worse survival included increasing age, male sex, diabetes, history of coronary artery disease, and inability to exercise (all p0.001) but not the type of SPECT protocol used to image patients.Patients determined to have a normal SPECT on the basis of stress imaging alone have a similar mortality rate as those who have a normal SPECT on the basis of evaluation of both stress and rest images. Our results support that additional rest imaging is not required in patients who have a normally appearing initial stress study. A significant reduction in radiation exposure can be achieved with such an approach.

ObjectivesThis study sought to examine the relationship between coronary artery calcium score (CACS) and single-photon emission computed tomography (SPECT) results for predicting the short- and long-term risk of cardiac events.BackgroundThe CACS and SPECT results both provide important prognostic information. It is unclear whether integrating these tests will better predict patient outcome.MethodsWe followed-up 1,126 generally asymptomatic subjects without previous cardiovascular disease who had a CACS and stress SPECT scan performed within a close time period (median 56 days). The median follow-up was 6.9 years. End points analyzed were total cardiac events and all-cause death/myocardial infarction (MI).ResultsAn abnormal SPECT result increased with increasing CACS from 400) (p < 0.001). Total cardiac events and death/MI also increased with increasing CACS and abnormal SPECT results (p < 0.001). In subjects with a normal SPECT result, CACS added incremental prognostic information, with a 3.55-fold relative increase for any cardiac event (2.75-fold for death/MI) when the CACS was severe (>400) versus minimal (≤10). Separation of the survival curves occurred at 3 years after initial testing for all cardiac events and at 5 years for death/MI.ConclusionsThe CACS and SPECT findings are independent and complementary predictors of short- and long-term cardiac events. Despite a normal SPECT result, a severe CACS identifies subjects at high long-term cardiac risk. After a normal SPECT result, our findings support performing a CACS in patients who are at intermediate or high clinical risk for coronary artery disease to better define those who will have a high long-term risk for adverse cardiac events.

ObjectivesThe purpose of this study is to investigate the morphologic characteristics of the cardiac interstitium in the hibernating human myocardium and evaluate whether active remodeling is present and is an important determinant of functional recovery.BackgroundMyocardial hibernation is associated with structural myocardial changes, which involve both the cardiomyocytes and the cardiac interstitium.MethodsWe evaluated 15 patients with coronary disease with two-dimensional echocardiography and thallium-201 (201Tl) tomography before coronary bypass surgery. During surgery, transmural myocardial biopsies were performed guided by transesophageal echocardiography. Myocardial biopsies were stained immunohistochemically to investigate fibroblast phenotype and examine evidence of active remodeling in the heart.ResultsAmong the 29 biopsied segments included in the study, 24 showed evidence of systolic dysfunction. The majority of dysfunctional segments (86.4%) were viable (201Tl uptake ≥60%). After revascularization, 12 dysfunctional segments recovered function as assessed with an echocardiogram three months after bypass surgery. Interstitial fibroblasts expressing the embryonal isoform of smooth muscle myosin heavy chain (SMemb) were noted in dysfunctional segments, predominantly located in border areas adjacent to viable myocardial tissue. Segments with recovery had higher SMemb expression (0.46 ± 0.16% [n = 12] vs. 0.10 ± 0.02% [n = 12]; p < 0.05) and a higher ratio of alpha-smooth muscle actin to collagen (0.14 ± 0.026 [n = 12] vs. 0.07 ± 0.01 [n = 12]; p < 0.05) compared with segments without recovery, indicating fibroblast activation and higher cellularity of the fibrotic areas. In addition, interstitial deposition of the matricellular protein tenascin, a marker of active remodeling, was higher in hibernating segments than in segments with persistent dysfunction (p < 0.05), suggesting an active continuous fibrotic process. Multiple logistic regression demonstrated a significant independent association between SMemb expression and functional recovery (p < 0.01).ConclusionsFibroblast activation and expression of SMemb and tenascin provide evidence of continuous remodeling in the cardiac interstitium of the hibernating myocardium, an important predictor of recovery of function after revascularization.

Added predictive ability of Agatston coronary artery calcium score (CACS) to coronary computed tomography angiography (CCTA) for prediction of adverse cardiovascular events has not been well described. A total of 805 patients without known history of coronary artery disease (CAD) who had chest pain

Coronary Computed Tomographic Angiography (CCTA) with prospective ECG gating (PG) reduces radiation exposure as compared to retrospective ECG gating (RG), yet its prognostic value is less validated. Our aim was to compare prognostic performance of 64 slice CCTA with PG vs RG in patients (pts) with

Computed tomography (CT) provides high-resolution images of the aortic valve with clear localization of calcium deposition. Three-dimensional (3D) stereolithographic printing can be used to convert these data into a physical model [(1,2)][1]. We hypothesized that patient-specific, multimaterial, 3D

Long-term prognostic value of renal function and coronary artery calcium score (CACS) in symptomatic patients without known coronary artery disease (CAD) is unclear. Renal function and CACS was assessed in patients without known CAD who presented with chest pain to our emergency room. Chronic

[Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4] A 40-year-old man with known cystic fibrosis (CF) was referred to our institution for cardiac evaluation as part of pre-lung transplantation assessment. Left heart catheterization revealed multiple fistulas arising from all 3