Differences and Disparities Among Self-Referred and Physician-Referred Populations Undergoing Coronary Artery Calcium Scanning.

BACKGROUND: Coronary artery calcium computed tomography (CAC) is an important tool for identifying subclinical atherosclerosis and cardiovascular risk stratification. Despite robust evidence and inclusion in current guidelines, CAC is considered investigational by some US insurance carriers and requires out-of-pocket expenses. CAC can be obtained via self-referral (SR) or physician referral (PR). We aimed to examine differences in patient, socioeconomic, and CAC characteristics between referral groups. METHODS: We evaluated demographic, medical history, and CAC results of consecutive patients with a CAC completed at one of multiple Wisconsin sites from March 1, 2019, to June 30, 2021. We separated patients into SR and PR groups. Through census data, we analyzed socioeconomic variables at the block level including race and ethnicity, median income, average household size, and high school completion in the areas where patients resided at the time of CAC. RESULTS: The final analysis included 19 726 patients: 13 835 (70.1%) PR and 5891 (29.9%) SR. Most patients in both groups were White (95.2% versus 95.1%), with the Black/African American population representing 2.7% (SR) and 2.3% (PR). The PR group had a higher prevalence of cardiovascular risk factors. SR patients were more likely to have a score of 0 (41.2% versus 38.1%; P<0.001); PR patients had a higher prevalence of CAC >300 (16.8% versus 14.8%; P<0.001). SR patients were more likely to be women (55.1% versus 48.9%; P<0.001) and were found to live in higher income areas (19.5% versus 16.4%; P<0.001). Patients from low-income areas comprised the smallest proportion in both groups (7.5%). CONCLUSIONS: Patients who obtain out-of-pocket CAC live predominantly in medium- and high-income areas, and patients from lower income locations are less likely to obtain CAC despite having more cardiovascular disease risk factors. Consideration should be made from a policy perspective to promote health equity and improve utilization of CAC testing among underrepresented groups.

Biventricular Arrhythmogenic Cardiomyopathy Mimicking Cardiac Sarcoidosis.

Noninvasive imaging is crucial for diagnosing and managing arrhythmogenic cardiomyopathy. Despite advanced multimodality imaging tools, challenges persist in differentiating it from other arrhythmogenic diseases (eg, cardiac sarcoidosis). We present a case of arrhythmogenic cardiomyopathy with an FLNC variant of uncertain significance exhibiting a phenocopy of cardiac sarcoidosis.

Hypertrophic Cardiomyopathy-Septal Perforator Arteries Doppler Flow Dynamics.

BACKGROUND: Patients with hypertrophic cardiomyopathy (HCM) have been shown to exhibit abnormal diastolic vessel flow; however, flow pattern profiles and their possible association with different grades of diastolic dysfunction have not been studied. Color Doppler two-dimensional echocardiography permits visualization of the septal perforator arteries, and pulsed-wave Doppler allows recording of diastolic septal artery flow (SAF). Through routine visualization of the septal perforator arteries and acquisition of SAF, we noticed 3 patterns of SAF in patients with HCM. In this study, we aimed to assess the feasibility of the acquisition of SAF and to describe types of SAF in an HCM cohort and their associations with diastolic function. METHODS: We reviewed two-dimensional echocardiograms and the electronic records of 108 patients with HCM in whom septal artery color and spectral Doppler had been performed. The peak diastolic and end-diastolic velocities, diastolic slope, diastolic flow time-velocity integral, and systolic flow reversal of the septal perforator arteries were recorded with pulsed-wave Doppler. Echocardiographic and clinical characteristics were analyzed. RESULTS: A reproducible pulsed-wave Doppler tracing was recorded in 54% of patients with HCM. Three distinct patterns of SAF were identified: type 1-smooth, linear holodiastolic velocity decrease; type 2-with presence of an atrial dip; and type 3-biphasic velocity decrease with an early, rapid diastolic slope and a mid-to-late gentle slope. These 3 SAFs correlated with different grades of diastolic dysfunction. CONCLUSION: Septal artery flow could be detected in more than 50% of patients with HCM. Three distinct types of SAF were identified, correlating with different grades of diastolic dysfunction. These 3 types of SAF can provide additional information about left ventricular end-diastolic pressure and diastolic function in patients with HCM in whom diastolic function may be difficult to determine.

Massive Left Ventricular Hypertrophy With Acromegaly: Hypertrophic Obstructive Cardiomyopathy Phenocopy or Phenotype?

Acromegaly-a rare endocrine disorder-results when a growth hormone-secreting somatotroph pituitary adenoma leads to increased insulin-like growth factor 1 production. Acromegaly is known to cause left ventricular hypertrophy. We present a case of acromegaly with massive left ventricular hypertrophy that was determined to be coexistent with gene-positive hypertrophic obstructive cardiomyopathy. (Level of Difficulty: Intermediate.).

Myocardial work indices in bileaflet mitral valve prolapse patients.

AIMS: Mechanisms underlying left ventricular dysfunction and arrhythmogenesis in bileaflet mitral valve prolapse (BMVP) patients are not well defined. Myocardial work index (MWI) is a noninvasive assessment that correlates with myocardial oxygen consumption. We aimed to compare global and regional MWI in BMVP patients with normal controls. METHODS AND RESULTS: In this retrospective study, we calculated MWI in BMVP patients and controls using GE EchoPAC (GE Healthcare, Chicago, IL) software. Of 147 BMVP patients (59% women, mean age 54 ± 15 years), 16 had a flail mitral leaflet. There was regional heterogeneity in MWIs, with increased posterolateral trident myocardial work (2099 ± 271 vs. 1895 ± 321 mm Hg%, P = .039), constructive work (2831 ± 366 vs. 2257 ± 338 mm Hg%, P < .001), wasted work (87 [52-194] vs. 71 [42-103] mm Hg%, P = .015), peak systolic strain (-23.0 ± 2.4 vs. -19 ± 3%, P < .001), and reduction in myocardial work efficiency (95.00 [93.50-97.75] vs. 96.75 (95.00-97.75) %, P = 0.020) in 100 BMVP patients compared with age- and sex-matched controls. BMVP patients' basal septal wall MWIs were lower than those of controls. The higher work indices in patients with BMVP were reduced in those who developed flail leaflets. No significant differences in work indices were seen between ventricular arrhythmia and non-arrhythmia BMVP patients. CONCLUSION: Regional differences in MWIs were noted in the BMVP patients compared with controls, with overall reduced myocardial efficiency in the posterolateral trident and basal septal regions. In cross-sectional analysis, MWIs were not different in patients with ventricular tachyarrhythmias. Impact of MWI in long-term prognosis needs to be determined.

Myocardial Work in Apical Hypertrophic Cardiomyopathy.

BACKGROUND: Pressure-strain loop analysis is a novel echocardiographic technique to calculate myocardial work indices that has not been applied to patients with apical hypertrophic cardiomyopathy (ApHCM). We hypothesized that myocardial work indices differ between patients with ApHCM and those with non-ApHCM. This study aimed to (1) evaluate myocardial work indices in patients with ApHCM compared with those with non-ApHCM, (2) describe associations with relevant clinical variables, and (3) examine associations with significant late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging. METHODS: We retrospectively identified 48 patients with ApHCM and 69 with non-ApHCM who had measurements of global longitudinal strain (GLS), global work index (GWI), global constructive work (GCW), global wasted work, and global work efficiency. We evaluated available cardiac magnetic resonance imaging data on 34 patients with ApHCM and 51 with non-ApHCM. Multivariable regression models correcting for traditional cardiac risk factors were used to evaluate the associations of myocardial work indices with relevant clinical variables. RESULTS: Median GLS (-11% vs -18%, P < .001), GWI (966 mm Hg% vs 1803 mm Hg%, P < .001), and GCW (1,050 mm Hg% vs 1,988 mm Hg%, P < .001) were significantly impaired in patients with ApHCM compared with those with non-ApHCM. Increasing N-terminal pro b-type natriuretic peptide, abnormal ultrasensitive troponin, and increasing maximal left ventricular wall thickness were significantly associated with reduced GWI and GCW in patients with ApHCM (P < .05). Global constructive work had only modest accuracy (area under the curve [AUC] = 0.70) to predict LGE in patients with ApHCM. However, in patients with non-ApHCM, GLS was the strongest predictor of LGE (AUC = 0.91), with a -17% cutoff yielding 81% sensitivity and 80% specificity. CONCLUSION: Myocardial work indices are significantly impaired in patients with ApHCM compared to those with non-ApHCM and correlate with important clinical variables. Global longitudinal strain, GWI, and GCW are more strongly predictive of fibrosis in patients with non-ApHCM than ApHCM.

Hypertrophic cardiomyopathy in identical twins: a case series.

Background: Hypertrophic cardiomyopathy in identical twins is rare. Cases of hypertrophic cardiomyopathy with homogenous and heterogeneous phenotypes have been described in the literature. Case summary: We report a pair of monozygotic twins (Twin A and Twin B) with identical morphological expression of hypertrophic cardiomyopathy. On initial evaluation, both twins had resting left ventricular outflow tract obstruction, Grade II diastolic dysfunction, and New York Heart Association (NYHA) Class II symptoms, but they had a different clinical course afterward. Twin A progressed from NYHA Class II to Class III with a high left ventricular outflow tract pressure gradient that was unresponsive to medical treatment and required alcohol septal ablation. Twin B responded very well to medical treatment. Both patients had no risk factors for sudden cardiac death, and neither required an implantable cardioverter defibrillator. Discussion: The morphology of hypertrophic cardiomyopathy has a strong genetic basis, but epigenetic factors may affect disease expression.

Pulmonary Artery Intimal Sarcoma Mimicking Right Ventricular Outflow Tract Thrombus.

A 72-year-old man presented with breathlessness and a systolic murmur. Extensive diagnostic work-up revealed a rare pulmonary artery intimal sarcoma mimicking a right ventricular outflow tract thrombus and impacting a cardiac pacemaker lead. Surgical resection, pathology confirmation, and management strategies are discussed, highlighting the challenges of treating this rare malignancy.

Feasibility of exercise treadmill 13N-ammonia positron emission tomography myocardial perfusion imaging using an off-site cyclotron.

BACKGROUND: Myocardial perfusion imaging with treadmill exercise nitrogen-13 (13N)-ammonia positron emission tomography (PET) presents a logistical challenge. We investigated the feasibility of exercise treadmill (GXT) 13N-ammonia PET MPI using an off-site cyclotron for production of 13N-ammonia. METHODS: Thirty-three patients underwent GXT 13N-ammonia PET MPI over 23 months. 13N-ammonia doses were prepared at an off-site cyclotron. Patients underwent 13N-ammonia resting and 13N-ammonia GXT emission and transmission scans at our facility. Image quality, perfusion data, and clinical variables were evaluated. RESULTS: We analyzed 33 patients (7/26 female/male). Mean age was 63 ± 12 years and mean BMI was 33.7 ± 6.9. GXT PET was feasible in all patients. Image quality was good in 29 patients, adequate in 3, and severely compromised in 1 patient. Summed stress score was 4.5 ± 5.7. Resting and GXT left ventricular ejection fractions were 63.7 ± 10.9% and 66.3 ± 13.1%. TID ratio was 1.0 ± 0.1. CONCLUSIONS: Treadmill exercise 13N-ammonia PET is feasible in a large medical center without access to an on-site cyclotron. This technique requires close coordination with an off-site cyclotron but expands the role of PET to patients for whom exercise is more appropriate than pharmacologic stress imaging.

Charcoal Heart: Metastatic Melanoma Mimicking Right Atrial Myxoma.

A 59-year-old woman with history of skin melanoma and complete excision presented with palpitations. Transthoracic echocardiogram revealed right atrial mass attached to interatrial septum. Cardiac magnetic resonance was suggestive of metastatic melanoma. Laboratory tests revealed elevated liver enzymes. Liver ultrasonography showed a large mass positive for metastatic melanoma by biopsy. (Level of Difficulty: Intermediate.).

Coronary-Bronchial Fistula: Coil the Steal.

Coronary artery fistula is a rare coronary anomaly with communication between the coronary artery and either the cardiac chamber or other vessels. We present a case of coronary-to-bronchial artery fistula that resulted in likely coronary artery steal and was treated with coil embolization. (Level of Difficulty: Beginner.).