Management of Cardiac Sarcoidosis Using Mycophenolate Mofetil as a Steroid-Sparing Agent.

BACKGROUND: Cardiac sarcoidosis (CS) is a major cause of morbidity and mortality in patients with systemic sarcoidosis. Steroid-sparing agents are increasingly used, despite a lack of randomized trials or published guidelines to direct treatment. METHODS AND RESULTS: This retrospective study included 77 patients with CS treated with prednisone monotherapy (n = 32) or a combination with mycophenolate mofetil (n = 45) between 2003 and 2018. Baseline characteristics and clinical outcomes were evaluated. The mean patient age was 53 ± 11 years at CS diagnosis, 66.2% were male, and 35.1% were Black. The total exposure to maximum prednisone dose (initial prednisone dose × days at dose) was lower in the combination therapy group (1440 mg [interquartile range (IQR), 1200-2760 mg] vs 2710 mg [IQR, 1200-5080 mg]; P = .06). On 18F-fluorodeoxyglucose positron emission tomography scans, both groups demonstrated a significant decrease in the cardiac maximum standardized uptake value after treatment: a median decrease of 3.9 (IQR 2.7-9.0, P = .002) and 2.9 (IQR 0-5.0, P = .001) for prednisone monotherapy and combination therapy, respectively. Most patients experienced improvement or complete resolution in qualitative cardiac 18F-fluorodeoxyglucose uptake (92.3% and 70.4% for the prednisone and combination therapy groups, respectively). Mycophenolate mofetil was well tolerated. CONCLUSIONS: Mycophenolate mofetil in combination with prednisone for the treatment of CS may minimize corticosteroid exposure and decrease cardiac inflammation without significant adverse effects.

Clinical and Imaging Response to Tumor Necrosis Factor Alpha Inhibitors in Treatment of Cardiac Sarcoidosis: A Multicenter Experience.

BACKGROUND: Cardiac sarcoidosis (CS) is an increasingly recognized cause of cardiomyopathy; however, data on immunosuppressive strategies are limited. Treatment with tumor necrosis factor (TNF) alpha inhibitors is not well described; moreover, there may be heart failure-related safety concerns. METHODS: Retrospective multicenter study of patients with CS treated with TNF alpha inhibitors. Baseline characteristics, treatments, and outcomes were adjudicated. RESULTS: Thirty-eight patients with CS (mean age 49.9 years, 42% women, 53% African American) were treated with TNF alpha inhibitor (30 infliximab, 8 adalimumab). Prednisone dose decreased from time of TNF alpha inhibitor initiation (21.7 ± 17.5 mg) to 6 months (10.4 ± 6.1 mg, P = .001) and 12 months (7.3 ± 7.3 mg, P = .002) after treatment. On pre-TNF alpha inhibitor treatment positron emission tomography with 18-flourodoxyglucose (FDG-PET), 84% of patients had cardiac FDG uptake. After treatment, there was a significant decrease in number of segments involved (3.5 ± 3.8 to 1.0 ± 2.5, P = .008) and maximum standardized uptake value (3.59 ± 3.70 to 0.57 ± 1.60, P = .0005), with 73% of patients demonstrating complete resolution or improvement of cardiac FDG uptake. The left ventricular ejection fraction remained stable (45.0 ± 16.5% to 47.0 ± 15.0%, P = .10). Four patients required inpatient heart failure treatment, and 8 had infections; 2 required treatment cessation. CONCLUSIONS: TNF alpha inhibitor treatment guided by FDG-PET imaging may minimize corticosteroid use and effectively reduce cardiac inflammation without significant adverse effect on cardiac function. However, infections were common, some of which were serious, and therefore patients require close monitoring for both infection and cardiac symptoms.

Coronary microvascular disease: coronary flow reserve and the complementary role of positron emission tomography and angiography.

We present the case of a woman in her 30s with chronic residual chest pain accompanied by dyspnoea on exertion despite multiple evaluations and cardiac testing over a period of nearly 14 years. Ultimately, she underwent N-13 Ammonia positron emission tomography (PET) myocardial perfusion imaging, which demonstrated mild three-vessel microvascular disease. Coronary microvascular disease (CMD) plays a major role in determining myocardial ischaemia in many cardiovascular conditions. The diagnosis relies on indirect measurement of coronary flow reserve (CFR), which may be evaluated both invasively and non-invasively. Assessing CFR not only allows for successful diagnosis of CMD but holds powerful prognostic value for cardiovascular mortality. PET myocardial perfusion imaging is a complementary tool to coronary angiography to achieve diagnosis in a non-invasive, highly accurate and reproducible manner.

Advanced imaging for risk stratification for ventricular arrhythmias and sudden cardiac death.

Sudden cardiac death (SCD) is a leading cause of mortality, comprising approximately half of all deaths from cardiovascular disease. In the US, the majority of SCD (85%) occurs in patients with ischemic cardiomyopathy (ICM) and a subset in patients with non-ischemic cardiomyopathy (NICM), who tend to be younger and whose risk of mortality is less clearly delineated than in ischemic cardiomyopathies. The conventional means of SCD risk stratification has been the determination of the ejection fraction (EF), typically via echocardiography, which is currently a means of determining candidacy for primary prevention in the form of implantable cardiac defibrillators (ICDs). Advanced cardiac imaging methods such as cardiac magnetic resonance imaging (CMR), single-photon emission computerized tomography (SPECT) and positron emission tomography (PET), and computed tomography (CT) have emerged as promising and non-invasive means of risk stratification for sudden death through their characterization of the underlying myocardial substrate that predisposes to SCD. Late gadolinium enhancement (LGE) on CMR detects myocardial scar, which can inform ICD decision-making. Overall scar burden, region-specific scar burden, and scar heterogeneity have all been studied in risk stratification. PET and SPECT are nuclear methods that determine myocardial viability and innervation, as well as inflammation. CT can be used for assessment of myocardial fat and its association with reentrant circuits. Emerging methodologies include the development of "virtual hearts" using complex electrophysiologic modeling derived from CMR to attempt to predict arrhythmic susceptibility. Recent developments have paired novel machine learning (ML) algorithms with established imaging techniques to improve predictive performance. The use of advanced imaging to augment risk stratification for sudden death is increasingly well-established and may soon have an expanded role in clinical decision-making. ML could help shift this paradigm further by advancing variable discovery and data analysis.

Large Pleural Effusion: A Pitfall in the Quantitation of 99m Tc-PYP Imaging for ATTR Cardiac Amyloidosis.

ABSTRACT: Transthyretin cardiac amyloidosis (ATTR and ATTRv) is an underrecognized cause of heart failure that results from myocardial deposition of misfolded protein (TTR or prealbumin). The diagnosis can be confirmed by uptake of 99m Tc-pyrophosphate ( 99m Tc-PYP) in the heart with serologic studies to rule out light chain disease. We present the case of a 70-year-old woman who underwent a 99m Tc-PYP scan. The patient had a large right-sided pleural effusion that lowered counts in the right chest on planar imaging, interfered with ratio-based grading of PYP uptake, and highlighted the importance of obtaining SPECT/CT for problem-solving in cases where uptake ratios may be spurious.

Association of True Positivity with Serum Prostate-Specific Antigen Levels and Other Clinical Factors in Indeterminate PSMA-RADS-3A Lesions Identified on 18F-DCFPyL PET/CT Scans.

The use of prostate-specific membrane antigen targeted PET imaging for the evaluation of prostate cancer has increased significantly in the last couple of decades. When evaluating these imaging findings based on the PSMA reporting and data system version 1.0, which categorize lesions based on their likelihood of prostate cancer involvement, PSMA-RADS-3A lesions are commonly seen, which are indeterminate for the presence of disease. A total of 28 patients with 171 PSMA-RADS-3A lesions on 18F-DCFPyL PET/CT scans from June 2016 to May 2017 who had follow-up cross-sectional imaging over time were included in this study. The PSA levels of patients with PSMA-RADS-3A lesions were categorized into four groups, 0-0.2, 0.2-1, 1-2, and >2 ng/mL. The pre-operative Gleason score of these patients was categorized into two groups, Gleason score < 7 or ≥7. The median age for these patients was 72.5 years (range 59-81). The median PSA value for patients with positive lesions was significantly higher than those with negative lesions (5.8 ng/mL vs. 0.2 ng/mL, p < 0.0001). The lesion positivity rate was significantly higher in patients with PSA > 1 ng/mL (18.2% vs. 81.9%, p < 0.001). On ROC analysis, the highest classification accuracy was seen at PSA ≥ 0.6 ng/mL of 80.12% (95% CI = 73.69-86.16%), and the area under the curve was 71.32% (95% CI = 61.9-80.7%, p < 0.0001). A total of 96.4% (108/112) of patients with positive lesions and 86.4% (51/59) of patients with negative lesions had a PSMA-RADS-4/5 lymph node on the initial 18F-DCFPyL PET/CT scan (p = 0.02). In patients with a Gleason score ≥ 7, the presence of positive PSMA-RADS-3A lesions was higher, compared to negative PSMA-RADS-3A lesions (p = 0.049). Higher PSA levels in patients with PSMA-RADS-3A lesions can point towards the presence of true positivity. PSA levels may be considered in deciding whether to call an indeterminate lesion on PSMA PET.

Current State and Future Directions of Multimodality Imaging in Cardiac Sarcoidosis.

Cardiac sarcoidosis (CS) is an increasingly recognized cause of heart failure and arrhythmia. Historically challenging to identify, particularly in the absence of extracardiac sarcoidosis, diagnosis of CS has improved with advancements in cardiac imaging. Recognition as well as management may require interpretation of multiple imaging modalities. Echocardiography may serve as an initial screening study for cardiac involvement in patients with systemic sarcoidosis. Cardiac magnetic resonance imaging (CMR) provides information on diagnosis as well as risk stratification, particularly for ventricular arrhythmia in the setting of late gadolinium enhancement. More recently, 18F-fluorodeoxyglucose position emission tomography (FDG-PET) has assumed a valuable role in the diagnosis and longitudinal management of patients with CS, allowing for the assessment of response to treatment. Hybrid FDG-PET/CT may also be used in the evaluation of extracardiac inflammation, permitting the identification of biopsy sites for diagnostic confirmation. Herein we examine the approach to diagnosis and management of CS using multimodality imaging via a case-based review.

Giant Primary Scrotal Lipoma: A Rare Entity with Diagnostic Pitfalls.

Primary scrotal lipomas are rare. We describe the case of a 47-year-old male with a giant scrotal lipoma who underwent a surgical excision. We report the clinical and radiological approach as well as the treatment of this atypical benign tumor.

Scheimpflug corneal power measurements for intraocular lens power calculation in cataract surgery.

PURPOSE: To compare the keratometric (K) readings from the Pentacam-HR (High Resolution) unit with the automated K values from the IOLMaster keratometer (KIOLM), and to evaluate them in the commonly used intraocular lens (IOL) power calculation formulas for routine cataract surgery. DESIGN: Prospective, comparative observational study. METHODS: setting: Private practice, Lynwood, California. study population: Fifty cataractous eyes scheduled for surgery between July and August 2012. observation procedure: The K readings from the Pentacam-HR unit taken at the 2-, 3-, 4-, and 5-mm zones and the 2-, 3-, 4-, and 5-mm rings, respectively, from 3 different maps: sagittal corneal front (KF), true net power (KTNP), and total refractive power (KRP) are compared with KIOLM. IOL power calculations were performed with each of the 25 sets of K readings. main outcome measures: The IOL prediction median absolute error (MedAE) obtained with each measurement. RESULTS: KF averaged 0.03-0.13 diopter (D) higher than KIOLM (P > .05), KTNP averaged 1.16-1.21 D lower than KIOLM (P > .001), and KRP averaged 0.23-0.72 D lower than KIOLM (P > .001), with large variations in the measurements. The MedAE obtained with the different Pentacam K readings ranged from 0.44-0.64 D vs 0.52 D obtained with KIOLM (P > .05). MedAE was lower in all categories when the pupil was 3 mm or smaller. CONCLUSION: The Pentacam KF values were the closest to KIOLM and the KF readings from the 2-mm ring yielded the best results for IOL power calculation.