Arrhythmogenic Left Ventricular Mass as the Initial Presentation of Cardiac Sarcoidosis.

Cardiac tumors of the left ventricle are rare, and cardiac magnetic resonance is the preferred imaging tool for evaluation given superior tissue characterization. We present a case of a patient with arrhythmia and left ventricular mass that was ultimately diagnosed with cardiac sarcoidosis, reminding us that tissue is the issue.

Cardiac Sarcoidosis.

Cardiac involvement is a major cause of morbidity and mortality in patients with sarcoidosis. It is important to distinguish between clinical manifest diseases from clinically silent diseases. Advanced cardiac imaging studies are crucial in the diagnostic pathway. In suspected isolated cardiac sarcoidosis, it's key to rule out alternative diagnoses. Therapeutic options can be divided into immunosuppressive agents, guideline-directed medical therapy, antiarrhythmic medications, device/ablation therapy, and heart transplantation.

Sleep-Related Hypoxia, Right Ventricular Dysfunction, and Survival in Patients With Group 1 Pulmonary Arterial Hypertension.

BACKGROUND: Group 1 pulmonary arterial hypertension (PAH) is a progressive fatal condition characterized by right ventricular (RV) failure with worse outcomes in connective tissue disease (CTD). Obstructive sleep apnea and sleep-related hypoxia may contribute to RV dysfunction, though the relationship remains unclear. OBJECTIVES: The aim of this study was to prospectively evaluate the association of the apnea-hypopnea index (AHI) and sleep-related hypoxia with RV function and survival. METHODS: Pulmonary Vascular Disease Phenomics (National Heart, Lung, and Blood Institute) cohort participants (patients with group 1 PAH, comparators, and healthy control participants) with sleep studies were included. Multimodal RV functional measures were examined in association with AHI and percentage of recording time with oxygen saturation <90% (T90) per 10-unit increment. Linear models, adjusted for demographics, oxygen, diffusing capacity of the lungs for carbon monoxide, pulmonary hypertension medications, assessed AHI and T90, and RV measures. Log-rank test/Cox proportional hazards models adjusted for demographics, oxygen, and positive airway pressure were constructed for transplantation-free survival analyses. RESULTS: Analysis included 186 participants with group 1 PAH with a mean age of 52.6 ± 14.1 years; 71.5% were women, 80.8% were Caucasian, and there were 43 events (transplantation or death). AHI and T90 were associated with decreased RV ejection fraction (on magnetic resonance imaging), by 2.18% (-2.18; 95% CI: -4.00 to -0.36; P = 0.019) and 0.93% (-0.93; 95% CI: -1.47 to -0.40; P < 0.001), respectively. T90 was associated with increased RV systolic pressure (on echocardiography), by 2.52 mm Hg (2.52; 95% CI: 1.61 to 3.43; P < 0.001); increased mean pulmonary artery pressure (on right heart catheterization), by 0.27 mm Hg (0.27; 95% CI: 0.05 to 0.49; P = 0.019); and RV hypertrophy (on electrocardiography), 1.24 mm (1.24; 95% CI: 1.10 to 1.40; P < 0.001). T90, but not AHI, was associated with a 17% increased 5-year risk for transplantation or death (HR: 1.17; 95% CI: 1.07 to 1.28). In non-CTD-associated PAH, T90 was associated with a 21% increased risk for transplantation or death (HR: 1.21; 95% CI: 1.08 to 1.34). In CTD-associated PAH, T90 was associated with RV dysfunction, but not death or transplantation. CONCLUSIONS: Sleep-related hypoxia was more strongly associated than AHI with measures of RV dysfunction, death, or transplantation overall and in group 1 non-CTD-associated PAH but only with RV dysfunction in CTD-associated PAH. (Pulmonary Vascular Disease Phenomics Program [PVDOMICS]; NCT02980887).

Optimal left ventricular ejection fraction in risk stratification of patients with cardiac sarcoidosis.

AIMS: Identifying patients with cardiac sarcoidosis (CS) who are at an increased risk of sudden cardiac death (SCD) poses a clinical challenge. We sought to identify the optimal cutoff for left ventricular ejection fraction (LVEF) in predicting ventricular arrhythmia (VA) and all-cause mortality and to identify clinical and imaging risk factors in patients with known CS. METHODS AND RESULTS: This retrospective cohort included 273 patients with well-established CS. The primary endpoint was a composite of VA and all-cause mortality. A modified receiver operating curve analysis was utilized to identify the optimal cutoff for LVEF in predicting the primary composite endpoint. Cox proportional hazard regression analysis was used to identify independent risk factors of the outcomes. At median follow-up of 7.9 years, the rate of the primary endpoint was 38% (83 VAs and 32 all-cause deaths). The 5-year overall survival rate was 97%. The optimal cutoff LVEF for the primary composite endpoint was 42% in the entire cohort and in subjects without a history of VA. Younger age, history of VA, lower LVEF, and any presence of scar by cardiac magnetic resonance (CMR) imaging and/or positron emission tomography (PET) were found to be independent risk factors for the primary endpoint and for VA, whereas lower LVEF, baseline NT-proBNP, and any presence of scar were independent risk factor of all-cause mortality. CONCLUSION: Among patients with CS, a mild reduction in LVEF of 42% was identified as the optimal cutoff for predicting VA and all-cause mortality. Prior VA and scar by CMR or PET are strong risk factors for future VA and all-cause mortality.

Impact of Age and Sex on Left Ventricular Remodeling in Patients With Aortic Regurgitation.

BACKGROUND: Current guidelines for aortic regurgitation (AR) recommend the same linear left ventricular (LV) dimension for intervention regardless of age and sex. OBJECTIVES: The purpose of this study was to evaluate the impact of age and sex on the degree of LV remodeling and outcomes. METHODS: We included consecutive patients with severe AR who were serially monitored by echocardiogram between 2010 and 2016. The 2 main endpoints were as follows: 1) LV end-systolic volume indexed to body surface area (LVESVi) and LV end-diastolic volume indexed to body surface area; and 2) adverse events (AE). We evaluated the longitudinal rate of LV remodeling and determined the association between LV volume and AE by age and sex. RESULTS: A total of 525 adult patients (26% women) with a median echocardiogram follow-up of 2.0 years (IQR: 1.0-3.6 years) were included. At baseline, older patients (age ≥60 years) had smaller LV volumes compared with younger patients (age <60 years), eg, the mean LVESVi was 27.3 mL/m2 vs 32.3 mL/m2, respectively. Similarly, women had smaller LV volumes compared with men (mean LVESVi was 23.3 mL/m2 vs 32.4 mL/m2). On serial evaluation, older patients and women maintained smaller LV volumes compared with younger patients and men, respectively. There were 210 (40%) AE during follow-up. The optimal discriminatory threshold for AE varies by age and sex, eg, the LVESVi threshold was highest for young men (50 mL/m2), intermediate for older men (35 mL/m2), and lowest for women (27 mL/m2). CONCLUSIONS: On serial evaluation, older patients and women with chronic AR maintained smaller LV volumes than younger patients and men, respectively, and develop AE at lower LV volumes.

Clinical Characteristics and Transplant-Free Survival Across the Spectrum of Pulmonary Vascular Disease.

BACKGROUND: PVDOMICS (Pulmonary Vascular Disease Phenomics) is a precision medicine initiative to characterize pulmonary vascular disease (PVD) using deep phenotyping. PVDOMICS tests the hypothesis that integration of clinical metrics with omic measures will enhance understanding of PVD and facilitate an updated PVD classification. OBJECTIVES: The purpose of this study was to describe clinical characteristics and transplant-free survival in the PVDOMICS cohort. METHODS: Subjects with World Symposium Pulmonary Hypertension (WSPH) group 1-5 PH, disease comparators with similar underlying diseases and mild or no PH and healthy control subjects enrolled in a cross-sectional study. PH groups, comparators were compared using standard statistical tests including log-rank tests for comparing time to transplant or death. RESULTS: A total of 1,193 subjects were included. Multiple WSPH groups were identified in 38.9% of PH subjects. Nocturnal desaturation was more frequently observed in groups 1, 3, and 4 PH vs comparators. A total of 50.2% of group 1 PH subjects had ground glass opacities on chest computed tomography. Diffusing capacity for carbon monoxide was significantly lower in groups 1-3 PH than their respective comparators. Right atrial volume index was higher in WSPH groups 1-4 than comparators. A total of 110 participants had a mean pulmonary artery pressure of 21-24 mm Hg. Transplant-free survival was poorest in group 3 PH. CONCLUSIONS: PVDOMICS enrolled subjects across the spectrum of PVD, including mild and mixed etiology PH. Novel findings include low diffusing capacity for carbon monoxide and enlarged right atrial volume index as shared features of groups 1-3 and 1-4 PH, respectively; unexpected, frequent presence of ground glass opacities on computed tomography; and sleep alterations in group 1 PH, and poorest survival in group 3 PH. PVDOMICS will facilitate a new understanding of PVD and refine the current PVD classification. (Pulmonary Vascular Disease Phenomics Program PVDOMICS [PVDOMICS]; NCT02980887).

Cardiac Magnetic Resonance Imaging Techniques and Applications for Pericardial Diseases.

Cardiac magnetic resonance imaging plays a central role among multimodality imaging modalities in the assessment, diagnosis, and surveillance of pericardial diseases. Clinicians and imagers should have a foundational understanding of the utilities, advantages, and limitations of cardiac magnetic resonance imaging and how they integrate with other diagnostic tools involved in the evaluation and management of pericardial diseases. This review aims to outline the contemporary magnetic resonance imaging sequences used to evaluate the pericardium, followed by exploring the main clinical applications of magnetic resonance imaging for identifying pericardial inflammation, constriction, and effusion.

Pericardial Manifestations of Thyroid Diseases.

PURPOSE OF REVIEW: Thyroid disorders, especially hypothyroidism, are known to be associated with pericardial diseases. The aim of this paper is to review the current knowledge of the pericardial manifestations of hypothyroidism and hyperthyroidism. RECENT FINDINGS: Many reports have described associations between dysthyroidism, which encompasses hypothyroidism and hyperthyroidism, and several pericardial diseases, including acute pericarditis, constrictive pericarditis, pericardial effusion, and tamponade. The diagnosis of dysthyroidism-induced pericardial diseases consists of a combination of thyroid blood levels that fall outside of the normal range and the exclusion of other causes. Treatment of the thyroid disorder is key, along with treatment of the pericardial disease as recommended by the guidelines. Early recognition of the thyroid disorder is key in patients with pericardial diseases, since treating the underlying cause should assist resolution of the pericardial issues and ideally prevent recurrence and possible future complications of suboptimally treated pericarditis or pericardial effusions.

Pericardial Involvement in Sarcoidosis.

Pericardial disease secondary to sarcoidosis is a rare clinical entity with no observational studies in previous research. Therefore, we evaluated reported cases of pericarditis because of sarcoidosis to further understand its diagnosis and management. We performed a systematic review of previous research until December 16, 2020 in MEDLINE, Embase, Scopus, Cochrane Central Register of Controlled Trials, and Web of Science. Case reports and case series demonstrating pericardial involvement in sarcoidosis were included. Fourteen reports with a total of 27 patients were identified. Dyspnea (82%) was the most common presentation, with the lungs being the primary site of sarcoidosis in most patients (77%). The most frequently encountered pericardial manifestations were pericardial effusion (89%), constrictive pericarditis and cardiac tamponade (48%). Management of these patients included use of corticosteroids (82%), colchicine (11%), and nonsteroidal anti-inflammatory agents (7%). Similar to the general population, the most common intervention in these patients was pericardiocentesis (59%), pericardial window (30%), and pericardiectomy (19%). Overall, the majority of this population (70%) achieved clinical improvement during median follow-up time of 8 months. In conclusion, the prevalence and incidence of sarcoid-induced pericarditial disease remain unclear. Clinical manifestations of pericardial involvement are variable, though many patients present with asymptomatic pericardial effusions. No consensus exists on the treatment of this special population, but corticosteroids and combination therapies are considered first-line therapies because of their efficacy in suppressing pericardial inflammation and underlying sarcoidosis. Patients with refractory cases of pericarditis may also benefit therapeutically from the addition of nonsteroidal anti-inflammatory agents, colchicine, and/or biologics.

Comprehensive echocardiographic evaluation of the right heart in patients with pulmonary vascular diseases: the PVDOMICS experience.

AIMS: There is a wide spectrum of diseases associated with pulmonary hypertension, pulmonary vascular remodelling, and right ventricular dysfunction. The NIH-sponsored PVDOMICS network seeks to perform comprehensive clinical phenotyping and endophenotyping across these disorders to further evaluate and define pulmonary vascular disease. METHODS AND RESULTS: Echocardiography represents the primary non-invasive method to phenotype cardiac anatomy, function, and haemodynamics in these complex patients. However, comprehensive right heart evaluation requires the use of multiple echocardiographic parameters and optimized techniques to ensure optimal image acquisition. The PVDOMICS echo protocol outlines the best practice approach to echo phenotypic assessment of the right heart/pulmonary artery unit. CONCLUSION: Novel workflow processes, methods for quality control, data for feasibility of measurements, and reproducibility of right heart parameters derived from this study provide a benchmark frame of reference. Lessons learned from this protocol will serve as a best practice guide for echocardiographic image acquisition and analysis across the spectrum of right heart/pulmonary vascular disease.

Worsened Tricuspid Regurgitation Following Pericardiectomy for Constrictive Pericarditis.

BACKGROUND: Worsening tricuspid regurgitation (TR) severity may occur after pericardiectomy surgery for constrictive pericarditis patients; however, its mechanisms and predictors are not well established. We evaluated the clinical characteristics, associated factors, and outcomes of worsening TR after pericardiectomy. METHODS: Consecutive patients undergoing pericardiectomy for constrictive pericarditis without tricuspid valve surgery and with pre- and postoperative echocardiography available during 2000 to 2017 were retrospectively studied. Clinical, imaging, hemodynamic, and mortality characteristics were analyzed by those with and without worsening TR by at least one grade. RESULTS: Among 381 patients (age 61 [17] years, 318 [83.5%] male), 193 (50.7%) had worsening TR post-operatively, and 75 died during the 2.5 (5.4) years follow-up. In univariable analysis, worsening TR was associated with a history of congestive heart failure (47.2% versus 31.9%, P=0.003), increased left atrial volume indexed (23 versus 20 mL/m2, P=0.020), reduced right ventricular fractional area change (47% versus 54%, P<0.001), and worsening mitral regurgitation (39.7% versus 16.6%, P<0.001). Worsened TR had a trend toward reduced survival during follow-up (log-rank P=0.080), especially those with worsened TR but no recovery of TR grade on subsequent echocardiography within the first year compared with those without worsened TR (log-rank P=0.02). In multivariable analysis, right ventricular fractional area change, left atrial volume indexed, left ventricular mass indexed, pulmonary artery systolic pressure, and right atrial pressure/pulmonary capillary wedge pressure ratio were most associated with worsened TR, while blood urea nitrogen, hematocrit, lateral and medial e' tissue Doppler and heart rate were most associated with mortality during follow-up. CONCLUSIONS: Worsening TR severity was prevalent after pericardiectomy and had a trend toward reduced survival, especially if TR severity did not recover on subsequent echocardiography. Presence of parameters associated with worsened TR and reduced survival should alert clinicians to carefully manage these patients during follow-up.

Case report of isolated cardiac sarcoidosis presenting as hypertrophic obstructive cardiomyopathy-a clinical picture printed on lenticular paper.

BACKGROUND: Cardiac sarcoidosis (CS) is an inflammatory granulomatous process of the myocardium that can be asymptomatic or have several different clinical phenotypes. One of its rarely described presentations consists of hypertrophy of the septal myocardium, similar to hypertrophic cardiomyopathy (HCM). Isolated cardiac sarcoidosis that haemodynamically mimics hypertrophic obstructive cardiomyopathy (HOCM) has been rarely described in the literature. CASE SUMMARY: A 64-year-old Caucasian female previously diagnosed with non-critical aortic stenosis presented with pre-syncope, and echocardiography showed significant obstruction based on left ventricular outflow tract gradients, confirmed by cardiac magnetic resonance (CMR), concerning for a phenocopy of HCM. Septal myectomy was performed and pathology specimen revealed non-caseating granulomata consistent with cardiac sarcoidosis. She was started on oral corticosteroids and initial cardiac fluorodeoxyglucose positron emission tomography (FDG-PET) done after 1 month of treatment was negative. Repeat FDG-PET 15 months later, in the setting of haemodynamic decompensation, demonstrated diffuse FDG uptake in the myocardium without extra-cardiac involvement. DISCUSSION: Our case brings together two entities: isolated cardiac sarcoidosis and its presentation mimicking HOCM, which has been very rarely described in the literature. And it also shows the scenario of surgical pathology diagnosis of sarcoidosis that was not suspected by initial CMR or FDG-PET, despite adequate preparation, only appearing on repeat FDG-PET done 15 months later. Isolated cardiac sarcoidosis should remain a differential diagnosis for any non-ischaemic cardiomyopathy without a clear cause, despite imaging evidence of HCM.

Recurrent pericarditis associated with human coronavirus (HKU1) infection in a patient with systemic lupus erythematosus (SLE).

The presence of human coronavirus HKU1 infection associated with pericardial inflammation is not reported. We are reporting a young woman with systemic lupus erythematosus, who was positive for HKU1 during her pericarditis flare. Diagnostic imaging demonstrated pericardial effusion, edema, and late gadolinium enhancement on cardiac magnetic resonance imaging and echocardiography. She was on multiple anti-inflammatory medications and achieved remission with anakinra. Her management and a brief literature review is also presented.

Predictors of Cardiac Implantable Electronic Device Artifact on Cardiac MRI: The Utility of a Device Related Score.

PURPOSE: Cardiac magnetic resonance imaging (CMR) image quality can be degraded by artifact in patients with cardiac implantable electronic devices (CIED). We aimed to establish a clinical risk score, so patient selection for diagnostic CMR could be optimised. METHODS: In this retrospective cohort study, CMRs performed for clinical use in subjects with CIED from January 2016 to May 2019 were reviewed. Subject anthropometry, CIED generator/lead specifications and pre-scan chest X-ray (CXR) measurements were collected. Generator-related artifact size was measured on axial steady state free precession images. Interpretability of late gadolinium enhancement (LGE) imaging was performed based on a three-grade visual score attributed to each of 17 myocardial segments. RESULTS: Fifty-seven (57) patients (59±16 years, 74% male) fitted the inclusion criteria. Artifact precluded left ventricle (LV) evaluation (≥5 segments) in 17 (30%). Artifact was more common with implantable cardioverter-defibrillators, related to generator volume, mass, height, width, thickness, and area, along with right ventricular (RV) lead length and diameter (all p<0.05). Artifact was associated with distance from generator to LV apex, generator to RV lead tip and shortest distance from generator to heart on CXR (all p<0.05). On multivariable regression modelling, RV lead diameter (OR 5.861, 95% CI 1.866-18.407, p=0.002) and distance from generator to LV apex (OR 0.693, 95% CI 0.511-0.940, p=0.019) were independent predictors of artifact. Multivariable predictors were used to develop Device Related CMR Artifact Prediction Score (DR-CAPS), where all patients with DR-CAPS=0 had fully interpretable LGE imaging. CONCLUSION: Simple, readily available measures, such as lead characteristics and pre-scan CXR measures, can stratify patients via an artifact prediction score to optimise selection for diagnostic CMR.

Arrhythmias in Cardiac Sarcoidosis Bench to Bedside: A Case-Based Review.

Cardiac sarcoidosis is a component of an often multiorgan granulomatous disease of still uncertain cause. It is being recognized with increasing frequency, mainly as the result of heightened awareness and new diagnostic tests, specifically cardiac magnetic resonance imaging and 18F-fluorodeoxyglucose positron emission tomography scans. The purpose of this case-based review is to highlight the potentially life-saving importance of making the early diagnosis of cardiac sarcoidosis using these new tools and to provide a framework for the optimal care of patients with this disease. We will review disease mechanisms as currently understood, associated arrhythmias including conduction abnormalities, and atrial and ventricular tachyarrhythmias, guideline-directed diagnostic criteria, screening of patients with extracardiac sarcoidosis, and the use of pacemakers and defibrillators in this setting. Treatment options, including those related to heart failure, and those which may help clarify disease mechanisms are included.