Non-Ischemic Scar Underlines Ventricular Arrhythmias in Kearns-Sayre Syndrome

Kearns-Sayre syndrome (KSS) is a rare mitochondrial disease in which cardiac involvement has been associated with poor prognosis. Although the most common clinical manifestation is progressive conduction system impairment, patients can suffer from ventricular arrhythmias. Yet, they show a high prevalence of sudden cardiac death, whose etiopathological mechanism is not completely understood. Cardiac magnetic resonance is a rising tool to detect subclinical heart involvement in many heart diseases and was recently able to detect nonischemic scar, which is an arrhythmogenic substrate, in patients affected by KSS

Biopsy-Proven Lymphocytic Myocarditis With Heart Failure in a Middle-Aged Female Patient With Mixed Connective Tissue Disease

Abstract A 56-year-old woman with mixed connective tissue disease, who was on maintenance immunosuppression, developed asymptomatic left ventricular dysfunction, ventricular arrhythmia, and high troponin I. Heart catheterization showed normal coronaries and biopsy-proven, virus-negative lymphocytic myocarditis. A biopsy-guided immunosuppression upgrade effectively treated autoimmune myocarditis, which resulted in ventricular function recovery, resolution of arrhythmia, and of troponin release. (Level of Difficulty: Advanced.

Atrial functional tricuspid regurgitation: a novel and underappreciated clinical entity

Abstract Functional or secondary tricuspid regurgitation (FTR) is a progressive disease with a significant negative impact on patient morbidity and mortality. Recently, atrial fibrillation (AF) has been recognized as a cause of FTR (with/without coexisting functional mitral regurgitation) by promoting right atrial (RA) remodeling and secondary tricuspid valve (TV) annulus dilation, even in the absence of right ventricular (RV) dilation or dysfunction. This distinct form of FTR has been called "atriogenic" or "atrial". Recent evidence suggests that the RA is an important player in FTR pathophysiology not only for patients with AF, but also for those in sinus rhythm. Preliminary reports on atrial FTR show that cardioversion with documented maintenance of sinus rhythm promotes TV annulus and RA reverse remodeling and may significantly reduce FTR severity at follow-up. Large-scale studies on the prognostic benefits of rhythm vs rate-control strategy in atrial FTR patients are needed to substantiate specific guidelines indications for this subset of patients

Fabry Disease: More than a Phenocopy of Hypertrophic Cardiomyopathy

Fabry disease (FD) is a genetic lysosomal storage disease with frequent cardiovascular involvement, whose presence is a major determinant of adverse clinical outcomes. As a potentially treatable cause of left ventricular hypertrophy (LVH) and heart failure with preserved ejection fraction, the early recognition of FD is crucial to initiate enzyme replacement therapy and improve long-term prognosis. Multimodality imaging plays a central role in the evaluation of patients with FD and helps in the differential diagnosis of other conditions presenting with LVH. In the present review, we explore the current applications of multimodality cardiac imaging, in particular echocardiography and cardiovascular magnetic resonance, in the diagnosis, prognostic assessment, and follow-up of patients with FD

Comparison of Demographic, Clinical, Biochemical, and Imaging Findings in Hypertrophic Cardiomyopathy Prognosis:A Network Meta-Analysis

Background: Despite hypertrophic cardiomyopathy (HCM) being the most common inherited heart disease and conferring increased risk for heart failure (HF) and sudden cardiac death (SCD), risk assessment in HCM patients is still largely unresolved. Objectives: This study aims to synthesize and compare the prognostic impact of demographic, clinical, biochemical, and imaging findings in patients with HCM. Methods: The authors searched PubMed, Embase, and Cochrane Library for studies published from 1955 to November 2020, and the endpoints were: 1) all-cause death; 2) an arrhythmic endpoint including SCD, sustained ventricular tachycardia, ventricular fibrillation, or aborted SCD; and 3) a composite endpoint including (1) or (2) plus hospitalization for HF or cardiac transplantation. The authors performed a pairwise meta-analysis obtaining the pooled estimate separately for the association between baseline variables and study endpoints. A random-effects network meta-analysis was subsequently used to comparatively assess the prognostic value of outcome associates. Results: A total of 112 studies with 58,732 HCM patients were included. Among others, increased brain natriuretic peptide/N-terminal pro–B-type natriuretic peptide, late gadolinium enhancement (LGE), positive genotype, impaired global longitudinal strain, and presence of apical aneurysm conferred increased risk for the composite endpoint. At network meta-analysis, LGE showed the highest prognostic value for all endpoints and was superior to all other associates except New York Heart Association functional class >class II. A multiparametric imaging-based model was superior in predicting the composite endpoint compared to a prespecified model based on conventional risk factors. Conclusions: This network meta-analysis supports the development of multiparametric risk prediction algorithms, including advanced imaging markers additively to conventional risk factors, for refined risk stratification in HCM. (Long-term prognosis of hypertrophic cardiomyopathy according to genetic, clinical, biochemical and imaging findings: a systemic review and meta-analysis; CRD42020185219

3D whole-heart grey-blood late gadolinium enhancement cardiovascular magnetic resonance imaging

Abstract Purpose To develop a free-breathing whole-heart isotropic-resolution 3D late gadolinium enhancement (LGE) sequence with Dixon-encoding, which provides co-registered 3D grey-blood phase-sensitive inversion-recovery (PSIR) and complementary 3D fat volumes in a single scan of  0.99) were obtained with the 2D and 3D PSIR LGE approaches with comparable total acquisition time (p = 0.29). Similar agreement in intra and inter-observer variability were obtained for the 2D and 3D acquisition respectively. Conclusion The proposed approach enabled the acquisition of free-breathing motion-compensated isotropic-resolution 3D grey-blood PSIR LGE and fat volumes. The proposed approach showed good agreement with conventional 2D LGE in terms of CR, scar depiction and scan time, while enabling free-breathing acquisition, whole-heart coverage, reformatting in arbitrary views and visualization of both water and fat information

Predictors of adverse prognosis in COVID-19: a systematic review and meta-analysis

Background: Identification of reliable outcome predictors in coronavirus disease 2019 (COVID-19) is of paramount importance for improving patient's management. Methods: A systematic review of literature was conducted until 24 April 2020. From 6843 articles, 49 studies were selected for a pooled assessment; cumulative statistics for age and sex were retrieved in 587 790 and 602 234 cases. Two endpoints were defined: (a) a composite outcome including death, severe presentation, hospitalization in the intensive care unit (ICU) and/or mechanical ventilation; and (b) in-hospital mortality. We extracted numeric data on patients’ characteristics and cases with adverse outcomes and employed inverse variance random-effects models to derive pooled estimates. Results: We identified 18 and 12 factors associated with the composite endpoint and death, respectively. Among those, a history of CVD (odds ratio (OR) = 3.15, 95% confidence intervals (CIs) 2.26-4.41), acute cardiac (OR = 10.58, 5.00-22.40) or kidney (OR = 5.13, 1.78-14.83) injury, increased procalcitonin (OR = 4.8, 2.034-11.31) or D-dimer (OR = 3.7, 1.74-7.89), and thrombocytopenia (OR = 6.23, 1.031-37.67) conveyed the highest odds for the adverse composite endpoint. Advanced age, male sex, cardiovascular comorbidities, acute cardiac or kidney injury, lymphocytopenia and D-dimer conferred an increased risk of in-hospital death. With respect to the treatment of the acute phase, therapy with steroids was associated with the adverse composite endpoint (OR = 3.61, 95% CI 1.934-6.73), but not with mortality. Conclusions: Advanced age, comorbidities, abnormal inflammatory and organ injury circulating biomarkers captured patients with an adverse clinical outcome. Clinical history and laboratory profile may then help identify patients with a higher risk of in-hospital mortality. © 2020 Stichting European Society for Clinical Investigation Journal Foundatio