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Objectives: The aim of the study was to assess cardiac involvement in patients with Wegener's granulomatosis (WG), who failed to achieve remission following >6 months induction therapy for life or organ threatening disease.Methods: Eleven WG patients (eight males, mean age 47 ± 13 years), who failed to achieve remission despite >6 months induction therapy, underwent transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR).Results: Cardiac involvement was present in 9 (82%) patients. Regional wall motion abnormalities were found in two individuals, but none had left ventricular (LV) ejection fraction <50%. Nine patients had late gadolinium enhancement (LGE) lesions involving LV myocardium and right ventricle free wall was involved in four patients. LGE lesions were found in subepicardial, midwall and subendocardial LV myocardial layers. CMR revealed myocarditis in six patients. Patients with myocarditis had a higher number of LV segments with LGE (5.2 ± 3.4 vs 1.0 ± 1.2, p = 0.03) and more frequent diastolic dysfunction by TTE (5 vs 0, p = 0.02) than those without. Pericardial effusion was observed in five patients, while localized pericardial thickening in six patients.Conclusions: In WG resistant to >6 months induction therapy cardiac involvement is frequent and is characterized by foci of LGE lesions and signs of myocardial inflammatory process. [ABSTRACT FROM AUTHOR]

Introduction: Myocarditis may be difficult to diagnose because of the variety of its clinical manifestations, and the clinical course of the disease can be unpredictable. Nevertheless, some patients may exhibit partial or full contractile recovery following myocarditis. Standard and speckle-tracking echocardiography may serve as tools to follow this recovery., Objectives: We aimed to evaluate predictors of positive left ventricular (LV) remodeling after active myocarditis (AM)., Patients and Methods: A database of a high‑volume, tertiary cardiology center was searched for patients with AM hospitalized between 2016 and 2019. They were included in the analysis based on clinical manifestations and presence of at least 1 of the following diagnostic criteria: positive findings on electrocardiography / Holter monitoring, echocardiography, elevated troponin T/I levels, functional or structural abnormalities on cardiac imaging, or tissue characterization by cardiac magnetic resonance. LV global longitudinal strain and mechanical dispersion (MD; defined as SD of the time to peak longitudinal strain derived from all LV segments in 3 apical views) were determined. Echocardiographic response (positive LV remodeling measured by transthoracic echocardiography) was defined as end‑systolic volume (ESV) reduction by 15% or greater or end-diastolic volume (EDV) reduction by 15% or greater from the baseline values., Results: A total of 61 consecutive patients were recruited. The median follow‑up was 1.4 years (range, 0.3-4). The mortality rate was 1.6%. Echocardiographic response was noted in 24 patients (39.4%). A multivariable Cox regression model including significant baseline differences as covariates showed that QRS duration (hazard ratio [HR], 1.31; 95% CI, 1.17-1.57; P = 0.049), MD (HR, 1.03; 95% CI, 1.01-1.07; P = 0.04), and mineralocorticoid receptor antagonist [MRA] use (HR, 8.60; 95% CI, 1.50-46.49; P = 0.01) were independently associated with positive LV remodeling with ESV reduction. MD (HR, 1.04; 95% CI, 1.02-1.06; P = 0.04) was also independently associated with positive LV remodeling with EDV reduction., Conclusions: Mechanical dispersion, QRS duration, and MRA use are independent predictors of positive LV remodeling in individuals with a history of AM.

Background: COVID-19 is a great medical challenge as it provokes acute respiratory distress and has pulmonary manifestations and cardiovascular (CV) consequences., Aims: This study compared cardiac injury in COVID-19 myocarditis patients with non-COVID-19 myocarditis patients., Methods: Patients who recovered from COVID-19 were scheduled for cardiovascular magnetic resonance (CMR) owing to clinical myocarditis suspicion. The retrospective non-COVID-19 myocarditis (2018-2019) group was enrolled (n = 221 patients). All patients underwent contrast-enhanced CMR, the conventional myocarditis protocol, and late gadolinium enhancement (LGE). The COVID study group included 552 patients at a mean (standard deviation [SD]) age of 45.9 (12.6) years., Results: CMR assessment confirmed myocarditis-like LGE in 46% of the cases (68.5% of the segments with LGE <25% transmural extent), left ventricular (LV) dilatation in 10%, and systolic dysfunction in 16% of cases. The COVID-19 myocarditis group showed a smaller median (interquartile range [IQR]) LV LGE (4.4% [2.9%-8.1%] vs. 5.9% [4.4%-11.8%]; P <0.001), lower LV end-diastolic volume (144.6 [125.5-178] ml vs. 162.8 [136.6-194] ml; P <0.001), limited functional consequence (left ventricular ejection fraction, 59% [54.1%-65%] vs. 58% [52%-63%]; P = 0.01), and a higher rate of pericarditis (13.6% vs. 6%; P = 0.03) compared to non-COVID-19 myocarditis. The COVID-19-induced injury was more frequent in septal segments (2, 3, 14), and non-COVID-19 myocarditis showed higher affinity to lateral wall segments (P <0.01). Neither obesity nor age was associated with LV injury or remodeling in subjects with COVID-19 myocarditis., Conclusions: COVID-19-induced myocarditis is associated with minor LV injury with a significantly more frequent septal pattern and a higher pericarditis rate than non-COVID-19 myocarditis.

Background: The incompetent bicuspid aortic valve (BAV) can be replaced or repaired using various surgical techniques. This study sought to assess the efficacy of external annuloplasty and postoperative reverse remodelling using cardiac magnetic resonance (CMR) and compare the results of external and subcommissural annuloplasty., Methods: Out of a total of 200 BAV repair performed between 2004 and 2018, 21 consecutive patients (median age 54 years) with regurgitation requiring valve repair with annuloplasty without concomitant aortic root surgery were prospectively referred for CMR and transthoracic echocardiography (TTE) one year after the operation. Two aortic annulus stabilization techniques were used: external, circumferential annuloplasty (EA), and subcommissural annuloplasty (SCA)., Results: 11 patients received EA and 10 patients were treated using SCA. There was no in-hospital mortality and all patients survived the follow-up period (median: 12.6 months (first quartile: 6.6; third quartile: 14.1). CMR showed strong correlation between postoperative aortic recurrent regurgitant fraction and left ventricular end-diastolic volume (r = 0.62; p = 0.003) as well as left ventricular ejection fraction (r = -0.53; p = 0.01). Patients treated with EA as compared with SCA had larger anatomic aortic valve area measured by CMR (3.5 (2.5; 4.0) vs. 2.5 cm 2 (2.0; 3.4); p = 0.04). In both EA and SCA group, aortic valve area below 3.5 cm 2 correlated with no regurgitation recurrency. EA (vs. SCA) was associated with lower peak transvalvular aortic gradients (10 (6; 17) vs. 21 mmHg (15; 27); p = 0.04)., Conclusions: The repair of the bicuspid aortic valve provides significant postoperative reverse remodelling, provided no recurrent regurgitation and durable reduction annuloplasty can be achieved. EA is associated with lower transvalvular gradients and higher aortic valve area assessed by CMR, compared to SCA.

Importance: Clinical decisions are frequently based on measurement of left ventricular ejection fraction (LVEF). Limited information exists regarding inconsistencies in LVEF measurements when determined by various imaging modalities and the potential impact of such variability., Objective: To determine the intermodality variability of LVEF measured by echocardiography, gated single-photon emission computed tomography (SPECT), and cardiovascular magnetic resonance (CMR) in patients with left ventricular dysfunction., Design, Setting, and Participants: International multicenter diagnostic study with LVEF imaging performed at 127 clinical sites in 26 countries from July 24, 2002, to May 5, 2007, and measured by core laboratories. Secondary study of clinical diagnostic measurements of LVEF in the Surgical Treatment for Ischemic Heart Failure (STICH), a randomized trial to identify the optimal treatment strategy for patients with LVEF of 35% or less and coronary artery disease. Data analysis was conducted from March 19, 2016, to May 29, 2018., Main Outcomes and Measures: At baseline, most patients had an echocardiogram and subsets of patients underwent SPECT and/or CMR. Left ventricular ejection fraction was measured by a core laboratory for each modality independent of the results of other modalities, and measurements were compared among imaging methods using correlation, Bland-Altman plots, and coverage probability methods. Association of LVEF by each method and death was assessed., Results: A total of 2032 patients (mean [SD] age, 60.9 [9.6] years; 1759 [86.6%] male) with baseline LVEF data were included. Correlation of LVEF between modalities was r = 0.601 (for biplane echocardiography and SPECT [n = 385]), r = 0.493 (for biplane echocardiography and CMR [n = 204]), and r = 0.660 (for CMR and SPECT [n = 134]). Bland-Altman plots showed only moderate agreement in LVEF measurements from all 3 core laboratories with no substantial overestimation or underestimation of LVEF by any modality. The percentage of observations that fell within a range of 5% ranged from 43% to 54% between different imaging modalities., Conclusions and Relevance: In this international multicenter study of patients with coronary artery disease and reduced LVEF, there was substantial variation between modalities in LVEF determination by core laboratories. This variability should be considered in clinical management and trial design., Trial Registration: Clinicaltrials.gov Identifier: NCT00023595.

Background: De novo missense variants in CDK13 have been described as the cause of syndromic congenital heart defects in seven individuals ascertained from a large congenital cardiovascular malformations cohort. We aimed to further define the phenotypic and molecular spectrum of this newly described disorder., Methods: To minimise ascertainment bias, we recruited nine additional individuals with CDK13 pathogenic variants from clinical and research exome laboratory sequencing cohorts. Each individual underwent dysmorphology exam and comprehensive medical history review., Results: We demonstrate greater than expected phenotypic heterogeneity, including 33% (3/9) of individuals without structural heart disease on echocardiogram. There was a high penetrance for a unique constellation of facial dysmorphism and global developmental delay, as well as less frequently seen renal and sacral anomalies. Two individuals had novel CDK13 variants (p.Asn842Asp, p.Lys734Glu), while the remaining seven unrelated individuals had a recurrent, previously published p.Asn842Ser variant. Summary of all variants published to date demonstrates apparent restriction of pathogenic variants to the protein kinase domain with clustering in the ATP and magnesium binding sites., Conclusions: Here we provide detailed phenotypic and molecular characterisation of individuals with pathogenic variants in CDK13 and propose management guidelines based upon the estimated prevalence of anomalies identified.

Background: Left ventricular noncompaction (LVNC) is a genetically and phenotypically heterogeneous disease and, although increasingly recognized in clinical practice, there is a lack of widely accepted diagnostic criteria. We sought to identify novel genetic causes of LVNC and describe genotype-phenotype correlations., Methods and Results: A total of 190 patients from 174 families with left ventricular hypertrabeculation (LVHT) or LVNC were referred for cardiac magnetic resonance and whole-exome sequencing. A total of 425 control individuals were included to identify variants of interest (VOIs). We found an excess of 138 VOIs in 102 (59%) unrelated patients in 54 previously identified LVNC or other known cardiomyopathy genes. VOIs were found in 68 of 90 probands with LVNC and 34 of 84 probands with LVHT (76% and 40%, respectively; P <0.001). We identified 0, 1, and ≥2 VOIs in 72, 74, and 28 probands, respectively. We found increasing number of VOIs in a patient strongly correlated with several markers of disease severity, including ratio of noncompacted to compacted myocardium ( P <0.001) and left ventricular ejection fraction ( P =0.01). The presence of sarcomeric gene mutations was associated with increased occurrence of late gadolinium enhancement ( P =0.004)., Conclusions: LVHT and LVNC likely represent a continuum of genotypic disease with differences in severity and variable phenotype explained, in part, by the number of VOIs and whether mutations are present in sarcomeric or nonsarcomeric genes. Presence of VOIs is common in patients with LVHT. Our findings expand the current clinical and genetic diagnostic approaches for patients with LVHT and LVNC., (© 2017 American Heart Association, Inc.)

Competing Interests: The authors declare no conflict of interest.

Primary malignant cardiac tumors are rare, and mostly manifest as sarcomas in various types. As non-invasive diagnostic modalities, e.g. echocardiography and magnetic resonance imaging, have become more sensitive, there is a marked increase in the number of patients diagnosed. Nevertheless, most patients die within one year of initial diagnosis, either because of the often asymptomatic presentation of cardiac tumors until advanced disease, or a low index of suspicion on the part of the physician. The presenting symptoms, treatment options and, indeed, prognosis are largely controlled by the tumor's anatomic location. Cardiac sarcomas may present with a variety of symptoms and are known to be great mimickers. A quick diagnosis facilitates the initiation of a proper treatment (surgical resection, adjuvant chemotherapy), which may in turn improve the prognosis. Metastases to the heart are far more common, unfortunately, clinical manifestations are mainly dominated by generalized tumor spread. The article summarizes epidemiology, symptoms, diagnostic modalities, and possible treatment options., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Aim: The aim of this study was to assess the relationship between late gadolinium-enhanced (LGE) cardiovascular magnetic resonance (CMR) and immunohistochemical markers of inflammation in patients with heart failure and a reduced ejection fraction (HFrEF)., Material and Methods: Endomyocardial biopsy and CMR were performed in 38 consecutive patients (24 males, average age 43.2 ± 6.9 years, New York Heart Association [NYHA] class II) with HFrEF and suspected myocarditis. The immunohistochemical evaluation was done by the En-Vision system using DAKO monoclonal antibodies. The presence of > 14 infiltrating cells together with myocardial damage and ≥ 2 + up-regulation of HLA class II was considered diagnostic for myocarditis. The results of LGE were compared with the immunohistochemical markers of inflammation. All patients underwent coronary angiography., Results: Twelve out of 38 (31.6%) patients met the immunohistological criteria for the diagnosis of myocarditis. Late gadolinium enhancement was present in 23 of 38 (60.5%) patients, mostly at the interventricular septum. No correlation was found between LGE and immunohistochemistry results (Kendall's tau; r = 0.21, p = 0.09)., Conclusions: Our study revealed no significant relationship between LGE cardiovascular magnetic resonance imaging and immunohistochemical markers of inflammation in patients with HFrEF.

We report a case of a 70-year-old woman admitted with symptoms of decompensated heart failure. Magnetic resonance imaging revealed lipomatous hypertrophy of the interatrial septum with partial upper right atrial inflow obstruction, partial obstruction of the right ventricular outflow tract, and excessive accumulation of adipose tissue in the pericardial space. The patient underwent excision of the septal lipomatous mass, which relieved the right ventricular outflow obstruction.

Background: The aim of this study was to determine the incremental prognostic value of myocardial contrast echocardiography (MCE) over two-dimensional echocardiography (2DE) in patients undergoing supine bicycle stress., Methods: Eighty-four patients with known or suspected coronary artery disease who underwent supine bicycle stress with 2DE and MCE (mean age, 58.5 +/- 9.7 years; 68 men) were followed up for 48.3 +/- 8.9 months for cardiac death (n = 1), nonfatal myocardial infarction (n = 9), and revascularization (n = 20)., Results: In sequential Cox models, the predictive power of the clinical model was strengthened by 2DE (chi(2) = 7.73 vs 12.92, P = .02) and further improved by MCE (chi(2) = 19.04, P = .01). On multivariate analysis, the only independent follow-up event predictor was ischemia on MCE (hazard ratio, 6.79; 95% confidence interval, 2.02-22.82; P = .001). Among patients with normal results on 2DE, those with normal results on MCE had greater 4.5-year event-free survival than those with abnormal results on MCE (93% vs 69%, P = .01)., Conclusions: MCE enhances the predictive power of supine bicycle stress 2DE and allows the risk stratification of patients with normal results on 2DE.

Background: If compared to two-dimensional echocardiography (2DE), myocardial contrast echocardiography (MCE) improves detection of coronary artery disease (CAD) during pharmacological stress, but data on MCE vs. 2DE during supine bicycle stress is limited. Although previous myocardial infarction (MI) influences sensitivity of 2DE, its effect on MCE has not been evaluated., Objectives: The study sought to determine the incremental benefit of MCE over 2DE for evaluation of CAD during supine bicycle stress and to assess the impact of previous MI on diagnostic values of both methods., Methods: We studied 103 consecutive patients scheduled for coronary angiography. Prior to coronary angiography, all patients underwent supine bicycle stress. 2DE and MCE were performed during this stress test. The diagnosis of obstructive CAD (> or =50% stenosis) was based on the presence of inducible wall motion and perfusion abnormalities., Results: Quantitative coronary angiography revealed > or =50% stenosis in 53 of 77 patients without previous MI and in 21 of 26 patients with previous MI. If compared to 2DE, MCE was more sensitive (68% vs. 86%; p<0.001) and more accurate (73% vs. 86%; p < 0.001) to detect > or =50% stenosis. In patients without previous MI, 2DE and MCE yielded sensitivity of 65% and 85% (p < 0.01) and accuracy of 71% and 85% (p < 0.01), whereas in patients with previous MI sensitivity was 79% and 90% (p=NS) and accuracy 79% and 88% (p = NS), respectively., Conclusions: MCE enhances sensitivity and accuracy of 2DE in detection of obstructive CAD during supine bicycle stress. The incremental benefit of MCE is especially present in patients without previous MI.