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Aims: In patients with recently diagnosed non‐ischaemic LV systolic dysfunction, left ventricular reverse remodelling (LVRR) and favourable prognosis has been documented in studies with short‐term follow‐up. The aim of our study was to assess the long‐term clinical course and stability of LVRR in these patients. Methods and results: We prospectively studied 133 patients (37 women; 55 [interquartile range 46, 61] years) with recently diagnosed unexplained LV systolic dysfunction, with heart failure symptoms lasting <6 months and LV ejection fraction <40% persisting after at least 1 week of therapy. All patients underwent endomyocardial biopsy (EMB) at the time of diagnosis and serial echocardiographic and clinical follow‐up over 5 years. LVRR was defined as the combined presence of (1) LVEF ≥ 50% or increase in LVEF ≥ 10% points and (2) decrease in LV end‐diastolic diameter index (LVEDDi) ≥ 10% or (3) LVEDDi ≤ 33 mm/m2. LVRR was observed in 46% patients at 1 year, in 60% at 2 years and 50% at 5 years. Additionally, 2% of patients underwent heart transplantation and 12% experienced heart failure hospitalization. During 5‐year follow‐up, 23 (17%) of the study cohort died. In multivariate analysis, independent predictors of mortality were baseline right atrial size (OR 1.097, CI 1.007–1.196), logBNP level (OR 2.02, CI 1.14–3.56), and PR interval (OR 1.02, CI 1.006–1.035) (P < 0.05 for all). The number of macrophages on EMB was associated with overall survival in univariate analysis only. LVRR at 1 year of follow‐up was associated with a lower rate of mortality and heart failure hospitalization (P = 0.025). In multivariate analysis, independent predictors of LVRR were left ventricular end‐diastolic volume index (OR 0.97, CI 0.946–0.988), LVEF (OR 0.89, CI 0.83–0.96), and diastolic blood pressure (OR 1.04, CI 1.01–1.08) (P < 0.05 for all). Conclusions: LVRR occurs in over half of patients with recent onset unexplained LV systolic dysfunction during first 2 years of optimally guided heart failure therapy and then remains relatively stable during 5‐year follow‐up. Normalization of adverse LV remodelling corresponds to a low rate of mortality and heart failure hospitalizations during long‐term follow‐up. [ABSTRACT FROM AUTHOR]

The article discusses the use of tailored immunosuppressive therapy in immune-mediated biopsy-proven myocarditis. It explains that myocarditis is an inflammatory disease of the myocardium and that endomyocardial biopsy is used to confirm the diagnosis and identify the underlying cause. The article summarizes a study that compared immunosuppressive therapy to conventional therapy in patients without cardiotropic viral infection. The study found that 5-year transplant-free survival was similar in both groups, and biventricular function and NYHA class were also comparable. The article emphasizes the importance of individualizing treatment based on the type and severity of myocarditis and the patient's response and tolerance, and recommends a multidisciplinary team approach for management. Further research is needed to determine the safety and effectiveness of immunosuppressive treatment in patients with myocarditis and viral presence. [Extracted from the article]

Sarcoidosis is a systemic granulomatous disease of unknown cause. Its clinical presentations are heterogeneous and virtually any organ system can be affected, most commonly lungs. The manifestations of cardiac sarcoidosis (CS) are heterogenous depending on the extent and location of the disease and range from asymptomatic forms to lifethreatening arrhythmias as well as to progressive heart failure. Cardiac involvement is associated with a worse prognosis. The diagnosis of CS is often challenging and requires a multimodality approach based on current international recommendations. Pharmacological treatment of CS is based on administration of anti-inflammatory therapy (mainly corticosteroids), which is often combined with heart failure medication and/or antiarrhythmics. Nonpharmacological therapeutic approaches in CS cover pacemaker or defibrillator implantation, catheter ablations and heart transplantation. This review aims to summarize the current understanding of CS including its epidemiology, etiopathogenesis, clinical presentations, diagnostic approaches, and therapeutic possibilities. [ABSTRACT FROM AUTHOR]

Purpose: Danon disease (DD) is a rare X‐linked disorder caused by pathogenic variants in LAMP2. DD primarily manifests as a severe cardiomyopathy. An early diagnosis is crucial for patient survival. The aim of the study was to determine the usefulness of ocular examination for identification of DD. Methods: Detailed ocular examination in 10 patients with DD (3 males, 7 females) and a 45‐year‐old asymptomatic female somatic mosaic carrier of a LAMP2 disease‐causing variant. Results: All patients with manifest cardiomyopathy had pigmentary retinopathy with altered autofluorescence and diffuse visual field loss. Best corrected visual acuity (BCVA) was decreased (<0.63) in 8 (40%) out of 20 eyes. The severity of retinal pathology increased with age, resulting in marked cone‐rod involvement overtime. Spectral‐domain optical coherence tomography in younger patients revealed focal loss of photoreceptors, disruption and deposition at the retinal pigment epithelium/Bruch's membrane layer (corresponding to areas of marked increased autofluorescence), and hyperreflective foci in the outer nuclear layer. Cystoid macular oedema was seen in one eye. In the asymptomatic female with somatic mosaicism, the BCVA was 1.0 bilaterally. An abnormal autofluorescence pattern in the left eye was present; while full‐field electroretinography was normal. Conclusions: Detailed ocular examination may represent a sensitive and quick screening tool for the identification of carriers of LAMP2 pathogenic variants, even in somatic mosaicism. Hence, further investigation should be undertaken in all patients with pigmentary retinal dystrophy as it may be a sign of a life‐threatening disease. [ABSTRACT FROM AUTHOR]

Background: Fabry cardiomyopathy (FC) and light‐chain amyloid cardiomyopathy (AL) present with concentric left ventricular (LV) hypertrophy/remodeling and diastolic rather than systolic dysfunction. Direct comparisons are difficult due to rarity and confounded by variability of LV thickness. Aims: To compare LV diastolic and systolic properties between patients with FC and AL in a cohort matched for interventricular septal thickness (IVS). Methods: A two‐center echocardiographic analysis was performed, comprising 118 patients with IVS ≥12 mm (FC and AL 59 patients each) matched by IVS. Results: Fabry cardiomyopathy patients had larger LV end‐diastolic diameter (47.7 [44.0–50.9] vs 45.0 [41.5–49.0] mm, P = 0.002), better LV ejection fraction (EF 68.7 [63.4–74.0] vs 63.0 [54.0–70.0]%, P = 0.001) and midwall fractional shortening (midFS 14.8 [13.0–16.1] vs 12.1 [8.9–15.0]%, P = 0.006). LV EF <40% was rare in both (2% vs 7%, P = 0.17). AL patients expressed higher LV diastolic dysfunction grade (III in 26% vs 4%, II in 21% vs 12% and I in 54% vs 84%, P = 0.004), with higher E/e' ratio (13.6 [10.2–18.8] vs 9.8 [7.5–12.3], P < 0.0001). Average E/e' ratio and midFS were significantly associated with NYHA severity in both groups (P < 0.05 for all). Conclusions: Matched AL patients had worse LV diastolic function than FC, driven by E/e'. Significant LV systolic dysfunction was rare overall. MidFS and E/e' were associated with heart failure severity in both groups. [ABSTRACT FROM AUTHOR]

Aim of the study: To assess the diagnostic utility of a simplified approach to relative apical sparing of longitudinal strain (RAS LS) using only an apical four‐chamber view (A4C) in patients with AL amyloid cardiomyopathy (ALAC). Methods: We retrospectively evaluated echocardiographic recordings of 20 patients with ALAC, 20 patients with Fabry disease‐related cardiomyopathy (FD), and 20 patients with concentric hypertensive left ventricular hypertrophy (HLVH) matched for mean LV mean thickness. Peak segmental LS values of the interventricular septum and lateral LV wall were measured in the A4C using two‐dimensional speckle‐tracking echocardiography. RAS LS was calculated as average apical LS/(average basal LS + average midventricular LS). Results: Relative apical sparing of longitudinal strain values in patients with ALAC (1.23 ± 0.64) were significantly higher than those in FD patients (0.75 ± 0.19, P < 0.05) as well as in individuals with HLVH (0.75 ± 0.23, P < 0.05), but with a significant overlap. The optimal RAS LS value differentiating ALAC from FD and HLVH with 70% sensitivity and 75% specificity was 0.88 (AUC 0.79). In multivariate modeling, RAS LS was significantly additive to traditional predictors of ALAC (low QRS voltage and pseudoinfarct ECG patterns, pericardial effusion, E/e′ ratio, E‐wave deceleration time; P < 0.05 for all models). Conclusions: Simplified RAS LS evaluation represents an attractive approach for diagnostics of ALAC. However, because of considerable overlap with other disorders with hypertrophic phenotype, the analysis of RAS LS in the A4C should be combined with other traditional echocardiographic and ECG predictors in differentiating ALAC from other forms of concentric LV wall thickening. [ABSTRACT FROM AUTHOR]

Background Left atrial (LA) enlargement is a predictor of worse outcome after catheter ablation for atrial fibrillation (AF). Widely used two-dimensional (2D)-echocardiography is inaccurate and underestimates real LA volume (LAV). We hypothesized that baseline clinical characteristics of patients can be used to adjust 2D-ECHO indices of LAV in order to minimize this disagreement. Methods The study enrolled 535 patients (59 ± 9 years; 67% males; 43% paroxysmal AF) who underwent catheter ablation for AF in three specialized centers. We investigated multivariately the relationship between 2D-echocardiographic indices of LA size, specifically LA diameter in M-mode in the parasternal long-axis view (LAD), LAV assessed by the prolate-ellipsoid method (LAVEllipsoid), LAV by the planimetric method (LAVPlanimetry), and LAV derived from 3D-electroanatomic mapping (LAVCARTO). Results Cubed LAD of 106 ± 45 ml, LAVEllipsoid of 72 ± 24 ml and LAVPlanimetry of 88 ± 30 ml correlated only modestly (r = 0.60, 0.69, and 0.53, respectively) with LAVCARTO of 137 ± 46 ml, which was significantly underestimated with a bias (±1.96 standard deviation) of -31 (-111; +49) ml, -64 (-132; +2) ml, and -49 (-125; +27) ml, respectively; p < 0.0001 for their mutual difference. LA enlargement itself, age, gender, type of AF, and the presence of structural heart disease were independent confounders of measurement error of 2D-echocardiographic LAV. Conclusion Accuracy and precision of all 2D-echocardiographic LAV indices are poor. Their agreement with true LAV can be significantly improved by multivariate adjustment to clinical characteristics of patients. [ABSTRACT FROM AUTHOR]

Background. Several studies have demonstrated the presence of the Borrelia burgdorferi (Bb) genome in the myocardium of patients with dilated cardiomyopathy (DCM). To further support a causal relationship between the presence of Bb in the heart muscle and the development of DCM, demonstration of the absence of Bb in the myocardium of subjects with normal left ventricular (LV) systolic function is needed. Aim. To determine the prevalence of Bb by polymerase chain reaction (PCR) and electron microscopy (EM) in individuals with normal LV systolic function and no history suggestive of myocarditis. Methods. We investigated 50 patients (67 ± 9 years, 15 women) with normal LV ejection fraction (EF) = 50% undergoing cardiac surgery. During surgery, four samples from the right atrial appendage were obtained and subsequently examined by PCR and EM for the presence of Bb, and by immunohistochemistry to detect inflammatory cells. Serological testing of antibodies against Bb was also performed. Results. Neither PCR nor EM detected Bb in any of the subjects. Immunohistological examination revealed myocardial inflammation in 2 individuals (4%). Serological analysis by enzyme-linked immunosorbent assay demonstrated IgM antibodies against Bb in 4% and IgG antibodies in 12% of the study cohort; Western blot revealed IgM as well as IgG positivity in 14% of patients. Conclusions. The absence of Bb in the myocardium of individuals who undergo cardiac surgery and have normal LV systolic function supports the idea of Bb pathogenicity in the development of DCM. [ABSTRACT FROM AUTHOR]

Eosinophilic myocarditis (EM) represents a rare form of myocardial inflammation with very heterogeneous aetiology. In developed countries, the most prevalent causes of EM are hypersensitivity or allergic reactions, as well as hematological diseases leading to eosinophilia. The disease may have a variable clinical presentation, ranging from asymptomatic forms to life-threatening conditions. Most patients with EM have marked eosinophilia in peripheral blood. Endomyocardial biopsy needs to be performed in most cases in order to establish a definitive diagnosis of EM. The therapy depends on the underlying aetiology. Immunosuppressive therapy represents the treatment mainstay in the majority of EM forms. [ABSTRACT FROM AUTHOR]

In the last decade, there has been increasing evidence that comprehensive evaluation of the left atrium is of utmost importance. Numerous studies have clearly demonstrated the prognostic value of left atrial volume for long-term outcome. Furthermore, advances in catheter ablation procedures used for the treatment of drug-refractory atrial fibrillation require the need for detailed knowledge of left atrial and pulmonary venous morphology as well of atrial wall characteristics. This review article discusses the role of cardiac magnetic resonance and computed tomography in assessment of left atrial size, its normal and abnormal morphology, and function. Special interest is paid to the utility of these rapidly involving noninvasive imaging methods before and after atrial fibrillation ablation. [ABSTRACT FROM AUTHOR]

Background: Left dominant arrhythmogenic cardiomyopathy (LDAC) is a rare condition characterised by progressive fibrofatty replacement of the myocardium of the left ventricle (LV) in combination with ventricular arrhythmias of LV origin. Case presentation: A thirty-five-year-old male was referred for evaluation of recurrent sustained monomorphic ventricular tachycardia (VT) of 200 bpm and right bundle branch block (RBBB) morphology. Cardiac magnetic resonance imaging showed late gadolinium enhancement distributed circumferentially in the epicardial layer of the LV free wall myocardium including the rightward portion of the interventricular septum (IVS). The clinical RBBB VT was reproduced during the EP study. Ablation at an LV septum site with absence of abnormal electrograms and a suboptimum pacemap rendered the VT of clinical morphology noninducible. Three other VTs, all of left bundle branch block (LBBB) pattern, were induced by programmed electrical stimulation. The regions corresponding to abnormal electrograms were identified and ablated at the mid-to-apical RV septum and the anteroseptal portion of the right ventricular outflow tract. No abnormalities were found at the RV free wall including the inferolateral peritricuspid annulus region. Histological examination confirmed the presence of abnormal fibrous and adipose tissue with myocyte reduction in endomyocardial samples taken from both the left and right aspects of the IVS. Conclusion: LDAC rarely manifests with sustained monomorphic ventricular tachycardia. In this case, several VTs of both RBBB and LBBB morphology were amenable to endocardial radiofrequency catheter ablation. [ABSTRACT FROM AUTHOR]

Background: Pulsed wave tissue Doppler echocardiography (PW-TDE) and color M-mode are new Doppler methods for left ventricular (LV) diastolic function assessment. To date, few studies have compared the data obtained by these methods in the same series of patients and compared them to the current clinical reference method of detecting LV diastolic function.Aims: To determine the utility of PW-TDE and color M-mode parameters in the assessment of LV diastolic function in the typical patient population encountered in daily clinical practice and to compare their discriminating power.Methods: Early diastolic septal mitral annular velocity (Em) determined by PW-TDE and color M-mode flow propagation velocity (Vp) were measured in 86 male patients and compared to LV filling patterns obtained using standard Doppler indices. Values of Em<0.08 m s−1 and Vp<0.5 m s−1 were considered as markers of abnormal LV diastolic function.Results: A value of Em<0.08 m s−1 distinguished mild to moderate LV diastolic dysfunction with higher sensitivity and specificity than Vp<0.5 m s−1 (96% and 87% vs. 73% and 84%, respectively). A comparison of receiver operating characteristic curves showed a significant difference for areas under the curve in favor of Em (p<0.01). In a stepwise multiple logistic regression analysis, a pseudonormal filling pattern and an EF > 60% were identified as significant predictors of Vp false negative results (p<0.05).Conclusions: Em appears to be superior to Vp in the detection of mild to moderate LV diastolic dysfunction. Vp failed to detect abnormal LV diastolic function in particular in patients with preserved LV systolic function and a pseudonormal filling pattern type. [ABSTRACT FROM AUTHOR]