A better understanding of the left ventricle (LV) and right ventricle (RV) functioning would help with the differentiation between athlete’s heart and dilated cardiomyopathy (DCM). We aimed to analyse deformation parameters in endurance athletes relative to patients with DCM using cardiac magnetic resonance feature tracking (CMR-FT). The study included males of a similar age: 22 ultramarathon runners, 22 patients with DCM and 21 sedentary healthy controls (41 ± 9 years). The analysed parameters were peak LV global longitudinal, circumferential and radial strains (GLS, GCS and GRS, respectively); peak LV torsion; peak RV GLS. The peak LV GLS was similar in controls and athletes, but lower in DCM (p < 0.0001). Peak LV GCS and GRS decreased from controls to DCM (both p < 0.0001). The best value for differentiation between DCM and other groups was found for the LV ejection fraction (area under the curve (AUC) = 0.990, p = 0.0001, with 90.9% sensitivity and 100% specificity for ≤53%) and the peak LV GRS diastolic rate (AUC = 0.987, p = 0.0001, with 100% sensitivity and 88.4% specificity for >−1.27 s⁻¹). The peak LV GRS diastolic rate was the only independent predictor of DCM (p = 0.003). Distinctive deformation patterns that were typical for each of the analysed groups existed and can help to differentiate between athlete’s heart, a nonathletic heart and a dilated cardiomyopathy.

Background: Peripartum (PPCM) and dilated (DCM) cardiomyopathies are distinct forms of cardiac disease that share certain aspects in clinical presentation. Aim: We hypothesized that different cardiac structural changes underlie PPCM and DCM, and we aimed to investigate them with cardiovascular magnetic resonance (CMR). Methods: We included 21 PPCM patients (30.5 ± 5.9 years) and 30 female DCM patients (41.5 ± 16.8 years) matched for left ventricular ejection fraction. Biventricular and biatrial volumetric and functional parameters were assessed along with ventricular and atrial strain indices based on feature-tracking techniques. The presence of late gadolinium enhancement (LGE) was also assessed. Results: In PPCM, the left ventricular (LV) stroke volume index was lower (p = 0.04), right atrial (RA) minimal and pre-systolic volumes were higher (p < 0.01 and p = 0.02, respectively), and the total RA ejection fraction was lower (p = 0.02) in comparison to DCM. Moreover, in PPCM, the LV global longitudinal strain (p = 0.03), global circumferential strain rate (p = 0.04), and global longitudinal strain rate (p < 0.01) were less impaired than in DCM. Both PPCM and DCM patients with LGE had more dilated ventricles and more impaired LV and left atrial function than in PPCM and DCM patients without LGE. Conclusions: Subtle differences appear on CMR between PPCM and DCM. Most importantly, the RA is larger and more impaired, and LV global longitudinal strain is less reduced in PPCM than in DCM. Furthermore, similarly to DCM, PPCM patients with LGE have more dilated and impaired ventricles than patients without LGE.

To investigate biatrial mechanics and their relation with left ventricular outflow tract (LVOT) obstruction (LVOTO), the degree of hypertrophy, indices of ventricular diastolic function and fibrosis in children with hypertrophic cardiomyopathy (HCM).

Background and aim: We sought to search for factors that are associated with the magnitude of trabeculation by cardiac magnetic resonance (CMR), and evaluate the impact of trabeculations on outcomes in patients with dilated cardiomyopathy (DCM). Methods: We evaluated clinical profiles and outcomes of 276 subjects with DCM (age - 33,2+/-13,3 years, 160 males). Trabeculation was quantified as trabeculated/total myocardial mass ratio (TM/M). Subjects were stratified into 3 subgroups (A, B, and C) according to the tertiles of rising TM/M values (33% ranges). A group of 30 healthy subjects served as controls. Patients were prospectively followed up in search for major adverse cardiovascular events for 2,4 years on average (range 0,2-3,9 years). Results: DCM patients had more trabeculation than controls (27,1+/-16,9% vs 17,3+/-8,1; p<0,01). Group C subjects had lowest NT-proBNP [1445 (984 – 3843) vs 873 (440-2633) vs 529 (206-1221) pg/ml; p<0,01], higher ejection fraction [23,9+/10,4 vs 25,0+/-9.2 vs 32,4+/-2,7%; p=0,03] and lower left ventricular mass index (LVMI) [91,3+/-21,5 vs 74,3+/-31,1 vs 55,7+/-23,2g/m2; p<0,01]. They also had less areas of lge [69(46,3%) vs 31(38,2%) vs 15 (32,6%); p=0,01]. Male sex (b=0,21, SE=0,13; p=0,01), LVMI (b=-0,32, SE=0,08; p<0,01) and NT-proBNP (b=-0,05, SE=0,02; p=0,02) independently related to TM/M. The magnitude of trabeculation was not a predictor of MACE-free survival. Prognosis was impacted by left ventricular end-diastolic volume index only (HR=2,538, 95%CI - 1,734-3,218; p<0,01). Conclusions: Trabeculation patterns do relate to cardiac function and neurohomonal activation but not to survival. Background and aim: We sought to search for factors that are associated with the magnitude of trabeculation by cardiac magnetic resonance (CMR), and evaluate the impact of trabeculations on outcomes in patients with dilated cardiomyopathy (DCM). Methods: We evaluated clinical profiles and outcomes of 276 subjects with DCM (age - 33,2+/-13,3 years, 160 males). Trabeculation was quantified as trabeculated/total myocardial mass ratio (TM/M). Subjects were stratified into 3 subgroups (A, B, and C) according to the tertiles of rising TM/M values (33% ranges). A group of 30 healthy subjects served as controls. Patients were prospectively followed up in search for major adverse cardiovascular events for 2,4 years on average (range 0,2-3,9 years). Results: DCM patients had more trabeculation than controls (27,1+/-16,9% vs 17,3+/-8,1; p<0,01). Group C subjects had lowest NT-proBNP [1445 (984 – 3843) vs 873 (440-2633) vs 529 (206-1221) pg/ml; p<0,01], higher ejection fraction [23,9+/10,4 vs 25,0+/-9.2 vs 32,4+/-2,7%; p=0,03] and lower left ventricular mass index (LVMI) [91,3+/-21,5 vs 74,3+/-31,1 vs 55,7+/-23,2g/m2; p<0,01]. They also had less areas of lge [69(46,3%) vs 31(38,2%) vs 15 (32,6%); p=0,01]. Male sex (b=0,21, SE=0,13; p=0,01), LVMI (b=-0,32, SE=0,08; p<0,01) and NT-proBNP (b=-0,05, SE=0,02; p=0,02) independently related to TM/M. The magnitude of trabeculation was not a predictor of MACE-free survival. Prognosis was impacted by left ventricular end-diastolic volume index only (HR=2,538, 95%CI - 1,734-3,218; p<0,01). Conclusions: Trabeculation patterns do relate to cardiac function and neurohomonal activation but not to survival.

Background: LMNA mutations are most frequently involved in the pathogenesis of dilated cardiomyopathy with conduction disease. The goal of this study was to identify LMNA mutations, estimate their frequency among Polish dilated cardiomyopathy patients and characterize their effect both in vivo and in vitro. Methods: Between January, 2008 and June, 2012 two patient populations were screened for the presence of LMNA mutations by direct sequencing: 66 dilated cardiomyopathy patients including 27 heart transplant recipients and 39 dilated cardiomyopathy patients with heart failure referred for heart transplantation evaluation, and 44 consecutive dilated cardiomyopathy patients, referred for a family evaluation and mutation screening. Results: We detected nine non-synonymous mutations including three novel mutations: p.Ser431*, p.Val256Gly and p.Gly400Argfs*11 deletion. There were 25 carriers altogether in nine families. The carriers were mostly characterized by dilated cardiomyopathy and heart failure with conduction system disease and/or complex ventricular arrhythmia, although five were asymptomatic. Among the LMNA mutation carriers, six underwent heart transplantation, fourteen ICD implantation and eight had pacemaker. In addition, we obtained ultrastructural images of cardiomyocytes from the patient carrying p.Thr510Tyrfs*42. Furthermore, because the novel p.Val256Gly mutation was found in a sporadic case, we verified its pathogenicity by expressing the mutation in a cellular model. Conclusions: In conclusion, in the two referral centre populations, the screening revealed five mutations among 66 heart transplant recipients or patients referred for heart transplantation (7.6%) and four mutations among 44 consecutive dilated cardiomyopathy patients referred for familial evaluation (9.1%). Dilated cardiomyopathy patients with LMNA mutations have poor prognosis, however considerable clinical variability is present among family members.