Mutations in the lamin A/C gene are variably phenotypically expressed; however, it is unclear whether circulating cardiac biomarkers are helpful in the detection and risk assessment of cardiolaminopathies. We sought to assess (1) clinical characteristics including serum biomarkers: high sensitivity troponin T (hsTnT) and N-terminal prohormone brain natriuretic peptide (NT-proBNP) in clinically stable cardiolaminopathy patients, and (2) outcome among pathogenic/likely pathogenic lamin A/C gene (LMNA) mutation carriers. Our single-centre cohort included 53 patients from 21 families. Clinical, laboratory, follow-up data were analysed. Median follow-up was 1522 days. The earliest abnormality, emerging in the second and third decades of life, was elevated hsTnT (in 12% and in 27% of patients, respectively), followed by the presence of atrioventricular block, heart failure, and malignant ventricular arrhythmia (MVA). In patients with missense vs. other mutations, we found no difference in MVA occurrence and, surprisingly, worse transplant-free survival. Increased levels of both hsTnT and NT-proBNP were strongly associated with MVA occurrence (HR > 13, p ≤ 0.02 in both) in univariable analysis. In multivariable analysis, NT-proBNP level > 150 pg/mL was the only independent indicator of MVA. We conclude that assessment of circulating cardiac biomarkers may help in the detection and risk assessment of cardiolaminopathies.

Use of preoperative echocardiography may help to identify patients with increased cardiac risk, who may benefit from modification of perioperative plan. The objective of our study was to evaluate the reliability of preoperative focused cardiac ultrasound (FoCUS) performed by an anaesthetist with basic ultrasound training and its impact on patient’s management. The prospective observational study was conducted in 159 adult patients, scheduled for elective operations. Cardiac ultrasound was performed by one anaesthetist with a limited experience of FoCUS. A simple, mnemonic scheme was used for the final reporting of each study. The same scheme was used by a cardiologist who produced an independent report based on digital video loops stored in the machine memory. Anaesthetists in-charge made final perioperative plan. Comparative analysis of anaesthetist and cardiologist performed ultrasound report was made. The incidence of modification of initial perioperative plan resulting from FoCUS report was analyzed. The average time required to complete the examination was 182 s 95 % CI [173–190]. Images of quality adequate to answer all questions from the scheme were obtained in 97.5 % (155/159) of patients. There was strong agreement between the anaesthetist and the cardiologist in 97.8 % (2274/2325) of the examined categories. In two categories (global and regional left ventricle contractility impairment) statistically significant discrepancies between both diagnosticians were confirmed (p McNemar <0.04). When compared with the cardiologist’s assessment the agreement of the anesthetist’s diagnosis had sensitivity of 0.84, specificity 0.99, positive predictive value 0.78 and negative predictive value 0.99. Kappa statistics showed good agreement between both examining doctors (κ = 0.797). Based on ultrasound findings, the preliminary anaesthetic plan was changed in relation to 20.8 % (33/159) of patients. An anaesthetist with limited training in FoCUS can perform a reliable preoperative examination which alters the perioperative management.

Background: Previously presented new electrocardiography (ECG) algorithm for localization of arrhythmogenic focus (AFo) in right ventricular outflow tract (RVOT) was based on spontaneous arrhythmia QRS morphology analysis. The aim of this study was to estimate the clinical value of our RVOT algorithm in a prospective study. Methods and results: Algorithm validation was made on 62 patients with RVOT arrhythmias (45 women), mean age 41.6 ± 14.3 years, scheduled for transcatheter ablation. Results of preablation ECG analysis with RVOT algorithm were matched with successful ablation sites and statistical indices: sensitivity (sens), specificity (spec), and positive and negative predictive values (PPV, NPV) were calculated for algorithm and for each of 9 RVOT zones (septal and free wall). An algorithm precisely localized AFo in 57 out of 62 patients (sens 91.3%, spec 99%, PPV 91%, NPV 98.8%). Sensitivity values for superior RVOT aspect (71% patients) varied from 88% to 100%, specificity from 95.9% to 100%; PPV values from 85.7% to 100%, NPV from 92.5% to 100%. Although the total number of patients was relatively small in the 2 remaining RVOT aspects (29% patients) high values (sens, spec, PPV, NPV) were gained for intermediate and inferior zones. Conclusions: On the basis of spontaneous arrhythmia QRS analysis, a novel algorithm was built for preablation localization of RVOT arrhythmia in 1 of the 9 RVOT zones. Prospective analysis of our ECG algorithm confirmed that it is a valuable tool to predict the site of successful ablation in patients with RVOT arrhythmias. Background: Previously presented new electrocardiography (ECG) algorithm for localization of arrhythmogenic focus (AFo) in right ventricular outflow tract (RVOT) was based on spontaneous arrhythmia QRS morphology analysis. The aim of this study was to estimate the clinical value of our RVOT algorithm in a prospective study. Methods and results: Algorithm validation was made on 62 patients with RVOT arrhythmias (45 women), mean age 41.6 ± 14.3 years, scheduled for transcatheter ablation. Results of preablation ECG analysis with RVOT algorithm were matched with successful ablation sites and statistical indices: sensitivity (sens), specificity (spec), and positive and negative predictive values (PPV, NPV) were calculated for algorithm and for each of 9 RVOT zones (septal and free wall). An algorithm precisely localized AFo in 57 out of 62 patients (sens 91.3%, spec 99%, PPV 91%, NPV 98.8%). Sensitivity values for superior RVOT aspect (71% patients) varied from 88% to 100%, specificity from 95.9% to 100%; PPV values from 85.7% to 100%, NPV from 92.5% to 100%. Although the total number of patients was relatively small in the 2 remaining RVOT aspects (29% patients) high values (sens, spec, PPV, NPV) were gained for intermediate and inferior zones. Conclusions: On the basis of spontaneous arrhythmia QRS analysis, a novel algorithm was built for preablation localization of RVOT arrhythmia in 1 of the 9 RVOT zones. Prospective analysis of our ECG algorithm confirmed that it is a valuable tool to predict the site of successful ablation in patients with RVOT arrhythmias.

Background: The impact of cardiac rehabilitation on the number of alerts in patients with remote monitoring (RM) of cardiac implantable electronic devices (CIEDs) is unknown. We compared alerts in RM and outcomes in patients with CIEDs undergoing hybrid comprehensive telerehabilitation (HCTR) versus usual care (UC). Methods: Patients with heart failure (HF) after a hospitalization due to worsening HF within the last 6 months (New York Heart Association (NYHA) class I-III and left ventricular ejection fraction (LVEF) ≤40%) were enrolled in the TELEREH-HF study and randomised 1:1 to HCTR or UC. Patients with HCTR and CIEDs received RM (HCTR-RM). Patients with UC and CIEDs were offered RM optionally (UC-RM). Data from the initial 9 weeks of the study were analysed. Results: Of 850 enrolled patients, 208 were in the HCTR-RM group and 62 in the UC-RM group. The HCTR-RM group was less likely to have alerts of intrathoracic impedance (TI) decrease (p < 0.001), atrial fibrillation (AF) occurrence (p = 0.031) and lower mean number of alerts per patient associated with TI decrease (p < 0.0001) and AF (p = 0.019) than the UC-RM group. HCTR significantly decreased the occurrence of alerts in RM of CIEDs, 0.360 (95%CI, 0.189–0.686; p = 0.002), in multivariable regression analysis. There were two deaths in the HCTR-RM group (0.96%) and no deaths in the UC-RM group (p = 1.0). There were no differences in the number of hospitalised patients between the HCTR-RM and UC-RM group (p = 1.0). Conclusions: HCTR significantly reduced the number of patients with RM alerts of CIEDs related to TI decrease and AF occurrence. There were no differences in mortality or hospitalisation rates between HCTR-RM and UC-RM groups.