Search

Background: The optimal cardiovascular assessment of liver transplant (LT) candidates is unclear. We aimed to evaluate the performance of CT‐based coronary tests (coronary artery calcium score [CACS] and coronary CT angiography [CCTA]) and a modification of the CAD‐LT score (mCAD‐LT, excluding family history of CAD) to diagnose significant coronary artery disease (CAD) before LT and predict the incidence of post‐LT cardiovascular events (CVE). Methods: We retrospectively analysed a single‐centre cohort of LT candidates who underwent non‐invasive tests; invasive coronary angiography (ICA) was performed depending on the results of non‐invasive tests. mCAD‐LT was calculated in all patients. Results: Six‐hundred‐and‐thirty‐four LT candidates were assessed and 351 of them underwent LT. CACS, CCTA and ICA were performed in 245, 123 and 120 LT candidates, respectively. Significant CAD was found in 30% of patients undergoing ICA. The AUROCs of mCAD‐LT (.722) and CCTA (.654) were significantly higher than that of CACS (.502) to predict the presence of significant CAD. Specificity of the tests ranged between 31% for CCTA and 53% for CACS. Among patients who underwent LT, CACS ≥ 400 and mCAD‐LT were independently associated with the incidence of CVE; in patients who underwent CCTA before LT, significant CAD at CCTA also predicted post‐LT CVE. Conclusion: In this cohort, mCAD‐LT score and CT‐based tests detect the presence of significant CAD in LT candidates, although they tend to overestimate it. Both mCAD‐LT score and CT‐based tests classify LT recipients according to their risk of post‐LT CVE and can be used to improve post‐LT risk mitigation. [ABSTRACT FROM AUTHOR]

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiac disease characterized by the presence of fibrofatty replacement of the right ventricular myocardium, which may cause ventricular arrhythmias and sudden cardiac death. Pathogenic mutations in several genes encoding mainly desmosomal proteins have been reported. Our aim is to perform genotype-phenotype correlations to establish the diagnostic value of genetics and to assess the role of mutation type in age-related penetrance in ARVC. Methods and Results: Thirty unrelated Spanish patients underwent a complete clinical evaluation. They all were screened for PKP2, DSG2, DSC2, DSP, JUP and TMEM43 genes. A total of 70 relatives of four families were also studied. The 30 patients fulfilled definite disease diagnostic criteria. Genetic analysis revealed a pathogenic mutation in 19 patients (13 in PKP2, 3 in DSG2, 2 in DSP, and 1 in DSC2). Nine of these mutations created a truncated protein due to the generation of a stop codon. Familial assessment revealed 28 genetic carriers among family members. Stop-gain mutations were associated to a later age of onset of ARVC, without differences in the severity of the pathology. Conclusions: Familial genetic analysis helps to identify the cause responsible for the pathology. In discrepancy with previous studies, the presence of a truncating protein does not confer a worse severity. This information could suggest that truncating proteins may be compensated by the normal allele and that missense mutations may act as poison peptides. [ABSTRACT FROM AUTHOR]

Abstract: Background: Cardiac acute beriberi (Shoshin syndrome) is a rare disease that may lead to a fatal outcome if not treated specifically. Objectives: We report a case of Shoshin syndrome with an unusual presentation of cardiogenic shock and an electrocardiographic pattern of severe myocardial ischemia suggesting left main coronary artery obstruction. Case Report: A 35-year-old man presented with chest discomfort, diffuse ST-segment depression in the 12-lead electrocardiogram (ECG) with ST-segment elevation in aVR, and rapidly evolving congestive heart failure leading to cardiogenic shock. Intensive support was required, including mechanical ventilation, high doses of inotropics and vasopressors, intra-aortic balloon counterpulsation, and continuous renal replacement therapy. An emergency coronary angiogram was performed that showed normal coronary arteries. Right heart catheterization showed a high-output state with elevated filling pressures suggesting high-output heart failure. The echocardiography confirmed normal left and right ventricular contraction. Thiamine deficiency was suspected as the cause of the high-output heart failure. After a single dose of intravenous thiamine (100 mg), the patient''s hemodynamic status improved dramatically within minutes, allowing a rapid discontinuation of hemodynamic support. Subsequent ECGs showed complete resolution of ST-segment abnormalities. Serial lactate measurements, red blood cell transketolase activity, and the thiamine pyrophosphate response test were concordant with a thiamine deficiency state. Conclusion: Shoshin syndrome may present as cardiogenic shock with an ECG mimicking severe myocardial ischemia, and if suspected, can be rapidly and effectively treated. [Copyright &y& Elsevier]

Providing therapies tailored to each patient is the vision of precision medicine, enabled by the increasing ability to capture extensive data about individual patients. In this position paper, we argue that the second enabling pillar towards this vision is the increasing power of computers and algorithms to learn, reason, and build the 'digital twin' of a patient. Computational models are boosting the capacity to draw diagnosis and prognosis, and future treatments will be tailored not only to current health status and data, but also to an accurate projection of the pathways to restore health by model predictions. The early steps of the digital twin in the area of cardiovascular medicine are reviewed in this article, together with a discussion of the challenges and opportunities ahead. We emphasize the synergies between mechanistic and statistical models in accelerating cardiovascular research and enabling the vision of precision medicine., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Objectives: This study assessed the feasibility and potential benefit of performing ventricular tachycardia (VT) substrate ablation procedures guided by cardiac magnetic resonance (CMR)-derived pixel signal intensity (PSI) maps., Background: CMR-aided VT ablation using PSI maps from late gadolinium enhancement-CMR (LGE-CMR), together with electroanatomical map (EAM) information, has been shown to improve outcomes of VT substrate ablation., Methods: Eighty-four patients with scar-dependent monomorphic VT who underwent substrate ablation were included in the study. In the last 28 (33%) consecutive patients, the procedure was guided by CMR. Procedural data, as well as acute and follow-up outcomes, were compared between patients who underwent guided CMR and 2 control groups: 1) patients who had PSI maps were available but the EAM was acquired and used to select the ablation targets (CMR aided); and 2) patients with no CMR-derived PSI maps available (no CMR)., Results: Mean procedure duration was lower in CMR-guided substrate ablation compared with CMR-aided and no CMR (107 ± 59 min vs. 203 ± 68 min and 227 ± 52 min; p < 0.001 for both comparisons). CMR-guided ablation required less fluoroscopy time than CMR-aided ablation and no CMR (10 ± 4 min vs. 23 ± 11 min and 20 ± 9 min, respectively; p < 0.001 for both comparisons) and less radiofrequency time (15 ± 8 min vs. 20 ± 15 min and 26 ± 10 min; p = 0.16 and p < 0.001, respectively). After substrate ablation, VT inducibility was lower in CMR-guided ablation compared with CMR-aided ablation and no CMR (18% vs. 32% and 46%; p = 0.35 and p = 0.04, respectively), without significant differences in complications. After 12 months, VT recurrence was lower in those who underwent CMR-guided ablation compared with no CMR (log-rank: 0.019), with no differences with CMR-aided ablation., Conclusions: CMR-guided VT ablation is feasible and safe, significantly reduces the procedural, fluoroscopy, and radiofrequency times, and is associated with a higher noninducibility rate and lower VT recurrence after substrate ablation., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)