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Aims Arrhythmogenic cardiomyopathy (AC) shows large heterogeneity in its clinical, genetic, and pathological presentation. This study aims to provide a comprehensive atlas of end-stage AC and illustrate the relationships among clinical characteristics, genotype, and pathological profiles of patients with this disease. Methods and results We collected 60 explanted AC hearts and performed standard pathology examinations. The clinical characteristics of patients, their genotype and cardiac magnetic resonance imaging findings were assessed along with pathological characteristics. Masson staining of six representative sections of each heart were performed. Digital pathology combined with image segmentation was developed to calculate distribution of myocardium, fibrosis, and adipose tissue. An unsupervised clustering based on fibrofatty distribution containing four subtypes was constructed. Patients in Cluster 1 mainly carried desmosomal mutations (except for desmoplakin) and were subjected to transplantation at early age; this group was consistent with classical 'desmosomal cardiomyopathy'. Cluster 2 mostly had non-desmosomal mutations and showed regional fibrofatty replacement in right ventricle. Patients in Cluster 3 showed parallel progression, and included patients with desmoplakin mutations. Cluster 4 is typical left-dominant AC, although the genetic background of these patients is not yet clear. Multivariate regression analysis revealed precordial QRS voltage as an independent indicator of the residual myocardium of right ventricle, which was validated in predicting death and transplant events in the validation cohort (n  = 92). Conclusion This study provides a novel classification of AC with distinct genetic backgrounds indicating different potential pathogenesis. Cluster 1 is distinct in genotype and clinicopathology and can be defined as 'desmosomal cardiomyopathy'. Precordial QRS amplitude is an independent indicator reflecting the right ventricular remodelling, which may be able to predict transplant/death events for AC patients. View large Download slide View large Download slide [ABSTRACT FROM AUTHOR]

The aim of this study is to report the combined application of extracorporeal membrane oxygenation (ECMO) with intra‐aortic balloon pumping (IABP) in postcardiotomy cardiac shock (PCS). A total of 60 consecutive patients who received both ECMO and IABP (concomitantly 24 hours) for PCS from February 2006 to March 2017 at Fuwai Hospital were included in our study. Clinical characteristics of the patients were collected retrospectively and compared between survivors and non‐survivors. Logistic regression analysis was used as predictors for survival to discharge. The study cohort had a mean age of 51.4±12.7 years with 75% males. ECMO was implanted intra‐operatively in 38 (63%) patients and post‐operatively in 22 (37%) patients. ECMO was implanted concurrently with IABP in 38 (63%) patients. Heart transplantation (38%) and coronary artery bypass graft (33%) were the main surgical procedures. ECMO was weaned successfully in 48% patients, and the rate of survival to discharge was 43%. Survivors showed less bedside ECMO implantation (12% vs. 41%, P=0.012) and more concurrent implantation of ECMO with IABP (81% vs. 50%, P=0.014). Concurrent implantation of IABP with ECMO (OR=0.177, P=0.015, 95% CI: 0.044‐0.718) was an independent predictor of survival to discharge. As for complications, the rate of renal failure (59% vs. 15%, P=0.001) and multiple organ dysfunction syndrome (29% vs. 0, P=0.003) was higher in patients who failed to survive to discharge. Patients who had heart transplantation had a better long‐term survival than others (P=0.0358). In summary, concurrent implantation of ECMO with IABP provides better short‐term outcome for PCS and combined application of ECMO with IABP for PCS after heart transplantation had a favorable long‐term outcome. [ABSTRACT FROM AUTHOR]

Heart failure (HF) is a clinical syndrome caused by a decline in cardiac systolic or diastolic function, which leaves the heart unable to pump enough blood to meet the normal physiological requirements of the human body. It is a serious disease burden worldwide affecting nearly 23 million patients. The concept that heart failure is "an engine out of fuel" has been generally accepted and metabolic remodeling has been recognized as an important aspect of this condition; it is characterized by defects in energy production and changes in metabolic pathways involved in the regulation of essential cellular functions such as the process of substrate utilization, the tricarboxylic acid cycle, oxidative phosphorylation, and high-energy phosphate metabolism. Advances in second-generation sequencing, proteomics, and metabolomics have made it possible to perform comprehensive tests on genes and metabolites that are crucial in the process of HF, thereby providing a clearer and comprehensive understanding of metabolic remodeling during HF. In recent years, new metabolic changes such as ketone bodies and branched-chain amino acids were demonstrated as alternative substrates in end-stage HF. This systematic review focuses on changes in metabolic substrate utilization during the progression of HF and the underlying regulatory mechanisms. Accordingly, the conventional concepts of metabolic remodeling characteristics are reviewed, and the latest developments, particularly multi-omics studies, are compiled. [ABSTRACT FROM AUTHOR]

The present study sought to compare postoperative bleeding and renal function in patients with multivessel coronary artery disease undergoing simultaneous hybrid coronary revascularization (HCR) and minimally invasive direct off-pump coronary artery bypass grafting (MIDCABG). The study retrospectively collected the data of 594 consecutive patients who underwent simultaneous HCR and 351 patients who underwent MIDCABG with planned staged HCR (MIDCABG first, then elective percutaneous coronary intervention) in Fuwai Hospital from June 2007 to December 2020. A total of 317 pairs of patients who were matched in a 1:1 ratio with propensity score matching were enrolled in this study. Bleeding and changes in renal function were compared between the 2 groups. Compared with patients who underwent MIDCABG, patients who underwent simultaneous HCR had significantly greater chest tube drainage on the day of the operation (492.7 ± 282.4 mL vs 441.0 ± 261.9 mL; P =.023), but no significant difference was detected in the total amount during the postoperative period (788.8 ± 458.9 mL vs 753.3 ± 409.8 mL; P =.74). The differences in re-exploration for bleeding (0.3% vs 1.6%; P =.13), blood transfusion (18.9% vs 16.4%; P =.13), acute kidney injury (23.3% vs 18.6%; P =.53), and in-hospital major adverse cardiovascular and cerebrovascular events (including all-cause death, myocardial infarction, stroke, and repeated revascularization) (2.5% vs 1.9%; P =.67) between the 2 groups did not reach statistical significance. Compared with MIDCABG, simultaneous HCR (MIDCABG first, instant percutaneous coronary intervention) did not increase postoperative bleeding or the incidence of acute kidney injury. [Display omitted] Compared with minimally invasive direct off-pump coronary artery bypass grafting (MIDCABG), simultaneous hybrid coronary revascularization (HCR) (MIDCABG first, instant percutaneous coronary intervention [PCI]) did not increase postoperative bleeding or renal dysfunction. The lower and upper borders of the box represent the lower and upper quartiles (25th percentile and 75th percentile). The middle horizontal line represents the median. The lower and upper whiskers represent the minimum and maximum values of nonoutliers. DAPT , Dual antiplatelet therapy; LIMA-LAD , left internal thoracic artery-left anterior descending coronary artery. [Display omitted] [ABSTRACT FROM AUTHOR]

For transposition of the great arteries with unrestricted ventricular septal defect and pulmonary stenosis, double-root translocation is reported to reconstruct ideal double artery roots with growth potential. However, prospective long-term studies describing the long-term outcomes are still scarce. Therefore, the aim was to assess development of double artery roots, hemodynamics, and freedom from death and heart failure 17 years after double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire procedures. In this prospective population-based study, 266 patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis (from July 2004 to August 2021) were consecutively included before surgery. All patients were divided into 3 groups based on the type of operation: double-root translocation (174), Rastelli (68), and Réparation à l'Etage Ventriculaire (24), who accepted postoperative evaluations annually. Generalized linear mixed model analysis was performed to determine growth potential of artery roots. Longitudinal repeated computed tomography measurements show the pulmonary root has significantly increased diameter (0.62 [0.03] mm/y, P <.001) over time and an adequate Z-score (−0.18) at the last follow-up only in the double-root translocation group. The pressure gradients of double outflow tracts in the double-root translocation group were the least among 3 groups. The probabilities of freedom from death/heart failure at the 15th year were 73.1%, 59.3%, and 60.9% in the double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire groups, respectively (double-root translocation vs Rastelli, P =.026; double-root translocation vs Réparation à l'Etage Ventriculaire, P =.009; Rastelli vs Réparation à l'Etage Ventriculaire, P =.449). By reconstructing ideal double artery roots, double-root translocation can provide postoperative long-term excellent hemodynamics and minimal death and heart failure for patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis. A prospective observed cohort study was conducted for patients with TGA/VSD/PS who received biventricular repair from July 2004 to August 2021. The study shows that the pulmonary root had optimal growth potential and superior long-term outcomes of hemodynamics for patients undergoing the DRT procedure. The probability of freedom from death/heart failure in the DRT group is significantly more than in the Rastelli or REV group. In biventricular repair for TGA/VSD/PS, the DRT procedure provided superior long-term outcomes compared with the Rastelli or REV procedure. [Display omitted] [ABSTRACT FROM AUTHOR]

We investigated the effect of repeated remote ischaemic post-conditioning (RIPoC) on the organ distribution of the intramyocardially injected MSCs in rat myocardial ischemia model. Myocardial ischemia of adult female Sprague-Dawley rats was induced by 30-min. obstruction of the left anterior descending coronary artery. Repeated RIPoC was induced after ischemia with three cycles of 5-min. occlusion and reperfusion of the limb with the frequency of half a day, 1 or 2 days, respectively. Compared with that by single RIPoC, repeated RIPoC transiently reduced oxidative stress, lipid peroxidation and inflammation in ischaemic myocardium; the gene expression of stromal cell-derived factor-1 alpha (SDF-1α) was consistently induced by repeated RIPoC procedures. A total of 4 × 106 male bone marrow-derived MSCs were intramyocardially injected into ischaemic myocardium at 1 week after reperfusion. Three weeks later, immunohistological examination and quantitative reverse transcriptase polymerase chain reaction demonstrated that repeated RIPoC significantly increased MSCs retention in myocardium and decreased MSCs distribution over the lungs, spleen and liver; echocardiography assessment revealed that further cardiac function enhancement imposed by repeated RIPoC procedure. Furthermore, blockade with the anti-CXCR4 antibody before cell transplantation markedly attenuated the benefits of therapeutic efficacy and cardiac function. Repeated RIPoC enhanced MSCs engraftment in ischaemic myocardium and reduced the distribution of MSCs over peripheral organs in a frequency-effect way, but it reached a ceiling of maximal effect when applied with every 1 day. The SDF1α-CXCR4 interaction played a major role in MSCs systemic redistribution induced by repeated RIPoC. [ABSTRACT FROM AUTHOR]