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Mutations in nuclear genes regulating mitochondrial DNA (mtDNA) replication are associated with mtDNA depletion syndromes. Using whole-genome sequencing, we identified a heterozygous mutation (c.272G>A:p.Arg91Gln) in single-stranded DNA-binding protein 1 (SSBP1), a crucial protein involved in mtDNA replisome. The proband manifested symptoms including sensorineural deafness, congenital cataract, optic atrophy, macular dystrophy, and myopathy. This mutation impeded multimer formation and DNA-binding affinity, leading to reduced efficiency of mtDNA replication, altered mitochondria dynamics, and compromised mitochondrial function. To correct this mutation, we tested two adenine base editor (ABE) variants on patient-derived fibroblasts. One variant, NG-Cas9-based ABE8e (NG-ABE8e), showed higher editing efficacy (≤30%) and enhanced mitochondrial replication and function, despite off-target editing frequencies; however, risks from bystander editing were limited due to silent mutations and off-target sites in non-translated regions. The other variant, NG-Cas9-based ABE8eWQ (NG-ABE8eWQ), had a safer therapeutic profile with very few off-target effects, but this came at the cost of lower editing efficacy (≤10% editing). Despite this, NG-ABE8eWQ-edited cells still restored replication and improved mtDNA copy number, which in turn recovery of compromised mitochondrial function. Taken together, base editing-based gene therapies may be a promising treatment for mitochondrial diseases, including those associated with SSBP1 mutations.
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OBJECTIVES: The optimal technique for repairing posterior mitral valve leaflet prolapse remains undetermined. We aimed to compare leaflet resection and neochordae implantation in patients undergoing mitral valve repair for posterior leaflet prolapse, focusing on transmitral pressure gradient and recurrence of mitral regurgitation. METHODS: We enrolled patients undergoing mitral valve repair using either leaflet resection or neochordae implantation for single-segment prolapse of posterior mitral valve leaflet between 2000 and 2021 at our institution. Longitudinal outcomes were evaluated after adjustments with inverse-probability-of-treatment weighting. Repeat echocardiographic measurements (n = 3473, 5.4/patient) of transmitral pressure gradient and significant (moderate or severe) mitral regurgitation recurrence were estimated using nonlinear mixed-effect models. Subgroup analyses were conducted based on the size and type of prosthesis. RESULTS: Among 639 patients, leaflet resection was used in 479 (75.0%) and neochordae implantation was used in 160 (25.0%). In the inverse-probability-of-treatment weighting adjusted cohort, the risk of death (P = .623) and mitral valve reoperation (P = .340) did not significantly differ between the 2 groups during a median follow-up of 97.3 months. Echocardiographic data showed comparable mean (at 5 years, 3.8 vs 4.0 mm Hg; P = .442) and peak (9.6 vs 10.4 mm Hg; P = .131) pressure gradients between groups, which persisted in most subgroup analyses. However, neochordae implantation was associated with a higher probability of significant mitral regurgitation recurrence compared with leaflet resection (at 5 years, 16.1% vs 7.0%; P < .001). CONCLUSIONS: Leaflet resection yielded similar clinical outcomes and transmitral pressure gradients compared with neochordae implantation after mitral valve repair, with a lower mitral regurgitation recurrence rate. These findings underscore the need to reassess the efficacy of neochordae implantation relative to leaflet resection.
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This study aimed to assess the lung transplantation (LT) outcomes of patients with right ventricular dysfunction (RVD), focusing on the impact of various extracorporeal membrane oxygenation (ECMO) configurations. We included adult patients who underwent LT with ECMO as a bridge-to-transplant from 2011 to 2021 at a single center. Among patients with RVD (n = 67), veno-venous (V-V) ECMO was initially applied in 79% (53/67) and maintained until LT in 52% (35/67). Due to the worsening of RVD, the configuration was changed from V-V ECMO to veno-arterial (V-A) ECMO or a right ventricular assist device with an oxygenator (Oxy-RVAD) in 34% (18/67). They showed that lactic acid levels (2-6.1 mmol/L) and vasoactive inotropic score (6.6-22.6) increased. V-A ECMO or Oxy-RVAD was initiated and maintained until LT in 21% (14/67) of cases. There was no significant difference in the survival rates among the three configuration groups (V-V ECMO vs. configuration changed vs. V-A ECMO/Oxy-RVAD). Our findings suggest that the choice of ECMO configuration for LT candidates with RVD should be determined by the patient's current hemodynamic status. Vital sign stability supports the use of V-V ECMO, while increasing lactic acid levels and vasopressor needs may require a switch to V-A ECMO or Oxy-RVAD.
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Oxigenación por Membrana Extracorpórea , Trasplante de Pulmón , Disfunción Ventricular Derecha , Humanos , Oxigenación por Membrana Extracorpórea/métodos , Masculino , Estudios Retrospectivos , Femenino , Persona de Mediana Edad , Disfunción Ventricular Derecha/terapia , Disfunción Ventricular Derecha/cirugía , Adulto , Resultado del Tratamiento , Corazón Auxiliar , AncianoRESUMEN
Supramolecular block copolymers, derived via seeded living polymerization, are increasingly recognized for their rich structural and functional diversity, marking them as cutting-edge materials. The use of metal complexes in supramolecular block copolymerization not only offers a broad range of block copolymers through the structural similarity in the coordination geometry of the central metal ion but also controls spectroscopic properties, such as emission wavelength, emission strength, and fluorescence lifetime. However, the exploration of metallosupramolecular multiblock copolymerization based on metal complexes remains quite limited. In this work, we present a pioneering synthesis of metallosupramolecular multiblock copolymers utilizing Eu3+ and Tb3+ complexes as building blocks. This is achieved through the strategic manipulation of nonequilibrium self-assemblies via a living supramolecular polymerization approach. Our comprehensive exploration of both thermodynamically and kinetically regulated metallosupramolecular polymerizations, centered around Eu3+ and Tb3+ complexes with bisterpyridine-modified ligands containing R-alanine units and a long alkyl group, has highlighted intriguing behaviors. The monomeric [R-L1Eu(NO3)3] complex generates a spherical structure as the kinetic product. In contrast, the monomeric [R-L1Eu2(NO3)6] complex generates fiber aggregates as a thermodynamic product through intermolecular interactions such as π-π stacking, hydrophobic interaction, and H-bonds. Utilizing the Eu3+ complex, we successfully conducted seed-induced living polymerization of the monomeric building unit under kinetically regulated conditions. This yielded a metallosupramolecular polymer of precisely controlled length with minimal polydispersity. Moreover, by copolymerizing the kinetically confined Tb3+ complex state ("A" species) with a seed derived from the Eu3+ complex ("B" species), we were able to fabricate metallosupramolecular tri- and pentablock copolymers with A-B-A, and B-A-B-A-B types, respectively, through a seed-end chain-growth mechanism.
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BACKGROUND AND OBJECTIVES: The number of sensitized heart failure patients on waiting lists for heart transplantation (HTx) is increasing. Using the Korean Organ Transplantation Registry (KOTRY), a nationwide multicenter database, we investigated the prevalence and clinical impact of calculated panel-reactive antibody (cPRA) in patients undergoing HTx. METHODS: We retrospectively reviewed 813 patients who underwent HTx between 2014 and 2021. Patients were grouped according to peak PRA level as group A: patients with cPRA ≤10% (n= 492); group B: patients with cPRA >10%, <50% (n=160); group C patients with cPRA ≥50% (n=161). Post-HTx outcomes were freedom from antibody-mediated rejection (AMR), acute cellular rejection, coronary allograft vasculopathy, and all-cause mortality. RESULTS: The median follow-up duration was 44 (19-72) months. Female sex, re-transplantation, and pre-HTx renal replacement therapy were independently associated with an increased risk of sensitization (cPRA ≥50%). Group C patients were more likely to have longer hospital stays and to use anti-thymocyte globulin as an induction agent compared to groups A and B. Significantly more patients in group C had positive flow cytometric crossmatch and had a higher incidence of preformed donor-specific antibody (DSA) compared to groups A and B. During follow-up, group C had a significantly higher rate of AMR, but the overall survival rate was comparable to that of groups A and B. In a subgroup analysis of group C, post-transplant survival was comparable despite higher preformed DSA in a desensitized group compared to the non-desensitized group. CONCLUSIONS: Patients with cPRA ≥50% had significantly higher incidence of preformed DSA and lower freedom from AMR, but post-HTx survival rates were similar to those with cPRA <50%. Our findings suggest that sensitized patients can attain comparable post-transplant survival to non-sensitized patients when treated with optimal desensitization treatment and therapeutic intervention.
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The effect of changes in immunosuppressive therapy during the acute phase post-heart transplantation (HTx) on clinical outcomes remains unclear. This study aimed to investigate the effects of changes in immunosuppressive therapy by corticosteroid (CS) weaning and everolimus (EVR) initiation during the first year post-HTx on clinical outcomes. We analyzed 622 recipients registered in the Korean Organ Transplant Registry (KOTRY) between January 2014 and December 2021. The median age at HTx was 56 years (interquartile range [IQR], 45-62), and the median follow-up time was 3.9 years (IQR 2.0-5.1). The early EVR initiation within the first year post-HTx and maintenance during the follow-up is associated with reduced the risk of primary composite outcome (all-cause mortality or re-transplantation) (HR, 0.24; 95% CI 0.09-0.68; p < 0.001) and cardiac allograft vasculopathy (CAV) (HR, 0.39; 95% CI 0.19-0.79; p = 0.009) compared with EVR-free or EVR intermittent treatment regimen, regardless of CS weaning. However, the early EVR initiation tends to increase the risk of acute allograft rejection compared with EVR-free or EVR intermittent treatment.
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Corticoesteroides , Everolimus , Rechazo de Injerto , Trasplante de Corazón , Inmunosupresores , Sistema de Registros , Humanos , Everolimus/administración & dosificación , Everolimus/uso terapéutico , Trasplante de Corazón/efectos adversos , Persona de Mediana Edad , Masculino , Femenino , Inmunosupresores/uso terapéutico , Inmunosupresores/administración & dosificación , República de Corea/epidemiología , Rechazo de Injerto/prevención & control , Corticoesteroides/administración & dosificación , Corticoesteroides/uso terapéutico , Resultado del Tratamiento , Supervivencia de Injerto , Estudios RetrospectivosRESUMEN
INTRODUCTION: This study aimed to compare postoperative outcomes after cardiac surgery in solid-organ transplant recipients and nontransplant patients. METHODS: We performed a retrospective analysis of 78 consecutive transplant recipients who underwent cardiac surgery at Asan Medical Center between 2000 and 2022 and were matched with 312 nontransplant patients who underwent cardiac surgery at a 1:4 ratio. The outcomes included 30-day mortality, all-cause death, cardiac death, readmission, and cardiac readmission. RESULTS: There was no significant difference in baseline characteristics between the two groups. The most common type of cardiac surgery performed in solid organ transplant recipients was isolated valve surgery, followed by isolated CABG. The 30-day mortality was not significantly different between transplant recipients and nontransplant patients (3.9% vs. 3.5%; P > .99). Solid organ transplant recipients showed a higher all-cause mortality compared to nontransplant patients (29.1% vs. 14.3% at 5 years; P = .001); however, there was no significant difference in cardiac death between the two groups (2.6% vs. 3.2% at 5 years; P = .80). In addition, the readmission and cardiac readmission rates showed comparable findings to that of mortality. CONCLUSION: Cardiac surgery can be performed safely in solid organ transplant recipients, with postoperative cardiovascular outcomes comparable to those observed in nontransplant patients.
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Procedimientos Quirúrgicos Cardíacos , Trasplante de Órganos , Humanos , Estudios Retrospectivos , Receptores de Trasplantes , Análisis por Apareamiento , Complicaciones Posoperatorias/etiología , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Trasplante de Órganos/efectos adversosRESUMEN
Background: This study compared the outcomes of surgical aortic valve replacement (AVR) in patients aged 50 to 70 years based on the type of prosthetic valve used. Methods: We compared patients who underwent mechanical AVR to those who underwent bioprosthetic AVR at our institution between January 2000 and March 2019. Competing risk analysis and the inverse probability of treatment weighting (IPTW) method based on propensity score were employed for comparisons. Results: A total of 1,580 patients (984 patients with mechanical AVR; 596 patients with bioprosthetic AVR) were enrolled. There was no significant difference in early mortality between the mechanical AVR and bioprosthetic AVR groups (0.9% vs. 1.7%, p=0.177). After IPTW adjustment, the risk of all-cause mortality was significantly higher in the bioprosthetic AVR group than in the mechanical AVR group (hazard ratio [HR], 1.39; 95% confidence interval [CI], 1.07-1.80; p=0.014). Competing risk analysis revealed lower risks of stroke (sub-distributional hazard ratio [sHR], 0.44; 95% CI, 0.28-0.67; p<0.001) and anticoagulation- related bleeding (sHR, 0.35; 95% CI, 0.23-0.53; p<0.001) in the bioprosthetic AVR group. Conversely, the risk of aortic valve (AV) reintervention was higher in the bioprosthetic AVR group (sHR, 6.14; 95% CI, 3.17-11.93; p<0.001). Conclusion: Among patients aged 50 to 70 years who underwent surgical AVR, those receiving mechanical valves showed better survival than those with bioprosthetic valves. The mechanical AVR group exhibited a higher risk of stroke and anticoagulation-related bleeding, while the bioprosthetic AVR group showed a higher risk of AV reintervention.
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Recently, cooperative supramolecular polymerization has garnered considerable attention due to its significant potential for enabling controlled chain-growth polymerization, which offers a route to achieving a well-defined degree of polymerization and low polydispersity. In this study, we synthesized two distinct alkynylplatinum(ii) complexes, one bearing a saturated long alkyl chain (Pt-Sat-C18) and another containing a diacetylene moiety within a long alkyl chain (Pt-DA-C25). Spectroscopic analyses revealed that Pt-Sat-C18 undergoes supramolecular polymerization via an isodesmic pathway, while Pt-DA-C25 assembles cooperatively. Intriguingly, the mechanism of supramolecular copolymerization could be tuned by varying the composition ratios: transitioning from an isodesmic to a cooperative pathway was achieved by increasing the proportion of Pt-DA-C25. Moreover, UV irradiation prompted a shift from an isodesmic to a cooperative assembly mechanism. Morphologically, self-assembled Pt-Sat-C18 resulted in left-handed fibrillar structures, whereas Pt-DA-C25 led to left-handed tubular assemblies. Supramolecular co-assembly further revealed helical ribbon or tubular structures. Photoluminescent properties were also observed, with emission spectra centered at approximately 650 nm, attributed to the formation of excimer species facilitated by strong Ptâ¯Pt interactions. To elucidate the mechanisms underlying these supramolecular polymerizations, temperature-dependent UV-visible spectroscopy was conducted during the cooling/heating processes, and thermodynamic parameters for both isodesmic and cooperative pathways were quantitatively assessed through curve fitting.
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INTRODUCTION: Although extracorporeal membrane oxygenation (ECMO) is a well-established treatment for supporting severe cardiopulmonary failure, the morbidity and mortality of patients requiring ECMO support remain high. Evaluating and correcting potential risk factors associated with any ECMO-related complications may improve care and decrease mortality. This study aimed to assess the predictors of ECMO-related vascular and cerebrovascular complications among adult patients and to test the hypothesis that ECMO-related complications are associated with higher in-hospital mortality rates. METHODS: This single-center, retrospective study included 856 ECMO runs administered via cannulation of the femoral vessels of 769 patients: venoarterial (VA) ECMO (n = 709, 82.8%) and venovenous (VV) ECMO (n = 147, 17.2%). The study outcomes included the occurrence of ECMO-related vascular and cerebrovascular complications and in-hospital death. The association of ECMO-related complications with the risk of in-hospital death was analyzed. RESULTS: The incidences of ECMO-related vascular and cerebrovascular complications were 20.2% and 13.6%, respectively. The overall in-hospital mortality rate was 48.7%: 52.8% among VA ECMO runs and 29.3% among VV ECMO runs. Multivariable analysis indicated that age (P < 0.01), cardiopulmonary cerebral resuscitation (P < 0.01), continuous renal replacement therapy (P < 0.01), and initial platelet count [<50×103/µL (P = 0.02) and 50-100(×103)/µL (P < 0.01)] were associated with an increased risk of in-hospital death. ECMO-related vascular and cerebrovascular complications were not independently associated with higher in-hospital mortality rates for VA or VV ECMO runs. CONCLUSION: ECMO-related vascular and cerebrovascular complications were not associated with an increased risk of in-hospital death among adult patients.
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Oxigenación por Membrana Extracorpórea , Adulto , Humanos , Oxigenación por Membrana Extracorpórea/efectos adversos , Estudios Retrospectivos , Mortalidad Hospitalaria , Cateterismo , Factores de RiesgoRESUMEN
BACKGROUND: Studies on the association between pleural effusion (PE) and left ventricular assist devices (LVADs) are limited. This study aimed to examine the characteristics and the clinical impact of PE following LVAD implantation. METHODS: This study is a prospective analysis of patients who underwent LVAD implantation from June 2015 to December 2022. We investigated the prognostic impact of therapeutic drainage (TD) on clinical outcomes. We also compared the characteristics and clinical outcomes between early and late PE and examined the factors related to the development of late PE. RESULTS: A total of 71 patients was analyzed. The TD group (n=45) had a longer ward stay (days; median [interquartile range]: 31.0 [23.0-46.0] vs. 21.0 [16.0-34.0], P=0.006) and total hospital stay (47.0 [36.0-82.0] vs. 31.0 [22.0-48.0], P=0.002) compared to the no TD group (n=26). Early PE was mostly exudate, left-sided, and neutrophil-dominant even though predominance of lymphocytes was the most common finding in late PE. Patients with late PE had a higher rate of reintubation within 14 days (31.8% vs. 4.1%, P=0.004) and longer hospital stays than those without late PE (67.0 [43.0-104.0] vs. 36.0 [28.0-48.0], P<0.001). Subgroup analysis indicated that female sex, low body mass index, cardiac resynchronization therapy, and hypoalbuminemia were associated with late PE. CONCLUSIONS: Compared to patients not undergoing TD, those undergoing TD had a longer hospital stay but not a higher 90-day mortality. Patients with late PE had poor clinical outcomes. Therefore, the correction of risk factors, like hypoalbuminemia, may be required.
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Background: Left ventricular assist devices (LVADs) are widely employed as a therapeutic option for end-stage heart failure. We evaluated the outcomes associated with centrifugal-flow LVAD implantation, comparing 2 device models: the Heartmate 3 (HM3) and the Heartware Ventricular Assist Device (HVAD). Methods: Data were collected from patients who underwent LVAD implantation between June 1, 2015 and December 31, 2022. We analyzed overall survival, first rehospitalization, and early, late, and LVAD-related complications. Results: In total, 74 patients underwent LVAD implantation, with 42 receiving the HM3 and 32 the HVAD. A mild Interagency Registry for Mechanically Assisted Circulatory Support score was more common among HM3 than HVAD recipients (p=0.006), and patients receiving the HM3 exhibited lower rates of preoperative ventilator use (p=0.010) and extracorporeal membrane oxygenation (p=0.039). The overall early mortality rate was 5.4% (4 of 74 patients), with no significant difference between groups. Regarding early right ventricular (RV) failure, HM3 implantation was associated with a lower rate (13 of 42 [31.0%]) than HVAD implantation (18 of 32 [56.2%], p=0.051). The median rehospitalization-free period was longer for HM3 recipients (16.9 months) than HVAD recipients (5.3 months, p=0.013). Furthermore, HM3 recipients displayed a lower incidence of late hemorrhagic stroke (p=0.016). In the multivariable analysis, preoperative use of continuous renal replacement therapy (odds ratio, 22.31; p=0.002) was the only significant predictor of postoperative RV failure. Conclusion: The LVAD models (HM3 and HVAD) demonstrated comparable overall survival rates. However, the HM3 was associated with a lower risk of late hemorrhagic stroke.
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Hypertrophic cardiomyopathy (HCM) is a genetic myocardial disease characterized by abnormal thickening of the myocardium caused by myocardial disarray and interstitial fibrosis. HCM is associated with sudden cardiac-related events, such as ventricular fibrillation, tachycardia, and syncope. Moreover, left ventricular or midcavity obstruction due to the thickened myocardium can result in severe heart failure and mortality in patients with HCM. Surgical myectomy is a standard treatment option for patients with symptomatic obstructive HCM; however, it is a complex procedure that requires careful planning and execution to avoid complications, such as residual flow obstruction, persistent obliteration of the left ventricular cavity in systole, or iatrogenic ventricular septal defects. Therefore, a thorough understanding of the mechanics of HCM and precise evaluation of the location and extent of the hypertrophic myocardium to be removed are crucial for preoperative planning. Multiphase cardiac CT postprocessing is important for preoperative evaluation and planning of surgical myectomy in patients with HCM. In this review, the authors highlight use of multiphase cardiac CT with step-by-step postprocessing methods to simulate successful surgical myectomy. The transaortic surgeon's view on end-diastolic phase images accurately represents the surgical field. Moreover, myocardial segmentation can be used to generate volume-rendered images and three-dimensional printing. CT evaluation can also assist in identifying concurrent abnormalities, such as mitral valve or papillary muscle abnormalities. In addition to CT, other imaging modalities for preoperative evaluation of HCM and postmyectomy evaluation methods are presented. ©RSNA, 2023 Test Your Knowledge questions in the supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article.
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Procedimientos Quirúrgicos Cardíacos , Cardiomiopatía Hipertrófica , Humanos , Procedimientos Quirúrgicos Cardíacos/métodos , Tabiques Cardíacos/cirugía , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/cirugía , Cardiomiopatía Hipertrófica/complicaciones , Miocardio , Tórax , Resultado del TratamientoRESUMEN
Metal-ligand binding plays a crucial role in regulating the photophysical properties of supramolecular gels. In this study, we designed 1-Pt complexes comprising a central benzene-1,3,5-tricarboxamide unit functionalized with three terpyridines, which can form supramolecular gels with Pt(II). The resulting supramolecular gel of 1-Pt exhibited strong orange emission, which was attributed to the metal-to-metal ligand charge transfer during gel formation. Furthermore, the temperature-dependent absorption spectrum changes of the supramolecular polymer 1-Pt exhibited a nonsigmoidal transition, following a cooperative pathway involving a nucleation-elongation mechanism. Additionally, the strategy for the co-assembling system involving 1-Pt with chiral molecules (D-form and L-form) induced the helical arrangement of 1-Ptvia chiral additives in supramolecular metallogels.
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BACKGROUND AND OBJECTIVES: To identify the factors associated with adverse outcomes following surgery for functional insufficiency of the mitral valve (MV) or tricuspid valve (TV) associated with atrial fibrillation (AF). METHODS: We evaluated 100 patients (age, 66.5±10.0 years; 47 males) who consecutively underwent surgery for functional insufficiency of the MV or TV associated with AF between January 2000 and December 2020 at our center. The primary outcome was a composite endpoint of all-cause death, valve reoperation, congestive heart failure (CHF) requiring rehospitalization, and stroke. RESULTS: During follow-up (532 patients-years [PYs]), adverse events included death in 16 (3.0%/yr), MV reoperation in 1 (0.2%/yr), CHF in 14 (2.6%/yr), and stroke in 5 (0.9%/yr) patients, demonstrating a 5-year rate of freedom from the primary endpoint of 69.5%. The rate of postoperative AF was high even in those who underwent AF ablation (n=92), with cumulative rates of 48.1% at 1 year and 60.2% at 5 years. In multivariable analyses, the primary outcome was significantly associated with age (adjusted hazard ratio [aHR], 1.06; 95% confidence interval [CI], 1.02-1.10; p=0.005), chronic kidney disease (aHR, 7.76; 95% CI, 2.28-26.38; p=0.001), left atrial appendage exclusion (aHR, 0.35; 95% CI, 0.16-1.78; p=0.010), and postoperative AF as a time-varying covariate (aHR, 3.33; 95% CI, 1.50-7.40; p=0.003). CONCLUSION: Among patients undergoing surgery for functional atrioventricular insufficiency associated with AF, a significant proportion showed recurrence of AF over time after concomitant AF ablation, which was significantly associated with poor clinical outcomes.
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Chiral metal-organic frameworks (CMOFs) and solid-state [2 + 2] photocyclization have been explored as independent areas in crystal engineering. We herein report the photoreactive CMOFs that undergo a [2 + 2] photocycloaddition reaction for the first time. Through the incorporation of a dipyridyl olefin ligand, 1,4-bis[2-(4-pyridyl)ethenyl]benzene, and d-camphoric acid or l-camphoric acid, we constructed a pair of homochiral Zn(II) CMOFs (d-1 or l-1) with a two-dimensional sql topology via a two-step procedure to avoid racemization. Both d-1 and l-1 were photoinert due to the large olefin bond separation. The removal of the solvent molecules between layers enabled them (d-1a and l-1a) to undergo [2 + 2] cycloaddition reactions; d-1a is more reactive (70%) than l-1a (20%) probably due to proper desolvation-induced rearrangement. The photoluminescence properties are also discussed. This work presents a new perspective on photoreactive homochiral network materials with diverse topologies and applications.
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AIMS: Few studies have reported data on the optimal timing of left ventricular (LV) unloading during venoarterial extracorporeal membrane oxygenation (VA-ECMO) for cardiac arrest or shock. This study evaluated the feasibility of an early LV unloading strategy compared with a conventional strategy in VA-ECMO. METHODS AND RESULTS: Between December 2018 and August 2022, 60 patients at two institutions were randomized in a 1:1 ratio to receive early (n = 30) or conventional (n = 30) LV unloading strategies. The early LV unloading strategy was defined as LV unloading performed at the time of VA-ECMO insertion. LV unloading was performed using a percutaneous transseptal left atrial cannulation via the femoral vein incorporated into the ECMO venous circuit. The early and conventional LV unloading groups included 29 (96.7%) and 23 (76.7%) patients, respectively (median time from VA-ECMO insertion to LV unloading: 48.4 h, interquartile range 47.8-96.5 h). The groups showed no significant differences in the rate of VA-ECMO weaning as the primary endpoint (70.0% vs. 76.7%; relative risk 0.91; 95% confidence interval 0.67-1.24; p = 0.386) and survival to discharge (53.3% vs. 50.0%, p = 0.796). However, the pulmonary congestion score index at 48 h after LV unloading was significantly improved only in the early LV unloading group (2.0 ± 0.7 vs. 1.7 ± 0.6 at baseline vs. at 48 h; p = 0.008). CONCLUSIONS: Compared with the conventional approach, early LV unloading did not improve the VA-ECMO weaning rate, despite the rapid improvement in pulmonary congestion. Therefore, the results of this study do not support the application of this strategy after VA-ECMO insertion.
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Oxigenación por Membrana Extracorpórea , Insuficiencia Cardíaca , Edema Pulmonar , Humanos , Insuficiencia Cardíaca/terapia , Choque Cardiogénico/etiología , Choque Cardiogénico/terapia , Oxigenación por Membrana Extracorpórea/métodos , Atrios Cardíacos , DescompresiónRESUMEN
BACKGROUND: Primary cardiac sarcomas are rare and their clinicopathologic features are heterogeneous. Among them, particularly intimal sarcoma is a diagnostic challenge due to nonspecific histologic features. Recently, MDM2 amplification reported to be a characteristic genetic event in the intimal sarcoma. In this study, we aimed to identify the types and incidence of primary cardiac sarcomas that occurred over 25 years in tertiary medical institutions, and to find clinicopatholgical significance through reclassification of diagnoses using additional immunohistochemistry (IHC). METHODS: We reviewed the primary cardiac sarcoma cases between January 1993 and June 2018 at Asan Medical Center, South Korea, with their clinicopathologic findings, and reclassified the subtypes, especially using IHC for MDM2 and then, analyzed the significance of prognosis. RESULTS: Forty-eight (6.8%) cases of a primary cardiac sarcoma were retrieved. The tumors most frequently involved the right atrium (n = 25, 52.1%), and the most frequent tumor subtype was angiosarcoma (n = 23, 47.9%). Seven cases (53.8%) were newly reclassified as an intimal sarcoma by IHC for MDM2. Twenty-nine (60.4%) patients died of disease (mean, 19.8 months). Four patients underwent a heart transplantation and had a median survival of 26.8 months. This transplantation group tended to show good clinical outcomes in the earlier stages, but this was not statistically significant (p = 0.318). MDM2 positive intimal sarcoma showed the better overall survival (p = 0.003) than undifferentiated pleomorphic sarcoma. Adjuvant treatment is beneficial for patient survival (p < 0.001), particularly in angiosarcoma (p < 0.001), but not in intimal sarcoma (p = 0.154). CONCLUSION: Our study supports the use of adjuvant treatment in primary cardiac sarcoma, as it was associated with a significantly better overall survival rate. Further consideration of tumor histology may be important in determining the optimal use of adjuvant treatment for different types of sarcomas. Therefore, accurate diagnosis by MDM2 test is important condsidering patient's prognosis and treatment.
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Neoplasias Cardíacas , Hemangiosarcoma , Sarcoma , Humanos , Terapia Combinada , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/genética , Neoplasias Cardíacas/terapia , Hemangiosarcoma/genética , Hemangiosarcoma/terapia , Pronóstico , Proteínas Proto-Oncogénicas c-mdm2/genética , Sarcoma/diagnóstico , Sarcoma/genética , Sarcoma/terapiaRESUMEN
BACKGROUND: Cardiac allograft vasculopathy (CAV) is a major obstacle limiting long-term graft survival. Effective noninvasive surveillance modalities reflecting both coronary artery and microvascular components of CAV are needed. OBJECTIVES: The authors evaluated the diagnostic performance of dynamic computed tomography-myocardial perfusion imaging (CT-MPI) and coronary computed tomography angiography (CCTA) for CAV. METHODS: A total of 63 heart transplantation patients underwent combined CT-MPI and CCTA plus invasive coronary angiography (ICA) with intravascular ultrasonography (IVUS) between December 2018 and October 2021. The median interval between CT-MPI and heart transplantation was 4.3 years. Peak myocardial blood flow (MBF) of the whole myocardium (MBFglobal) and minimum MBF (MBFmin) among the 16 segments according to the American Heart Association model, except the left ventricular apex, were calculated from CT-MPI. CCTA was assessed qualitatively, and the degree of coronary artery stenosis was recorded. CAV was diagnosed based on both ICA (ISHLT criteria) and IVUS. Patients were followed up for a median time of 2.3 years after CT-MPI and a median time of 5.7 years after transplantation. RESULTS: Among the 63 recipients, 35 (55.6%) had diagnoses of CAV. The median MBFglobal and MBFmin were significantly lower in patients with CAV (128.7 vs 150.4 mL/100 mL/min; P = 0.014; and 96.9 vs 122.8 mL/100 mL/min; P < 0.001, respectively). The combined use of coronary artery stenosis on CCTA and MBFmin showed the highest diagnostic performance with an area under the curve of 0.886 (sensitivity: 74.3%, specificity: 96.4%, positive predictive value: 96.3%, and negative predictive value: 75.0%). CONCLUSIONS: The combination of CT-MPI and CCTA demonstrated excellent diagnostic performance for the detection of CAV. One-stop evaluation of the coronary artery and microvascular components involved in CAV using combined CCTA and CT-MPI may be a potent noninvasive screening method for early detection of CAV.
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Enfermedad de la Arteria Coronaria , Estenosis Coronaria , Imagen de Perfusión Miocárdica , Humanos , Angiografía Coronaria/métodos , Angiografía por Tomografía Computarizada/métodos , Valor Predictivo de las Pruebas , Tomografía Computarizada por Rayos X/métodos , Miocardio , Aloinjertos , Perfusión , Enfermedad de la Arteria Coronaria/diagnóstico por imagen , Imagen de Perfusión Miocárdica/métodosRESUMEN
BACKGROUND AND OBJECTIVES: Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) as a bridge to eventual heart transplantation (HT) is increasingly used worldwide. However, the effect of different VA-ECMO types on HT outcomes remains unclear. METHODS: This was a retrospective observational study of 111 patients receiving VA-ECMO and awaiting HT. We assessed 3 ECMO configuration groups: peripheral (n=76), central (n=12), and peripheral to central ECMO conversion (n=23). Cox proportional hazards regression and landmark analysis were conducted to analyze the effect of the ECMO configuration on HT and in-hospital mortality rates. We also evaluated adverse events during ECMO support. RESULTS: HT was performed in the peripheral (n=48, 63.2%), central (n=10, 83.3%), and conversion (n=11, 47.8%) ECMO groups (p=0.133) with a median interval of 10.5, 16, and 30 days, respectively (p<0.001). The cumulative incidence of HT was significantly lower in the conversion group (hazard ratio, 0.292, 95% confidence interval, 0.145-0.586, p=0.001). However, there was no difference in in-hospital mortality (log-rank p=0.433). In the landmark analysis, in-hospital mortality did not differ significantly among the 3 groups. Although we did note a trend toward lower HT in the conversion group, the difference was not statistically significant. Surgical site bleeding occurred mainly in the central, while limb ischemia occurred mainly in the peripheral groups. CONCLUSIONS: We suggest that if patients are being stably supported with their initial ECMO configuration, whether it is central or peripheral, it should be maintained, and ECMO conversion should only be cautiously performed when necessary.