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INTRODUCTION: Tricuspid regurgitation is associated with significant morbidity and mortality, and occurs at a higher rate in patients with cardiovascular implantable electronic devices. Percutaneous strategies for managing tricuspid regurgitation are evolving, including the development of bicaval valve implantation which has been successfully used in patients with pacing leads. METHODS AND RESULTS: We present the first documented case of lead failure following TricValve® implantation, a dedicated self-expanding system for bicaval valve implantation, and the first successful lead revision procedure in this setting. CONCLUSION: The case illustrates important considerations in undertaking percutaneous intervention in patients with cardiovascular implantable electronic devices, and their ongoing management.
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Desfibriladores Implantáveis , Falha de Equipamento , Insuficiência da Valva Tricúspide , Humanos , Insuficiência da Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Próteses Valvulares Cardíacas , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/efeitos adversos , Masculino , Idoso , Resultado do Tratamento , Reoperação , Desenho de Prótese , Remoção de Dispositivo , Feminino , Cardioversão Elétrica/instrumentação , Cardioversão Elétrica/efeitos adversosRESUMO
BACKGROUND: Despite its wide diffusion in surgical procedures, very few experiences are reported so far about the possible applications of remote proctoring (RP) in structural heart interventions. AIMS: Our aim was to demonstrate the feasibility, safety, and efficacy of RP during transcatheter edge-to-edge repair using a mixed reality-based head-mounted display (HMD). METHODS: Two users (a doctor and a proctor), wearing HMDs, were connected through a 5G network, allowing them to share audio and video signals. During the procedure, the proctor was located in a contiguous room, without any direct connection with the operator. The primary endpoint was noninferiority of HMD-mediated interaction if compared with direct in-person interaction. It was assessed using an operator-proctor interaction test based on six questions asked by the doctor to the proctor on six key procedural steps. RESULTS: Between September 2021 and April 2022, nine patients were enrolled in the study (15 clips delivered). The primary endpoint was reached in 9/15 clips implanted (60%). The first three failures were due to a chat software problem, and the others were linked with a 5G network malfunction. However, the HMD and its mixed reality tools were well tolerated by the operators, and no interference with the procedure was registered in any of the cases. CONCLUSIONS: In our experience, RP during structural heart interventions is feasible and efficacious. Wearing the HMD is comfortable and does not affect safety and effectiveness of interventional procedures. However, especially if considering a wide geographic use of this tool, a stable internet connection is imperative.
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BACKGROUND: Predictors of permanent pacemaker implantation (PPMI) after self-expanding transcatheter aortic valve implant (TAVI) were described. Is unknown if PPMI predictors remain in the era of high implants using the cusp overlap (COP). METHODS: Single-center, prospective, consecutive case series of patients undergoing self-expanding TAVI with the COP approach. The status of PPMI and other clinical events were ascertained at 30 days. RESULTS: A total of 261 patients were included (84% with Evolut, n = 219). Implant depth >4 mm was infrequent (13.8%). TAVI depth (OR 1.259; p = 0.005), first or second-degree auriculo-ventricular block (OR 3.406; p = 0.033), right-bundle (OR 15.477; p < 0.0001), and incomplete left-bundle branch block (OR 7.964; p = 0.036) were found to be independent predictors of PPMI. The risk of PPMI with deep implant and no electrical disturbances was 3%, and 0% with high implant and no prior electrical disturbances. Those who received PPMI had no statistically significant increased risk of death, myocardial infarction, stroke, bleeding events, or vascular complications at 30 days, but longer hospital stay (mean difference 1.43 days more, p = 0.003). CONCLUSIONS: Implant depth and prior conduction abnormalities remain the main predictors of PPMI using self-expanding TAVI in the COP era. Patients with high implants and no prior conduction abnormalities may be candidates for early discharge after uneventful self-expanding TAVI, while the rest may need inpatient monitoring regardless of achieving a high implant. The need for PPMI was associated with longer hospital stays.
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BACKGROUND: The exclusion/occlusion of the left atrial appendage (LAA) is a treatment option for atrial fibrillation (AF) patients who are at high risk of stroke and high risk of bleeding. As the role of the LAA is not well understood or explored, this study aims to assess its role on flow dynamics in the left atrium. METHODS: Computational fluid dynamics (CFD) simulations were carried out for nine AF patients before and after LAA exclusion. The flow parameters investigated included the LA velocities, Time Averaged Wall Shear Stress (TAWSS), Oscillatory Shear Index (OSI), Relative Residence Time (RRT), and Pressure in the LA. RESULTS: This study shows that, on average, a decrease in TAWSS (1.82 ± 1.85 Pa to 1.27 ± 0.96 Pa, p < 0.05) and a slight increase in OSI (0.16 ± 0.10 to 0.17 ± 0.10, p < 0.05), RRT (1.87 ± 1.84 Pa-1 to 2.11 ± 1.78 Pa-1, p < 0.05), and pressure (-19.2 ± 6.8 mmHg to -15.3 ± 8.3 mmHg, p < 0.05) were observed in the LA after the exclusion of the LAA, with a decrease in low-magnitude velocities. CONCLUSION: The exclusion of the LAA seems to be associated with changes in LA flow dynamics. Further studies are needed to elucidate the clinical implications of these changes.
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Despite progressively uncommon in Western countries, rheumatic heart disease still portrays a significant global burden. In elderly or high-surgical risk patients, plurivalvular disease may require a complex percutaneous approach. Transcatheter aortic valve implantation (TAVI) in patients with previous monoleaflet mitral prosthesis is challenging due to interference between the aortic valve and the rigid mitral mechanical prosthesis "ring." Prior cases report the use of CoreValve or Edwards Sapien aortic valves in patients with adequate mitro-aortic distance. Performing a second major procedure, such as tricuspid valve-in-valve (TVIV), sequentially during a single percutaneous intervention, increases treatment complexity. An 83-year-old woman with rheumatic heart disease, with previous implantation of a Bjork-Shiley monoleaflet mitral prosthesis, and Carpentier-Edwards 29 tricuspid bioprosthesis presented with decompensated heart failure due to severe aortic stenosis and tricuspid bioprosthesis stenosis. After HeartTeam discussion, the patient was deemed as inoperable due to a prohibitive surgical risk. As an alternative, a TAVI (Navitor FlexNav) and a transcatheter TVIV replacement (Edwards Sapiens 3 Ultra) were discussed and proposed, with both techniques being performed sequentially in a single procedure. TAVI in a patient with a previous monoleaflet mitral mechanical prosthesis and TVIV may be a feasible approach in inoperable patients with plurivalvular disease.
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Estenose da Valva Aórtica , Valva Aórtica , Bioprótese , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Valva Mitral , Desenho de Prótese , Cardiopatia Reumática , Substituição da Valva Aórtica Transcateter , Valva Tricúspide , Humanos , Feminino , Idoso de 80 Anos ou mais , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/efeitos adversos , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia , Valva Tricúspide/fisiopatologia , Resultado do Tratamento , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/fisiopatologia , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Valva Mitral/fisiopatologia , Cardiopatia Reumática/diagnóstico por imagem , Cardiopatia Reumática/fisiopatologia , Cardiopatia Reumática/cirurgia , Cardiopatia Reumática/terapia , Substituição da Valva Aórtica Transcateter/instrumentação , Substituição da Valva Aórtica Transcateter/efeitos adversos , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Valva Aórtica/fisiopatologia , Cateterismo Cardíaco/instrumentação , Recuperação de Função Fisiológica , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/terapia , Insuficiência Cardíaca/diagnóstico por imagemRESUMO
Subclinical bioprosthetic valve thrombosis (BPVT) is a relatively common finding in asymptomatic patients during follow-up imaging. However, its clinical significance is unclear. Data from registries associate BPVT with elevated valve gradients, thromboembolic complications, recurrence, and valve degeneration. Given the dynamic nature of the disease process, management is challenging. The duration of anticoagulation is unpredictable, and the need for frequent monitoring of BPVT, even in subclinical scenarios, is unclear. Our report is shedding the light on the clinical implications BPVT.
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Estenose da Valva Aórtica , Valva Aórtica , Bioprótese , Próteses Valvulares Cardíacas , Recidiva , Trombose , Substituição da Valva Aórtica Transcateter , Humanos , Substituição da Valva Aórtica Transcateter/efeitos adversos , Substituição da Valva Aórtica Transcateter/instrumentação , Valva Aórtica/cirurgia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/fisiopatologia , Resultado do Tratamento , Trombose/etiologia , Trombose/diagnóstico por imagem , Idoso de 80 Anos ou mais , Masculino , Desenho de Prótese , Feminino , Anticoagulantes/uso terapêutico , Idoso , Fatores de TempoRESUMO
BACKGROUND: Early detection and intervention for preclinical heart failure (HF) are crucial for restraining the potential increase in patients with HF. Thus, we designed and conducted a single-center retrospective cohort study to confirm the efficacy of B-type natriuretic peptide (BNP) for the early detection of preclinical HF in a primary care setting. METHODSâANDâRESULTS: We investigated 477 patients with no prior diagnosis of HF who were under the care of general practitioners. These patients were categorized into 4 groups based on BNP concentrations: Category 1, 0 pg/mL≤BNP≤35 pg/mL; Category 2, 35 pg/mL
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Insuficiência Cardíaca , Peptídeo Natriurético Encefálico , Atenção Primária à Saúde , Humanos , Peptídeo Natriurético Encefálico/sangue , Idoso , Estudos Retrospectivos , Feminino , Masculino , Pessoa de Meia-Idade , Insuficiência Cardíaca/sangue , Insuficiência Cardíaca/diagnóstico , Idoso de 80 Anos ou mais , Diagnóstico Precoce , Biomarcadores/sangueRESUMO
PURPOSE OF THE REVIEW: This review aims to provide a profound overview on most recent studies on the clinical significance of Cardiovascular Computed Tomography (CCT) in diagnostic and therapeutic pathways. Herby, this review helps to pave the way for a more extended but yet purposefully use in modern day cardiovascular medicine. RECENT FINDINGS: In recent years, new clinical applications of CCT have emerged. Major applications include the assessment of coronary artery disease and structural heart disease, with corresponding recommendations by major guidelines of international societies. While CCT already allows for a rapid and non-invasive diagnosis, technical improvements enable further in-depth assessments using novel imaging parameters with high temporal and spatial resolution. Those developments facilitate diagnostic and therapeutic decision-making as well as improved prognostication. This review determined that recent advancements in both hardware and software components of CCT allow for highly advanced examinations with little radiation exposure. This particularly strengthens its role in preventive care and coronary artery disease. The addition of functional analyses within and beyond coronary artery disease offers solutions in wide-ranging patient populations. Many techniques still require improvement and validation, however, CCT possesses potential to become a "one-stop-shop" examination.
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Ventricular premature beats (VPBs) can potentially lead to life-threatening arrhythmias, especially in patients with structural heart disease (SHD). However, identifying dangerous VPBs has always been a topic and challenge in clinical research. This study aimed to evaluate the relationship of postextrasystolic repolarization changes of VPBs with SHD and its possible additional prognostic value. 125 cases of frequent VPBs with SHD and 156 cases without SHD were included. VPBs were stratified selected from 24 h Holter recording according to the scale of heart rate. Average QTDV (difference value of QT interval between the first beat follow VPB with beats preceding VPB) and max QTDV were significantly longer in SHD group than that in the non-SHD group. For identifying patients with SHD, the best cutoff value were 19 ms for average QTDV (AUC = 0.931) and 29 ms for max QTDV (AUC = 0.910) respectively. For Tu morphology analysis, PT2 (postextrasystolic T wave amplitude change ≥2 mV), reversed T wave, and Pu (postextrasystolic u wave) change were all highly specific, but low sensitive as identification of SHD. Compared with average QTDV < 19 ms patients, average QTDV ≥ 19 ms patients had significantly larger left heart size and wores left cardiac function. The presence of non-persistent ventricular tachycardia runs was higher in average QTDV ≥ 19 ms group and positive Pu change group than that in control groups. The findings indicated that postextrasystolic repolarization changes of VPBs correlated with SHD and suggested potential value in prognosis asssessment.
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Eletrocardiografia Ambulatorial , Complexos Ventriculares Prematuros , Humanos , Masculino , Complexos Ventriculares Prematuros/fisiopatologia , Feminino , Prognóstico , Pessoa de Meia-Idade , Sensibilidade e Especificidade , Idoso , Frequência Cardíaca/fisiologiaRESUMO
In the dynamic field of interventional cardiology, significant strides have been made in reducing periprocedural complications. Echocardiography, particularly transesophageal echocardiography, plays a key role in ensuring the safety and success of structural heart interventions. Its real-time imaging capabilities allow for precise monitoring of device positioning, deployment, and procedural outcomes. By adhering to established imaging protocols and acquiring standard imaging planes, periprocedural echocardiography has become an essential tool for the successful performance of many structural heart interventions. In this manuscript, we present a series of unusual yet significant complications that we encountered during structural interventional procedures in our catheter laboratory. These complications, detected through echocardiography, underscore the critical role of imaging guidance in recognizing, and addressing unforeseen challenges, such as device malposition, thrombus formation in cardiac chambers during structural heart interventions, and cardiac tamponade during transcatheter mitral valve procedures. Through these cases, we highlight the effectiveness of transesophageal echocardiography in promptly identifying complications, allowing for timely intervention and resolution.
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BACKGROUND: The impact of sex on outcomes following surgical aortic valve replacement (SAVR) remains unclear. It has been proposed that females experience inferior outcomes, but this has yet to be conclusively established, particularly in the long term. The objective of this study is to identify discrepancies in postoperative outcomes between males and females following SAVR to better inform consideration for surgical intervention. METHOD: We retrospectively reviewed the outcomes of 4,927 patients who underwent SAVR from 2004 to 2018 at our centre. In total, 531 propensity-matched males and females were included in the final analysis. The primary outcome was mortality at any point during the follow-up period. Secondary outcomes included various measures of postoperative morbidity. Follow-up duration was 15 years. RESULTS: In SAVR all-comers, females experienced inferior short-term mortality, but equivalent mid-term and long-term mortality. Rates of mediastinal bleeding, sternal wound infections, sepsis, heart failure, and pacemaker insertion were all equivalent between the sexes; however, males experienced a higher rate of acute kidney injury and readmission for stroke at the longest follow-up while females experienced a longer intensive care unit and hospital length of stay. In a sub-analysis of isolated SAVR, males and females experienced equivalent early, mid, and late mortality. Of note, a trend towards increased aortic valve reoperation was noted in females at the longest follow-up. CONCLUSIONS: Males and females experience equivalent long-term mortality following isolated SAVR. Sex is not an independent risk factor of poor outcomes post-SAVR; however, the increased preoperative risk profile of females requires diligent consideration.
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BACKGROUND: Electroanatomical mapping (EAM)-guided stereotactic arrhythmia radioablation (STAR) is a novel noninvasive therapy option for patients with monomorphic ventricular tachycardia (VT) refractory to antiarrhythmic drugs and/or urgent catheter ablation (CA). Data on success rates in an emergency situation such as electrical storm (ES) are rare. We present a case of a patient with an initially very poor life expectancy after extensive myocardial infarction with therapy-resistant ES, not amendable for further antiarrhythmic drug therapy, implantable cardioverter-defibrillator implantation, or repeated CA who was introduced to the radiation oncology department for emergency STAR as a bail-out therapy. METHODS: Target volume definition and transfer from EAM to CT were validated and quality assured with a semi-automatic, dedicated visualization tool (CARDIO-RT). Emergency STAR was performed with 25â¯Gy in the framework of the RAVENTA study. The VT burden gradually decreased after STAR; however, a second VT morphology occurred, which was successfully treated with EAM-guided CA 12 days after STAR. RESULTS: The second EAM-guided CA showed areas of low voltage in the irradiated segments, indicating a precise targeting and early functional response to STAR. The patient remained free of any VT recurrence or any radiation-related toxicities and in good general condition during the recent follow-up of 18 months. CONCLUSION: The case highlights the possible approach, caveats, difficulties, and prognosis of a patient severely affected by therapy-resistant VT in whom CA could not lead to VT suppression. Further studies of putative mechanisms of STAR in the acute and chronic phase of this novel therapy are warranted.
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Ablação por Cateter , Taquicardia Ventricular , Humanos , Taquicardia Ventricular/radioterapia , Taquicardia Ventricular/cirurgia , Antiarrítmicos/uso terapêutico , Coração , Ablação por Cateter/efeitos adversos , Prognóstico , Resultado do TratamentoRESUMO
BACKGROUND: Single-session cardiac stereotactic radiation therapy (SBRT) has demonstrated promising results for patients with refractory ventricular tachycardia (VT). However, the full safety profile of this novel treatment remains unknown and very limited data from prospective clinical multicenter trials are available. METHODS: The prospective multicenter multiplatform RAVENTA (radiosurgery for ventricular tachycardia) study assesses high-precision image-guided cardiac SBRT with 25â¯Gy delivered to the VT substrate determined by high-definition endocardial and/or epicardial electrophysiological mapping in patients with refractory VT ineligible for catheter ablation and an implanted cardioverter defibrillator (ICD). Primary endpoint is the feasibility of full-dose application and procedural safety (defined as an incidence of serious [grade ≥â¯3] treatment-related complications ≤â¯5% within 30 days after therapy). Secondary endpoints comprise VT burden, ICD interventions, treatment-related toxicity, and quality of life. We present the results of a protocol-defined interim analysis. RESULTS: Between 10/2019 and 12/2021, a total of five patients were included at three university medical centers. In all cases, the treatment was carried out without complications. There were no serious potentially treatment-related adverse events and no deterioration of left ventricular ejection fraction upon echocardiography. Three patients had a decrease in VT episodes during follow-up. One patient underwent subsequent catheter ablation for a new VT with different morphology. One patient with local VT recurrence died 6 weeks after treatment in cardiogenic shock. CONCLUSION: The interim analysis of the RAVENTA trial demonstrates early initial feasibility of this new treatment without serious complications within 30 days after treatment in five patients. Recruitment will continue as planned and the study has been expanded to further university medical centers. TRIAL REGISTRATION NUMBER: NCT03867747 (clinicaltrials.gov). Registered March 8, 2019. Study start: October 1, 2019.
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Radiocirurgia , Taquicardia Ventricular , Humanos , Radiocirurgia/métodos , Volume Sistólico , Estudos Prospectivos , Qualidade de Vida , Estudos de Viabilidade , Função Ventricular Esquerda , Taquicardia Ventricular/radioterapia , Taquicardia Ventricular/cirurgia , Resultado do TratamentoRESUMO
PHACES syndrome is a multiple congenital disorder with unknown etiology that is characterized by Posterior fossa anomalies, Hemangioma, Arterial lesions, Cardiac abnormalities/coarctation of the aorta, Eye anomalies, and Sternal cleft. Compound heterozygous or homozygous TMEM260 variants cause structural heart defects and renal anomalies syndrome (SHDRA). We describe a 10-year-old male patient with a PHACES-like syndrome and TMEM260 compound heterozygous variants who demonstrated overlapping phenotypes between the two syndromes. He presented with truncus arteriosus, supraumbilical raphe, ophthalmological abnormality, vertebral abnormality, borderline intellectual disability, and hearing loss. He had normal serum creatinine. In proband exome sequencing, compound heterozygous TMEM260 variants (NM_017799.4 c.1617delG p.(Trp539Cysfs*9)/c.1858C > T p.(Gln620*)) were identified. Twelve patients have been reported with TMEM260-related SHDRA: 10 had truncus arteriosus and 6 had renal failure. One previously reported patient had facial port wine nevus and another patient had supraumbilical raphe, which are the cardinal signs for PHACES syndrome. TMEM260-related SHDRA could share overlapping clinical features with PHACES syndrome. This report expands the phenotypic spectrum of a TMEM260-related disorder.
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Coartação Aórtica , Anormalidades do Olho , Cardiopatias Congênitas , Síndromes Neurocutâneas , Masculino , Humanos , Síndrome , Coartação Aórtica/diagnóstico , Coartação Aórtica/genética , Anormalidades do Olho/diagnóstico , Anormalidades do Olho/genética , Síndromes Neurocutâneas/patologiaRESUMO
Transcatheter edge-to-edge repair (TEER) is used extensively in patients with symptomatic severe mitral regurgitation and high surgical risk. While prosthetic valve endocarditis is well documented, infective endocarditis (IE) after TEER is a rare occurrence. To date, no study has been conducted on this complication. We report the case of an 85-year-old man who developed IE 3 months after undergoing TEER, and we systematically review 26 previously published cases of this complication. Our review findings indicate that discussion within the heart team is essential for the decision-making process and treatment strategy determination.
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Endocardite Bacteriana , Endocardite , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Insuficiência da Valva Mitral , Masculino , Humanos , Idoso de 80 Anos ou mais , Endocardite Bacteriana/diagnóstico por imagem , Endocardite Bacteriana/etiologia , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/etiologia , Insuficiência da Valva Mitral/cirurgia , Resultado do Tratamento , Endocardite/diagnóstico por imagem , Endocardite/etiologia , Implante de Prótese de Valva Cardíaca/efeitos adversosRESUMO
AIMS: Assess prevalence, risk factors, and management of patients with intra-cardiac thrombus referred for scar-related ventricular tachycardia (VT) ablation. METHODS AND RESULTS: Consecutive VT ablation referrals between January 2015 and December 2019 were reviewed (n = 618). Patients referred for de novo, scar-related VT ablation who underwent pre-procedure cardiac computed tomography (cCT) were included. We included 401 patients [61 ± 14 years; 364 male; left ventricular ejection fraction (LVEF) 40 ± 13%]; 45 patients (11%) had cardiac thrombi on cCT at 49 sites [29 LV; eight left atrial appendage (LAA); eight right ventricle (RV); four right atrial appendage]. Nine patients had pulmonary emboli. Overall predictors of cardiac thrombus included LV aneurysm [odds ratio (OR): 6.6, 95%, confidence interval (CI): 3.1-14.3], LVEF < 40% (OR: 3.3, CI: 1.5-7.3), altered RV ejection fraction (OR: 2.3, CI: 1.1-4.6), and electrical storm (OR: 2.9, CI: 1.4-6.1). Thrombus location-specific analysis identified LV aneurysm (OR: 10.9, CI: 4.3-27.7) and LVEF < 40% (OR: 9.6, CI: 2.6-35.8) as predictors of LV thrombus and arrhythmogenic right ventricular cardiomyopathy (OR: 10.6, CI: 1.2-98.4) as a predictor for RV thrombus. Left atrial appendage thrombi exclusively occurred in patients with atrial fibrillation. Ventricular tachycardia ablation was finally performed in 363 including 7 (16%) patients with thrombus but refractory electrical storm. These seven patients had tailored ablation with no embolic complications. Only one (0.3%) ablation-related embolic event occurred in the entire cohort. CONCLUSION: Cardiac thrombus can be identified in 11% of patients referred for scar-related VT ablation. These findings underscore the importance of systematic thrombus screening to minimize embolic risk.
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Ablação por Cateter , Cardiopatias , Taquicardia Ventricular , Trombose , Humanos , Masculino , Taquicardia Ventricular/epidemiologia , Taquicardia Ventricular/cirurgia , Taquicardia Ventricular/diagnóstico , Volume Sistólico , Prevalência , Cicatriz , Função Ventricular Esquerda , Cardiopatias/diagnóstico por imagem , Cardiopatias/epidemiologia , Cardiopatias/complicações , Trombose/diagnóstico por imagem , Trombose/epidemiologia , Ablação por Cateter/efeitos adversos , Fatores de Risco , Resultado do TratamentoRESUMO
AIMS: Kleefstra syndrome (KS), often diagnosed in early childhood, is a rare genetic disorder due to haploinsufficiency of EHMT1 and is characterized by neuromuscular and intellectual developmental abnormalities. Although congenital heart disease (CHD) is common, the prevalence of arrhythmias and CHD subtypes in KS is unknown. METHODS AND RESULTS: Inspired by a novel case series of KS patients with atrial tachyarrhythmias in the USA, we evaluate the two largest known KS registries for arrhythmias and CHD: Radboudumc (50 patients) based on health record review at Radboud University Medical Center in the Netherlands and GenIDA (163 patients) based on worldwide surveys of patient families. Three KS patients (aged 17-25 years) presented with atrial tachyarrhythmias without manifest CHD. In the international KS registries, the median [interquartile range (IQR)] age was considerably younger: GenIDA/Radboudumc at 10/13.5 (12/13) years, respectively. Both registries had a 40% prevalence of cardiovascular abnormalities, the majority being CHD, including septal defects, vascular malformations, and valvular disease. Interestingly, 4 (8%) patients in the Radboudumc registry reported arrhythmias without CHD, including one atrial fibrillation (AF), two with supraventricular tachycardias, and one with non-sustained ventricular tachycardia. The GenIDA registry reported one patient with AF and another with chronic ectopic atrial tachycardia (AT). In total, atrial tachyarrhythmias were noted in six young KS patients (6/213 or 3%) with at least four (three AF and one AT) without structural heart disease. CONCLUSION: In addition to a high prevalence of CHD, evolving data reveal early-onset atrial tachyarrhythmias in young KS patients, including AF, even in the absence of structural heart disease.
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Fibrilação Atrial , Deleção Cromossômica , Anormalidades Craniofaciais , Cardiopatias Congênitas , Deficiência Intelectual , Humanos , Pré-Escolar , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/epidemiologia , Fibrilação Atrial/genética , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/genética , Taquicardia , Epigênese Genética , Cromossomos Humanos Par 9RESUMO
Left ventricular pseudoaneurysm (PSA) after surgical aortic valve replacement (AVR) is a known but uncommon complication. It is associated with risks such as thromboembolism and life-threatening rupture. Surgical repair has traditionally been utilized in low-risk patients but transcatheter closure has become a promising therapeutic option. This case report describes the utility of multimodality imaging in pre-, intra-, and post-procedural evaluation of transcatheter PSA closure and is among the first to demonstrate the utility of 3D print model.
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Falso Aneurisma , Implante de Prótese de Valva Cardíaca , Substituição da Valva Aórtica Transcateter , Humanos , Falso Aneurisma/diagnóstico por imagem , Falso Aneurisma/cirurgia , Cateterismo Cardíaco/métodos , Implante de Prótese de Valva Cardíaca/efeitos adversos , Valva Aórtica/cirurgia , Substituição da Valva Aórtica Transcateter/métodos , Imagem Multimodal , Resultado do TratamentoRESUMO
Treatments for structural heart diseases (SHD) have been considerably evolved by the widespread of transcatheter approach in the last decades. The progression of transcatheter treatments for SHD was feasible due to the improvement of devices and the advances in imaging techniques. In this setting, the cardiovascular imaging is pivotal not only for the diagnosis but even for the treatment of SHD. With the aim of fulfilling these tasks, a multimodality imaging approach with new imaging tools for pre-procedural planning, intra-procedural guidance, and follow-up of SHD was developed. This review will describe the current state-of-the-art imaging techniques for the most common percutaneous interventions as well as the new imaging tools. The imaging approaches will be addressed describing the use in pre-procedural planning, intra-procedural guidance, and follow-up.
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Transseptal puncture is an increasingly common procedure undertaken to gain access to the left side of the heart during structural heart disease interventions. Precision guidance during this procedure is paramount to ensure success and patient safety. As such, multimodality imaging, such as echocardiography, fluoroscopy, and fusion imaging, is routinely used to guide safe transseptal puncture. Despite the use of multimodal imaging, there is currently no uniform nomenclature of cardiac anatomy between the various imaging modes and proceduralists, and echocardiographers tend to use imaging modality-specific terminology when communicating among the various imaging modes. This variability in nomenclature among imaging modes stems from differing anatomic descriptions of cardiac anatomy. Given the required level of precision in performing transseptal puncture, a clearer understanding of the basis of cardiac anatomic nomenclature is required by both echocardiographers as well as proceduralists; enhanced understanding can help facilitate communication across specialties and possibly improve communication and safety. In this review, the authors highlight the variation in cardiac anatomy nomenclature among various imaging modes.