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3.
Am J Cardiol ; 125(6): 977-981, 2020 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-31959431

RESUMO

The rationale for timing of pulmonary valve replacement (PVR) in patients with repaired Tetralogy of Fallot (rTOF) has focused on pre-PVR threshold values of indexed right ventricular end-diastolic volume (RVEDVi) that lead to normalization of right ventricular (RV) size after valve implantation. The goal of this study was to determine whether persistent RV dilation after PVR is associated with adverse clinical outcomes. Subjects with rTOF who underwent PVR and had a cardiac magnetic resonance (CMR) exam after valve implantation at a single center from 2001 to 2017 were included. The composite clinical outcome after PVR included: death, aborted sudden cardiac death, sustained ventricular tachycardia (VT), or NYHA class ≥3. In 189 rTOF subjects, the mean age at PVR was 23.5 ± 11.7 years, median follow-up was 6.0 years (IQR 3.4 to 8.7), and the primary outcome occurred in 14 subjects (7%). The 5- and 10-year event-free rates were 97% and 91%, respectively. Post-PVR RVEDVi was not associated with the composite outcome (p = 0.59). Independent predictors of the outcome were older age at PVR (hazard ratios [HR] 1.06; 95% confidence interval [CI] 1.02 to 1.11; p = 0.004), post-PVR lower RV ejection fraction (HR 0.91; 95% CI 0.86 to 0.97; p = 0.002), and post-PVR atrial tachyarrhythmia (HR 7.60, 95% CI 1.65 to 35.05, p = 0.009). Our study shows that post-PVR RV dilation as measured by CMR-derived RVEDVi was not associated with the composite adverse clinical outcome in this cohort. These findings challenge the validity of current guidelines for PVR, which are based on pre-procedural threshold values of RVEDVi aimed at achieving normal post-procedural RV volumes.

4.
Am J Obstet Gynecol ; 222(1): 77.e1-77.e11, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31310750

RESUMO

BACKGROUND: Although consensus guidelines on the management of cardiovascular disease in pregnancy reserve cesarean delivery for obstetric indications, there is a paucity of data to support this approach. OBJECTIVE: The objective of the study was to compare cardiovascular and obstetric morbidity in women with cardiovascular disease according to the plan for vaginal birth or cesarean delivery. STUDY DESIGN: We assembled a prospective cohort of women delivering at an academic tertiary care center with a protocolized multidisciplinary approach to management of cardiovascular disease between September 2011 and December 2016. Our practice is to encourage vaginal birth in women with cardiovascular disease unless there is an obstetric indication for cesarean delivery. We allow women attempting vaginal birth a trial of Valsalva in the second stage with the ability to provide operative vaginal delivery if pushing leads to changes in hemodynamics or symptoms. Women were classified according to planned mode of delivery: either vaginal birth or cesarean delivery. We then used univariate analysis to compare adverse outcomes according to planned mode of delivery. The primary composite cardiac outcome of interest included sustained arrhythmia, heart failure, cardiac arrest, cerebral vascular accident, need for cardiac surgery or intervention, or death. Secondary obstetric and neonatal outcomes were also considered. RESULTS: We included 276 consenting women with congenital heart disease (68.5%), arrhythmias (11.2%), connective tissue disease (9.1%), cardiomyopathy (8.0%), valvular disease (1.4%), or vascular heart disease (1.8%) at or beyond 24 weeks' gestation. Seventy-six percent (n = 210) planned vaginal birth and 24% (n = 66) planned cesarean delivery. Women planning vaginal birth had lower rates of left ventricular outflow tract obstruction, multiparity, and preterm delivery. All women attempting vaginal birth were allowed Valsalva. Among planned vaginal deliveries 86.2% (n = 181) were successful, with a 9.5% operative vaginal delivery rate. Five women underwent operative vaginal delivery for the indication of cardiovascular disease without another obstetric indication at the discretion of the delivering provider. Four of these patients tolerated trials of Valsalva ranging from 15 to 75 minutes prior to delivery. Adverse cardiac outcomes were similar between planned vaginal birth and cesarean delivery groups (4.3% vs 3.0%, P = 1.00). Rates of postpartum hemorrhage (1.9% vs 10.6%, P < .01) and transfusion (1.9% vs 9.1%, P = .01) were lower in the planned vaginal birth group. There were no differences in adverse cardiac, obstetric, or neonatal outcomes in the cohort overall or the subset of women with high-risk cardiovascular disease or a high burden of obstetric comorbidity. CONCLUSION: These findings suggest that cesarean delivery does not reduce adverse cardiovascular outcomes and lend support to a planned vaginal birth for the majority of women with cardiovascular disease including those with high-risk disease.

5.
Int J Cardiol ; 2019 Oct 12.
Artigo em Inglês | MEDLINE | ID: mdl-31668657

RESUMO

BACKGROUND: Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations. METHODS: This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines. RESULTS: We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53-70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported. CONCLUSIONS: Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.

6.
J Am Coll Cardiol ; 74(18): 2295-2312, 2019 Nov 05.
Artigo em Inglês | MEDLINE | ID: mdl-31672187

RESUMO

In the contemporary era, nearly 85% of children with congenital heart disease will reach adulthood. Despite optimal medical and surgical treatment, many will experience a progressive decline in cardiopulmonary function leading to advanced heart failure. These patients present unique anatomic and physiological challenges to the care team, and unlike adults with acquired heart disease who progress to severe heart failure, advanced treatment options such as mechanical circulatory support and cardiac transplant may be limited. Severe ventricular dysfunction and/or pulmonary hypertension may not be amenable to corrective repair. Heart transplantation with or without mechanical circulatory support may be the only option for highly selected patients. The aim of this review is to describe advanced heart failure therapies for adults with congenital heart disease, including the general approach to evaluation and management, pre- and post-operative care, anticipated short- and long-term outcomes, and future directions for clinical care and research.

7.
Congenit Heart Dis ; 14(6): 1013-1023, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31642600

RESUMO

INTRODUCTION: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures. METHODS: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for patients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low-risk vs high-risk Fontan patients across regional programs. RESULTS: Fifty-six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the patient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less frequent in low-risk Fontan patients compared to high-risk Fontan patients. Counseling practices were similar for both low-risk and high-risk Fontan patients. Aspirin monotherapy was recommended by 82% of providers for low-risk Fontan patients, while anticoagulation regimens were more varied for the high-risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low-risk and high-risk Fontan patients. There were no other major differences in testing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice. CONCLUSION: This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence-based guidelines to streamline the approach to manage these complex patients.

8.
Am Heart J ; 217: 112-120, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31520896

RESUMO

BACKGROUND: Validated protocols for diagnostic testing and management of pregnant women with cardiovascular disease (CVD) do not exist. Our objective was to establish a prospective standardized protocol for the clinical evaluation of pregnant women with CVD. METHODS: The Standardized Outcomes in Reproductive Cardiovascular Care (STORCC) initiative prospectively enrolled pregnant women with CVD into a standardized diagnostic testing and assessment protocol. Detailed cardiac and obstetric data were collected during the antepartum, intrapartum, and postpartum periods. Each woman was assigned a STORCC color code of perceived risk at a monthly multidisciplinary conference. RESULTS: In 250 pregnancies of 207 women with CVD, the standardized care protocol was followed in 136 and routine care in 114. The median age of the subjects was 32 years, and the most common form of heart disease was congenital heart disease (77%). Women enrolled in standardized care protocol had high compliance with second- and third-trimester visits (93%) and postpartum visits (76%). Maternal cardiac complications occurred in 10%. The STORCC cardiac and obstetric color codes predicted adverse outcomes within each respective category (P = .02, .01). CONCLUSIONS: The STORCC protocol for prospective diagnostic testing and follow-up of pregnant women with CVD was successfully established, and compliance was high. The strength of a standardized testing and care protocol as well as detailed classification of labor and delivery characteristics allows for robust analyses into specific questions regarding testing protocols, and mode and timing of delivery.

9.
J Nucl Cardiol ; 2019 Aug 12.
Artigo em Inglês | MEDLINE | ID: mdl-31407235

RESUMO

INTRODUCTION: The prevalence of defects and effective radiation dose from various myocardial perfusion imaging (MPI) strategies in congenital heart disease (CHD) is unknown. METHODS: We studied 75 subjects with complex CHD (ages 5 to 80 years) referred for MPI between 2002 and 2015. A rest and exercise or pharmacologic stress MPI was performed using 99mTechnetium sestamibi, 82rubidium or 13N-ammonia, and Sodium iodide SPECT (single-photon emission computed tomography), SPECT/CT or Cadmium zinc telluride (CZT) SPECT or PET (positron emission tomography)/CT scanners. Deidentified images were interpreted semi-quantitatively in three batches: stress only MPI, stress/rest MPI, and stress/rest MPI with taking into account a history of ventricular septal defect repair. Effective radiation dose was estimated for stress/rest MPI and predicted for 1-day stress-first (normal stress scans), and for 2-day stress/rest MPI (abnormal stress scans). RESULTS: The median age was 18.6 years. The most common type of CHD was transposition of the great arteries (63%). Rest/stress MPI was abnormal in 43% of subjects and 25% of the abnormal scans demonstrated reversible defects. Of the subjects with abnormal MPI, 33% had significant underlying anatomic coronary artery obstruction. Estimated mean effective radiation dose ranged from 2.1 ± 0.6 mSv for 13N-ammonia PET/CT to 12.5 ± 0.9 mSv for SPECT/CT. Predicted effective radiation dose was significantly lower for stress-first MPI and for 2-day stress/rest protocols. CONCLUSIONS: Due to the relatively high prevalence of abnormal stress MPI, tailored protocols with a stress-first MPI as well as the use of 2-day protocols and advanced imaging technologies including CZT SPECT, novel image reconstruction software, and PET MPI could substantially reduce radiation dose in complex CHD.

11.
J Am Coll Cardiol ; 73(4): 457-476, 2019 02 05.
Artigo em Inglês | MEDLINE | ID: mdl-30704579

RESUMO

Cardiovascular disease complicating pregnancy is rising in prevalence secondary to advanced maternal age, cardiovascular risk factors, and the successful management of congenital heart disease conditions. The physiological changes of pregnancy may alter drug properties affecting both mother and fetus. Familiarity with both physiological and pharmacological attributes is key for the successful management of pregnant women with cardiac disease. This review summarizes the published data, available guidelines, and recommendations for use of cardiovascular medications during pregnancy. Care of the pregnant woman with cardiovascular disease requires a multidisciplinary team approach with members from cardiology, maternal fetal medicine, anesthesia, and nursing.


Assuntos
Fármacos Cardiovasculares/uso terapêutico , Complicações Cardiovasculares na Gravidez/tratamento farmacológico , Aleitamento Materno , Fármacos Cardiovasculares/farmacocinética , Doenças do Tecido Conjuntivo/complicações , Feminino , Fármacos Hematológicos/uso terapêutico , Hemodinâmica , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Hipertensão Pulmonar/tratamento farmacológico , Gravidez , Complicações Cardiovasculares na Gravidez/etiologia , Teratogênios
17.
Int J Cardiol ; 271: 60-65, 2018 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-30223379

RESUMO

BACKGROUND: Myocardial dysfunction has been implicated in gradual heart failure in transposition of the great arteries (TGA) with a systemic right ventricle (RV). Fibrosis can be assessed using the extracellular volume fraction (ECV). Our aim was to measure ECV and determine its associations with clinical findings and outcomes. METHODS: We prospectively measured ECV in systemic RV subjects (either D-loop after atrial switch or L-loop) and healthy controls. T1 measurements for a single mid-ventricular short-axis plane before and 3, 7, and 15 min after gadolinium contrast were used to quantify systemic ventricular ECV. Individuals with elevated ECV were compared to those without. RESULTS: In 53 TGA subjects (age 34.6 ±â€¯10.3 years, 41% female) the mean ECV for the systemic RV (28.7 ±â€¯4.4%) was significantly higher than the left ventricle in 22 controls (26.1 ±â€¯2.8%, P = 0.0104). Those with an elevated ECV (n = 15, 28.3%) had a higher b-type natriuretic peptide (BNP) (P < 0.011) and a longer 6-min walk distance (P = 0.021), but did not differ by age, arrhythmia history, ventricular volume, function, or circulating collagen byproducts. At follow-up (median 4.4 years), those experiencing major cardiovascular endpoints (new arrhythmia, arrhythmia device, heart failure hospitalization, listing for transplantation, mechanical support, or cardiovascular death, n = 14) had a higher ECV. ECV, age, and BNP were independent predictors of cardiac events in Cox-proportional hazard models. CONCLUSIONS: Myocardial fibrosis is common in the systemic RV and associated with a higher BNP. Elevated CMR-derived ECV was associated with adverse clinical outcome. The findings suggest a role of diffuse myocardial fibrosis in clinical deterioration of the systemic RV.


Assuntos
Insuficiência Cardíaca/diagnóstico por imagem , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/cirurgia , Adulto , Feminino , Fibrose , Seguimentos , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Estudos Prospectivos , Transposição dos Grandes Vasos/fisiopatologia , Resultado do Tratamento , Disfunção Ventricular Direita/fisiopatologia
18.
Semin Thorac Cardiovasc Surg ; 30(4): 462-469, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30063966

RESUMO

While the Fontan procedure has improved life expectancy, patients with single ventricle physiology have impaired exercise capacity due to limited increase in pulmonary blood flow during activity. Enhancing the "thoracic pump" using inspiratory muscle training (IMT) may ameliorate this impairment. Adult nonsmokers with Fontan physiology were recruited through Boston Children's Hospital's outpatient clinic. Participants underwent cardiopulmonary exercise testing and pulmonary function testing, followed by 12 weeks of IMT and then repeat testing. The primary endpoint was change in % predicted peak oxygen consumption (VO2). Secondary endpoints were changes in other exercise metrics. Eleven patients (6 male) were enrolled. Median ages at time of enrollment and Fontan completion were 28.8 years (25.7, 45.5) and 7.8 years (3.9, 16.5), respectively. Average baseline maximal inspiratory pressure (MIP) was normal; only 2 patients had MIP <70% predicted. Peak work rate improved significantly from baseline after 12 weeks of IMT (116.5 ± 45.0 to 126.8 ± 47.0 W, P = 0.019). Peak VO2 tended to improve (baseline 68.1 ± 14.3, change + 5.3 ± 9.6% predicted, P = 0.12), as did VE/VCO2 slope (34.1 ± 6.7 to 31.4 ± 3.6, P = 0.12). There was no change in peak tidal volume or MIP. In a small cohort of Fontan patients with mostly normal MIP, IMT was associated with significant improvement in peak work rate and a trend toward higher peak VO2 and improved ventilatory efficiency. Larger studies are needed to determine if this reflects true lack of effect or whether this pilot study was underpowered for effect size, and whether IMT is more narrowly useful for patients with impaired MIP.


Assuntos
Exercícios Respiratórios/métodos , Tolerância ao Exercício , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Inalação , Músculos Respiratórios/fisiopatologia , Adolescente , Adulto , Exercícios Respiratórios/instrumentação , Criança , Pré-Escolar , Feminino , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/fisiopatologia , Humanos , Capacidade Inspiratória , Masculino , Pessoa de Meia-Idade , Consumo de Oxigênio , Projetos Piloto , Estudos Prospectivos , Recuperação de Função Fisiológica , Fatores de Tempo , Resultado do Tratamento
19.
Eur Heart J ; 39(34): 3253-3261, 2018 09 07.
Artigo em Inglês | MEDLINE | ID: mdl-30010900

RESUMO

Aims: Despite the well-defined association of high-sensitivity hsCRP with cardiovascular outcomes in apparently healthy adults and those with acquired heart disease, the relevance of this inflammatory marker in adults with congenital heart disease (ACHD) remains unclear. We aimed to examine the clinical correlates and prognostic value of high-sensitivity C-reactive protein levels in ACHD. Methods and results: We conducted a prospective cohort study of (n = 707) outpatient ACHD (age 39 ± 14 years, 49% women), enrolled mainly at a referral centre, who had serum hsCRP measured in conjunction with a clinical assessment between 2012 and 2016. We analysed clinical correlates of hsCRP and its association with adverse events including the primary combined outcome of all-cause mortality or non-elective cardiovascular hospitalization. Higher hsCRP was strongly associated with measures of functional status including New York Heart Association class and peak V̇O2, and with comorbidities such as atrial arrhythmia. During average follow-up of 815 ± 536 days, 114 patients (16%) experienced the primary outcome, including 29 deaths. Having elevated hsCRP, in the highest (≥2.98 mg/L) compared with the lower three quartiles, conferred increased risk for the primary outcome [30.5% vs. 11.3%, adjusted hazard ratio (HR) = 2.00, 95% confidence interval (CI) 1.35-2.97; P = 0.0006] and all-cause mortality (11.9% vs. 1.5%, adjusted HR = 4.23, 95% CI 1.87-9.59; P = 0.0006). Elevated hsCRP was associated with adverse outcomes across ACHD subgroups and other patient characteristics. Conclusion: Adults with congenital heart disease with elevated hsCRP have not only worse functional status and exercise capacity, but also greater risk for death or non-elective cardiovascular hospitalization. Further study is warranted to characterize the role of inflammation in the pathophysiology of ACHD.


Assuntos
Bancos de Espécimes Biológicos/estatística & dados numéricos , Proteína C-Reativa/metabolismo , Cardiopatias Congênitas/sangue , Adulto , Biomarcadores/sangue , Boston , Feminino , Seguimentos , Humanos , Masculino , Prognóstico , Estudos Prospectivos , Fatores de Risco
20.
Circulation ; 138(19): 2106-2115, 2018 11 06.
Artigo em Inglês | MEDLINE | ID: mdl-30030416

RESUMO

BACKGROUND: Risk factors for adverse clinical outcomes have been identified in patients with repaired tetralogy of Fallot before pulmonary valve replacement (PVR). However, pre-PVR predictors for post-PVR sustained ventricular tachycardia and death have not been identified. METHODS: Patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort (International Multicenter TOF Registry), a 4-center international cohort study, who had a comprehensive preoperative evaluation and subsequently underwent PVR were included. Preprocedural clinical, ECG, cardiovascular magnetic resonance, and postoperative outcome data were analyzed. Cox proportional hazards multivariable regression analysis was used to evaluate factors associated with time from pre-PVR cardiovascular magnetic resonance until the primary outcome: death, aborted sudden cardiac death, or sustained ventricular tachycardia. RESULTS: Of the 452 eligible patients (median age at PVR, 25.8 years), 36 (8%) reached the primary outcome (27 deaths, 2 resuscitated death, and 7 sustained ventricular tachycardia) at a median time after PVR of 6.5 years. Cox proportional hazards regression identified pre-PVR right ventricular ejection fraction <40% (hazard ratio, 2.39; 95% CI, 1.18-4.85; P=0.02), right ventricular mass-to-volume ratio ≥0.45 g/mL (hazard ratio, 4.08; 95% CI, 1.57-10.6; P=0.004), and age at PVR ≥28 years (hazard ratio, 3.10; 95% CI, 1.42-6.78; P=0.005) as outcome predictors. In a subgroup analysis of 230 patients with Doppler data, predicted right ventricular systolic pressure ≥40 mm Hg was associated with the primary outcome (hazard ratio, 3.42; 95% CI, 1.09-10.7; P=0.04). Preoperative predictors of a composite secondary outcome, postoperative arrhythmias and heart failure, included older age at PVR, pre-PVR atrial tachyarrhythmias, and a higher left ventricular end-systolic volume index. CONCLUSIONS: In this observational investigation of patients with repaired tetralogy of Fallot, an older age at PVR and pre-PVR right ventricular hypertrophy and dysfunction were predictive of a shorter time to postoperative death and sustained ventricular tachycardia. These findings may inform the timing of PVR if confirmed by prospective clinical trials.


Assuntos
Procedimentos Cirúrgicos Cardíacos/mortalidade , Implante de Prótese de Valva Cardíaca/mortalidade , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Taquicardia Ventricular/mortalidade , Tetralogia de Fallot/cirurgia , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Ecocardiografia Doppler , Eletrocardiografia , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Hemodinâmica , Humanos , Hipertrofia Ventricular Direita/etiologia , Hipertrofia Ventricular Direita/mortalidade , Hipertrofia Ventricular Direita/fisiopatologia , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/etiologia , Estenose da Valva Pulmonar/mortalidade , Estenose da Valva Pulmonar/fisiopatologia , Sistema de Registros , Medição de Risco , Fatores de Risco , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/fisiopatologia , Tetralogia de Fallot/mortalidade , Tetralogia de Fallot/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/mortalidade , Disfunção Ventricular Direita/fisiopatologia , Função Ventricular Direita , Adulto Jovem
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