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The antibiotic doxycycline is known to inhibit inflammation and was therefore considered as a therapeutic to prevent abdominal aortic aneurysm (AAA) growth. Yet mitochondrial dysfunction is a key-characteristic of clinical AAA disease. We hypothesize that doxycycline impairs mitochondrial function in the aorta and aortic smooth muscle cells (SMCs). Doxycycline induced mitonuclear imbalance, reduced proliferation and diminished expression of typical contractile smooth muscle cell (SMC) proteins. To understand the underlying mechanism, we studied krüppel-like factor 4 (KLF4). The expression of this transcription factor was enhanced in SMCs after doxycycline treatment. Knockdown of KLF4, however, did not affect the doxycycline-induced SMC phenotypic changes. Then we used the bioenergetics drug elamipretide (SS-31). Doxycycline-induced loss of SMC contractility markers was not rescued, but mitochondrial genes and mitochondrial connectivity improved upon elamipretide. Thus while doxycycline is anti-inflammatory, it also induces mitochondrial dysfunction in aortic SMCs and causes SMC phenotypic switching, potentially contributing to aortic aneurysm pathology. The drug elamipretide helps mitigate the harmful effects of doxycycline on mitochondrial function in aortic SMC, and may be of interest for treatment of aneurysm diseases with pre-existing mitochondrial dysfunction.
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Aneurisma da Aorta Abdominal , Doenças Mitocondriais , Humanos , Doxiciclina/efeitos adversos , Doxiciclina/metabolismo , Aorta/metabolismo , Aneurisma da Aorta Abdominal/induzido quimicamente , Aneurisma da Aorta Abdominal/prevenção & controle , Aneurisma da Aorta Abdominal/genética , Miócitos de Músculo Liso/metabolismo , Doenças Mitocondriais/metabolismo , Doenças Mitocondriais/patologiaRESUMO
INTRODUCTION: Increasing survival of adult congenital heart disease (ACHD) patients comes at the price of a range of late complications-arrhythmias, heart failure, and valvular dysfunction. Transcatheter valve interventions have become a legitimate alternative to conventional surgical treatment in selected acquired heart disease patients. However, literature on technical aspects, hemodynamic effects, and clinical outcomes of percutaneous atrioventricular (AV) valve interventions in ACHD patients is scarce. METHOD: This is a descriptive cohort from CAHAL (Center of Congenital Heart Disease Amsterdam-Leiden). ACHD patients with severe AV valve regurgitation who underwent a transcatheter intervention in the period 2020-2022 were included. Demographic, clinical, procedural, and follow-up data were collected from patient records. RESULTS: Five ACHD patients with severe or torrential AV valve regurgitation are described. Two patients underwent a transcatheter edge-to-edge repair (TEER), one patient underwent a valve-in-valve procedure, one patient received a Cardioband system, and one patient received both a Cardioband system and TEER. No periprocedural complications occurred. Post-procedural AV valve regurgitation as well as NYHA functional class improved in all patients. The median post-procedural NYHA functional class improved from 3.0 (IQR [2.5-4.0]) to 2.0 (IQR [1.5-2.5]). One patient died 9 months after the procedure due to advanced heart failure with multiorgan dysfunction. CONCLUSION: Transcatheter valve repair is feasible and safe in selected complex ACHD patients. A dedicated heart team is essential for determining an individualized treatment strategy as well as pre- and periprocedural imaging to address the underlying mechanism(s) of AV regurgitation and guide the transcatheter intervention. Long-term follow-up is essential to evaluate the clinical outcomes of transcatheter AV valve repair in ACHD patients.
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OBJECTIVE: Quantitative extracellular volume fraction (ECV) mapping with MRI is commonly used to investigate in vivo diffuse myocardial fibrosis. This study aimed to validate ECV measurements against ex vivo histology of myocardial tissue samples from patients with aortic valve stenosis or hypertrophic cardiomyopathy. MATERIALS AND METHODS: Sixteen patients underwent MRI examination at 3 T to acquire native T1 maps and post-contrast T1 maps after gadobutrol administration, from which hematocrit-corrected ECV maps were estimated. Intra-operatively obtained myocardial tissue samples from the same patients were stained with picrosirius red for quantitative histology of myocardial interstitial fibrosis. Correlations between in vivo ECV and ex vivo myocardial collagen content were evaluated with regression analyses. RESULTS: Septal ECV was 30.3% ± 4.6% and correlated strongly (n = 16, r = 0.70; p = 0.003) with myocardial collagen content. Myocardial native T1 values (1206 ± 36 ms) did not correlate with septal ECV (r = 0.41; p = 0.111) or with myocardial collagen content (r = 0.32; p = 0.227). DISCUSSION: We compared myocardial ECV mapping at 3 T against ex vivo histology of myocardial collagen content, adding evidence to the notion that ECV mapping is a surrogate marker for in vivo diffuse myocardial fibrosis.
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Estenose da Valva Aórtica , Cardiomiopatias , Cardiomiopatia Hipertrófica , Humanos , Imagem Cinética por Ressonância Magnética , Valor Preditivo dos Testes , Biópsia , Reprodutibilidade dos Testes , Miocárdio/patologia , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/patologia , Imageamento por Ressonância Magnética , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/patologia , Colágeno , Fibrose , Espectroscopia de Ressonância Magnética , Meios de ContrasteRESUMO
OBJECTIVES: To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA). METHODS: Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR. RESULTS: In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor. CONCLUSIONS: Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.
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Síndromes do Arco Aórtico , Anel Vascular , Gravidez , Lactente , Recém-Nascido , Feminino , Humanos , Criança , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Ultrassonografia Pré-Natal , Estudos Retrospectivos , Diagnóstico Pré-Natal , Síndromes do Arco Aórtico/diagnóstico por imagem , Síndromes do Arco Aórtico/cirurgiaRESUMO
Marfan syndrome (MFS) is a connective tissue disorder causing aortic aneurysm formation. Currently, only prophylactic aortic surgery and blood pressure-lowering drugs are available to reduce the risk of aortic rupture. Upon whole genome sequencing of a Marfan family, we identified a complement gene C1R variant (p.Ser152Leu), which is associated with severe aortic patients. Therefore, we assessed the role of complement activation in MFS aortic tissue. Expression of various complement genes and proteins was detected in human and murine MFS aneurysm tissue, which prompted us to study complement inhibition in MFS mice. Treatment of the Fbn1C1041G/+ MFS mice with human plasma-derived C1-esterase inhibitor Cetor® resulted in reduced complement deposition, decreased macrophage influx in the aorta, and lower circulating TNFα levels. However, in line with previous anti-inflammatory treatments, complement inhibition did not change the aortic dilatation rate in this MFS mouse model. Thus, while complement factors/component 3 activation were detected in human/murine MFS aorta, Cetor® had no effect on aortic dilatation in MFS mice, indicating that complement inhibition is not a suitable treatment strategy in MFS.
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This report describes the case of a symptomatic patient with a right coronary artery fistula draining into the coronary sinus who underwent transcatheter closure, which was deployed in the drainage site to seal off the exit of the fistula. (Level of Difficulty: Advanced.).
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OBJECTIVES: The objective of this study was to assess our 43-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) by analysing cardiac outcome measures (hospital and late mortality, reoperations and catheter interventions, significant coronary artery obstruction) and to identify risk factors for reoperation and catheter interventions. METHODS: A total of 490 patients who underwent ASO for TGA from 1977 to 2020 were included in this retrospective, single-centre study. Data on reoperation and catheter intervention of hospital survivors were estimated by the Kaplan-Meier method and compared using a long-rank test. Risk factors for reoperation and/or catheter intervention were assessed by multivariate Cox regression analysis. RESULTS: Hospital mortality occurred in 43 patients (8.8%), late death in 12 patients (2.9%) and 43 patients were lost to follow-up. Median follow-up time of 413 hospital survivors was 15.6 (interquartile range 7.0-22.4) years. Reoperations were performed in 83 patients (117 reoperations). Neoaortic valve regurgitation with root dilatation was the second most common indication for reoperation (15/83 patients, 18.1%) after right ventricular outflow tract obstruction (50/83 patients, 60.2%). Risk factors for any reoperation on multivariable analysis were: TGA morphological subtype [TGA with ventricular septal defect: hazard ratio (HR) = 1.99, 95% confidence interval (CI) 1.18-3.36; P = 0.010 and Taussig-Bing: HR = 2.17, 95% CI 1.02-4.64; P = 0.045], aortic arch repair associated with ASO (HR = 3.03, 95% CI 1.62-5.69; P = 0.001) and a non-usual coronary artery anatomy (HR = 2.41, 95% CI 1.45-4.00; P = 0.001). One hundred and one catheter interventions were performed in 54 patients, usually for relief of supravalvular pulmonary stenosis (44/54 patients, 81.5%) or arch obstruction (10/54 patients, 18.5%). Main risk factor for catheter intervention on multivariable analysis was aortic arch repair associated with ASO (HR = 2.95, 95% CI 1.37-6.36; P = 0.006). Significant coronary artery stenosis was relatively uncommon (9/413 patients, 2.2%) but may be underrepresented. CONCLUSIONS: Patients after ASO typically have good long-term clinical outcomes but reoperations and interventions remain necessary in some patients. Neoaortic valve regurgitation with root dilatation is the second most common indication for reoperation after right ventricular outflow tract obstruction and an increasing need for neoaortic valve and root redo surgery in future is to be expected.
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Transposição das Grandes Artérias , Transposição dos Grandes Vasos , Seguimentos , Humanos , Lactente , Complicações Pós-Operatórias/cirurgia , Reoperação , Estudos Retrospectivos , Transposição dos Grandes Vasos/cirurgia , Resultado do TratamentoRESUMO
OBJECTIVES: Tracheobronchomalacia (TBM) is characterized by collapse of trachea, bronchi or both, leading to dyspnoea, expiratory stridor, coughing or recurrent airway infections. Surgical treatment with aortopexy is warranted for severe TBM. We describe a modified aortopexy technique with aortic wall strap sutures that evenly distributes the traction force over the full width of the aortic arch. The aim of this study was to determine the outcomes of this modified anterior aortopexy technique. METHODS: Retrospective chart review of all patients undergoing aortopexy with aortic wall strap sutures for TBM between January 2010 and June 2020 in 2 tertiary hospitals in the Netherlands. RESULTS: Twenty-four patients [median age 9 months (interquartile range 2-117 months); 71% male] underwent aortopexy with the modified technique for TBM (52%), tracheomalacia (40%) or bonchomalacia (8%). Aortopexy was successful in 91.7%, defined as relief or decrease of respiratory symptoms and no need for respiratory support. Complications occurred in 8.3% and mortality was 4%. CONCLUSIONS: Aortopexy with non-absorbable strap sutures seems an effective and safe treatment for severe TBM. This study supports the hypothesis that strap sutures provide a solid and reliable traction force, but future comparative studies should confirm the benefit of strap sutures over conventional techniques.
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Traqueobroncomalácia , Traqueomalácia , Brônquios , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Traqueia , Traqueobroncomalácia/diagnóstico por imagem , Traqueobroncomalácia/cirurgiaAssuntos
Ecocardiografia/métodos , Endocardite/etiologia , Comunicação Interventricular/etiologia , Imagem Multimodal/métodos , Neurocisticercose/complicações , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Endocardite/diagnóstico , Feminino , Comunicação Interventricular/diagnóstico , Humanos , Neurocisticercose/diagnóstico , Adulto JovemRESUMO
BACKGROUND: Kommerell's diverticulum is a rare vascular anomaly characterized as an outpouch at the onset of an aberrant subclavian artery. In the variant of a right-sided aortic arch, the trachea and esophagus are enclosed dorsally by the arch. In the configuration of an aberrant left subclavian artery, a Kommerell's diverticulum and persisting ductus arteriosus or ductal ligament enclose the lateral side, forming a vascular ring which may result in (symptomatic) esophageal or tracheal compression. Spontaneous rupture of an aneurysmatic Kommerell's diverticulum has also been reported. Due to the rarity of this condition and underreporting in the literature, the clinical implications of a Kommerell's diverticulum are not well defined. CASE SUMMARY: We describe seven consecutive adult patients with a right-sided aortic arch and an aberrant course of the left subclavian artery (arteria lusoria), and a Kommerell's diverticulum, diagnosed in our tertiary hospital. One patient had severe symptoms related to the Kommerell's diverticulum and underwent surgical repair. In total, two of the patients experienced mild non-limiting dyspnea complaints and in four patients the Kommerell's diverticulum was incidentally documented on a computed tomography (CT) scan acquired for a different indication. The size of the Kommerell's diverticulum ranged from 19 × 21 mm to 30 × 29 mm. In the six patients that did not undergo surgery, a strategy of periodic follow-up with structural imaging was pursued. No significant growth of the Kommerell's diverticulum was observed and none of the patients experienced an acute aortic syndrome to date. DISCUSSION: Kommerell's diverticulum in the setting of a right-sided aortic arch with an aberrant left subclavian artery is frequently associated with tracheal and esophageal compression and this may result in a varying range of symptoms. Guidelines on management of Kommerell's diverticulum are currently lacking. This case series and literature overview suggests that serial follow-up is warranted in adult patients with a Kommerell's diverticulum with small dimensions and no symptoms, however, that surgical intervention should be considered when patients become symptomatic or when the diameter exceeds 30 mm in the absence of symptoms.
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BACKGROUND: Proton magnetic resonance spectroscopy (1 H-MRS) of the human heart is deemed to be a quantitative method to investigate myocardial metabolite content, but thorough validations of in vivo measurements against invasive techniques are lacking. PURPOSE: To determine measurement precision and accuracy for quantifications of myocardial total creatine and triglyceride content with localized 1 H-MRS. STUDY TYPE: Test-retest repeatability and measurement validation study. SUBJECTS: Sixteen volunteers and 22 patients scheduled for open-heart aortic valve replacement or septal myectomy. FIELD STRENGTH/SEQUENCE: Prospectively ECG-triggered respiratory-gated free-breathing single-voxel point-resolved spectroscopy (PRESS) sequence at 3 T. ASSESSMENT: Myocardial total creatine and triglyceride content were quantified relative to the total water content by fitting the 1 H-MR spectra. Precision was assessed with measurement repeatability. Accuracy was assessed by validating in vivo 1 H-MRS measurements against biochemical assays in myocardial tissue from the same subjects. STATISTICAL TESTS: Intrasession and intersession repeatability was assessed using Bland-Altman analyses. Agreement between 1 H-MRS measurements and biochemical assay was tested with regression analyses. RESULTS: The intersession repeatability coefficient for myocardial total creatine content was 41.8% with a mean value of 0.083% ± 0.020% of the total water signal, and 36.7% for myocardial triglyceride content with a mean value of 0.35% ± 0.13% of the total water signal. Ex vivo myocardial total creatine concentrations in tissue samples correlated with the in vivo myocardial total creatine content measured with 1 H-MRS: n = 22, r = 0.44; P < 0.05. Likewise, ex vivo myocardial triglyceride concentrations correlated with the in vivo myocardial triglyceride content: n = 20, r = 0.50; P < 0.05. DATA CONCLUSION: We validated the use of localized 1 H-MRS of the human heart at 3 T for quantitative assessments of in vivo myocardial tissue metabolite content by estimating the measurement precision and accuracy. LEVEL OF EVIDENCE: 2 TECHNICAL EFFICACY STAGE: 2.
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Creatina , Miocárdio , Coração/diagnóstico por imagem , Humanos , Espectroscopia de Prótons por Ressonância Magnética , TriglicerídeosRESUMO
BACKGROUND: Prolonged or excessive bleeding after cardiac surgery can lead to a broad spectrum of secondary complications. One of the underlying causes is incomplete wound drainage, with subsequent accumulation of blood and clots in the pericardium. We developed the continuous postoperative pericardial flushing (CPPF) therapy to improve wound drainage and reduce postoperative blood loss and bleeding-related complications after cardiac surgery. This study compared CPPF to standard care in patients after coronary artery bypass grafting (CABG). METHODS: This is a single center, open label, randomized trial that enrolled patients at the Amsterdam UMC, location AMC, Amsterdam, the Netherlands. The study was registered at the 'Netherlands Trial Register', study identifier NTR5200 [1]. Adults undergoing CABG were randomly assigned to receive CPPF therapy or standard care, participants and investigators were not masked to group assignment. The primary end point was postoperative blood loss in the first 12-hours after surgery. FINDINGS: Between the January 15, 2014 and the March 13, 2017, 169 patients were enrolled and assigned to CPPF therapy (study group; n = 83) or standard care (control group; n = 86). CPPF reduced postoperative blood loss when compared to standard care (median differences -385 ml, reduction 76% p=≤0.001), with the remark that these results are overestimated due to a measurement error in part of the study group. None of patients in the study group required reoperation for non-surgical bleeding versus 3 (4%, 95% CI -0.4% to 7.0%) in the control group. None of the patients in the study group suffered from cardiac tamponade, versus 3 (4%, 95% CI -0,4% to 7.0%) in the control group. The incremental cost-effectiveness ratio was 116.513 (95% bootstrap CI -882.068 to +897.278). INTERPRETATION: The use of CPPF therapy after CABG seems to reduce bleeding and bleeding related complications. With comparable costs and no improvement in Qualty of Life (QoL), cost consideration for the implementation of CPPF is not relevant. None of the patients in the study group required re-interventions for non-surgical bleeding or acute cardiac tamponade, which underlines the proof of concept of this novel therapy. FUNDING: This study was funded by ZonMw, the Netherlands organization for health research and development (project 837001405).
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The thymus is a primary lymphoid organ necessary for optimal T cell development. Here, we show that liver X receptors (LXRs)-a class of nuclear receptors and transcription factors with diverse functions in metabolism and immunity-critically contribute to thymic integrity and function. LXRαß-deficient mice develop a fatty, rapidly involuting thymus and acquire a shrunken and prematurely immunoinhibitory peripheral T cell repertoire. LXRαß's functions are cell specific, and the resulting phenotypes are mutually independent. Although thymic macrophages require LXRαß for cholesterol efflux, thymic epithelial cells (TECs) use LXRαß for self-renewal and thymocytes for negative selection. Consequently, TEC-derived LXRαß protects against homeostatic premature involution and orchestrates thymic regeneration following stress, while thymocyte-derived LXRαß limits cell disposal during negative selection and confers heightened sensitivity to experimental autoimmune encephalomyelitis. These results identify three distinct but complementary mechanisms by which LXRαß governs T lymphocyte education and illuminate LXRαß's indispensable roles in adaptive immunity.
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Receptores X do Fígado/fisiologia , Fígado/metabolismo , Linfócitos T/fisiologia , Timo/fisiologia , Imunidade Adaptativa , Animais , Apoptose , Feminino , Citometria de Fluxo , Homeostase , Humanos , Metabolismo dos Lipídeos , Receptores X do Fígado/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Reação em Cadeia da Polimerase em Tempo Real , Linfócitos T/metabolismo , Timo/metabolismoRESUMO
OBJECTIVE: Marfan syndrome (MFS) is caused by mutations in FBN1 (fibrillin-1), an extracellular matrix (ECM) component, which is modified post-translationally by glycosylation. This study aimed to characterize the glycoproteome of the aortic ECM from patients with MFS and relate it to aortopathy. Approach and Results: ECM extracts of aneurysmal ascending aortic tissue from patients with and without MFS were enriched for glycopeptides. Direct N-glycopeptide analysis by mass spectrometry identified 141 glycoforms from 47 glycosites within 35 glycoproteins in the human aortic ECM. Notably, MFAP4 (microfibril-associated glycoprotein 4) showed increased and more diverse N-glycosylation in patients with MFS compared with control patients. MFAP4 mRNA levels were markedly higher in MFS aortic tissue. MFAP4 protein levels were also increased at the predilection (convexity) site for ascending aorta aneurysm in bicuspid aortic valve patients, preceding aortic dilatation. In human aortic smooth muscle cells, MFAP4 mRNA expression was induced by TGF (transforming growth factor)-ß1 whereas siRNA knockdown of MFAP4 decreased FBN1 but increased elastin expression. These ECM changes were accompanied by differential gene expression and protein abundance of proteases from ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) family and their proteoglycan substrates, respectively. Finally, high plasma MFAP4 concentrations in patients with MFS were associated with a lower thoracic descending aorta distensibility and greater incidence of type B aortic dissection during 68 months follow-up. CONCLUSIONS: Our glycoproteomics analysis revealed that MFAP4 glycosylation is enhanced, as well as its expression during the advanced, aneurysmal stages of MFS compared with control aneurysms from patients without MFS.
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Aorta/química , Matriz Extracelular/química , Glicopeptídeos/análise , Síndrome de Marfan/metabolismo , Proteômica/métodos , Aneurisma da Aorta Torácica/metabolismo , Proteínas de Transporte/sangue , Proteínas de Transporte/genética , Proteínas de Transporte/fisiologia , Proteínas da Matriz Extracelular/sangue , Proteínas da Matriz Extracelular/genética , Proteínas da Matriz Extracelular/fisiologia , Fibrilina-1/genética , Glicoproteínas/sangue , Glicoproteínas/genética , Glicoproteínas/fisiologia , Glicosilação , Humanos , Miócitos de Músculo Liso/metabolismo , Remodelação VascularRESUMO
OBJECTIVES: The aim of this retrospective study was to compare subcuticular sutures and Steri-Strip™ S in closing median sternotomy incisions in children with regard to wound healing and scar formation. METHODS: Fifty-three children and adolescents were enrolled in this study who all underwent a median sternotomy at age 0-18 years and had their presternal cutaneous wounds closed with either a running subcuticular suture (Group 1) or Steri-Strip™ S (Group 2). Their scars were assessed using the Patient and Observer Scar Assessment Scale (POSAS). Secondary outcome measures were the scar measurements and the incidence of wound problems post-surgery. RESULTS: A significant difference was found between both groups in median POSAS observer scale scores for the items thickness (p = .027), pliability (p = .045), surface area (p = .045) and the total score (p = .048). All in favor of the subcuticular suture group. There were no significant differences concerning the POSAS patient scale scores. Middle parts of scars of patients in Group 2 were significantly broader (p = .001) than scars of patients in Group 1. No significant differences concerning wound problems were found. CONCLUSIONS: There are, according to our results, no significant differences in wound healing of median sternotomy incisions in children closed with either a subcuticular suture or Steri-Strip™ S. Significant differences do exist regarding scar formation and final cosmetic results of the scars, in favor of subcuticular closure.
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Esternotomia , Suturas , Técnicas de Fechamento de Ferimentos/instrumentação , Adolescente , Criança , Pré-Escolar , Cicatriz/etiologia , Cicatriz/patologia , Estudos Transversais , Estética , Feminino , Humanos , Masculino , Estudos Retrospectivos , Cicatrização , Adulto JovemRESUMO
Aims: Functional tricuspid regurgitation (TR) associated with atrial septal defects (ASDs) is frequently present due to right-sided volume-overload. Tricuspid valve (TV) repair is often considered in candidates for surgical ASD closure, and percutaneous TV repair is currently under clinical investigation. In this study, we develop a prediction model to identify patients with residual moderate/severe TR after percutaneous ASD closure. Methods and results: In this observational study, 172 adult patients (26% male, age 49 ± 17 years) with successful percutaneous ASD closure had pre- and post-procedural echocardiography. Right heart dimensions/function were measured. TR was assessed semi-quantitatively. A prediction model for 6-month post-procedural moderate/severe TR was derived from uni-and multi-variable logistic regression. Clinical follow-up (FU) was updated and adverse events were defined as cardiovascular death or hospitalization for heart failure. Pre-procedural TR was present in 130 (76%) patients (moderate/severe: n = 64) of which 72 (55%) had ≥1 grade reduction post-closure. Independent predictors of post-procedural moderate/severe TR (n = 36) were age ≥60 years [odds ratio (OR) 2.57; P = 0.095], right atrial end-diastolic area ≥10cm2/m2 (OR 3.36; P = 0.032), right ventricular systolic pressure ≥44 mmHg (OR 6.44; P = 0.001), and tricuspid annular plane systolic excursion ≤2.3 cm (OR 3.29; P = 0.037), producing a model with optimism-corrected C-index = 0.82 (P < 0.001). Sensitivity analysis excluding baseline none/mild TR yielded similar results. Patients with moderate/severe TR at 6-month FU had higher adverse event rates [hazard ratio = 6.2 (95% confidence interval 1.5-26); log-rank P = 0.004] across a median of 45 (30-76) months clinical FU. Conclusion: This study shows that parallel to reduction of volume-overload and reverse remodelling after percutaneous ASD closure, TR improved substantially despite significant TR at baseline. Our proposed risk model helps identify ASD patients in whom TR regression is unlikely after successful percutaneous closure.
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Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interatrial/cirurgia , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Ecocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Retrospectivos , Insuficiência da Valva Tricúspide/fisiopatologiaRESUMO
OBJECTIVES: Surgical repair of coarctation of the aorta (CoA) is often possible through left thoracotomy and without the use of cardiopulmonary bypass. Recent studies reporting the outcome after CoA repair through left thoracotomy are limited. Therefore, the aim of this study is to evaluate the results of CoA repair through left thoracotomy in children who were operated on in our centre over the past 21 years. METHODS: From January 1995 to December 2016, 292 patients younger than 18 years underwent primary CoA repair through left thoracotomy at our 2 institutions. Peri- and postoperative data and follow-up data collected from our hospital and the referring hospitals were retrospectively reviewed. RESULTS: Median age at operation was 64 days (range 2 days-17 years). Most patients underwent the resection of the CoA followed by an (extended) end-to-end anastomosis (93%). Six patients died perioperatively and 2 more patients died during the follow-up, of which 7 patients had other major comorbidities. Actuarial survival was 97% at 5 years, 96% at 10 years and 96% at 15 years. Second arch interventions due to recoarctation were performed in 9.9% (n = 29) of patients, consisting of balloon dilatation in all but 2 patients. Recoarctation occurred significantly more often after initial repair in the neonatal period (21%) and could occur as late as 14 years after initial surgery. There were 7 re-recoarctations, and 14% of patients were on hypertensive medication during the follow-up. CONCLUSIONS: Repair of CoA through left thoracotomy is a safe procedure with low rates of mortality. The long-term follow-up is necessary due to the significant risk of recoarctation requiring reintervention.