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Congenital heart disease (CHD) affects approximately one million people in the USA with the number increasing by 5% each year. Patients are usually both diagnosed and treated in infancy, however many of them may have subclinical CHD that remains undiagnosed until late adulthood. Patients with complex CHD tend to be symptomatic and are diagnosed at a younger age than those with a single defect. CHDs can be divided into three categories, including cardiac, great vessels and coronary artery anomalies. Recent advances in computed tomography (CT) technology with faster acquisition time and improved spatial resolution allow for detailed evaluation of cardiac morphology and function. The concomitant increased utilization of CT has simultaneously led to more sensitive detection and more thorough diagnosis of CHD. Recognition of and understanding the imaging attributes specific to each anomaly is important for radiologists in order to make a correct and definite diagnosis. This article reviews the spectrum of CHDs, which persist into adulthood that may be encountered by radiologists on CT.  相似文献   

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ObjectiveThe study objective was to examine pulmonary function and quality of life improvement after robotic-assisted thoracoscopic tracheobronchoplasty for patients with different degrees of obstructive airway disease.MethodsWe performed a retrospective review of a prospective database of patients who underwent robotic-assisted thoracoscopic tracheobronchoplasty between 2013 and 2020.ResultsA total of 118 patients underwent robotic-assisted thoracoscopic tracheobronchoplasty. Preoperative and postoperative pulmonary function tests were available for 108 patients. Postoperative pulmonary function tests at a median of 16 months demonstrated a significant increase in percent predicted forced expiratory volume in 1 second (preoperative median: 76.76% predicted, postoperative: 83% predicted, P = .002). Preoperative and postoperative St George Respiratory Questionnaires were available for 64 patients with a significant decrease in postoperative score at a median of 7 months (preoperative median: 61, postoperative: 41.60, P < .001). When stratified by preoperative degree of obstruction, robotic-assisted thoracoscopic tracheobronchoplasty improved forced expiratory volume in 1 second in moderate to very severe obstruction with a statistically significant improvement in moderate (preoperative median: 63.91% predicted, postoperative median: 73% predicted, P = .001) and severe (preoperative median: 44% predicted, postoperative median: 57% predicted, P = .007) obstruction. St George Respiratory Questionnaire scores improved for all patients. Improvement for mild (preoperative median: 61.27, postoperative median: 36.71, P < .001) and moderate (preoperative median: 57.15, postoperative median: 47.52, P = .03) obstruction was statistically significant.ConclusionsRobotic-assisted thoracoscopic tracheobronchoplasty improves obstruction and symptoms. With limited follow-up, subgroup analysis showed forced expiratory volume in 1 second improved in severe preoperative obstruction and quality of life improved in moderate obstruction. Future follow-up is required to determine robotic-assisted thoracoscopic tracheobronchoplasty effects on the most severe group, but we cannot conclude that increased degree of preoperative obstruction precludes surgery.  相似文献   

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IntroductionDystrophic epidermolysis bullosa (DEB) is one of the most severe forms of congenital epidermolysis bullosa and characterized by the formation of many surgical complications. Esophageal stenosis is a common complication of DEB and occurs in almost 76% of cases. Balloon dilatation (BD) under X-ray control is the main therapeutic technique, however conservative treatment is necessary to prevent restenosis. The use of the drug losartan is promising due to its antifibrotic effect through the suppression of transforming growth factor-β1 (TGF-β1).PurposeTo evaluate the efficacy of losartan in the prevention of restenosis after BD of esophageal stenosis in children with DEB.Materials and methodsThe study included 19 children from 2 to 16 years old (mean age 9.2 ± 3.58 years) with DEB and X-ray confirmed esophageal stenosis. All children underwent BD. In the main group 9 children after BD have received losartan, in the control group of 10 children - only standard therapy. The observation period was 12 months.ResultsIn the main group, 1 child (11.1%) required repeated dilatation, in the control group - 4 children (40%). Indicators of nutritional deficiency (THINC scale) and the disease severity index (EBDASI) were significantly lower in the group of children treated with losartan. No undesirable actions of the drug were recorded.ConclusionsIn this study losartan showed its safety, contributed to a decrease in the restenosis frequency and an improvement in the nutritional status of children with DEB after BD. However, further studies are required to confirm its effectiveness.Level of EvidenceIV.  相似文献   

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BackgroundCongenital chylothorax (CCT) and postoperative chylothorax (POCT) are rare and difficult to treat. We report our treatment strategy and outcomes for chylothorax, including thoracoscopic surgery with indocyanine-green (ICG) near-infrared fluorescence lymphangiography.MethodsA retrospective review of patients with CCT and POCT from 2014 to 2021 was performed. After definitive diagnosis, conservative treatments with octreotide, followed by intravenous steroids as needed, were performed. Patients who were refractory to conservative treatment were transferred to surgical treatment, consisting of thoracoscopic lymphatic leak ligations using ICG intraoperative lymphangiography. The effectiveness of conservative and surgical treatment was then examined.ResultsWe included 19 cases of CCT and 31 cases of POCT. The 31 POCT patients included 23 of 84 postoperative patients with congenital diaphragmatic hernia (CDH), 7 of 54 postoperative patients with esophageal atresia (EA), and 1 of 3 postoperative patients with lymphatic malformation. The efficacy of conservative treatment was 12/19 for CCT, 22/23 for CDH, and 4/7 for EA. Surgical intervention was performed in 10 patients, and the rate of resolution of chylothorax within 3 weeks after surgery was 90%.ConclusionThoracoscopic lymphatic leak ligations with intraoperative ICG lymphangiography are feasible and useful in patients with chylothorax refractory to conservative treatment.Level of evidenceLevel IV  相似文献   

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《Injury》2022,53(12):3938-3944
PurposeFracture-related infection (FRI) is one of the most serious complications in orthopedic trauma surgery. Despite its widespread use, the role of Negative Pressure Wound Therapy (NPWT) remains controversial in the management pathway of FRI. The aim of this study was to assess the relationship between the application of NPWT and its duration and recurrence of infection in operatively treated FRI patients.Patients and MethodsThis is a retrospective cohort study based on the FRI database of three level 1 Trauma Centres. Included patients had to be at least 16 years of age and surgically treated for FRI between January 1st 2015 and September 1st 2020. Patients were subdivided in either the NPWT group, when NPWT was applied as part of the FRI treatment, or in the control group, when no NPWT had been applied. To limit confounding, patients were excluded if they (also) underwent NPWT prior to the diagnosis of FRI. The relation between the duration of NPWT during FRI treatment and the recurrence rate of infection was analyzed using a multivariable logistic regression model.ResultsA total of 263 patients were included, 99 in the NPWT group and 164 in the control group. The median duration of NPWT was 18.0 (IQR 15.8) days. In the NPWT group, 28 patients (28.3%) developed a recurrent FRI. In the control group, 19 patients (11.6%) had a recurrent FRI (p = 0.001, 95% CI [0.174 – 0.635]). In the NPWT group there were no significant differences in baseline characteristics between the recurrence and non-recurrence group. The duration of NPWT was associated with a higher risk of recurrence of infection (p = 0.013, OR 1.036, 95% CI [1.008 – 1.066]).ConclusionDelayed wound closure with the application of NPWT increased the risk of recurrence of infection in patients with soft tissue defects after FRI treatment. Therefore, it is advised to consider NPWT only as a short-term (e.g. few days) necessity to bridge the period until definitive wound closure can be established.  相似文献   

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BackgroundMany breast cancer clinical trials with PARPi have been completed or are currently carried out, either by monotherapy or combined with chemotherapy. We aim to assess the efficacy and safety of PARPi in breast cancer patients as compared to chemotherapy.MethodsA comprehensive literature search of PubMed, EMBASE, CENTRAL, conference meetings and clinical trial registry was performed. The primary outcomes were progression-free survival (PFS), overall survival (OS), overall response rate (ORR). The secondary outcome was safety profile. The comparative effects were measured using hazard ratio (HR) or relative risk (RR) with 95% confidence interval. Subgroup analyses were conducted based on types of intervention and baseline characteristics of patients.ResultsSix RCTs (n = 1953) were included. Two RCTs were recognized as high risk. PARPi was associated with an improved PFS (HR, 0.65; 95% CI, 0.56–0.74), OS (HR, 0.86; 95% CI, 0.73–1.01), and a higher ORR (RR, 1.38; 95% CI, 1.05–1.82). PARPi, however, significantly increased risk of grade 3–4 thrombocytopenia (RR, 1.63; 95% CI, 1.06–2.52). Monotherapy was observed with lower risk of disease progression and higher ORR rate than combination therapy, 0.56 to 0.65 and 2.21 to 1.05, respectively. For patients without prior platinum treatment, PARPi significantly improved PFS (HR, 0.64; 95% CI, 0.52–0.79).ConclusionsPARPi was observed with a significantly improved efficacy in aspects of PFS and ORR, but also higher risk of grade 3–4 thrombocytopenia as compared to chemotherapy. PARPi was a better choice for patients who had not received previous platinum treatment.  相似文献   

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BackgroundSevere primary graft failure is a life-threatening complication of heart transplantation that may require venoarterial extracorporeal membrane oxygenation (VA-ECMO) support. Surgical practices and management strategies regarding VA-ECMO vary between and within centers.MethodsWe performed a single-center retrospective cohort study on adult patients who received VA-ECMO for primary graft failure between 2013 and 2020. Clinical data were obtained from chart review and national databases. Patients were stratified by transplantation before or after 2017, when our center adopted additional objective criteria for VA-ECMO, adopted partial-flow support, and changed from central cannulation to chimney graft arterial cannulation of brachiocephalic, axillary, or aorta. The primary outcome was survival to device weaning. Secondary outcomes were survival to discharge, survival to 1 year, complications on support, and time to sedation weaning and extubation.ResultsFrom 276 heart transplant recipients, 39 severe primary graft failure patients requiring VA-ECMO were identified. Incidence of graft failure was 13% (n = 18 of 135) pre-2017 and 15% (n = 21 of 141) post-2017. Survival at all time points improved significantly after 2017, with greatest difference in survival to device weaning (61% pre-2017 vs 100% post-2017). After controlling for other factors in multivariable Cox regression modeling, transplantation after 2017 was a predictor of reduced mortality (hazard ratio, 0.209; 95% CI, 0.06-0.71; P = .01). Significant differences were not observed in other secondary outcomes of recovery.ConclusionsThe new VA-ECMO strategy displayed reasonable survival and a remarkable improvement from the prior system.  相似文献   

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