Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 277
Filtrar
1.
JACC Cardiovasc Interv ; 13(13): 1529-1540, 2020 Jul 13.
Artigo em Inglês | MEDLINE | ID: mdl-32646693

RESUMO

OBJECTIVES: The aim of this analysis was to evaluate outcomes following transcatheter reintervention for degenerated transcatheter pulmonary valves (TPVs). BACKGROUND: TPV replacement (TPVR) with the Melody valve demonstrated sustained relief of right ventricular outflow tract (RVOT) obstruction and pulmonary regurgitation. METHODS: All patients who underwent TPVR with a Melody valve as part of 3 Medtronic-sponsored prospective multicenter studies were included. Transcatheter reinterventions included balloon dilation of the previously implanted Melody valve, placement of a bare-metal stent within the implanted TPV, or placement of a new TPV in the RVOT (TPV-in-TPV). Indications for reintervention, decisions to reintervene, and the method of reintervention were at physician discretion. All patients provided written informed consent to participate in the trials, and each trial was approved by local or central Institutional Review Boards or ethics committees at participating sites. RESULTS: A total of 309 patients who underwent TPVR were discharged from the implantation hospitalization with Melody valves in place. Transcatheter reintervention on the TPV was performed in 46 patients. The first transcatheter reintervention consisted of TPV-in-TPV in 28 patients (median 6.9 years [quartile 1 to quartile 3: 5.2 to 7.8 years] after TPVR), simple balloon dilation of the implanted Melody valve in 17 (median 4.9 years [quartile 1 to quartile 3: 4.0 to 6.0 years] after TPVR), and bare-metal stent placement alone in 1 (4.4 years after TPVR). There were no major procedural complications. Overall, 4-year freedom from explant and from any later RVOT reintervention after the first reintervention were 83% and 60%, respectively. Freedom from repeat RVOT reintervention was longer in patients undergoing TPV-in-TPV than balloon dilation (71% vs. 46% at 4 years; p = 0.027). CONCLUSIONS: TPV-in-TPV can be an effective and durable treatment for Melody valve dysfunction. Although balloon dilation of the Melody valve was also acutely effective at reducing RVOT obstruction, the durability of this therapy was limited in this cohort compared with TPV-in-TPV.

2.
Pediatr Transplant ; 24(5): e13738, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32525246

RESUMO

TDI is considered superior to conventional echocardiography for detecting changes in graft function during rejection in adults but has not demonstrated after pediatric OHT. We retrospectively analyzed echocardiograms performed within 24 hours of biopsy in 122 recipients with median age of 8.7 years. Using biopsy findings as the gold standard, we compared paired rejection and non-rejection echocardiograms using each patient as their own control. We included pairs of LV dimensions, FS, volumes, mass, mass/volume, sphericity, wall stress, SSI, SVI, and TDI velocities in this comparison. C-statistic was used to assess discrimination for individual echo variables and combinations of variables. Overall, 647 non-rejection and 24 rejection biopsy-echo pairs were identified. There was a significant decline in TDI velocities and their Z-scores during rejection but not in conventional variables (P ≤ .005). The variable that best discriminated rejection from non-rejection was LV S', with C-statistic = 0.93. Conventional echo variables performed less well with C-statistic range 0.65-0.67 for LV EF, shortening fraction, and mass. TDI is superior to conventional echocardiography measures for discriminating rejection from non-rejection. The use of newer non-invasive parameters to detect myocardial dysfunction and shifting the paradigm of rejection surveillance to detection of non-rejection together provide a promising approach to reducing the need for biopsy in pediatric heart recipients.

3.
Cardiol Young ; 30(6): 890-891, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32425140

RESUMO

We present a rare case of incidentally diagnosed Twiddler's syndrome in a child 7 years after implantation of a dual-chamber pacemaker system with epicardial leads. During revision, an insulation defect of the ventricular lead was evident, despite unremarkable prior pacemaker lead testing. The lead was repaired and a new generator was suture-fixated to prevent re-occurrence of generator manipulation.

4.
Cardiol Young ; 30(5): 698-709, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32364090

RESUMO

OBJECTIVE: Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality. MATERIAL AND METHODS: We performed a retrospective observational study. From the total cohort of 439 Fontan patients treated in our institution during the study period 1986-2019, 30 patients (6.8%) with protein-losing enteropathy were identified. Perioperative, clinical, echocardiographic, laboratory, and invasive haemodynamic findings and treatment details were analysed. RESULTS: Median follow-up after disease onset was 13.1 years [interquartile range 10.6]. Twenty-five patients received surgical or interventional treatment for haemodynamic restrictions. Medical treatment, predominantly pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide, was initiated in 28 patients. In 15 patients, a stable remission could be achieved by medical or surgical procedures (n = 3 each), by combined multimodal therapy (n = 8), or ultimately by cardiac transplantation (n = 1). Phrenic palsy, bradyarrhythmia, Fontan pathway stenosis, and absence of a fenestration were significantly associated with development of protein-losing enteropathy (p = 0.001-0.48). Ten patients (33.3%) died during follow-up; 5-year survival estimate was 96.1%. In unadjusted analysis, medical therapy with budesonide and pulmonary vasodilator therapy in combination was associated with improved survival. CONCLUSIONS: Protein-losing enteropathy is a serious condition limiting survival after the Fontan procedure. Comprehensive assessment and individual treatment strategies are mandatory to achieve best possible outcome. Nevertheless, relapse is frequent and long-term mortality substantial. Cardiac transplantation should be considered early as treatment option.

5.
J Clin Med ; 9(5)2020 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-32414075

RESUMO

INTRODUCTION: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. OBJECTIVE: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. METHODS AND RESULTS: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients´ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan-Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. CONCLUSIONS: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

6.
J Am Heart Assoc ; 9(10): e015289, 2020 May 18.
Artigo em Inglês | MEDLINE | ID: mdl-32410525

RESUMO

Background Variants of the desmosomal protein desmoplakin are associated with arrhythmogenic cardiomyopathy, an important cause of ventricular arrhythmias in children and young adults. Disease penetrance of desmoplakin variants is incomplete and variant carriers may display noncardiac, dermatologic phenotypes. We describe a novel cardiac phenotype associated with a truncating desmoplakin variant, likely causing mechanical instability of myocardial desmosomes. Methods and Results In 2 young brothers with recurrent myocarditis triggered by physical exercise, screening of 218 cardiomyopathy-related genes identified the heterozygous truncating variant p.Arg1458Ter in desmoplakin. Screening for infections yielded no evidence of viral or nonviral infections. Myosin and troponin I autoantibodies were detected at high titers. Immunohistology failed to detect any residual DSP protein in endomyocardial biopsies, and none of the histologic criteria of arrhythmogenic cardiomyopathy were fulfilled. Cardiac magnetic resonance imaging revealed no features associated with right ventricular arrhythmogenic cardiomyopathy, but multifocal subepicardial late gadolinium enhancement was present in the left ventricles of both brothers. Screening of adult cardiomyopathy cohorts for truncating variants identified the rare genetic variants p.Gln307Ter, p.Tyr1391Ter, and p.Tyr1512Ter, suggesting that over subsequent decades critical genetic/exogenous modifiers drive pathogenesis from desmoplakin truncations toward different end points. Conclusions The described novel phenotype of familial recurrent myocarditis associated with a desmoplakin truncation in adolescents likely represents a serendipitously revealed subtype of arrhythmogenic cardiomyopathy. It may be caused by a distinctive adverse effect of the variant desmoplakin upon the mechanical stability of myocardial desmosomes. Variant screening is advisable to allow early detection of patients with similar phenotypes.

7.
Eur J Cardiothorac Surg ; 57(6): 1038-1050, 2020 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-32364221

RESUMO

OBJECTIVES: A second paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS: Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (≤19 years of age) performed from 1 January 2000 to 1 July 2019 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events including neurological dysfunction, device malfunction, major infection and bleeding. RESULTS: Twenty-nine hospitals contributed 398 registered implants in 353 patients (150 female, 203 male) to the registry. The most frequent aetiology of heart failure was any form of cardiomyopathy (61%), followed by congenital heart disease and myocarditis (16.4% and 16.1%, respectively). Competing outcomes analysis revealed that a total of 80% survived to transplant or recovery or are ongoing; at the 2-year follow-up examination, 20% died while on support. At 12 months, 46.7% received transplants, 8.7% were weaned from their device and 18.5% died. The 3-month adverse events rate was 1.69 per patient-year for device malfunction including pump exchange, 0.48 for major bleeding, 0.64 for major infection and 0.78 for neurological events. CONCLUSIONS: The overall survival rate was 81.5% at 12 months following ventricular assist device implant. The comparison of survival rates of the early and later eras shows no significant difference. A focus on specific subgroups showed that survival was less in patients of younger age (<1 year of age) (P = 0.01) and lower weight (<20 kg) (P = 0.015). Transplant rates at 6 months continue to be low (33.2%) The fact that the EUROMACS registry is embedded within the European Association for Cardio-Thoracic Surgery Quality Improvement Programme offers opportunities to focus on improving outcomes.

8.
Cardiol Young ; 30(6): 769-773, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32340633

RESUMO

BACKGROUND: Thrombocytopenia is a risk factor for patent ductus arteriosus. Immature and mature platelets exhibit distinct haemostatic properties; however, whether platelet maturity plays a role in postnatal, ductus arteriosus closure is unknown. METHODS: In this observational study, counts of immature and mature platelets (=total platelet count - immature platelet count) were assessed on days 1, 3, and 7 of life in very low birth weight infants (<1500 g birth weight). We performed echocardiographic screening for haemodynamically significant patent ductus arteriosus on day 7. RESULTS: Counts of mature platelets did not differ on day 1 in infants with (n = 24) and without (n = 45) haemodynamically significant patent ductus arteriosus, while infants with significant patent ductus arteriosus exhibited lower counts of mature platelet on postnatal days 3 and 7. Relative counts of immature platelets (fraction, in %) were higher in infants with patent ductus arteriosus on day 7 but not on days 1 and 3. Receiver operating characteristic curve analysis unraveled associations between both lower mature platelet counts and higher immature platelet fraction (percentage) values on days 3 and 7, with haemodynamically significant ductus arteriosus. Logistic regression analysis revealed that mature platelet counts, but not immature platelet fraction values, were independent predictors of haemodynamically significant patent ductus arteriosus. CONCLUSION: During the first week of postnatal life, lower counts of mature platelets and higher immature platelet fraction values are associated with haemodynamically significant patent ductus arteriosus. Lower counts of mature platelet were found to be independent predictors of haemodynamically significant patent ductus arteriosus.

9.
Pediatr Crit Care Med ; 21(6): e316-e324, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32343108

RESUMO

OBJECTIVES: Extracorporeal cardiopulmonary resuscitation in children with refractory cardiac arrest has been shown to improve survival, however, risk factors associated with mortality and neurologic impairments are not well defined. We analyzed our recent institutional experience with pediatric extracorporeal cardiopulmonary resuscitation to identify variables associated with survival and neurocognitive outcome. DESIGN: Retrospective observational study. SETTING: Pediatric cardiology and congenital heart surgery departments of a tertiary referral heart center. PATIENTS: Seventy-two consecutive children (median age, 0.3 yr [0.0-1.9 yr]) who underwent extracorporeal cardiopulmonary resuscitation at our institution during the study period from 2005 to 2016. INTERVENTIONS: Not applicable. MEASUREMENTS AND MAIN RESULTS: Median duration of resuscitation was 60 minutes (42-80 min) and median extracorporeal support duration was 5.4 days (2.2-7.9 d). Forty-three (59.7%) extracorporeal cardiopulmonary resuscitation events occurred during off-hours, however, neither duration of resuscitation (65 min [49-89 min] vs 51 min [35-80 min]; p = 0.16) nor survival (34.9% vs 37.9%; p = 0.81) differed significantly compared to working hours. Congenital heart disease was present in 84.7% of the patients. Survival to hospital discharge was 36.1%; younger age, higher lactate levels after resuscitation, acute kidney injury, renal replacement therapy, hepatic injury, and complexity of prior cardiothoracic surgical procedures were significantly associated with mortality. At mid-term follow-up (median, 4.1 yr [3.7-6.1 yr]), 22 patients (84.6% of discharge survivors) were still alive with 77.3% having a favorable neurologic outcome. High lactate levels, arrest location other than ICU, and requirement for renal replacement therapy were associated with unfavorable neurologic outcome. Interestingly, longer duration of resuscitation did not negatively impact survival or neurologic outcome. CONCLUSIONS: Extracorporeal cardiopulmonary resuscitation is a valuable tool for the treatment of children with refractory cardiac arrest and a favorable neurologic outcome can be achieved in the majority of survivors even after prolonged resuscitation. Mortality after extracorporeal cardiopulmonary resuscitation in postcardiac surgery children is associated with procedural complexity.

10.
Cardiol Young ; 30(5): 629-632, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32279698

RESUMO

OBJECTIVES: The aim of our study was to compare post-operative outcome after total cavopulmonary connection between patients operated during winter and summer season. METHODS: We retrospectively studied 211 patients who underwent extracardiac total cavopulmonary connection completion at our institution between 1995 and 2015 (median age 4 (1-42) years). Seventy (33%) patients were operated during winter (November to March) and 141 (67%) patients during summer season (April to October). RESULTS: Patients operated during winter and summer season showed no difference in early mortality (7% versus 5%, p = 0.52) and severe morbidity like need for early Fontan takedown (1% versus 1%, p = 0.99) and need for mechanical circulatory support (9% versus 4%, p = 0.12). The post-operative course and haemodynamic outcome were comparable between both groups of patients (ICU (4 versus 3 days, p = 0.44) and hospital stay (15 versus 14 days, p = 0.28), prolonged pleural effusions (36% versus 31%, p = 0.51), need for dialysis (16% versus 11%, p = 0.37), ascites (37% versus 33%, p = 0.52), supraventricular tachyarrhythmia (16% versus 13%, p = 0.56) and chylothorax (26% versus 16%, p = 0.12), change of antibiotic treatment (47% versus 36%, p = 0.06), prolonged inotropic support (24% versus 14%, p = 0.05), intubation time (15 versus 12 hours, p = 0.33), and incidence of fast-track extubation (11% versus 22%, p = 0.06). CONCLUSION: Outcomes after total cavopulmonary connection completion during winter and summer season were comparably related to mortality, severe morbidity, or longer hospital stay in the early post-operative period. These results suggest that total cavopulmonary connection completion during winter season is as safe as during summer season.

11.
Eur J Cardiothorac Surg ; 58(1): 171-176, 2020 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-32236473

RESUMO

OBJECTIVES: In revised 2018 American Heart Association/American College of Cardiology guideline for the management of adults with congenital heart disease (ACHD), the committee introduced a classification that combines lesion anatomy and physiological status: ACHD anatomic physiological (AP) classification. Anatomy is described as of simple (I), moderate (II) or great (III) complexity, whereas physiology is listed in 4 categories of increasing severity (A, B, C and D). Can this classification predict early postoperative mortality? METHODS: ACHD AP classification was determined for 339 adults who underwent open-heart surgery between September 2012 and August 2018. In addition, the adult congenital heart surgery (ACHS) and Society of Thoracic Surgery-European Association for Cardio-Thoracic Surgery (STAT) mortality scores were calculated. A model based on binary logistic regression was applied. The event was early postoperative death. Mortality scores were estimated for each ACHD AP class. RESULTS: All patients could be categorized by the ACHD AP classification. The 354 procedures were performed with an early mortality of 3.4% (12/354). The mortality risk for the new mortality score, simply called ACAP score, ranged from 0.2% (95% confidence interval 0.08-0.41%) for ACHD AP class IA to 20% (16.04-24.64%) for IIID class. Observed over expected ratios of early mortality amounted to 0.87, 1.54 and 1.14, whereas areas under the curve of receiver operator characteristic were found to be 0.78, 0.64 and 0.88 for STAT, ACHS and ACAP scores, respectively. CONCLUSIONS: ACHD AP classification could embrace all procedures. In our setting, the ACAP score was more predictive of early mortality than the ACHS and STAT mortality scores. It should be validated by further studies and other centres.

13.
BMJ Open ; 10(3): e034853, 2020 03 25.
Artigo em Inglês | MEDLINE | ID: mdl-32213521

RESUMO

OBJECTIVE: We aimed to investigate the combined effects of arterial hypertension, bicuspid aortic valve disease (BAVD) and age on the distensibility of the ascending and descending aortas in patients with aortic coarctation. DESIGN: Cross-sectional study. SETTING: The study was conducted at two university medical centres, located in Berlin and London. PARTICIPANTS: A total of 121 patients with aortic coarctation (ages 1-71 years) underwent cardiac MRI, echocardiography and blood pressure measurements. OUTCOME MEASURES: Cross-sectional diameters of the ascending and descending aortas were assessed to compute aortic area distensibility. Findings were compared with age-specific reference values. The study complied with the Strengthening the Reporting of Observational Studies in Epidemiology statement and reporting guidelines. RESULTS: Impaired distensibility (below fifth percentile) was seen in 37% of all patients with coarctation in the ascending aorta and in 43% in the descending aorta. BAVD (43%) and arterial hypertension (72%) were present across all ages. In patients >10 years distensibility impairment of the ascending aorta was predominantly associated with BAVD (OR 3.1, 95% CI 1.33 to 7.22, p=0.009). Distensibility impairment of the descending aorta was predominantly associated with arterial hypertension (OR 2.8, 95% CI 1.08 to 7.2, p=0.033) and was most pronounced in patients with uncontrolled hypertension despite antihypertensive treatment. CONCLUSION: From early adolescence on, both arterial hypertension and BAVD have a major impact on aortic distensibility. Their specific effects differ in strength and localisation (descending vs ascending aorta). Moreover, adequate blood pressure control is associated with improved distensibility. These findings could contribute to the understanding of cardiovascular complications and the management of patients with aortic coarctation.

14.
Heart ; 106(13): 1007-1014, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32161041

RESUMO

OBJECTIVE: To assess the utility of machine learning algorithms for automatically estimating prognosis in patients with repaired tetralogy of Fallot (ToF) using cardiac magnetic resonance (CMR). METHODS: We included 372 patients with ToF who had undergone CMR imaging as part of a nationwide prospective study. Cine loops were retrieved and subjected to automatic deep learning (DL)-based image analysis, trained on independent, local CMR data, to derive measures of cardiac dimensions and function. This information was combined with established clinical parameters and ECG markers of prognosis. RESULTS: Over a median follow-up period of 10 years, 23 patients experienced an endpoint of death/aborted cardiac arrest or documented ventricular tachycardia (defined as >3 documented consecutive ventricular beats). On univariate Cox analysis, various DL parameters, including right atrial median area (HR 1.11/cm², p=0.003) and right ventricular long-axis strain (HR 0.80/%, p=0.009) emerged as significant predictors of outcome. DL parameters were related to adverse outcome independently of left and right ventricular ejection fraction and peak oxygen uptake (p<0.05 for all). A composite score of enlarged right atrial area and depressed right ventricular longitudinal function identified a ToF subgroup at significantly increased risk of adverse outcome (HR 2.1/unit, p=0.007). CONCLUSIONS: We present data on the utility of machine learning algorithms trained on external imaging datasets to automatically estimate prognosis in patients with ToF. Due to the automated analysis process these two-dimensional-based algorithms may serve as surrogates for labour-intensive manually attained imaging parameters in patients with ToF.

15.
Pediatr Cardiol ; 41(4): 736-746, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32006084

RESUMO

Fontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). In this study, we performed a detailed hemodynamic and hepatic assessment to analyze the incidence and spectrum of FALD and its association with patients' hemodynamics. From 2017 to 2019, 145 patients underwent a detailed, age-adjusted hepatic examination including laboratory analysis (FibroTest®, n = 101), liver ultrasound (n = 117) and transient elastography (FibroScan®, n = 61). The median patient age was 16.0 years [IQR 14.2], and the median duration of the Fontan circulation was 10.3 years [IQR 14.7]. Hemodynamic assessment was performed using echocardiography, cardiopulmonary exercise capacity testing and cardiac catheterization. Liver ultrasound revealed hepatic parenchymal changes in 83 patients (70.9%). Severe liver cirrhosis was detectable in 20 patients (17.1%). Median liver stiffness measured by FibroScan® was 27.7 kPa [IQR 14.5], and the median Fibrotest® score was 0.5 [IQR 0.3], corresponding to fibrosis stage ≥ 2. Liver stiffness values and Fibrotest® scores correlated significantly with Fontan duration (P1 = 0.013, P2 = 0.012). Exercise performance was significantly impaired in patients with severe liver cirrhosis (P = 0.003). Pulmonary artery pressure and end-diastolic pressure were highly elevated in cirrhotic patients (P1 = 0.008, P2 = 0.003). Multivariable risk factor analysis revealed Fontan duration to be a major risk factor for the development of FALD (P < 0.001, OR 0.77, CI 0.68-0.87). In the majority of patients, hepatic abnormalities suggestive of FALD were detectable by liver ultrasound, transient elastography and laboratory analysis. The severity of FALD correlated significantly with Fontan duration and impaired Fontan hemodynamics. A detailed hepatic assessment is indispensable for long-term surveillance of Fontan patients.


Assuntos
Técnica de Fontan/efeitos adversos , Cirrose Hepática/etiologia , Adulto , Cateterismo Cardíaco/efeitos adversos , Pré-Escolar , Estudos Transversais , Ecocardiografia , Técnicas de Imagem por Elasticidade , Feminino , Hemodinâmica , Humanos , Cirrose Hepática/diagnóstico por imagem , Cirrose Hepática/patologia , Masculino , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Ultrassonografia
16.
ACS Nano ; 2020 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-31999430

RESUMO

Solution-processed networks of semiconducting, single-walled carbon nanotubes (SWCNTs) have attracted considerable attention as materials for next-generation electronic devices and circuits. However, the impact of the SWCNT network composition on charge transport on a microscopic level remains an open and complex question. Here, we use charge-modulated absorption and photoluminescence spectroscopy to probe exclusively the mobile charge carriers in monochiral (6,5) and mixed SWCNT network field-effect transistors. Ground-state bleaching and charge-induced trion absorption features as well as exciton quenching are observed depending on applied voltage and modulation frequency. Through correlation of the modulated mobile carrier density and the optical response of the nanotubes, we find that charge transport in mixed SWCNT networks depends strongly on the diameter and thus bandgap of the individual species. Mobile charges are preferentially transported by small bandgap SWCNTs especially at low gate voltages, whereas large bandgap species only start to participate at higher carrier concentrations. Our results demonstrate the excellent suitability of modulation spectroscopy to investigate charge transport in nanotube network transistors and highlight the importance of SWCNT network composition for their performance.

17.
Ther Hypothermia Temp Manag ; 10(1): 60-70, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30973305

RESUMO

Therapeutic hypothermia during cardiac surgery has been widely used for neuroprotection and to attenuate the systemic inflammatory response due to cardiopulmonary bypass (CPB). Experimental data suggest that cold-shock protein RNA-binding motif 3 (RBM3), which is induced in response to hypothermia, plays a key role in hypothermia-induced organ protection. To date, investigation on RBM3 has been performed exclusively in vitro or in animal models, and the detection and regulation of RBM3 in human blood has not been investigated until now. The aim of this study was to investigate the level of RBM3 protein and cytokine expression profile involved in the inflammatory response in patients with congenital heart disease undergoing cardiac surgery involving CPB and therapeutic hypothermia. A single-center prospective trial with 23 patients undergoing cardiac surgery with CPB was performed. RBM3 protein was quantified in blood serum samples collected from patients and healthy individuals employing a new developed enzyme-linked immunosorbent assay. Cytokine levels were analyzed from dry blood spot samples using a Quanterix Simoa Immunoassay. For the first time, RBM3 protein was detected in blood samples of patients with congenital heart disease undergoing cardiac surgery. Hereby, RBM3 protein concentrations were significantly elevated in patients after cardiac surgery with CPB and mild hypothermia as compared with pre-surgery levels. Moreover, a complex immune reaction with significant induction of pro-inflammatory cytokines (interleukin [IL]-1 beta, IL-6, IL-8, IL-16, IL-18, monocyte chemotactic protein 1, CC-chemokine ligand [CCL]3, CCL4, intercellular adhesion molecule-1) in response to CPB was detected. Significantly elevated vascular endothelial growth factor and matrix metallopeptidase 3 concentrations reflecting ischemia/reperfusion-induced injury were observed 24 hours after weaning from CPB. The use of CPB is still associated with a complex inflammatory response. RBM3 protein is measurable in blood samples of patients with significantly higher concentrations after cardiac surgery with CPB and mild-to-moderate hypothermia. RBM3 is a new candidate as a biomarker for therapeutic hypothermia and a possible new therapeutic target for organ protection.

18.
Int J Cardiol ; 303: 36-40, 2020 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-31611088

RESUMO

BACKGROUND: Endomyocardial biopsies (EMB) are the gold standard for the diagnosis of myocarditis in children and adults. The existing WHO/ISFC criteria for lymphocytic cell infiltrates by are based on the myocardium of adults. The aim of this study was to present a paediatric control cohort for the evaluation of inflammation in EMB of children. METHODS: In this study endomyocardial tissue from 62 children under 4 years of age was investigated, being collected during a planned open heart surgery with routine resection from ventricular site. Patients had no history of infection or myocardial inflammation. The heart tissue was formalin fixed and embedded in paraffin. Four µm thick tissue sections were stained with haematoxylin and eosin, Masson's trichrome, and Giemsa. Immunohistochemical stainings included quantitative evaluation of CD3+ T cells, CD20+ B cells, CD68+ macrophages and MHCII expression. RESULTS: The myocardium was obtained in 96.8% (n = 60) of the cases from the right and in 3.2% (n = 2) from the left ventricle. The median age (interquartile range) at biopsy was 0.5 years (0.3-0.9), 66.1% male. Within this cohort, a median of 2.5/mm2 (1.0-4.0) CD3+ T cells, 0.5/mm2 (0.0-0.6) CD20+ B cells and 4.0/mm2 (2.5-6.0) CD68+ macrophages were detected. The MHC II grade was 0 in 71.0% (n = 44) and 1 in 29.0% (n = 18). CONCLUSION: This is the first paediatric control cohort being relevant for the correct interpretation of inflammatory heart diseases in EMB. The lymphocytic cell numbers in children needing congenital heart surgery without myocardial inflammation are below the existing values in adults.

19.
Eur J Cardiothorac Surg ; 57(1): 133-141, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31131388

RESUMO

OBJECTIVES: Complex blood flow profiles in the aorta are known to contribute to vessel dilatation. We studied flow profiles in the aorta in patients with aortic valve disease before and after surgical aortic valve replacement (AVR). METHODS: Thirty-four patients with aortic valve disease underwent 4-dimensional velocity-encoded magnetic resonance imaging before and after AVR (biological valve = 27, mechanical valve = 7). Seven healthy volunteers served as controls. Eccentricity (ES) and complex flow scores (CFS) were determined from the degree of helicity, vorticity and eccentricity of flow profiles in the aorta. Model-based therapy planning was used in 4 cases to improve in silico postoperative flow profiles by personalized adjustment of size, rotation and angulation of the valve as well as aorta diameter. RESULTS: Patients with aortic valve disease showed more complex flow than controls [median ES 2.5 (interquartile range (IQR) 2.3-2.7) vs 1.0 (IQR 1.0-1.0), P < 0.001, median CFS 4.7 (IQR 4.3-4.8) vs 1.0 (IQR 1.0-2.0), P < 0.001]. After surgery, flow complexity in the total patient cohort was reduced, but remained significantly higher compared to controls [median ES 2.3 (IQR 1.9-2.3) vs 1.0 (IQR 1.0-1.0), P < 0.001, median CFS 3.8 (IQR 3.0-4.3) vs 1.0 (IQR 1.0-2.0), P < 0.001]. In patients after mechanical AVR, flow complexity fell substantially and showed no difference from controls [median ES 1.0 (IQR 1.0-2.3) vs 1.0 (IQR 1.0-1.0), P = 0.46, median CFS 1.0 (IQR 1.0-3.3) vs 1.0 (IQR 1.0-2.0), P = 0.71]. In all 4 selected cases (biological, n = 2; mechanical, n = 2), model-based therapy planning reduced in silico complexity of flow profiles compared to the existing post-surgical findings [median ES 1.7 (IQR 1.4-1.7) vs 2.3 (IQR 2.3-2.3); CFS 1.7 (IQR 1.4-2.5) vs 3.8 (IQR 3.3-4.3)]. CONCLUSIONS: Abnormal flow profiles in the aorta more frequently persist after surgical AVR. Model-based therapy planning might have the potential to optimize treatment for best possible individual outcome. CLINICAL TRIAL REGISTRATION NUMBER: clinicaltrials.gov NCT03172338, 1 June 2017, retrospectively registered; NCT02591940, 30 October 2015, retrospectively registered.

20.
Sci Rep ; 9(1): 19063, 2019 Dec 13.
Artigo em Inglês | MEDLINE | ID: mdl-31836860

RESUMO

MicroRNAs (miRNAs) play an important role in guiding development and maintaining function of the human heart. Dysregulation of miRNAs has been linked to various congenital heart diseases including Tetralogy of Fallot (TOF), which represents the most common cyanotic heart malformation in humans. Several studies have identified dysregulated miRNAs in right ventricular (RV) tissues of TOF patients. In this study, we profiled genome-wide the whole transcriptome and analyzed the relationship of miRNAs and mRNAs of RV tissues of a homogeneous group of 22 non-syndromic TOF patients. Observed profiles were compared to profiles obtained from right and left ventricular tissue of normal hearts. To reduce the commonly observed large list of predicted target genes of dysregulated miRNAs, we applied a stringent target prediction pipeline integrating probabilities for miRNA-mRNA interaction. The final list of disease-related miRNA-mRNA pairs comprises novel as well as known miRNAs including miR-1 and miR-133, which are essential to cardiac development and function by regulating KCNJ2, FBN2, SLC38A3 and TNNI1. Overall, our study provides additional insights into post-transcriptional gene regulation of malformed hearts of TOF patients.

SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA