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1.
Heart Vessels ; 38(4): 570-580, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36305895

RESUMO

Evaluating the relation of non-cardiac comorbidity and socio-demographic factors to physical and mental health-related quality of life (QOL) which has been partially found at elevated risk in young adults after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA). In a prospective reassessment study, results of 92 unselected young adults (22.8 ± 2.6 years) having undergone evaluation of QOL (SF-36) were related to non-cardiac comorbidity with special respect to neurologic and psychiatric comorbidity and to socio-demographic parameters. Neurologic (14%) contrary to psychiatric comorbidities (6.5%) were more frequent than in the general population. The educational level was higher, the rate of unemployment was double as high compared to the average German population. Significant inverse relations (p = 0.006 to 0.033) existed between physical health domains (physical functioning and general health perception) and non-cardiac, neurologic, and psychiatric comorbidity, as well as correlations between the latter domains and socio-economic status, educational level, and worse employment status (Spearman 0.22-0.41, p < 0.0001 to 0.036). Mental health domains (vitality, social functioning, psychical health) were significantly inversely related with neurologic and psychiatric comorbidity (p = 0.002 to 0.048) and correlated with higher educational level (Spearman 0.25, p = 0.019). Neurologic and psychiatric comorbidities and socio-demographic parameters are significant risk factors for a reduced QOL concerning physical and mental health in young adults with TGA after ASO. Standardized QOL measurement should be part of routine screening programs to detect subclinical physical, neurodevelopmental, and psychosocial comorbidity.


Assuntos
Transposição das Grandes Artérias , Transposição dos Grandes Vasos , Recém-Nascido , Humanos , Adulto Jovem , Transposição das Grandes Artérias/efeitos adversos , Transposição dos Grandes Vasos/cirurgia , Qualidade de Vida , Estudos Prospectivos , Comorbidade , Artérias
2.
Cardiol Young ; 33(2): 227-234, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-35193726

RESUMO

OBJECTIVE: The aim of this study was to evaluate the association of bicuspid aortic valve on contemporary outcomes, including reoperation rates, after one-stage correction for interrupted aortic arch with ventricular septal defect or for aortic coarctation with hypoplastic aortic arch and ventricular septal defect. METHODS: Seventy-four consecutive patients (35 boys, 47% and 39 girls, 53%) with interrupted aortic arch (n = 41, 55%) or aortic coarctation with hypoplastic aortic arch (n = 33, 45%) with ventricular septal defect underwent early one-stage correction. Twenty (27%) patients had bicuspid aortic valve, and the remaining 54 (73%) had a tricuspid aortic valve. The median aortic valve annulus diameter was 6.0 mm (IQR: 2.0). Patients' median age was 7 ± 29 days (range, 2-150); median weight was 3.3 ± 0.7 kg (range, 1.5-6.0), with 21 (28%) patients <3.0 kg. Selective brain perfusion through the innominate artery and selective coronary perfusion through the aortic root during aortic arch reconstruction were used in all patients. Statistical analysis was performed using SPSS version 20.0 software (SPSS Inc., Chicago, IL, USA). RESULTS: The early mortality was 1.3%. One premature neonate died in the hospital with extracorporeal membrane oxygenation after aortic coarctation plus ventricular septal defect repair. There was no further mortality. Median follow-up was 5.7 years (IQR: 10.48). Reinterventions occurred in 36 (49%) patients: balloon angioplasty in 18 (24%) patients, reoperations in 4 (5%) patients, and both in 14 (19%) patients. A total of 86 follow-up procedures were required in these 36 (49%) patients: aortic valve valvulopasty (n = 6, 8%), stent implantation (n = 8, 11%), balloon dilatation (n = 39, 53%), and reoperation (n = 33, 45%). The median time to reinterventions was 9.094 years (SE 0.890). A potential risk factor for reintervention after interrupted aortic arch and aortic coarctation with ventricular septal defect repair was bicuspid aortic valve (p = 0.019, Chi2 (1) = 5.457). In addition, a multivariate Cox analysis with backward selection and significance level <0.015 was applied to all variables that showed significant effects in univariable analyzes. This regression confirmed that bicuspid aortic valve (HR = 0.381, p = .016), and interrupted aortic arch (HR = 0.412, p = 0.043) were predictors of late reintervention. All patients had no obvious neurologic impairment in routine examinations at last follow-up. CONCLUSION: Bicuspid aortic valve was a significant risk factor for valve-related reintervention after one-stage repair for aortic arch obstruction with ventricular septal defect due to later development of stenosis associated with higher late morbidity and mortality. Particularly neonates with bicuspid aortic valve will possibly require reintervention in the future. Regular lifelong cardiac follow-up is recommended.


Assuntos
Coartação Aórtica , Doenças da Aorta , Doença da Válvula Aórtica Bicúspide , Comunicação Interventricular , Recém-Nascido , Masculino , Feminino , Humanos , Lactente , Coartação Aórtica/complicações , Coartação Aórtica/cirurgia , Aorta Torácica/cirurgia , Comunicação Interventricular/complicações , Comunicação Interventricular/cirurgia , Aorta , Doenças da Aorta/cirurgia , Reoperação , Resultado do Tratamento , Estudos Retrospectivos
4.
Mol Cell Pediatr ; 9(1): 5, 2022 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-35307783

RESUMO

BACKGROUND: To examine whether uni-ventricular palliation (UVP) and bi-ventricular repair (BVR) result in a different pattern of systemic inflammatory response to pediatric cardiac surgery with extra-corporeal circulation (ECC). METHODS: In 20 children (median age 39.5 months) undergoing either UVP (n = 12) or BVR (n = 8), plasma levels of the inflammatory cytokines TNF-α, IL-6, IL-10, and IL-12 and of procalcitonin (PCT), were measured before, during and after open cardiac surgery up to postoperative day (POD) 10. RESULTS: Epidemiologic, operative- and outcome variables were similar in both groups but post-operative central venous pressure that was higher in UVP. In the whole cohort, the inflammatory response was characterized by an early important, significant and parallel increase of IL-6 and IL-10 that reached their peak values either at the end of ECC (IL-10) or 4 h postoperatively (IL-6), respectively and by a significant and parallel decrease of TNF-α and IL-12 levels after connection to ECC, followed by a bi-phasic significant increase with a first peak 4 h after ECC and a second at POD 10, respectively. Patients after UVP showed a shift of the cytokine balance with lower IL-6- (p = 0.01) after connection to ECC, lower early post-operative TNF-α - (p = 0.02) and IL-12- (p = 0.04) concentrations and lower TNF-α/IL-10-ratio (p = 0.03) as compared with patients with BVR. Levels of PCT were similar in both groups. CONCLUSIONS: UVP is associated with an anti-inflammatory shift of the inflammatory response to cardiac surgery that might be related to the particular hemodynamic situation of patients with UVP.

5.
Thorac Cardiovasc Surg ; 69(S 03): e68-e75, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34953470

RESUMO

OBJECTIVES: Patients with repaired complete atrioventricular septal defect (CAVSD) represent an increasing portion of grown-ups with congenital heart disease. For repair of CAVSD, the single-patch technique has been employed first. This technique requires division of the bridging leaflets, thus, among other issues, long-term function of the atrioventricular valves is of particular concern. METHODS: Between 1978 and 2001, 100 consecutive patients with isolated CAVSD underwent single-patch repair in our institution. Hospital mortality was 11%. Primary endpoints were clinical status, atrioventricular valve function, and freedom from reoperation in long term. Follow-up was obtained contacting the patient and/or caregiver, and the referring cardiologist. RESULTS: Eighty-three patients were eligible for long-term follow-up (21.0 ± 8.7, mean ± standard deviation [21.5; 2.1-40.0, median; min-max] years after surgical repair). Actual long-term mortality was 3.4%. Quality of life (QoL; self- or caregiver-reported in patients with Down syndrome) was excellent or good in 81%, mild congestive heart failure was present in 16%, moderate in 3.6% as estimated by New York Heart Association classification. Echocardiography revealed normal systolic left ventricular function in all cases. Regurgitation of the right atrioventricular valve was mild in 48%, mild-moderate in 3.6%, and moderate in 1.2%. The left atrioventricular valve was mildly stenotic in 15% and mild to moderately stenotic in 2%; regurgitation was mild in 54%, mild to moderate in 13%, and moderate in 15% of patients. Freedom from left atrioventricular-valve-related reoperation was 95.3, 92.7, and 89.3% after 5, 10, and 30 years, respectively. Permanent pacemaker therapy, as an immediate result of CAVSD repair (n = 7) or as a result of late-onset sick sinus syndrome (n = 5), required up to six reoperations in single patients. Freedom from pacemaker-related reoperation was 91.4, 84.4, and 51.5% after 5, 10, and 30 years, respectively. CONCLUSION: Up to 40 years after single-patch repair of CAVSD, clinical status and functional results are promising, particularly, in terms of atrioventricular valve function. Permanent pacemaker therapy results in a life-long need for surgical reinterventions.


Assuntos
Defeitos dos Septos Cardíacos , Doenças das Valvas Cardíacas , Adulto , Criança , Seguimentos , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/cirurgia , Doenças das Valvas Cardíacas/cirurgia , Humanos , Lactente , Qualidade de Vida , Reoperação , Estudos Retrospectivos , Resultado do Tratamento
6.
J Clin Med ; 10(22)2021 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-34830578

RESUMO

Aortopathy is a known complication whose incidence is growing within the population of tetralogy of Fallot (TOF) patients. Its pathology and relationship with other comorbidities remain unclear. This study was designed to determine the prevalence and predictors of proximal aortic dilatation after TOF repair. We retrospectively investigated all patients who underwent follow-up cardiac magnetic resonance imaging (CMR; at least 4 years after TOF repair) between March 2004 and December 2019. The dimensions at the ascending aorta (AAo) and sinus of Valsalva (SoV) levels were measured. Aortic dilatation was defined as an internal aortic diameter that was >2 standard deviation of the previously published normal values. We included 77 patients (mean age 28.9 ± 10.5 years, 41.5% female, mean follow-up of 24.5 ± 8.1 years). AAo and SoV were dilated in 19 (24.6%) and 43 (55.8%) patients, respectively. Patients with dilated AAo and SoV were older during the corrective surgery (p < 0.001 and p = 0.004, respectively) and during CMR (p = 0.002 and 0.024, respectively) than patients without AAo and SoV dilatation. Patients of the dilated AAo group were more likely to have prior palliative shunt (p = 0.008), longer shunt duration (p = 0.005), and a higher degree of aortic valve regurgitation (AR) fraction (p < 0.001) and to undergo pulmonary (PVR) and/or aortic valve replacement (p < 0.001 and p = 0.013, respectively). PVR (p = 0.048, odds ratio = 6.413, and 95% CI = 1.013-40.619) and higher AR fraction (p = 0.031, odds ratio = 1.194, and 95% CI = 1.017-1.403) were independent predictors for AAo dilatation. Aortopathy is a common progressive complication that may require reintervention and lifelong follow-up. Our study shows that proximal aortic dilatation may be attributed to factors that increase the volume overload across the proximal aorta, including late corrective surgery and palliative shunt. We also found that PVR and higher AR fraction are independent predictors of AAo dilatation.

7.
Front Pediatr ; 9: 729198, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34631625

RESUMO

Background: Estrogen receptors (ERs) relate to cardio-protection in adults, but their role in younger patients is not known. We aimed to assess the myocardial expression of ERα- and ERß- mRNA in young patients with congenital cardiac disease and to analyze their putative protective role. Patients and Methods: Twenty children and young adults (seven females and 13 males) with a median age of 13.8 years (interquartile range: 12.3 years) were enrolled in this prospective study. The myocardial expression of ER-mRNA and genes involved in inflammation, growth, and stress response was assessed by real-time PCR and was correlated to post-operative (po) outcome. Results: ER-mRNA was detected in the myocardium of all patients, independently of gender and age. The expression of ER-mRNA correlated with that of mRNA coding for brain natriuretic peptide and for all cytokines tested. A higher ERα-mRNA expression correlated with lower troponin T concentrations at 24 h po (p = 0.032), higher PaO2/FiO2 ratio at 4 h po (p = 0.059), lower fluid retention at 4 h po (p = 0.048), and lower aspartate aminotransferase (AST) levels at 24 h po (p = 0.047). A higher ERß-mRNA expression was also correlated with lower fluid retention at 24 h po (p = 0.048). Patients in whom the levels of ERα- and ERß-mRNA were >P50 had lower troponin T (p = 0.003, respectively) and lower AST concentrations at 24 h po (p = 0.043, respectively) than the others. Conclusions: The expression of ERα- and ERß-mRNA is present in the myocardium of children and young adults with congenital cardiac defect and is associated with lower markers of po organ damage. This suggests that ERs may provide perioperative organ protection in this population.

8.
Mol Genet Metab Rep ; 25: 100673, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33209585

RESUMO

Variants in Phosphomannomutase 2 (PMM2) lead to PMM2-CDG, the most frequent congenital disorder of glycosylation (CDG). We here describe the disease course of a ten-month old patient who presented with the classical PMM2-CDG symptoms as cerebellar hypoplasia, retinitis pigmentosa, seizures, short stature, hepato- and splenomegaly, anaemia, recurrent vomiting and inverted mamillae. A severe form of tetralogy of Fallot was diagnosed and corrective surgery was performed at the age of 10 months. At the end of the cardiopulmonary bypass, a sudden oedematous reaction of the myocardium accompanied by biventricular pump failure was observed immediately after heparin antagonization with protamine sulfate. The patient died seven days after surgery, since myocardial function did not recover on ECMO support. We here describe the first patient carrying the homozygous variant g.18313A > T in the PMM2 gene (NG_009209.1) that either can lead to c.394A > T (p.I132F) or even loss of 100 bp due to exon 5 skipping (c.348_447del; p.G117Rfs*4) which is comparable to a null allele. Proliferation and doubling time of the patient's fibroblasts were affected. In addition, we show that the induction of cellular stress by elevating the cell culture temperature to 40 °C led to a decrease of the patients' PMM2 transcript as well as PMM2 protein levels and subsequently to a significant loss of residual activity. We assume that metabolic stressful processes occurring after cardiac surgery led to the drop of the patient's PMM activity below a life-sustaining niveau which paved the way for the fatal outcome.

9.
Front Pediatr ; 8: 40, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32117843

RESUMO

Introduction: Myocardial remodeling due to large atrial septum defect (ASD) is macroscopically characterized by dilation of the right-sided cardiac cavities secondary to volume overload, the cellular mechanisms of which are not yet understood. We postulated that inflammation, fibrosis, and cell death are actors of right atrial remodeling secondary to ASD. Patients and Methods: In 12 children with large ASD (median age: 63 months), expression of genes coding for proteins involved in the response to cell stress and -protection, inflammation, growth and angiogenesis, fibrosis, and apoptosis was assessed by RT-PCR in right atrial myocardial biopsies taken during cardiac surgery. The presence of cytokines in myocardial cells was confirmed by immunohistochemistry and effective apoptosis by TUNEL assay. Results: In all patients investigated, a cellular response to early mechanical stress with the initiation of early protective mechanisms, of inflammation (and its control), -growth, and -angiogenesis, of fibrosis and apoptosis was present. The apoptotic index assessed by TUNEL assay averaged 0.3%. Conclusions: In children with large ASD, macroscopic right atrial remodeling relates to cellular mechanisms involving the expression of numerous genes that either still act to protect cells and tissues but that also harm as they initiate and/or sustain inflammation, fibrosis, and cell death by apoptosis. This may contribute to long term morbidity in patients with ASD.

10.
J Biomed Mater Res B Appl Biomater ; 108(4): 1198-1212, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31408584

RESUMO

Tissue-engineered constructs have great potential in many intervention strategies. In order for these constructs to function optimally, they should ideally mimic the cellular alignment and orientation found in the tissues to be treated. Here we present a simple and reproducible method for the production of cell-laden pure fibrin micro-fibers with longitudinal topography. The micro-fibers were produced using a molding technique and longitudinal topography was induced by a single initial stretch. Using this method, fibers up to 1 m in length and with diameters of 0.2-3 mm could be produced. The micro-fibers were generated with embedded endothelial cells, smooth muscle cell/fibroblasts or Schwann cells. Polarized light and scanning electron microscopy imaging showed that the initial stretch was sufficient to induce longitudinal topography in the fibrin gel. Cells in the unstretched control micro-fibers elongated randomly in both the floating and encapsulated environments, whereas the cells in the stretched micro-fibers responded to the introduced topography by adopting a similar orientation. Proof of concept bottom-up tissue engineering (TE) constructs are shown, all displaying various anisotropic organization of cells within. This simple, economical, versatile and scalable approach for the production of highly orientated and cell-laden micro-fibers is easily transferrable to any TE laboratory.


Assuntos
Fibrina/química , Fibroblastos/metabolismo , Células Endoteliais da Veia Umbilical Humana/metabolismo , Miócitos de Músculo Liso/metabolismo , Células de Schwann/metabolismo , Alicerces Teciduais/química , Humanos , Engenharia Tecidual
11.
Interact Cardiovasc Thorac Surg ; 25(4): 589-594, 2017 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-28605441

RESUMO

OBJECTIVES: Aortopexy is widely used; however, many surgeons still regard it with suspicion. To date, there are only a few large series and minimal long-term data. Against this background, our goal was to report our experience, particularly with regard to the recent expansion of indications and modification of diagnostic routine and surgical strategy. METHODS: Between 1994 and 2012, 53 patients (mean age: 1.1 years; 5 weeks-10.2 years) were operated on. Tracheomalacia after the operation for oesophageal atresia was the main indication for aortopexy (74%), followed by tracheal compression by the innominate artery (17%) and other selected indications (9%). Computed tomography angiography has emerged in recent years as the method of choice for preoperative diagnosis. Median sternotomy has replaced lateral thoracotomy, and intraoperative bronchoscopy has become the standard. RESULTS: In contrast to magnetic resonance imaging, computed tomography angiography resulted in perfect visualization of the condition. Median sternotomy and simultaneous bronchoscopy led to immediate readjustment of the aortopexy sutures in 6 patients. There were no surgical deaths or serious morbidities. The mean follow-up was 4.9 (0.3-14.9) years. Two patients with additional complex diseases died during the follow-up period. In all survivors, symptoms improved markedly or disappeared. However, we observed an increased susceptibility to lower respiratory tract infections over the long-term (32%). CONCLUSIONS: Aortopexy is effective for treating tracheomalacia of different origins and other pathological conditions as well. Preoperative computed tomography angiography offers excellent visualization of the condition. Median sternotomy and intraoperative bronchoscopy provide a combination for reliable operative and long-term results. However, many patients still have an increased susceptibility to lower respiratory tract infections.


Assuntos
Obstrução das Vias Respiratórias/etiologia , Aorta Torácica/cirurgia , Atresia Esofágica/complicações , Técnicas de Sutura , Traqueia/diagnóstico por imagem , Traqueomalácia/complicações , Procedimentos Cirúrgicos Vasculares/métodos , Obstrução das Vias Respiratórias/diagnóstico , Obstrução das Vias Respiratórias/cirurgia , Broncoscopia/métodos , Criança , Pré-Escolar , Angiografia por Tomografia Computadorizada , Feminino , Seguimentos , Previsões , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Traqueomalácia/diagnóstico , Traqueomalácia/cirurgia
12.
Thorac Cardiovasc Surg ; 65(1): 31-35, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27177266

RESUMO

Background Understanding the anatomy and physiology of congenital heart defects is crucial for planning interventions in these patients. Congenital heart procedures often involve complex three-dimensional (3D) reconstructions. Excellent imaging techniques are required to depict all anatomical details. We have used and evaluated fast 3D prototyping technology for reconstruction and planning of corrections of complex congenital heart defects. Materials and Methods 3D physical models were constructed from contrast-enhanced computed tomography (CT) datasets of patients with complex congenital heart defect. Two different commercially available printing technologies were used and their clinical application compared. Results Physical models of three different patients were used for preoperative surgical planning. All models showed good correspondence to patient anatomy. Both printing technologies gave excellent results. Conclusion Physical models could be easily constructed with the use of CT datasets. The printing process could be done efficiently, quite rapidly, and cost effectively. Surgical corrections could be planned based on these models.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Modelos Anatômicos , Modelos Cardiovasculares , Modelagem Computacional Específica para o Paciente , Impressão Tridimensional , Interpretação de Imagem Radiográfica Assistida por Computador , Tomografia Computadorizada por Raios X , Humanos , Lactente , Recém-Nascido , Masculino , Valor Preditivo dos Testes
13.
Int J Artif Organs ; 38(10): 548-56, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26541279

RESUMO

PURPOSE: Implanting the largest valved conduit possible - oversizing - to reconstruct an absent connection from the right ventricle to the pulmonary artery in certain types of congenital heart defects has been suggested as a compensating measure for somatic outgrowth of the patient. However, one effect that has not been investigated yet is the hemodynamic consequence. For this purpose, virtual implantation and flow simulations were conducted in this study. METHODS: To isolate the effects of conduit oversizing on the hemodynamics observed after conduit implantation and outgrowth, calculated wall shear stresses (WSS) of image-based computational fluid dynamic (CFD) simulations were used as indicators. Three different sizes of valved conduits (20 mm, 22 mm, and 24 mm), including the largest possible conduit size, virtually implanted in a child-sized healthy pulmonary artery and the corresponding adult-sized model were investigated. RESULTS: The child and adult models show a decrease of the mean WSS (approx. 26%) in the whole domain with an increase of the conduit size. When looking at the mean WSS at the anastomosis, for the child model the WSS is significantly increased (approx. 40%) when oversizing (Z-score +3.21). In contrast, the stresses are decreased for the adult model (34%) when using the largest conduit (Z-score +0.25). CONCLUSIONS: Based on the results of this study, it must be considered that choosing a prosthesis size that will lead to high WSS and an associated intimal reaction, possibly leading to stenosis, can defeat the benefit of having a nominally larger orifice area directly after implantation.


Assuntos
Cardiopatias Congênitas/cirurgia , Implante de Prótese de Valva Cardíaca/métodos , Próteses Valvulares Cardíacas , Hemodinâmica/fisiologia , Modelos Cardiovasculares , Valva Pulmonar/cirurgia , Adolescente , Bioprótese , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino
14.
Future Cardiol ; 11(3): 297-307, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-26021636

RESUMO

BACKGROUND: Right ventricular remodeling due to pulmonary stenosis increases morbidity in children. Its pathophysiology needs to be clarified. METHODS: Six newborn lambs underwent pulmonary arterial banding, seven sham operation. mRNA encoding for cytokines, growth factors and regulators of apoptosis was sequentially measured in myocardium and blood before and up to 12 weeks postoperatively. RESULTS: Experimental animals showed hypertrophy and fibrosis of the right ventricular myocardium, myocardial over-expression of CT-1-mRNA and higher blood concentrations of mRNA encoding for VEGF, TGF-ß, Bak and BcL-xL than controls, respectively. CONCLUSION: Neonatal pulmonary stenosis leads to myocardial hypertrophy that is associated with CT-1 gene expression and with activation of growth- and apoptosis pathways in blood cells.


Assuntos
Apoptose/genética , Citocinas/genética , DNA/genética , Regulação da Expressão Gênica no Desenvolvimento , Peptídeos e Proteínas de Sinalização Intercelular/genética , Miocárdio/patologia , Estenose da Valva Pulmonar/genética , Animais , Animais Recém-Nascidos , Citocinas/biossíntese , Modelos Animais de Doenças , Peptídeos e Proteínas de Sinalização Intercelular/biossíntese , Microscopia Eletrônica , Miocárdio/metabolismo , Estenose da Valva Pulmonar/metabolismo , Estenose da Valva Pulmonar/patologia , Reação em Cadeia da Polimerase em Tempo Real , Ovinos
15.
Ann Thorac Surg ; 97(3): 945-9, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24480260

RESUMO

BACKGROUND: Bicarotid trunk is the most common variation of aortic arch branching patterns. Bicarotid trunk can be a decisive factor in the surgical management of congenital heart defects. Our objective was to determine the prevalence of the bicarotid trunk among pediatric patients with congenital heart defects and to identify whether there is an association between bicarotid trunk and aortic coarctation. METHODS: From a total of 4,371 pediatric cardiac catheterizations between the years 1979 and 2010, a group of 2,033 patients were selected. Prevalence of the aortic coarctation in patients with bicarotid trunk and with a normal branching pattern was determined by evaluating the catheterization reports. In addition, associated congenital defects, vascular anomalies, and genetic syndromes were also assessed. RESULTS: Of 2,033 patients in our database, 84.8% of patients had a normal branching pattern of the aortic arch and 15.2% had a bicarotid trunk. The most frequent congenital heart defects in patients with a bicarotid trunk were ventricular septal defects, valvular pulmonary stenosis, and atrial septal defect. The most common associated vascular anomaly was the aberrant right subclavian artery. Most frequent genetic syndromes were trisomy 21, Williams syndrome, and CHARGE syndrome. CONCLUSIONS: The prevalence of bicarotid trunk of 17.6% among patients with aortic coarctation and 15.2% among the entire population studied made it not at all a rare phenomenon. Because bicarotid trunk can be a risk factor for surgery, the anatomy of the aortic arch needs to be clearly depicted and described. If ultrasonography examination cannot exclude bicarotid trunk, enhanced magnetic resonance imaging or aortic arch angiography may be necessary.


Assuntos
Anormalidades Múltiplas/epidemiologia , Aorta Torácica/anormalidades , Coartação Aórtica/epidemiologia , Coartação Aórtica/etiologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Criança , Feminino , Humanos , Masculino , Prevalência , Estudos Retrospectivos
16.
Ann Thorac Surg ; 97(1): 328-9, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24384187

RESUMO

We have previously reported an 18-year-old girl with a congenital heart defect who developed complete heart block after one of her corrective surgeries and who needed an epicardial pacemaker implantation. She developed contact sensitivity to silicone compounds. The problem was solved by implanting a silicone-free pacemaker system utilizing silicone-free transvenous leads. The patient was readmitted 2 years later due to lead failure. As no silicone-free epicardial leads were available, we decided to use standard silicone epicardial leads and enclose the whole system in Gore-Tex material (W.L. Gore & Associates, Flagstaff, AZ). Based on our experience we would discourage the use of silicone-free transvenous pacing leads for epicardial use.


Assuntos
Bloqueio Atrioventricular/terapia , Estimulação Cardíaca Artificial/efeitos adversos , Marca-Passo Artificial/efeitos adversos , Politetrafluoretileno/farmacologia , Silicones/efeitos adversos , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/cirurgia , Adolescente , Bloqueio Atrioventricular/etiologia , Estimulação Cardíaca Artificial/métodos , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/métodos , Materiais Revestidos Biocompatíveis , Dermatite de Contato/etiologia , Dermatite de Contato/terapia , Feminino , Seguimentos , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/cirurgia , Humanos , Retratamento , Medição de Risco , Técnicas de Sutura , Resultado do Tratamento
17.
Future Cardiol ; 10(1): 53-62, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24344663

RESUMO

AIM: Cardiotrophin-1 (CT-1) is upregulated by hypoxemia and hemodynamic overload and is characterized by potent hypertrophic and protective properties on cardiac cells. This study aimed to investigate whether CT-1 is differentially induced in the myocardium of infants with congenital cardiac defects depending on hypoxemia. METHODS & RESULTS: Infants with Tetralogy of Fallot (n = 8) or with large nonrestrictive ventricular septal defect (n = 8) undergoing corrective surgery were investigated. Expression of CT-1 was assessed at mRNA and protein levels in the right atrial and ventricular myocardium. The activation of the STAT-3 and VEGF were measured. Degradation of cardiac troponin-I served as a marker of myocardial damage. CT-1 was detected in all patients with levels negatively correlating to the arterial oxygen saturation. Higher CT-1 expression in Tetralogy of Fallot patients was associated with activation of the JAK/STAT pathway and higher cardiac troponin-I degradation. CONCLUSION: CT-1 may mediate myocardial hypertrophy and dysfunction in infants with congenital cardiac defects, particularly in those with hypoxemia.


Assuntos
Cardiomegalia/etiologia , Citocinas/metabolismo , Comunicação Interventricular/metabolismo , Hipóxia/metabolismo , Tetralogia de Fallot/metabolismo , Citocinas/genética , Feminino , Comunicação Interventricular/etiologia , Comunicação Interventricular/cirurgia , Humanos , Hipóxia/etiologia , Hipóxia/cirurgia , Lactente , Recém-Nascido , Masculino , Miocárdio/metabolismo , RNA Mensageiro/metabolismo , Fator de Transcrição STAT3/metabolismo , Transdução de Sinais/fisiologia , Tetralogia de Fallot/etiologia , Tetralogia de Fallot/cirurgia , Fator A de Crescimento do Endotélio Vascular/metabolismo
18.
Artif Organs ; 37(6): 541-8, 2013 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23578310

RESUMO

The majority of cases involving the surgical treatment of congenital heart disease require implementation of cardiopulmonary bypass (CPB). However, neonates and infants are particularly prone to serious complications associated with CPB as a result of capillary leak due to cardiovascular failure. These complications are related to the transfusion of foreign blood, the disproportionately large area of contact between the patient's blood and foreign material, as well as the systemic inflammatory response induced by hemolysis. To attenuate these risks, we developed a novel, highly integrative, miniaturized heart-lung machine (MiniHLM) with a static priming volume of only 102 mL. This prototype was tested in comparison with a conventional heart-lung machine (static priming volume 213 mL) using a rabbit animal model. The animals were anesthetized, sternotomized, and connected to CBP via the aorta and right atrium. The aorta was cross-clamped for 1 h. Blood samples for examination were taken at regular intervals. Biopsies of the right atrial appendage (RAA) were removed directly after initiation and after cessation of CPB. After gradual reduction of perfusion with the HLM, all rabbits were successfully weaned from CPB, and the sternum was closed. Foreign blood was not administered in all cases. After cryopreservation of the RAA tissue, de novo transcription of inflammatory cytokines was measured by means of real-time polymerase chain reaction using the comparative CT method. No significant differences in the expression of the inflammatory parameters of the myocardial tissue samples were found between the study groups.


Assuntos
Ponte Cardiopulmonar , Cardiopatias Congênitas/cirurgia , Máquina Coração-Pulmão , Inflamação/patologia , Miocárdio/patologia , Animais , Desenho de Equipamento , Feminino , Cardiopatias Congênitas/metabolismo , Cardiopatias Congênitas/patologia , Inflamação/metabolismo , Modelos Animais , Miocárdio/metabolismo , Coelhos
19.
ASAIO J ; 59(2): 152-6, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23438778

RESUMO

The utilization of a heart-lung machine (HLM) for the correction of congenital heart defects can lead to various complications, which can culminate in multiorgan failure and death. To reduce the considerable risk of complications, we developed a miniaturized, highly integrated HLM (MiniHLM) for use in infants and children. For the purpose of testing the MiniHLM, we developed a new rabbit animal model. In all, surgery was performed on 32 rabbits. In the first series, 13 New Zealand white rabbits were placed on cardiopulmonary bypass (CPB) for 1 hour with the use of an initial version of the MiniHLM. In the second series, we operated on 19 Chinchilla Bastard rabbits using the further developed MiniHLM 02 or the Dideco Kids D100 system. While several adjustments had to be made to the operating protocol in the first series in order to lower the mortality rate, 15 of the 19 rabbits were successfully weaned from the HLM in the second series. Blood tests pertaining to hemolysis and the expression of inflammation were performed. In addition, tissue samples were taken from the right atrial auricle for the purpose of investigating the expression of inflammatory parameters. The newly developed MiniHLM prototype was tested successfully in an animal model in terms of technical function, hemolysis, and the expression of inflammation. On account of the comparability of their blood values, as well as their anatomy, Chinchilla Bastard rabbits serve as excellent models for the testing of CPB and support systems for infants and children that do not require the administration of foreign blood.


Assuntos
Máquina Coração-Pulmão , Animais , Ponte Cardiopulmonar , Máquina Coração-Pulmão/efeitos adversos , Humanos , Modelos Animais , Oxigênio/sangue , Coelhos
20.
Ann Thorac Surg ; 95(1): 183-8, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23084416

RESUMO

BACKGROUND: The prevention of the pericardial adhesions largely accountable for the technical difficulty and risk of injury inherent to resternotomy continues to gain in importance with the increasing frequency of reoperations. The hemostatic sponge TachoSil (Nycomed Austria GmbH, Linz, Austria), has shown promising results in adhesion prevention in several regions of the body. This study was designed to evaluate its effectiveness in the prevention of pericardial adhesions in comparison with the Gore-Tex (W. L. Gore and Assoc, Flagstaff, AZ) surgical membrane and a control. METHODS: Twenty-four rabbits were distributed into 3 groups: TachoSil, Gore-Tex, or no barrier agent (control). After median sternotomy and pericardiotomy, the cardial surface was exposed to the aggravating effects of room air, irrigation, and gauze abrasion for one hour. A pericardial defect was created and repaired with one of the barrier agents, or left uncovered (control). Resternotomy was performed after 6 months for the evaluation of adhesion formation. RESULTS: Significantly fewer macroscopic adhesions were observed with TachoSil than Gore-Tex in all regions (p < 0.05) excluding the coronary arteries, where the difference in favor of TachoSil did not achieve significance (0.05< p-value <0.10). TachoSil also demonstrated significantly fewer retrosternal adhesions than the control, as well as a universal non-significant trend of fewer adhesions in all regions. The limited lesions present in the TachoSil group were filmy in nature and removed with blunt dissection relatively easily. No significant differences were found between Gore-Tex and the control. Microscopically, the least pronounced fibrosis formation and inflammatory reaction was detected with TachoSil. CONCLUSIONS: TachoSil is effective in the prevention of pericardial adhesions.


Assuntos
Fibrinogênio , Cardiopatias/prevenção & controle , Pericárdio/patologia , Complicações Pós-Operatórias/prevenção & controle , Procedimentos Cirúrgicos Torácicos/efeitos adversos , Trombina , Aderências Teciduais/prevenção & controle , Animais , Modelos Animais de Doenças , Combinação de Medicamentos , Feminino , Cardiopatias/etiologia , Cardiopatias/patologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/patologia , Período Pós-Operatório , Coelhos , Tampões de Gaze Cirúrgicos , Aderências Teciduais/etiologia , Aderências Teciduais/patologia
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