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The following video tutorial presents the surgical correction of the left circumflex aortic arch in a 6-month-old boy with severe respiratory distress and stridor. The diagnosis was confirmed using cardiac catheterization and computed tomography. Intraoperative bronchoscopy showed marked compression of the trachea. An operation was planned to translocate the aortic arch anteriorly and to close the atrial septal defect. After a median sternotomy, the mediastinal structures were carefully mobilized and dissected. The trachea was carefully mobilized and the right ligamentum arteriosum was clipped and divided. Control of the aortic arch vessels, as well as the aberrant right subclavian artery from the right descending aorta, was achieved using vessel loops. An arterial line inserted in the femoral artery was connected to the heart-lung machine. Hence the surgical procedure was undertaken in selective antegrade cerebral perfusion combined with distal body perfusion, avoiding the need for deep hypothermic arrest. Careful mobilization of the complete course of the proximal and distal sections of the circumflex arch allowed its translocation from its retro-oesophageal course. The aortic stump distal to the left subclavian artery was closed by running polypropylene suture. An appropriate site on the ascending aorta was selected to ensure tension- and torsion-free anastomoses. Postoperative bronchoscopy confirmed relief of the tracheal compression.
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Aorta Torácica , Humanos , Masculino , Aorta Torácica/cirugía , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/anomalías , Lactante , Arteria Subclavia/cirugía , Arteria Subclavia/anomalías , Tomografía Computarizada por Rayos X , Procedimientos Quirúrgicos Vasculares/métodosRESUMEN
OBJECTIVES: The objective of the study was to improve postoperative risk assessment in congenital heart surgery by developing a machine-learning model based on readily available peri- and postoperative parameters. METHODS: Our bicentric retrospective data analysis from January 2014 to December 2019 of established risk parameters for dismal outcome was used to train and test a model to predict postoperative survival within the first 30 days. The Freiburg training data consisted of 780 procedures; the Heidelberg test data comprised 985 procedures. STAT mortality score, age, aortic cross-clamp time and postoperative lactate values over 24 h were considered. RESULTS: Our model showed an area under the curve (AUC) of 94.86%, specificity of 89.48% and sensitivity of 85.00%, resulting in 3 false negatives and 99 false positives.The STAT mortality score and the aortic cross-clamp time each showed a statistically highly significant impact on postoperative mortality. Interestingly, a child's age was barely statistically significant. Postoperative lactate values indicated an increased mortality risk if they were either constantly at a high level or low during the first 8 h postoperatively with an increase afterwards.When considering parameters available before, at the end of and 24 h after surgery, the predictive power of the complete model achieved the highest AUC. This, compared to the already high predictive power alone (AUC 88.9%) of the STAT mortality score, translates to an error reduction of 53.5%. CONCLUSIONS: Our model predicts postoperative survival after congenital heart surgery with great accuracy. Compared with preoperative risk assessments, our postoperative risk assessment reduces prediction error by half. Heightened awareness of high-risk patients should improve preventive measures and thus patient safety.
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BACKGROUND: Hybrid procedure with ductal stenting and bilateral pulmonary banding offers a temporary approach in high-risk neonates with complex congenital heart defects aiming biventricular repair. This procedure may also have negative impact concerning post-banding pulmonary stenosis resulting in right ventricular pressure load. METHODS: Between 2010 and 2021 we identified 5 patients with interrupted aortic arch and complex congenital heart defect who underwent hybrid procedure and staged biventricular repair ("hybrid-group"). Other 7 cases with interrupted aortic arch were corrected in the neonatal phase without hybrid procedure ("nonhybrid-group"). Detailed intra- and extracardiac features and surgical procedures were documented as well as pulmonary interventions during follow up. Pulmonary vessel size was assessed by diameter of left and right pulmonary artery in absolute and indexed values. RV pressure was evaluated invasively via catheterization. RESULTS: Survival in cases with hybrid procedure and staged biventricular repair was 91% for a follow-up time of 40.7 months (95% CI 26-55 months) and 100% in the non-hybrid-group. Postoperative results concerning left ventricular function showed normal LV dimensions and systolic function without relevant stenosis on distal aortic arch. Hybrid procedure was associated with impaired local pulmonary arterial diameter after debanding resulting in increased right ventricular pressure and need for interventions (number intervention per patient: hybrid group 1.7 ± 0.95, non-hybrid group 0.17 ± 0.41; P 0.003). CONCLUSIONS: Hybrid procedure in high-risk cases with interrupted aortic arch and staged biventricular repair shows good postoperative results with low perioperative mortality and normal left ventricular function. Due to potential risk of relevant pulmonary stenosis and right ventricular pressure load, follow up examinations must not only focus on left but also on the right heart.
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Coartación Aórtica , Cardiopatías Congénitas , Defectos del Tabique Interventricular , Hipertensión Pulmonar , Estenosis de la Válvula Pulmonar , Recién Nacido , Humanos , Lactante , Arteria Pulmonar/cirugía , Defectos del Tabique Interventricular/cirugía , Resultado del Tratamiento , Coartación Aórtica/cirugía , Ventrículos Cardíacos/cirugíaRESUMEN
BACKGROUND: Neonatal coarctation of the aorta (CoA) is primarily treated by surgical repair. However, under certain high-risk constellations, initial stent angioplasty may be considered followed by surgical repair. We report our experience with this staged approach. Methods: All patients undergoing surgical CoA repair following prior stenting at our institution between January 2011 and December 2019 were included in this retrospective analysis. The patients were classified to be at high risk because of cardiogenic shock, associated complex cardiac malformations, neonatal infection, necrotizing enterocolitis, and extracardiac conditions, respectively. Outcomes were analyzed and compared with neonates who underwent surgical CoA repair without prior stenting in the same observation period. RESULTS: Twenty-six neonates received stent implantation at a median age of 20 days (IQR 9-33 days). Subsequent surgical repair was conducted at an age of 4.2 months (IQR 3.2-6.1 months) with a median body weight of 5.6â kg (IQR 4.5-6.5â kg). Cardiopulmonary bypass was applied in 96% of cases. Extended end-to-end anastomosis was possible in 11 patients. Extended reconstruction with patch material was necessary in the remaining patients. One fatality (3.8%) occurred 33 days postoperatively. At a median follow-up of 5.2 years after initial stenting, all remaining patients were alive; 15/25 patients (60%) were free from re-intervention. Of note, re-intervention rates were comparable in neonates (n = 76) who were operated on with native CoA (28/74 patients; 38%; P = .67). CONCLUSIONS: Neonatal stent angioplasty for CoA results in increased complexity of the subsequent surgical repair. Nevertheless, this staged approach allows to bridge high-risk neonates to later surgical repair with reduced perioperative risk and acceptable midterm outcomes.
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Coartación Aórtica , Humanos , Lactante , Recién Nacido , Angioplastia , Coartación Aórtica/cirugía , Estudios Retrospectivos , Stents , Resultado del TratamientoRESUMEN
BACKGROUND: Currently, over 90% of children with congenital heart disease (CHD) survive into adulthood. As a consequence the psychosocial impact on children and their families has become an important outcome measure. Therefore, the goal of this study was to assess the psychosocial impact from a parent's perspective and to identify possible predictors. METHODS: We included all parents of children who underwent open-heart surgery in the years 2010 and 2011 at the Department of Cardiothoracic Surgery at University Hospital Heidelberg and invited them to complete standardized questionnaires. Psychosocial outcome was measured via parent self- and proxy reporting of family burden (Family Burden Questionnaire, FaBel), health-related quality of life (KidScreen-10), developmental problems (Five-to-Fifteen, FTF), and mental health problems (Strength and Difficulties Questionnaire, SDQ). RESULTS: In total, 113 families returned the questionnaires completely (71.5%). The Aristotle Basic Complexity score and the STAT 2020 Score overall did not predict the psychosocial impact, whereas the number of surgical operations did significantly predict psychosocial impact across all domains in this study cohort. CONCLUSIONS: These data suggest that the number of surgical operations might be a relevant predictor for the long-term psychosocial impact on families suffering from CHD and a potential connecting factor for specialized psychological support. When setting up screening instruments or support programs the entire family must be considered.
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Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Adulto , Niño , Cardiopatías Congénitas/cirugía , Humanos , Padres , Calidad de Vida , Encuestas y CuestionariosRESUMEN
We aimed at identifying the developmental stage at which leukemic cells of pediatric T-ALLs are arrested and at defining leukemogenic mechanisms based on ATAC-Seq. Chromatin accessibility maps of seven developmental stages of human healthy T cells revealed progressive chromatin condensation during T-cell maturation. Developmental stages were distinguished by 2,823 signature chromatin regions with 95% accuracy. Open chromatin surrounding SAE1 was identified to best distinguish thymic developmental stages suggesting a potential role of SUMOylation in T-cell development. Deconvolution using signature regions revealed that T-ALLs, including those with mature immunophenotypes, resemble the most immature populations, which was confirmed by TF-binding motif profiles. We integrated ATAC-Seq and RNA-Seq and found DAB1, a gene not related to leukemia previously, to be overexpressed, abnormally spliced and hyper-accessible in T-ALLs. DAB1-negative patients formed a distinct subgroup with particularly immature chromatin profiles and hyper-accessible binding sites for SPI1 (PU.1), a TF crucial for normal T-cell maturation. In conclusion, our analyses of chromatin accessibility and TF-binding motifs showed that pediatric T-ALL cells are most similar to immature thymic precursors, indicating an early developmental arrest.
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Células Precursoras de Linfocitos T , Leucemia-Linfoma Linfoblástico de Células T Precursoras , Niño , Cromatina , Humanos , Oncogenes , Leucemia-Linfoma Linfoblástico de Células T Precursoras/genética , Unión ProteicaRESUMEN
INTRODUCTION: Congenital heart surgery with cardiopulmonary bypass (CPB) initiates an immune response which frequently leads to organ dysfunction and a systemic inflammatory response. Complications associated with exacerbated immune responses may severely impact the postoperative recovery. The objective was to describe the characteristics of monocyte subpopulations and neutrophils at the level of pattern recognition receptors (PRR) and the cytokine response after CPB in infants. METHODS: An observational cohort study was conducted between June 2016 and June 2017 of infants < 2 years of age, electively admitted for surgical correction of acyanotic congenital heart defects using CPB. Fourteen blood samples were collected sequentially and processed immediately during and up to 48 h following cardiac surgery for each patient. Flow cytometry analysis comprised monocytic and granulocytic surface expression of CD14, CD16, CD64, TLR2, TLR4 and Dectin-1 (CLEC7A). Monocyte subpopulations were further defined as classical (CD14++/CD16-), intermediate (CD14++/CD16+) and nonclassical (CD14+/CD16++) monocytes. Plasma concentrations of 14 cytokines, including G-CSF, GM-CSF, IL-1ß, IL-1RA, IL-4, IL-6, IL-8, IL-10, IL-12p40, IL-12p70, TNF-α, IFN-γ, MIP-1ß (CCL4) and TGF-ß1, were measured using multiplex immunoassay for seven points in time. RESULTS: Samples from 21 infants (median age 7.4 months) were analyzed by flow cytometry and from 11 infants, cytokine concentrations were measured. Classical and intermediate monocytes showed first receptor upregulation with an increase in CD64 expression four hours post CPB. CD64-expression on intermediate monocytes almost tripled 48 h post CPB (p < 0.0001). TLR4 was only increased on intermediate monocytes, occurring 12 h post CPB (p = 0.0406) along with elevated TLR2 levels (p = 0.0002). TLR4 expression on intermediate monocytes correlated with vasoactive-inotropic score (rs = 0.642, p = 0.0017), duration of ventilation (rs = 0.485, p = 0.0259), highest serum creatinine (rs = 0.547, p = 0.0102), postsurgical transfusion (total volume per kg bodyweight) (rs = 0.469, p = 0.0321) and lowest mean arterial pressure (rs = -0.530, p = 0.0135). Concentrations of IL-10, MIP-1ß, IL-8, G-CSF and IL-6 increased one hour post CPB. Methylprednisolone administration in six patients had no significant influence on the studied surface receptors but led to lower IL-8 and higher IL-10 plasma concentrations. CONCLUSIONS: Congenital heart surgery with CPB induces a systemic inflammatory process including cytokine response and changes in PRR expression. Intermediate monocytes feature specific inflammatory characteristics in the 48 h after pediatric CPB and TLR4 correlates with poorer clinical course, which might provide a potential diagnostic or even therapeutic target.
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Cardiopatías Congénitas/metabolismo , Monocitos/metabolismo , Receptores de IgG/metabolismo , Receptor Toll-Like 2/metabolismo , Receptor Toll-Like 4/metabolismo , Presión Arterial/fisiología , Citocinas/metabolismo , Femenino , Cardiopatías Congénitas/cirugía , Humanos , Lactante , Recién Nacido , Inflamación/metabolismo , Masculino , Neutrófilos/metabolismo , Estudios ProspectivosRESUMEN
BACKGROUND: Depth of anesthesia may be insufficient in pediatric cardiac anesthesia if a total intravenous anesthetic regimen with opioids and midazolam is used during cardiopulmonary bypass. The advantages of sevoflurane-based balanced anesthesia may be (1) a more graduated regulation of the depth of anesthesia during cardiopulmonary bypass and (2) a reduction in postoperative ventilation time for children in comparison with total intravenous anesthesia. AIM: To evaluate a possibly positive effect of sevoflurane-based balanced anesthesia in children undergoing cardiac surgery we analyzed whether this anesthetic regimen had a significant effect related to (1) depth of anesthesia, (2) the need for opioids during cardiopulmonary bypass as well as on postoperative characteristics such as (3) time of postoperative ventilation, and (4) duration of stay in the intensive care unit in comparison with total intravenous anesthesia. METHODS: In a retrospective analysis, data from heart-lung machine protocols from 2013 to 2016 were compared according to anesthetic regimen (sevoflurane-balanced anesthesia, n = 70 vs. total intravenous anesthesia, n = 65). Children (age: 8 weeks to 14 years) undergoing cardiac surgery with cardiopulmonary bypass were included. As a primary outcome measure, we compared Narcotrend® system-extracted data to detect insufficient phases of anesthetic depth during extracorporeal circulation under moderate hypothermia. Postoperatively, we measured the postoperative ventilation time and the number of days in the intensive care unit. Furthermore, we analyzed patients' specific characteristics such as opioid consumption during cardiopulmonary bypass. Regression analysis relating primary objectives was done using the following variables: anesthetic regimen, age, severity of illness/surgery, and cumulative dosage of opiates during cardiopulmonary bypass. RESULTS: No significant differences were observed in descriptive patient characteristics (age, body weight, height, and body temperature) between the two groups. Further, no significant differences were found in depth of anesthesia by analyzing phases of superficial B1-C2-electroencephalography Narcotrend® data. No marked difference between the groups was observed for the duration of postoperative intensive care unit stay. However, the postoperative ventilation time (median (95% CI, hours)) was significantly lower in the sevoflurane-based balanced anesthesia group (6.0 (2.0-15.0)) than in the total intravenous anesthesia group (13.5 (7.0-25)). A higher dosage of opioids and midazolam was required in the total intravenous anesthesia group to maintain adequate anesthesia during cardiopulmonary bypass. Regression analysis showed an additional, significant impact of the following factors: severity of illness and severity grade of cardiac surgery (according to Aristotle) on the primary endpoint. CONCLUSION: In children undergoing cardiac surgery in our department, the use of sevoflurane-balanced anesthesia during cardiopulmonary bypass showed no superiority of inhalational agents over total intravenous anesthesia with opioids and benzodiazepines preventing phases of superficial anesthesia, but a marked advantage for the postoperative ventilation time compared with total intravenous anesthesia.
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Anestésicos por Inhalación/uso terapéutico , Anestésicos Intravenosos/uso terapéutico , Procedimientos Quirúrgicos Cardíacos/métodos , Circulación Extracorporea/métodos , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Estudios RetrospectivosRESUMEN
BACKGROUND: Different methods for surgical correction of partial anomalous pulmonary venous connection (PAPVC) exist. We evaluated the outcomes of four techniques regarding morbidity and mortality. METHODS: A total of 116 patients underwent PAPVC repair in our institution over a period of 20 years. Single-patch technique (n = 82 [71%], mean age: 18.59 ± 20.49 years), double-patch technique (n = 13 [11%], mean age: 43.18 ± 25.14 years), Warden's technique (n = 7 [6%], mean age: 10.04 ± 10.47 years), and direct implantation of anomalous pulmonary veins (n = 14 [12%], mean age: 14.42 ± 18.58 years) were examined. RESULTS: Out of the 116 patients, one patient (0.9%) developed pulmonary hypertension after discharge and three patients (2.6%) with normal right cardiac function showed right ventricular failure. In total, a pacemaker was inserted in seven cases (6%). Three patients (2.6%) presented with persistent nonsinus rhythm during follow-up. This complication was most frequently seen in the double-patch group being significantly increased compared with the other groups (p = 0.035). One patient presented with a mild stenosis of the superior vena cava. There were two early, nonsurgery-related deaths and no late mortality. CONCLUSION: Operative therapy of PAPVC has low postoperative morbidity and mortality. Therefore, surgical repair of this cardiac anomaly is a safe and reproducible treatment independent of the applied method. The surgical technique must be selected based on the anatomy and possible accompanying congenital heart defects. Special care should be taken when using the double-patch technique because of significant more frequent nonsinus rhythm events postoperatively.
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Procedimientos Quirúrgicos Cardíacos , Venas Pulmonares/cirugía , Síndrome de Cimitarra/cirugía , Adolescente , Adulto , Anciano , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/mortalidad , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/etiología , Circulación Pulmonar , Venas Pulmonares/anomalías , Venas Pulmonares/diagnóstico por imagen , Venas Pulmonares/fisiopatología , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Síndrome de Cimitarra/diagnóstico por imagen , Síndrome de Cimitarra/mortalidad , Síndrome de Cimitarra/fisiopatología , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
Anomalous connection of the superior vena cava to the morphologic left atrium is a rare congenital systemic venous abnormality. As opposed to pulmonary venous anomalies, the significant right-to-left shunt in these patients warrants a correction early in life. Optimal repair technique for combined pulmonary and systemic venous anomalies is not defined yet. Herein, we describe a neonate with such a diagnosis treated with cavoatrial anastomosis, known as Warden procedure with excellent results.
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Procedimientos Quirúrgicos Cardíacos/métodos , Atrios Cardíacos/cirugía , Cardiopatías Congénitas/cirugía , Vena Cava Superior/cirugía , Anastomosis Quirúrgica/métodos , Atrios Cardíacos/anomalías , Humanos , Recién Nacido , MasculinoRESUMEN
BACKGROUND: Even after successful aortic coarctation (CoA) repair, hypertension causes premature morbidity and mortality. The mechanisms are not clear. The aim was to evaluate elastic wall properties and aortic morphology and to correlate these results with severity of restenosis, hypertension, aortic arch geometry, noninvasive pressure gradients, and time and kind of surgical procedure. METHODS: Eighty-nine patients (17 ± 6.3 years) and 20 controls (18 ± 4.9 years) were examined using magnetic resonance imaging (MRI). In addition to contrast-enhanced MR angiography and flow measurements, CINE MRI was performed to assess the relative change of aortic cross-sectional areas at diaphragm level to calculate aortic compliance (C). RESULTS: Fifty-four percent of all patients showed hypertension (> 95th percentile), but more than half of them had no significant stenosis (defined as ≥30%). C was lower in CoA than in controls (3.30 ± 2.43 vs. 4.67 ± 2.21 [10-5 Pa-1 m-2]; p = 0.024). Significant differences in compliance were found between hyper- and normotensive patients (2.61 ± 1.60 vs. 4.11 ± 2.95; p = 0.01), and gothic and Romanesque arch geometry (2.64 ± 1.58 vs. 3.78 ± 2.81; p = 0.027). There was a good correlation between C and hypertension (r = 0.671; p < 0.01), but no correlation between C (and hypertension) and time or kind of repair, restenosis, or pressure gradients. CONCLUSION: The decreased compliance, a high rate of hypertension without restenosis, and independency of time and kind of repair confirm the hypothesis that CoA may not be limited to isthmus region but rather be a widespread (systemic) vascular anomaly at least in some of the CoA patients. Therefore, aortic compliance should be assessed in these patients to individually tailor treatment of CoA patients with restenosis and/or hypertension.
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Aorta/diagnóstico por imagen , Coartación Aórtica/diagnóstico por imagen , Presión Arterial , Hipertensión/diagnóstico por imagen , Angiografía por Resonancia Magnética , Imagen por Resonancia Cinemagnética , Rigidez Vascular , Adolescente , Adulto , Antihipertensivos/uso terapéutico , Aorta/anomalías , Aorta/fisiopatología , Aorta/cirugía , Coartación Aórtica/complicaciones , Coartación Aórtica/fisiopatología , Coartación Aórtica/cirugía , Presión Arterial/efectos de los fármacos , Niño , Preescolar , Femenino , Humanos , Hipertensión/tratamiento farmacológico , Hipertensión/etiología , Hipertensión/fisiopatología , Masculino , Valor Predictivo de las Pruebas , Pronóstico , Estudios Prospectivos , Factores de Riesgo , Rigidez Vascular/efectos de los fármacos , Adulto JovenRESUMEN
While Wilms tumors (WT) typically present solely with an abdominally palpable mass, rare cases exhibiting vascular tumor growth can also present with circulatory problems. Here, we report the case of a 2.5-year-old girl presenting with upper venous congestion and arterial hypertension as the primary symptoms of intraventricular tumor growth exhibiting remarkable tubular and perfused morphology. Clinical situation stabilized after initiation of neoadjuvant chemotherapy (NAC) with actinomycin D and vincristine, followed by surgical resection via laparotomy and sternotomy supported by cardiopulmonary bypass and deep hypothermia. Our results highlight the previously reported feasibility of this approach, even in primarily unstable patients.
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Cor triatriatum sinister is a rare congenital abnormality, with varying signs and symptoms related to the anatomic features and the components of the accompanying cardiac abnormalities. We present a 3-year-old girl who underwent successful simultaneous operation with complete loss of the membrane and ligation of vertical vein by anomalous venous return. The principles for surgical management of cor triatriatum can be summarised as radical resection of the membrane and correction of other intracardiac anomalies.
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Anomalías Múltiples , Procedimientos Quirúrgicos Cardíacos/métodos , Corazón Triatrial/diagnóstico , Síndrome de Cimitarra/diagnóstico , Cateterismo Cardíaco , Preescolar , Corazón Triatrial/cirugía , Ecocardiografía , Electrocardiografía , Femenino , Humanos , Síndrome de Cimitarra/cirugíaRESUMEN
BACKGROUND: Congenital cardiovascular malformations (CCVM) may cause infrequently airway pathologies (AP) in children and are of prognostic and therapeutic relevance. While computed tomography (CT) is considered first-line imaging modality in many centres, we started using magnetic resonance imaging (MRI) more and more in the last years to detect CCVM and AP to avoid radiation in this patient group. OBJECTIVE: The aim of this retrospective study was to determine and to compare the diagnostic accuracy of CT and MRI when used to detect CCVM and/or AP. METHODS: All patients suspected to have CCVM and/or AP and examined either by CT or MRI between 2000 and 2013 in our hospital were included. Extension and type of CCVM, as well as their relationship to esophagus, trachea or bronchi were assessed and related to findings of tracheobronchoscopy, cardiac catheterization or surgery if available. RESULTS: One hundred six patients (median [range] 4 years [2 days to 66 years]) were examined by CT (n = 27) or MRI (n = 79). In 78 patients (74%), CCVM and/or AP were found with either of the imaging methods. CCVM were found in 63 subjects. Forty-six of 63 subjects had both, CCVM and AP. The presence of CCVM was always detected correctly by CT or MRI, although both techniques had a weakness detecting atretic segments directly. AP (n = 61) were correctly diagnosed in all patients not intubated for artificial ventilation by CT (n = 17) and in all but 2 patients by MRI (39 out of 41). CONCLUSIONS: MRI is sensitive to detect CCVM associated with AP equally to CT without any radiation exposure.
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Cardiopatías Congénitas/diagnóstico por imagen , Imagen por Resonancia Magnética , Tomografía Computarizada Multidetector , Anillo Vascular/diagnóstico por imagen , Adolescente , Adulto , Anciano , Enfermedades Bronquiales/diagnóstico por imagen , Niño , Preescolar , Constricción Patológica/diagnóstico por imagen , Esófago/diagnóstico por imagen , Femenino , Humanos , Imagenología Tridimensional , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tráquea/diagnóstico por imagen , Estenosis Traqueal/diagnóstico por imagen , Traqueobroncomalacia/diagnóstico por imagen , Adulto JovenRESUMEN
BACKGROUND: Surgery of the pulmonary valve, right ventricular outflow tract, and pulmonary artery falls under the domain of paediatric cardiac surgery. However, 97 adult patients underwent such operations in our institution from 1993 to 2016. AIM: This study aims to analyse preoperative risk factors, intraoperative data, postoperative outcomes, and long-term survival to identify the potential predictors of mortality and high-risk patients. METHODS: We divided our patient cohort into three groups in accordance with surgical indications: 17 patients with pulmonary valve endocarditis (group A), 70 patients with congenital defects involving the pulmonary valve (group B), and 10 patients who underwent pulmonary valve surgery for other indications, such as tumour or other acquired valvular disease (group C). RESULTS: Gender distribution was comparable in all the three groups, with about 40% of the total number of patients being female. The mean age was 35.9 ± 15.7 years. Sixty (61.9%) patients had a history of cardiac surgery. Various concomitant cardiac surgical procedures were necessary in 49 (50.5%) cases. There were two (11.8%) in-hospital deaths in group A, two (2.9%) in group B, and none in group C. Within the mean follow-up time of 6.6 ±7.2 years, three (17.7%) patients in group A, two (2.9%) in group B, and four (40%) in group C died. CONCLUSIONS: Adult patients with pulmonary valve disease are often previously heart-operated and often need concomitant procedures. The operative risk in patients with pulmonary valve endocarditis is high. Surgery of congenital defects of the pulmonary valve is safe and can be performed with excellent outcomes.
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Procedimientos Quirúrgicos Cardíacos/estadística & datos numéricos , Endocarditis/cirugía , Ventrículos Cardíacos/cirugía , Válvula Pulmonar/cirugía , Tetralogía de Fallot/cirugía , Adulto , Anciano , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Femenino , Cardiopatías Congénitas/cirugía , Humanos , Masculino , Persona de Mediana Edad , Resultado del Tratamiento , Adulto JovenRESUMEN
A newborn presented with an aorto-right ventricular tunnel, a defect connecting the left aortic sinus to the right ventricle. The patient underwent repair on 4th day of life.
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Anomalías Múltiples/cirugía , Aorta/anomalías , Aorta/cirugía , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Procedimientos Quirúrgicos Cardíacos , Humanos , Recién NacidoRESUMEN
We report the case of a patient with tetralogy of Fallot with absent pulmonary valve and familial Alagille syndrome who successfully underwent cardiac repair. The patient's sister had liver and congenital heart disease. The father had undergone liver transplantation but showed no significant cardiac abnormalities. A yet-unknown mutation of the JAG1 gene was discovered in this family with variable expression of Alagille syndrome.
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Síndrome de Alagille/genética , Proteínas de Unión al Calcio/genética , Péptidos y Proteínas de Señalización Intercelular/genética , Proteínas de la Membrana/genética , Mutación , Niño , Femenino , Cardiopatías Congénitas , Humanos , Recién Nacido , Proteína Jagged-1 , Masculino , Fenotipo , Proteínas Serrate-Jagged , Adulto JovenRESUMEN
Hypoplastic left heart syndrome (HLHS) is one of the most challenging congenital heart defects. At present, it is expected that - at best - 70% of newborns with HLHS will reach adulthood. This review addresses the problems of right ventricular (RV) failure and insufficient growth of pulmonary vasculature in these patients. In order to further improve long-term prognosis translational research to control RV function, growth of pulmonary arteries and progress in chronic circulatory support are clearly needed to provide a further improvement for adults with HLHS.
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BACKGROUND: Patent ductus arteriosus in preterm neonates leads to significant morbidity. Surgery is indicated when pharmacological treatment fails or is contraindicated, but the optimal timing remains unclear. METHODS: We retrospectively studied all 41 preterm neonates with symptomatic ductus arteriosus who underwent ligation between 1988 and 2009. We compared early complications rates and late neurological outcomes of patients operated on before 21 days of age with these operated on later. RESULTS: The median gestational age at birth was 26 weeks (range 23-31 weeks) and median weight at birth was 930 g (range 510-1500 g); 34 (82.9%) received pharmacological treatment before surgery. Fourteen (34.1%) patients underwent surgical closure before 21 days of age and 27 (65.9%) after 21 days. The 2 groups did not differ significantly in gestational age and weight at birth, but those operated on after 21 days received significantly more pharmacological treatment cycles. Patients in the early closure group had shorter intubation times: median 23 days (range 13-35 days) vs. 43 days (range 27-84 days; p < 0.001) and shorter neonatal intensive care unit stay: median 44 days (range 31-66 days) vs. 76 days (range 41-97 days; p < 0.001), with significantly lower rates of bronchopulmonary dysplasia, intraventricular hemorrhage, and acute renal failure, and significantly better neurological outcomes. DISCUSSION: Performing early ligation of symptomatic ductus arteriosus after unsuccessful pharmacological therapy in preterm neonates might lower complication rates and improve neurological outcome. Prospective randomized studies are needed to determine the optimal treatment.