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INTRODUCTION: Vascular Rings (VRs) are congenital malformations of the aortic arch that can compress the trachea and esophagus producing symptoms. Approximately, 5%-10% of patients experience persistent symptoms postrepair and 9% require reintervention. There are various approaches to repair-simple ligation and division of the ring or more complex procedures. Our objective was to describe outcomes for VR repair using a "simple" surgical approach. METHODS: We identified patients who underwent VR repair from 2012 to 2022 at our institution. Clinical and surgical data, demographics, intraoperative, and postoperative outcomes were extracted from their electronic medical records. Outcomes were analyzed and regression analysis was used to identify risk factors for residual symptoms after repair. RESULTS: Ninety three patients with VR repair were included. Prevalence of symptoms were reduced following surgery (80% preoperative versus 13% postoperative, P 0.03). Tracheomalacia also decreased from 16% to 7% postrepair. No deaths were reported in our cohort and 2% of our patients required reintervention. Multivariable regression analysis showed that preoperative tracheomalacia was associated with having persistent symptoms after VR repair (odds ratio 6.2, 95% CI 1.02-37.6). CONCLUSIONS: Our institutional experience using a simple surgical approach showed a significant decrease in symptoms, a very low reintervention rate and no mortality. Preoperative tracheomalacia can be a risk factor for persistent symptoms postrepair. We believe a simple surgical approach for children with complete VR repair can be effective in relieving symptoms.
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Single ventricle (SV) cardiac lesions and tetralogy of Fallot (TOF) are both common forms of cyanotic congenital heart disease. With advances in perioperative care and longitudinal follow-up, survival of these patients has dramatically improved and the majority survive to adulthood. This study compares health-related quality of life (HRQoL) of adult SV and TOF patients to each other and the general population. HRQoL of all surviving, non-transplanted SV and TOF patients 21 years of age and older at our institution was assessed with the SF-36 questionnaire via phone. Additional data including demographic parameters and information related to comorbidities and healthcare utilization were also analyzed. Among 81 eligible SV patients and 207 TOF patients, 33 (41%) and 75 (36%) completed the SF-36 phone survey, respectively. The mean age of SV patients was 32 vs. 38 years in the TOF group (p=0.01). SV patients reported more hepatic, pulmonary, and renal comorbidities. TOF patients were more likely to complete advanced degrees and more likely to have children (p=0.03). SV physical functioning scores were worse compared to TOF. In other domains of the SF-36 questionnaire, SV and TOF scores were similar. Compared to the general population, both groups reported worse bodily pain and mental health, but other aspects of psychosocial and general health were comparable. Overall HRQoL is good for both SV and TOF patients through early and mid-adulthood. Some QoL metrics were modestly worse in the SV patients. While these patients may have some physical limitations, psychosocial wellbeing appears preserved.
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Cardiopatias Congênitas , Tetralogia de Fallot , Coração Univentricular , Adulto , Criança , Humanos , Qualidade de Vida/psicologia , Cardiopatias Congênitas/cirurgia , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Informed consent for surgery is a complex process particularly in paediatrics. Complexity increases with procedures such as CHD surgery. Regulatory agencies outline informed consent contents for surgery. We assessed and described CHD surgical informed consent contents through survey dissemination to paediatric CHD centres across United States of America. METHODS: Publicly available email addresses for 125 paediatric cardiac clinicians at 70 CHD surgical centres were obtained. Nine-item de-identified survey assessing adherence to The Joint Commission informed consent standards was created and distributed via RedCap® 14 March, 2023. A follow-up email was sent 29 March, 2023. Survey link was closed 18 April, 2023. RESULTS: Thirty-seven surveys were completed. Results showed informed consent documents were available in both paper (25, 68%) and electronic (3, 8%) format. When both (9, 24%) formats were available, decision on which format to use was based on centre protocols (1, 11%), clinician personal preference (3, 33%), procedure being performed (1, 11%), or other (4, 45%). Five (13%) centres' informed consent documents were available only in English, with 32 (87%) centres also having a Spanish version. Review of informed consent documents demonstrated missing The Joint Commission elements including procedure specific risks, benefits, treatment alternatives, and expected outcomes. CONCLUSIONS: Informed consent for CHD surgery is a complex process with multiple factors involved. Majority of paediatric CHD surgical centres in the United States of America used a generic informed consent document which did not uniformly contain The Joint Commission specified information nor reflect time spent in discussion with families. Further research is needed on parental comprehension during the informed consent process.
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Calcium channel blocker (CCB) toxicity carries a high mortality and is the sixth most fatal drug class reported to US poison centers. Amlodipine overdose is characterized by a life-threatening arterial vasodilation that compromises organ perfusion. The management of CCB intoxication is focused on maintaining adequate organ perfusion. In cases refractory to medical therapies, hemodynamic support with extracorporeal membrane oxygenation (ECMO) is warranted necessitating higher flows than usual to compensate for the vasodilation and requiring central cannulation. We present a case of a 12-year-old with severe dihydropyridine CCB ingestion, refractory to medical management and successfully treated with central ECMO cannulation. The patient was discharged home with no significant disability. Central ECMO cannulation may be helpful to facilitate adequate flows in vasodilatory shock such as CCB overdose.
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Di-Hidropiridinas , Oxigenação por Membrana Extracorpórea , Humanos , Criança , Bloqueadores dos Canais de Cálcio , Perfusão , CateterismoRESUMO
INTRODUCTION: In addition to an organ donor shortage, racial disparities exist at different stages of the transplantation process. Xenotransplantation (XTx) could alleviate these issues. This study describes racial differences in attitudes to XTx among populations who may need a transplant or are transplant recipients. METHODS: A Likert-scale survey was distributed at outpatient clinics to parents of children with congenital heart disease (CHD) and kidney patients on their attitudes to pig organ XTx. Data from these two groups were stratified by race and compared. RESULTS: Ninety-seven parents of children with CHD (74.2% White and 25.8% Black) and 148 kidney patients (50% White and 50% Black) responded to our survey. Black kidney patients' acceptance of XTx although high (70%) was lower than White kidney patients (91%; P .003). White kidney patients were more likely to accept XTx if results are similar to allotransplantation (OR 4.14; 95% CI 4.51-11.41), and less likely to be concerned with psychosocial changes when compared to Black kidney patients (receiving a pig organ would change your personality OR 0.08; 95% CI 0.01-0.67 and would change social interaction OR 0.24; 95% CI 0.07-0.78). There were no racial differences in attitudes to XTx among parents of children with CHD. CONCLUSION: There are differences in attitudes to XTx particularly among Black kidney patients. Because kidneys may be the first organ for clinical trials of XTx, future studies that decrease scientific mistrust and XTx concerns among the Black community are needed to prevent disparities in uptake of possible future organ transplant alternatives.
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Atitude , Doadores de Tecidos , Animais , Xenoenxertos , Humanos , Fatores Raciais , Suínos , Transplante HeterólogoRESUMO
BACKGROUND: Scientific advancements are occurring in cardiac xenotransplantation (XTx). However, there have been religious and social concerns surrounding this allotransplantation alternative. The purpose of this study was to explore the acceptance of XTx among stakeholders of the congenital heart disease (CHD) community. METHODS: A Likert-scale anonymous survey was distributed to physicians and nurses who care for children with CHD and parents of children with CHD. Psychosocial and clinical attitudes were compared across all groups to identify differences, and regression analysis was performed to identify factors associated with XTx acceptance. RESULTS: A total of 297 responded to the survey: 134 physicians, 62 nurses, and 101 parents. Potential acceptance of XTx if outcomes were similar to allotransplantation was high overall (75.3%), but different between the groups (physicians 86%; nurses 71%, parents 64%; P < .0001). Regression analysis showed respondents who reported religion would influence medical decision making (OR 0.48; 95%CI 0.24-0.97) and those who would not use a pig heart transplant as a bridge until a human heart became available were less likely to accept XTx (OR 0.09; 95%CI 0.04-0.21). Psychosocial concerns to XTx were minimal but were also associated with XTx acceptance particularly among parents (OR 0.17; 95%CI 0.03-0.80). CONCLUSIONS: Potential acceptance of XTx is high, assuming results are similar to allotransplantation. Religious beliefs and attitudes toward the use of XTx as a bridge to allotransplant may present barriers to XTx acceptance. Future research is needed to assess potential attitude differences in light of ethical, psychosocial, and religious objections to XTx.
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Atitude do Pessoal de Saúde , Atitude Frente a Saúde , Cardiopatias Congênitas/cirurgia , Transplante de Coração/métodos , Pais/psicologia , Transplante Heterólogo/psicologia , Adulto , Animais , Criança , Estudos Transversais , Feminino , Transplante de Coração/psicologia , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Enfermeiras e Enfermeiros/psicologia , Pediatria , Médicos/psicologia , Religião e Medicina , Religião e Psicologia , Inquéritos e Questionários , Suínos , Estados UnidosRESUMO
BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic poses broad challenges to healthcare systems and providers. The manifestations of this disease are still being described in a variety of different contexts and patient populations. RESULTS: We report the case of a neonate who demonstrated COVID-19 after surgical correction of transposition of the great arteries. In addition, the patient demonstrated an evolving and persistent tachyarrhythmia consistent with neither the most likely postoperative complications nor typical COVID-19. DISCUSSION: The patient had negative preoperative testing for the virus and presented with profound oxygen desaturation and respiratory failure several days postoperatively. This raised concern for a complication of his arterial switch operation. It was found that one of the patient's caregivers was an asymptomatic carrier of COVID-19, and imaging ruled out intracardiac shunting. After initiating treatment for COVID-19, the patient's oxygen requirements and need for anti-arrhythmic agents improved. CONCLUSION: We propose that, despite negative preoperative testing, coronavirus infection may present as refractory tachyarrhythmia, and may be considered along with surgical complications as a cause for unexplained hypoxemia postoperatively.
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COVID-19 , Transposição dos Grandes Vasos , Artérias , Humanos , Recém-Nascido , SARS-CoV-2 , Taquicardia/etiologia , Transposição dos Grandes Vasos/cirurgiaRESUMO
BACKGROUND: Stroke as a complication of infective endocarditis portends a poor prognosis, yet risk factors for stroke subtypes have not been well defined. AIM: To identify risk factors associated with ischaemic and haemorrhagic strokes. METHODS: A retrospective patient chart review was performed at a single US academic centre to identify risk factors and imaging for patients who were 18 years or older with infectious endocarditis (IE) and stroke diagnoses. Differences in patient characteristics by stroke status were assessed using univariate analysis, χ2 or student's t-test as well as logistic regression models for multivariable analyses and correlation matrices to identify possible collinearity between variables and to obtain odds ratios (OR) and their 95% confidence intervals. RESULTS: A final sample of 1157 participants was used for this analysis. The total number of non-surgical strokes was 178, with a prevalence of 15.4% (78% ischaemic, 10% parenchymal haemorrhages, 8% subarachnoid haemorrhages and 4% mixed ischaemic/haemorrhagic). Multivariate risk factors for ischaemic stroke included prior stroke (OR 2.0, 1.3-3.1), Staphylococcus infection (OR 2.0, 1.3-3.0), mitral vegetations (OR 2.2, 1.4-3.3) and valvular abscess (OR 2.7, 1.7-4.3). Risk factors for haemorrhagic stroke included fungal infection (OR 6.4, 1.2-34.0), male gender (OR 3.5, 1.4-8.3) and rheumatic heart disease (OR 3.3, 1.1-10.4). CONCLUSION: Among patients with IE, there exist characteristics that relate differentially to ischaemic and haemorrhagic stroke risk.
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Isquemia Encefálica/complicações , Endocardite/complicações , Hemorragias Intracranianas/complicações , Acidente Vascular Cerebral/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Micoses/complicações , New York/epidemiologia , Estudos Retrospectivos , Cardiopatia Reumática/complicações , Fatores de Risco , Índice de Gravidade de Doença , Infecções Estafilocócicas/complicações , Adulto JovemRESUMO
Heart rate variability (HRV) has become an important clinical marker of cardiovascular health and a research measure for the study of the cardiac conduction system and its autonomic controls. While the zebrafish (Danio rerio) is an ideal vertebrate model for understanding heart development, HRV has only recently been investigated in this system. We have previously demonstrated that nkx2.5 and nkx2.7, two homologues of Nkx2-5 expressed in zebrafish cardiomyocytes, play vital roles in maintaining cardiac chamber-specific characteristics. Given observed defects in ventricular and atrial chamber identities in nkx2.5-/- embryos coupled with conduction system abnormalities in murine models of Nkx2.5 insufficiency, we postulated that reduced HRV would serve as a marker of poor cardiac health in nkx2.5 mutants and in other zebrafish models of human congenital heart disease. Using live video image acquisition, we derived beat-to-beat intervals to compare HRV in wild-type and nkx2.5-/- embryos. Our data illustrate that the nkx2.5 loss-of-function model exhibits increased heart rate and decreased HRV when compared with wild type during embryogenesis. These findings validate HRV analysis as a useful quantitative tool for assessment of cardiac health in zebrafish and underscore the importance of nkx2.5 in maintaining normal heart rate and HRV during early conduction system development.
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Desenvolvimento Embrionário/fisiologia , Frequência Cardíaca/fisiologia , Proteína Homeobox Nkx-2.5/metabolismo , Proteínas de Peixe-Zebra/metabolismo , Peixe-Zebra/embriologia , Peixe-Zebra/fisiologia , AnimaisRESUMO
BACKGROUND: Acute kidney injury (AKI) is a significant complication of surgical aortic valve replacement (SAVR). This study sought to describe AKI following SAVR, its risk factors, predictors and effect on long-term survival. METHODS: We retrospectively reviewed 2169 patients who underwent isolated SAVR between 2000 and 2012. The main end-points were occurrence of AKI, postoperative complications, and short- and long-term survival rates following SAVR. Patients were divided into two groups: AKI+ (n = 181) and AKI- (n = 1945). RESULTS: AKI occurred in 8.5% of patients, of which 3.9% (n = 7) needed dialysis. Predictors of AKI after SAVR were body mass index (BMI) and intraoperative packed red blood cells (PRBC) transfusion. AKI+ patients had a more complicated postoperative course and higher cumulative mortality (25% vs. 17%, p = 0.012) with a median follow-up of 4.1 years. AKI was not found to be an independent predictor of mortality. CONCLUSIONS: Predictors of AKI after SAVR are increased BMI and intraoperative PRBC transfusion. AKI conferred an increase in hospital length of stay and cumulative mortality while the need for postoperative dialysis was associated with the most complicated hospital stays and the highest in-hospital and cumulative mortalities; therefore careful recognition of patients at risk of AKI is warranted for a better preoperative renal optimization. However, incidence of AKI was lower than what is reported after both on-CPB cardiac surgeries and transcatheter aortic valve replacement, moreover AKI was not found to be an independent predictor of mortality.
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Injúria Renal Aguda , Valva Aórtica/cirurgia , Cardiopatias Congênitas/cirurgia , Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca , Complicações Pós-Operatórias , Injúria Renal Aguda/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Doença da Válvula Aórtica Bicúspide , Índice de Massa Corporal , Diálise , Transfusão de Eritrócitos , Feminino , Previsões , Implante de Prótese de Valva Cardíaca/mortalidade , Mortalidade Hospitalar , Humanos , Incidência , Cuidados Intraoperatórios , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Taxa de SobrevidaRESUMO
BACKGROUND: Concomitant tricuspid valve repair (TVr) for functional tricuspid regurgitation (TR) at the time of left-sided valve surgery has become increasingly more common over the past decade. The impact of residual post-repair TR on late outcomes remains unclear. METHODS: All patients undergoing TVr during concomitant left-sided valve surgery at our institution from 2005-2012 were retrospectively reviewed. Patients were stratified into 2 groups according to the degree of post-cardiopulmonary bypass TR observed on intraoperative transesophageal echocardiography; 0-1+ TR (No TR, n = 246) and ≥2+ TR (Residual TR, n = 26). Primary outcomes of interest were 30-day survival, 4-year survival, and follow-up TR grade. A propensity-matched subgroup analysis was performed in addition to the overall cohort analysis. RESULTS: Mean age for all patients was 70.3 ± 13.0 years and 107 (39%) patients were male. There was no difference in 30-day survival between groups (92% No TR versus 96% Residual TR, P = .70). Kaplan-Meier analysis of overall 4-year survival showed a trend toward worsened survival in the Residual TR group (log rank P = .17) and propensity-matched subgroup analysis showed significantly worse 4-year survival for Residual TR (log rank P = .02). At mean echocardiographic follow up of 11.9 ± 22.5 months, TR grade was significantly worse in the Residual TR group compared to No TR (1.5 ± 0.8 Residual TR versus 0.9 ± 0.9 No TR, P = .005), although TR severity was significantly improved from immediately post-bypass. CONCLUSIONS: Patients left with residual TR following TVr during concomitant left-sided valve surgery have significantly decreased late survival compared to patients left with no post-repair TR.
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Insuficiência da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Idoso , Ponte Cardiopulmonar , Ecocardiografia Transesofagiana , Feminino , Humanos , Período Intraoperatório , Tempo de Internação , Masculino , Recidiva , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento , Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/mortalidadeRESUMO
BACKGROUND AND AIMS: The immediate postischemic period is marked by elevated intracellular calcium levels, which can lead to irreversible myocyte injury. Del Nido cardioplegia was developed for use in the pediatric population to address the inability of immature myocardium to tolerate high levels of intracellular calcium following cardiac surgery. Our aim in this study is to determine if this solution can be used safely and effectively in an adult, reoperative population. METHODS: All patients undergoing isolated reoperative aortic valve replacement at our institution from 2010 to 2012 were retrospectively reviewed. Demographics, comorbidities, operative variables, postoperative complications, and patient outcomes were collected. Patients were divided into two groups based on cardioplegia strategy used: whole blood cardioplegia (WB, n = 61) and del Nido cardioplegia (DN, n = 52). RESULTS: Mean age in the study was 73.4 ± 14.3 years and 86 patients were male (76.1%). Eighty-four patients had undergone prior coronary artery bypass graft (CABG) (74.3%). Patients in the DN group required significantly lower total volume of cardioplegia (1147.6 ± 447.2 mL DN vs. 1985.4 ± 691.1 mL WB, p < 0.001) and retrograde cardioplegia dose (279.3 ± 445.1 mL DN vs. 1341.2 ± 690.8 mL WB, p < 0.001). There were no differences in cross-clamp time, bypass time, postoperative complication rate, or patient outcomes between groups. CONCLUSIONS: Del Nido cardioplegia use in an adult, reoperative aortic valve population offers equivalent postoperative outcomes when compared with whole blood cardioplegia. In addition, use of del Nido solution requires lower total and retrograde cardioplegia volumes in order to achieve adequate myocardial protection.
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Soluções Cardioplégicas , Parada Cardíaca Induzida/métodos , Idoso , Idoso de 80 Anos ou mais , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/cirurgia , Soluções Cardioplégicas/administração & dosagem , Feminino , Implante de Prótese de Valva Cardíaca , Humanos , Masculino , Pessoa de Meia-Idade , Reoperação , Estudos Retrospectivos , Resultado do TratamentoRESUMO
For children with complex congenital heart problems, cardiac allotransplantation is sometimes the best therapeutic option. However, availability of hearts for pediatric patients is limited, resulting in a long and growing waitlist, and a high mortality rate while waiting. Cardiac xenotransplantation has been proposed as one therapeutic alternative for neonates and infants, either in lieu of allotransplantation or as a bridge until an allograft becomes available. Scientific and clinical developments in xenotransplantation appear likely to permit cardiac xenotransplantation clinical trials in adults in the coming years. The ethical issues around xenotransplantation of the heart and other organs and tissues have recently been examined, but to date, only limited literature is available on the ethical issues that are attendant with pediatric heart xenotransplantation. Here, we summarize the ethical issues, focusing on (1) whether cardiac xenotransplantation should proceed in adults or children first, (2) pediatric recipient selection for initial xenotransplantation trials, (3) special problems regarding informed consent in this context, and (4) related psychosocial and public perception considerations. We conclude with specific recommendations regarding ethically informed design of pediatric heart xenotransplantation trials.
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The Norwood operation has become common practice to palliate patients with hypoplastic left heart structures. Surgical technique and postoperative care have improved; yet, there remains significant attrition prior to stage II palliation. The objective of this study is to report outcomes before and after standardizing our approach to the Norwood operation. Patients who underwent the Norwood operation at Children's of Alabama were identified, those who underwent hybrid palliation operations were excluded. Pre- (2015-2020) and post- (2020-January 2023) standardization groups were compared and outcomes analyzed. Ninety-one patients were included (pre-standardization 44 (48.3%) and 47 (51.7%) post-standardization). There were no differences in baseline and intraoperative characteristics at Norwood between the pre- and post-standardization groups. Compared with pre-standardization, post-standardization was associated with decreased time to extubation (OR 0.87, 95%CI 0.79-0.96), inotrope duration (OR 0.92, 95%CI 0.86-0.98) and hospital length of stay (OR 0.98, 95%CI 0.96-0.99). There was a trend toward decreased cardiac arrest, reintervention rates, and interstage mortality for the post-standardization group. A standardized approach to complex neonatal cardiac operations such as the Norwood procedure may improve morbidity and decrease hospital resource utilization. We recommend establishing protocols at an institutional level to optimize outcomes in such high-risk patient populations.
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We describe a hybrid thrombectomy and central extracorporeal membrane oxygenation for a child in cardiogenic shock due to a massive pulmonary embolism.
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Oxigenação por Membrana Extracorpórea , Embolia Pulmonar , Trombectomia , Humanos , Embolia Pulmonar/cirurgia , Embolia Pulmonar/terapia , Embolia Pulmonar/complicações , Oxigenação por Membrana Extracorpórea/métodos , Trombectomia/métodos , Choque Cardiogênico/etiologia , Choque Cardiogênico/terapia , Choque Cardiogênico/cirurgia , Masculino , Criança , FemininoRESUMO
OBJECTIVES: Recommendations for surgical repair of a congenital heart defect in children with trisomy 13 or trisomy 18 remain controversial, are subject to biases, and are largely unsupported with limited empirical data. This has created significant distrust and uncertainty among parents and could potentially lead to suboptimal care for patients. A working group, representing several clinical specialties involved with the care of these children, developed recommendations to assist in the decision-making process for congenital heart defect care in this population. The goal of these recommendations is to provide families and their health care teams with a framework for clinical decision making based on the literature and expert opinions. METHODS: This project was performed under the auspices of the AATS Congenital Heart Surgery Evidence-Based Medicine Taskforce. A Patient/Population, Intervention, Comparison/Control, Outcome process was used to generate preliminary statements and recommendations to address various aspects related to cardiac surgery in children with trisomy 13 or trisomy 18. Delphi methodology was then used iteratively to generate consensus among the group using a structured communication process. RESULTS: Nine recommendations were developed from a set of initial statements that arose from the Patient/Population, Intervention, Comparison/Control, Outcome process methodology following the groups' review of more than 500 articles. These recommendations were adjudicated by this group of experts using a modified Delphi process in a reproducible fashion and make up the current publication. The Class (strength) of recommendations was usually Class IIa (moderate benefit), and the overall level (quality) of evidence was level C-limited data. CONCLUSIONS: This is the first set of recommendations collated by an expert multidisciplinary group to address specific issues around indications for surgical intervention in children with trisomy 13 or trisomy 18 with congenital heart defect. Based on our analysis of recent data, we recommend that decisions should not be based solely on the presence of trisomy but, instead, should be made on a case-by-case basis, considering both the severity of the baby's heart disease as well as the presence of other anomalies. These recommendations offer a framework to assist parents and clinicians in surgical decision making for children who have trisomy 13 or trisomy 18 with congenital heart defect.
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Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Cirurgia Torácica , Lactente , Criança , Humanos , Estados Unidos , Síndrome da Trissomía do Cromossomo 18/diagnóstico , Síndrome da Trissomia do Cromossomo 13/diagnóstico , Consenso , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/cirurgiaRESUMO
We describe the hybrid Norwood as first-stage palliation for a patient with hypoplastic left heart syndrome, right aortic arch, right descending aorta, bilateral ductus arteriosus, and left innominate artery arising from the left ductus.
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Permeabilidade do Canal Arterial , Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Humanos , Aorta Torácica , Cuidados Paliativos , Artéria Pulmonar , Resultado do TratamentoRESUMO
BACKGROUND: Right-sided aortic arch obstruction is an extremely rare congenital anomaly. A variety of surgical approaches have been described. This study reviews our institutional experience over the last 30 years. METHODS: Our surgical database at the University of Alabama at Birmingham and Children's Hospital of Alabama from 1992 to 2022 was reviewed to include all patients who underwent surgical repair for right-sided aortic arch obstruction. RESULTS: A total of nine patients underwent surgical repair for right-sided aortic arch obstruction. Surgical approach was via thoracotomy (n = 2, 22%), sternotomy (n = 5, 56%), or combined (n = 2, 22%). Primary extended end-to-end anastomosis was utilized for patients with discrete coarctation (n = 1, 11%), reverse subclavian flap for coarctation with associated distal arch hypoplasia (n = 2, 22%), GORE-TEX® tube graft for circumflex aorta (n = 1, 11%), and aortic arch advancement (n = 5, 56%) with or without patch augmentation for those with an interrupted or severely hypoplastic aortic arch. Reintervention was required in one patient (11%) for recoarctation. All patients were discharged in good condition. There was no hospital mortality and at 10.5 years (mean) follow-up there was one late death. CONCLUSION: Right aortic arch obstruction is a rare entity. Surgical approach should be tailored to the anatomy and associated intracardiac defects. Preoperative imaging with a CT angiogram is useful for operative planning. Sternotomy with single-stage primary repair is safe, effective, and our preferred surgical approach for patients with right aortic arch obstruction and associated intracardiac pathology.
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Coartação Aórtica , Doenças da Aorta , Criança , Humanos , Lactente , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Aorta Torácica/anormalidades , Estudos Retrospectivos , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/cirurgia , Aorta/cirurgia , Toracotomia/métodos , Anastomose Cirúrgica , Doenças da Aorta/cirurgia , Resultado do TratamentoRESUMO
BACKGROUND: Right ventricle (RV) to pulmonary artery (PA) shunts have become the shunt of choice at many centers for use during the Norwood procedure for single ventricle palliation. Some centers have begun to use cryopreserved femoral or saphenous venous homografts as an alternative to polytetrafluoroethylene (PTFE) for shunt construction. The immunogenicity of these homografts is unknown, and potential allosensitization could have significant implications on transplant candidacy. METHODS: All patients undergoing Glenn procedure at our center between 2013 and 2020 were screened. Patients who initially underwent Norwood procedure with either PTFE or venous homograft RV-PA shunt and had available pre-Glenn serum were included in the study. The primary outcome of interest was panel reactive antibody (PRA) level at the time of Glenn surgery. RESULTS: Thirty-six patients met inclusion criteria (N = 28 PTFE, N = 8 homograft). Patients in the homograft group had significantly higher median PRA levels at the time of Glenn surgery (0% [IQR 0-18] PTFE vs 94% [IQR 74-100] homograft, P = .003). There were no other differences between the two groups. CONCLUSIONS: Despite potential improvements in PA architecture, the use of venous homografts for RV-PA shunt construction at the time of Norwood procedure is associated with significantly elevated PRA level at the time of Glenn surgery. Centers should carefully consider the use of currently available venous homografts given the high percentage of these patients who may require future transplantation.
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Procedimentos de Norwood , Veia Safena , Humanos , Transplante Homólogo , Politetrafluoretileno , AloenxertosRESUMO
BACKGROUND: The Norwood operation is a complex neonatal surgery. There are limited data to inform the timing of sternal closure. After the Norwood operation, delayed sternal closure (DSC) is frequent. We aimed to examine the association of DSC with outcomes, with a particular interest in how sternal closure at the time of surgery compared with the timing of DSC. Our outcomes included mortality, length of ventilation, length of stay, and postoperative complications. METHODS: This retrospective study included neonates who underwent a Norwood operation reported in the Pediatric Cardiac Critical Care Consortium registry from February 2019 through April 2021. Outcomes of patients with closed sternum were compared to those with sternal closure prior to postoperative day 3 (early closure) and prior to postoperative day 6 (intermediate closure). RESULTS: The incidence of DSC was 74% (500 of 674). The median duration of open sternum was 4 days (interquartile range 3-5 days). Comparing patients with closed sternum to patients with early sternal closure, there was no statistical difference in mortality rate (1.1% vs 0%) and the median hospital postoperative stay (30 days vs 31 days). Compared with closed sternum, patients with intermediate sternal closure required longer mechanical ventilation (5.9 days vs 3.9 days) and fewer subsequent sternotomies (3% vs 7.5%). CONCLUSIONS: For important outcomes following the Norwood operation there is no advantage to chest closure at the time of surgery if the chest can be closed prior to postoperative day 3.