The care of the patients with hipoplastic left heart syndrome in places of social and economic vulneability. An ethical analysis / O cuidado de pacientes com síndrome do coração esquerdo hipoplásico em localidades de vulnerabilidade social e econômica. Uma análise bioética

ABSTRACT The birth of a child means hope and joy, particularly for the parents and the healthcare team. When this child is born with a severe malformation and a poor prognosis, as in the case of hypoplastic left heart syndrome, the scenario is one of great uncertainty and emotional suffering. The role of the health team becomes fundamental for the identification of conflicts of values and for the search for shared decisions that promote the best benefit to the child. When the diagnosis is made during fetal life, it is necessary to develop counseling strategies appropriate to the context of each family. In places with limited care resources, precarious prenatal care and short temporal conditions, the recommended counseling is compromised. Indication of treatment requires technical competence and a detailed analysis of ethical issues, and consultation with institutional clinical bioethics services or commissions is important. The article proposes to address the moral conflicts of two clinical cases and the respective bioethical analysis that involves principles and values in contexts of vulnerability and uncertainty, contrasting two situations where the indication of treatment was based on accessibility to treatment.

RESUMO O nascimento de uma criança significa esperança e alegria, particularmente para os pais e para a equipe de saúde. Quando essa criança nasce com uma malformação grave e de prognóstico reservado, como acontece na SHCE, o cenário é de grande incerteza e sofrimento emocional. O papel da equipe de saúde torna-se fundamental para a identificação de conflitos de valores e para a busca de decisões compartilhadas que promovam o melhor benefício à criança. Quando o diagnóstico é feito em vida fetal, é necessário a elaboração de estratégias de aconselhamento adequadas ao contexto de cada família. Em locais com recursos assistenciais limitados, acompanhamento prénatal precário e condição temporal curta, o aconselhamento preconizado fica comprometido. A indicação do tratamento requer competência técnica e uma análise pormenorizada de questões éticas, sendo importante a consultoria aos serviços ou comissões de bioética clínica institucional. O artigo propõe a abordar os conflitos morais de dois casos clínicos e a respectiva análise bioética que envolve princípios e valores em contextos de vulnerabilidade e incertezas, contrapondo duas situações onde a indicação do tratamento baseou-se na acessibilidade ao tratamento.

Resultados Precoces do Procedimento de Norwood em um Centro de Referência no Brasil / Early Outcomes of the Norwood Procedure in a Reference Center in Brazil

Resumo Fundamento Apenas dois artigos abordam os resultados precoces de pacientes com síndrome do coração esquerdo hipoplásico (SHCE) submetidos à operação de Norwood, no Brasil. Objetivos Avaliamos pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood para identificar os fatores preditivos de mortalidade precoce (nos primeiros 30 dias após a cirurgia) e intermediária (desde a sobrevida precoce até o procedimento de Glenn). Métodos Foram incluídos pacientes com SHCE submetidos em nosso serviço ao primeiro estágio da operação de Norwood de janeiro de 2016 a abril de 2019. Dados demográficos, anatômicos e cirúrgicos foram analisados. Os desfechos foram mortalidade precoce (nos primeiros 30 dias após a cirurgia), mortalidade intermediária (desde a sobrevida precoce até o procedimento de Glenn) e a necessidade de suporte pós-operatório com ECMO. Foram realizadas análises univariadas e multivariadas e calculados odds ratios, com intervalos de confiança de 95%. Um valor de p < 0,05 foi considerado estatisticamente significativo. Resultados Um total de 80 pacientes com SHCE foram submetidos ao primeiro estágio da operação de Norwood. A taxa de sobrevida em 30 dias foi de 91,3% e a taxa de sobrevida intermediária foi de 81,3%. Quatorze pacientes (17,5%) necessitaram de suporte com ECMO. Menor peso (p=0,033), estenose aórtica (vs atresia aórtica; p=0,036) e necessidade de suporte pós-operatório com ECMO (p=0,009) foram fatores preditivos independentes para mortalidade em 30 dias. A estenose da valva mitral ( vs atresia da valva mitral; p=0,041) foi um fator preditivo independente para mortalidade intermediária. Conclusão O presente estudo inclui a maior coorte brasileira de pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood na era recente. Nossas taxas de sobrevida foram comparáveis às mais altas taxas de sobrevida relatadas globalmente. Baixo peso corporal, estenose valvar aórtica e necessidade de suporte pós-operatório com ECMO foram preditores independentes para mortalidade em 30 dias. A estenose da valva mitral foi o único fator preditivo independente para mortalidade intermediária.

Abstract Background Only two papers have addressed the early outcomes of patients with hypoplastic left heart syndrome (HLHS) undergoing the Norwood operation, in Brazil. Objectives We evaluated patients with HLHS undergoing the first-stage Norwood operation in order to identify the predictive factors for early (within the first 30 days after surgery) and intermediate (from early survival up to the Glenn procedure) mortality. Methods Patients with HLHS undergoing the stage I Norwood procedure from January 2016 through April 2019, in our service, were enrolled. Demographic, anatomical, and surgical data were analyzed. Endpoints were early mortality (within the first 30 days after surgery), intermediate mortality (from early survival up to the Glenn procedure) and the need for postoperative ECMO support. Univariate and multivariate analyses were performed, and odds ratios, with 95% confidence intervals, were calculated. A p-value <0.05 was considered statistically significant. Results A total of 80 patients with HLHS underwent the stage I Norwood procedure. The 30-day survival rate was 91.3% and the intermediate survival rate 81.3%. Fourteen patients (17.5%) required ECMO support. Lower weight (p=0.033), aortic stenosis (vs aortic atresia; p=0.036), and the need for postoperative ECMO support (p=0.009) were independent predictive factors for 30-day mortality. Mitral valve stenosis (vs mitral valve atresia; p=0.041) was an independent predictive factor for intermediate mortality. Conclusion The present study includes the largest Brazilian cohort of patients with HLHS undergoing the stage I Norwood procedure in the recent era. Our survival rates were comparable to the highest survival rates reported globally. Low body weight, aortic valve stenosis, and the need for postoperative ECMO support were independent predictors for 30-day mortality. Mitral valve stenosis was the only independent predictive factor for intermediate mortality.

Experience of one center on the Norwood stage Ⅰ procedure / 中华胸心血管外科杂志

Objective To review the clinical experience with the Norwood stage Ⅰprocedure.Methods Between June 2016 to October 2018, totally 5 neonates underwent Norwood stage Ⅰ procedure.There were 3 boys and 2 girls, weighing(2. 98 ±0.60)kg with median of 2.95 kg.Age at surgeries ranged from 1 to 8 days.All 5 cases underwent the Norwood stage Ⅰprocedure under deep hypothermic circulatory arrest, including 3 cases of modified Blalock-Taussig shunts (MBTS) and 2 ca-ses of RV-PA shunt.Results The third case was successfully closed the chest on postoperative day 2 and extubated, but died from DIC due to severe infection on the postoperative day 6.The fifth case died from pericardiol tamponade at 10 hours after the operation.The first,second and fourth cases were followed up 1 month after discharge with NYHA Ⅰ, but the first and second cases dropped out of follow-up due to some personal resaons.The fourth case underwent the bidirectional Glenn procedure 9 monthes after the stage Ⅰ procedure and recovered smoothly.Conclusion The application of these modified methods and te-chiques based on the classical Norwood procedure help to accumulate experience in sugical treatment of HLHS in China .

Extracardiac Conduit Fontan Operation with Reduction Aortoplasty for Left Pulmonary Artery Compression after a Norwood Procedure in a Patient with Double-Inlet Left Ventricle / 대한흉부외과학회지

Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure of the neoaorta to systemic pressure, which may induce adverse effects on adjacent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure.

Reflection of the perioperative managements of Norwood stage Ⅰ procedure for hypoplastic left heart syndrome / 中华胸心血管外科杂志

Objective Norwood Stage Ⅰ is the standard procedure to cope with hypoplastic left heart syndrome (HLHS),which continues to be the most challenging congenital heart disease.The aim of this study is to retrospectively compare the classical perioperative management of Norwood Stage] with the modified strategy.Methods Between June 2010 and November 2017,totally 10 patients with HLHS underwent the standard Norwood Stage Ⅰ procedure.They are stratified to two tiers:Group A,from June 2010 to August 2014,there were 5 boys.Age at surgeries ranged from 29 to 75 days,and weight 2.57-3.50 kg with median of 3.13 kg.Group B,from August 2014 to November 2017,there were 4 boys and 1 girl.Age at surgeries ranged from 6 to 22 days,and weight from 2.0-3.1 kg.In Group A,all 5 cases underwent the standard Norwood Stage.procedure under deep hypothermic circulatory arrest,including 4 cases of modified Blalock-Taussig shunt (MBTS) and 1 case of RV-PA shunt.In Group B,all 5 cases adopted side graft technique and RV-PA shunt,aortic arch and ascending aorta were reconstructed with treated bovine pericardial patch.Group B used incubators to adjust systemic vascular resistance instead of vasodilators.Results Group A's early mortality is 40％;Group B's early mortality is 20％,1 case died of tamponade.Conclusion The standard Norwood Stage Ⅰ procedure is a complex procedure,which demands multidisplinary cooperation,to palliatively correct HLHS.To adjust and find a suitable perioperative managements can improve the results.Sharing experiences on perioperative managements of Norwood Stage Ⅰ between heart centers in China will be helpful to decrease the mortality and morbidity in relatively short period.

Early experience on the standard Norwood stageⅠprocedure for hypoplastic left heart syndrome / 中华胸心血管外科杂志

Objective Norwood StageⅠis the standard procedure to cope with hypoplastic left heart syndrome(HLHS), which continues to be the most challenging congenital heart disease .The aim of this study is to retrospectively analyse the perio-perative management of Norwood StageⅠ.Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood StageⅠprocedure.They were all boys.Age at surgeries ranged from 29 to 75 days, and weight from 2.57-3.50 kg with median of 3.13 kg.Case 1, 2 and 3 received standard medical regimen after accessing NICU, which included intravenous prostaglandin E1 and mechanical ventilation.The 3 infants underwent emergent operations because of unstable hemodynamics.Case 4 and 5 received no medical intervention before the urgent surgeries.All 5 cases underwent the standard Norwood StageⅠprocedure under deep hypothermic circulatory arrest, including 4 cases of modified Blalock-Taus-sig shunt( MBTS) and 1 case of Sano shunt.Results The case with Sano shunt died from severe hypoxemia and persistent aci-dosis 32 hours after the operation, another case died from low cardiac output syndrome after cardiopulmonary bypass.The first case underwent bidirectional Glenn procedure 12 months after Norwood Stage I, the postoperative heart function was NYHA I and the oxygen saturation was 0.90-0.95 in room air, but he died from accidental brain injury 3 months after stage Ⅱ.The second case was followed up 3 months after stage I procedure with NYHA I and oxygen saturation of 0.78-0.83 in room air, and lost the follow-up after.The fifth case was followed up 3 months after stageⅠprocedure with NYHAⅠ, confluent MBTS and oxygen saturation of 0.84, the patient is being followed up and waiting for further evaluation for stageⅡprocedures.Con-clusion The standard Norwood StageⅠprocedure is a complex procedure, which demands multidisplinary cooperation, to pal-liatively correct HLHS .Sharing expericences on perioperative managements of Norwood Stage I between heart centers in China will be helpful to decrease the mortality and morbidity in relatively short period .

Anesthetic management of the standard Norwood stage Ⅰ procedure for hypoplastic left heart syndrome / 中华胸心血管外科杂志

Objective To retrospectively analyse the anaesthetic management of Norwood Stage Ⅰ.Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood Stage I procedure .They were all boys.Age at surgeries ranged from 29 to 75 days with median 36 days, and weight from 2.57 -3.50 kg with median of 3.13kg.The first three cases were received intravenous prostaglandin E1 before they were sent to the operation theatre and were under mechanical ventilation .They were received emergent operations because of unstable hemodynamic situation .The other two cases were relatively stable without mechanical ventilation and were received restrict surgery .All 5 cases received the stand-ard Norwood Stage Ⅰprocedure under intravenous-inhalation balance-general anaesthesia with cardiopulmonary bypass.The technique of deep hypothermia and circulatory arrest were used in all five cases .Results The fourth case died from low cardi-ac output syndrome after cardiopulmonary bypass .The other 4 cases were transferred to the paediatric intensive care unit after withdrawal from bypass.One of the four cases died after 32 h after surgery.Conclusion The standard Norwood Stage Ⅰ pro-cedure is aquite complex procedure, which demands multidisplinary cooperation, to palliatively correct HLHS.We retrospect the experiences of the anesthetic management in our centre and hope it will be helpful to decrease the mortality and morbidity in relatively short period.

Translocation of the Aortic Arch with Norwood Procedure for Hypoplastic Left Heart Syndrome Variant with Circumflex Retroesophageal Aortic Arch

Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.