Impact of Medical Interventions and Comorbidities on Norwood Admission for Patients with Hypoplastic Left Heart Syndrome.

The purpose of these analyses was to determine how specific comorbidities and medical interventions impact risk of inpatient mortality in those with hypoplastic left heart syndrome undergoing Norwood procedure. The secondary aims were to determine the impact of these on billed charges, postoperative length of stay, and risk of cardiac arrest. Admissions from 2004 to 2015 in the Pediatric Health Information System database with hypoplastic left heart syndrome and Norwood procedure were identified. Admission characteristics, patient interventions, and the presence of comorbidities were captured. A total of 5,138 admissions were identified meeting inclusion criteria. Of these 829 (16.1%) experienced inpatient mortality, and 352 (6.7%) experienced cardiac arrest. The frequency of inpatient mortality did not significantly change over the course of the study era. The frequency of cardiac arrest significantly decreased from 7.4% in 2004 to 4.3% in 2015 (p = 0.04). The frequency of pharmacologic therapies, particularly vasoactive use, decreased as the study period progressed. Regression analyses demonstrated a significant association between cardiac arrest and inpatient mortality with arrhythmias, acute kidney injury, and pulmonary hypertension. Similarly, regression analyses demonstrated a significant association between increase in billed charges and length of stay with year of surgery, presence of heart failure, syndromes, and acute kidney injury. For patients with hypoplastic left heart syndrome undergoing the Norwood procedure, the frequency of pharmacologic therapies and cardiac arrest has decreased over time. There are significant associations between acute kidney injury, arrythmias, and pulmonary hypertension with cardiac arrest and mortality.

Management Options and Outcomes for Neonatal Hypoplastic Left Heart Syndrome in the Early Twenty-First Century.

Without surgical treatment, neonatal hypoplastic left heart syndrome (HLHS) mortality in the first year of life exceeds 90 % and, in spite of improved surgical outcomes, many families still opt for non-surgical management. The purpose of this study was to investigate trends in neonatal HLHS management and to identify characteristics of patients who did not undergo surgical palliation. Neonates with HLHS were identified from a serial cross-sectional analysis using the Healthcare Cost and Utilization Project's Kids' Inpatient Database from 2000 to 2012. The primary analysis compared children undergoing surgical palliation to those discharged alive without surgery using a binary logistic regression model. Multivariate logistic regression was conducted to determine factors associated with treatment choice. A total of 1750 patients underwent analysis. Overall hospital mortality decreased from 35.3 % in 2000 to 22.9 % in 2012. The percentage of patients undergoing comfort care discharge without surgery also decreased from 21.2 to 14.8 %. After controlling for demographics and comorbidities, older patients at presentation were less likely to undergo surgery (OR 0.93, 0.91-0.96), and patients in 2012 were more likely to undergo surgery compared to those in prior years (OR 1.5, 1.1-2.1). Discharge without surgical intervention is decreasing with a 30 % reduction between 2000 and 2012. Given the improvement in surgical outcomes, further dialogue about ethical justification of non-operative comfort or palliative care is warranted. In the meantime, clinicians should present families with surgical outcome data and recommend intervention, while supporting their option to refuse.

Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial.

BACKGROUND: In the Single Ventricle Reconstruction (SVR) trial, 1-year transplantation-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock-Taussig shunt (MBTS). At 3 years, we compared transplantation-free survival, echocardiographic right ventricular ejection fraction, and unplanned interventions in the treatment groups. METHODS AND RESULTS: Vital status and medical history were ascertained from annual medical records, death indexes, and phone interviews. The cohort included 549 patients randomized and treated in the SVR trial. Transplantation-free survival for the RVPAS versus MBTS groups did not differ at 3 years (67% versus 61%; P=0.15) or with all available follow-up of 4.8±1.1 years (log-rank P=0.14). Pre-Fontan right ventricular ejection fraction was lower in the RVPAS group than in the MBTS group (41.7±5.1% versus 44.7±6.0%; P=0.007), and right ventricular ejection fraction deteriorated in RVPAS (P=0.004) but not MBTS (P=0.40) subjects (pre-Fontan minus 14-month mean, -3.25±8.24% versus 0.99±8.80%; P=0.009). The RVPAS versus MBTS treatment effect had nonproportional hazards (P=0.004); the hazard ratio favored the RVPAS before 5 months (hazard ratio=0.63; 95% confidence interval, 0.45-0.88) but the MBTS beyond 1 year (hazard ratio=2.22; 95% confidence interval, 1.07-4.62). By 3 years, RVPAS subjects had a higher incidence of catheter interventions (P<0.001) with an increasing HR over time (P=0.005): <5 months, 1.14 (95% confidence interval, 0.81-1.60); from 5 months to 1 year, 1.94 (95% confidence interval, 1.02-3.69); and >1 year, 2.48 (95% confidence interval, 1.28-4.80). CONCLUSIONS: By 3 years, the Norwood procedure with RVPAS compared with MBTS was no longer associated with superior transplantation-free survival. Moreover, RVPAS subjects had slightly worse right ventricular ejection fraction and underwent more catheter interventions with increasing hazard ratio over time. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.

Patients with single ventricle physiology undergoing noncardiac surgery are at high risk for adverse events.

BACKGROUND: Patients with single ventricle physiology are at increased anesthetic risk when undergoing noncardiac surgery. OBJECTIVE: To review the outcomes of anesthetics for patients with single ventricle physiology undergoing noncardiac surgery. METHODS: This study is a retrospective chart review of all patients who underwent a palliative procedure for single ventricle physiology between January 1, 2007 and January 31, 2014. Anesthetic and surgical records were reviewed for noncardiac operations that required sedation or general anesthesia. Any noncardiac operation occurring prior to completion of a bidirectional Glenn procedure was included. Diagnostic procedures, including cardiac catheterization, insertion of permanent pacemaker, and procedures performed in the ICU, were excluded. RESULTS: During the review period, 417 patients with single ventricle physiology had initial palliation. Of these, 70 patients (16.7%) underwent 102 anesthetics for 121 noncardiac procedures. The noncardiac procedures included line insertion (n = 23); minor surgical procedures such as percutaneous endoscopic gastrostomy or airway surgery (n = 38); or major surgical procedures including intra-abdominal and thoracic operations (n = 41). These interventions occurred on median day 60 of life (1-233 days). The procedures occurred most commonly in the operating room (n = 79, 77.5%). Patients' median weight was 3.4 kg (2.4-15 kg) at time of noncardiac intervention. In 102 anesthetics, 26 patients had an endotracheal tube or tracheostomy in situ, 57 patients underwent endotracheal intubation, and 19 patients had a natural or mask airway. An intravenous induction was performed in 77 anesthetics, an inhalational induction in 17, and a combination technique in 8. The median total anesthetic time was 126 min (14-594 min). In 22 anesthetics (21.6%), patients were on inotropic support upon arrival; an additional 24 patients required inotropic support (23.5%), of which dopamine was the most common medication. There were 10 intraoperative adverse events (9.8%) including: arrhythmias requiring treatment (n = 4), conversion from sedation to a general anesthetic (n = 2), difficult airway (n = 1), inadvertent extubation with desaturation and bradycardia (n = 1), hypotension and desaturation (n = 1), and cardiac arrest (n = 1). Postoperative events (<48 h) included ST segment changes requiring cardiac catheterization (n = 1), and cardiorespiratory arrest (n = 1). Age, size, gender, type of cardiac palliation, patient location, procedure location, and type of procedure were not associated with adverse outcome. After 62 anesthetics (60.8%), patients went postoperatively to the cardiac ICU. There were no deaths at 48 h. CONCLUSION: We observed no mortality during or after noncardiac surgery in a high-risk subgroup of palliated cardiac patients with single ventricle physiology. However, 11.8% of patients had an adverse event associated with their anesthetic.

Acute effects of vasoactive medications in patients with parallel circulation awaiting hybrid or Norwood procedure.

Background: Vasoactive medications are frequently used in the preoperative stage to balance the pulmonary and systemic blood flow. However, not much is known about the effects of these agents during this stage. Aims: The primary objective of this study was to characterize the acute effects of vasoactive medications in children with parallel circulation before either the hybrid or Norwood procedure. Setting and Designs: This is a single-center, cross-sectional, retrospective study. Methods: Hemodynamic and systemic oxygen delivery data were captured from patients' vital signs, arterial blood gases, near-infrared spectroscopy monitoring (NIRS). Data for each patient were collected before the initiation of a vasoactive medication and again 6 h after. Statistical Analysis: Data were analyzed using paired t-tests, and analysis of covariance. Results: A total of 139 patients were identified. After data extraction the following patients were included before the initial intervention: 7 were on milrinone, 22 were on dopamine, and 17 were on dobutamine. Dopamine and dobutamine were found to significantly increase systolic blood pressure. Only dopamine increased pH (mean difference 0.04), decreased paCO2 (mean difference -7.1), decreased lactate (mean difference -0.6 mmol/L), and decreased in bedside Qp: Qs (mean difference -7.5) after continuous infusion for 6 h. Milrinone was not associated with any significant hemodynamic change. Conclusion: In this study, dopamine was independently associated with improvement in markers of systemic oxygen delivery 6 h after initiation. Dobutamine and dopamine were associated with increased in blood pressure. Well-powered studies are required to detect changes in lactate and NIRS.

Improved outcomes in neonates who require venoarterial extracorporeal membrane oxygenation after the Norwood procedure.

BACKGROUND: Extracorporeal membrane oxygenation after the Norwood procedure has historically been associated with poor outcomes, with reported hospital survival rates of 13%-48%. We hypothesized that contemporary outcomes in this population have improved. We aimed to compare clinical outcomes of contemporary cohorts of patients with functional single ventricle physiology who did and did not receive extracorporeal membrane oxygenation after the Norwood procedure. METHODS: Single-center retrospective cohort study of patients with single ventricle anatomy who underwent the Norwood procedure between 2009 and 2017 was performed. Kaplan-Meier survival curves were constructed, and Cox proportional hazard regression analyses were performed to compare transplant-free survival in patients who did and did not receive venoarterial extracorporeal membrane oxygenation. RESULTS: In total, 85 patients met inclusion criteria. Venoarterial extracorporeal membrane oxygenation was utilized in 25 patients (29%). A total of 18 patients (72%) who received venoarterial extracorporeal membrane oxygenation survived to hospital discharge, compared to 54 patients (92%) who did not receive venoarterial extracorporeal membrane oxygenation (p = 0.013). Post-discharge transplant-free survival was not significantly different between patients who did and did not receive venoarterial extracorporeal membrane oxygenation (log-rank p value = 0.28). Cox proportional hazard regression analysis revealed that the occurrence of cardiac arrest requiring cardiopulmonary resuscitation (hazard ratio = 4.5; 95% confidence interval = 2.0-10.1) during the perioperative period was independently associated with death or transplantation, whereas venoarterial extracorporeal membrane oxygenation was not an independent risk factor for death or transplantation (hazard ratio = 2.0; 95% confidence interval = 0.8-4.9). CONCLUSION: In our cohort of children who received venoarterial extracorporeal membrane oxygenation after the Norwood procedure, hospital survival was improved compared to historical data. In addition, venoarterial extracorporeal membrane oxygenation utilization was not independently associated with worse outcomes.

Impact of Initial Shunt Type on Echocardiographic Indices in Children After Single Right Ventricle Palliations.

Background Heart size and function in children with single right ventricle (RV) anomalies may be influenced by shunt type at the Norwood procedure. We sought to identify shunt-related differences during early childhood after staged surgical palliations using echocardiography. Methods We compared echocardiographic indices of RV, neoaortic, and tricuspid valve size and function at 14 months, pre-Fontan, and 6 years in 241 subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt or RV-to-pulmonary-artery shunt. Results At 6 years, the shunt groups did not differ significantly in any measure except for increased indexed neoaortic area in the modified Blalock-Taussig shunt. RV ejection fraction improved between pre-Fontan and 6 years in the RV-to-pulmonary artery shunt group but was stable in the modified Blalock-Taussig shunt group. For the entire cohort, RV diastolic and systolic size and functional indices were improved at 6 years compared with earlier measurements, and indexed tricuspid and neoaortic annular area decreased from 14 months to 6 years. The prevalence of ≥moderate tricuspid and neoaortic regurgitation was uncommon and did not vary by group or time period. Diminished RV ejection fraction at the 14-month study was predictive of late death/transplant; the hazard of late death/transplant when RV ejection fraction was <40% was tripled (hazard ratio, 3.18; 95% CI, 1.41-7.17). Conclusions By 6 years after staged palliation, shunt type has not impacted RV size and function, and RV and valvar size and function show beneficial remodeling. Poor RV systolic function at 14 months predicts worse late survival independent of the initial shunt type. Clinical Trial Registration URL: https://www.clinicaltrials.gov . Unique identifier: NCT00115934.

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

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.

Early results of aortic arch reconstruction and bilateral pulmonary artery banding: modification of the Norwood operation for treatment of hypoplastic left heart syndrome.

BACKGROUND: In the period from 2003 to the end of 2015, 96 Norwood I procedures were performed in the Paediatric Heart Surgery Department in Katowice, Poland, in children with hypoplastic left heart syndrome (HLHS). AIM: This paper presents a retrospective analysis of early surgical results. METHODS: The patients consisted of two groups: group I included 59 children operated on in the years 2003-2012, in whom the stage I Norwood procedure with the Sano modification was performed with the aortic arch reconstructed by use of a ho-mogenous pulmonary artery patch or a bovine pericardial patch. Group II included 37 children after our modification of the Norwood I procedure, in which the aortic arch was reconstructed with an extracellular matrix patch and bilateral pulmonary artery banding was done. RESULTS: Aortic cross-clamping time was significantly shorter in group II (mean 52; range 38-62 min) than in group I (mean 57; range 39-72 min; p < 0.009). Eighteen (30.5%) children in group I and six (16.2%) in group II died. Although this dif-ference did not reach statistical significance (p = 0.12), it suggested that improved outcomes with the modified procedure are possible. The cause of death in group I was significantly more frequently due to massive postoperative bleeding (n = 6; 33.3%) than in group II (n = 1; 16.7%; p = 0.046). CONCLUSIONS: The introduction of this new surgical technique reduced postoperative bleeding rates, shortened the operation time, and might improve the mortality rate in the first-stage surgical treatment of children with HLHS.

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.

Unplanned cardiac reoperations and interventions during long-term follow-up after the Norwood procedure†.

OBJECTIVES: Patients with hypoplastic left heart syndrome or related malformations are predominantly treated with a 3-stage palliation. Anatomic or physiologic problems can lead to unplanned additional surgical or catheter interventions during single ventricle palliation. Changes in operative technique may have an impact on the reoperation rate. METHODS: Between 1997 and 2014, 317 Norwood procedures were performed at our centre. A retrospective single centre investigation was carried out concerning incidence, timing, indication and type of unplanned interstage cardiac reoperations and catheter interventions during follow-up of Norwood patients. Patients were followed from birth until the end of 2015. Cardiac procedures taking place at the time of the bidirectional Glenn or Fontan procedure or heart transplantation were not included. RESULTS: Sixty-five of the Norwood patients (20.5%) had at least one additional surgical cardiac procedure. Nine patients (2.8%) needed open procedures prior to the Norwood operations, 11.0% had procedures in the interstage I, 3.5% in the interstage II and 9.1% of the Fontan patients had cardiac reoperations afterwards. Main indications for unplanned surgery were insufficient pulmonary perfusion and tricuspid regurgitation. Eighty-one patients (25.6%) had at least one interstage catheter intervention during follow-up mainly addressing stenosis of the pulmonary arteries, aortic arch stenosis or aortopulmonary collaterals. CONCLUSIONS: The number of unplanned reoperations and interventions during staged palliation is remarkably high showing surgical peaks in the interstage I and after the Fontan procedure and an interventional peak in the interstage II. Thorough early information of the parents about possibly anticipated additional procedures is necessary.

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.

Older age at the time of the Norwood procedure is a risk factor for early postoperative mortality†.

OBJECTIVES: The Norwood procedure is commonly used as first-step palliation in children with hypoplastic left heart syndrome or related congenital malformations. In most cases, the operation is carried out during the first week of life. Excessive pulmonary blood flow in univentricular physiology can soon lead to compromised cardiac function and pulmonary hypertension and could have an influence on postoperative outcomes. METHODS: Between 1997 and 2011, 257 Norwood procedures were performed at the Kinderherz-Zentrum Linz. Twenty-seven patients were older than 20 days at the time of the Norwood operation without previous pulmonary artery banding. A retrospective analysis of the two age groups was carried out concerning early mortality, interstage mortality and major events until the bidirectional Glenn operation. RESULTS: Patients older than 20 days had a significantly higher mortality rate before the bidirectional Glenn operation than younger patients (44.4 vs 20.3%; P = 0.002). They also showed a significantly higher need for postoperative inhalative nitric oxide therapy (40.7 vs 14.5%, P = 0.002). Summarized risk for either successful postoperative cardiopulmonary resuscitation (CPR), extracorporeal membrane oxygenation therapy or mortality before the bidirectional Glenn operation was 59.3% in the older age group vs 23.8% in the younger age group (P < 0.001). CONCLUSIONS: Age older than 20 days seems to be an independent risk factor for early postoperative mortality and fatal events after the Norwood procedure. Long-persisting excessive pulmonary blood flow and preoperative cardiac decompensation can be the reason for postoperative lethal pulmonary hypertensive crisis and compromised ventricular function.

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.