Prenatal diagnosis of hypoplastic left heart syndrome: current knowledge / Diagnóstico pré-natal da síndrome hipoplásica do coração esquerdo: conhecimentos atuais

Abstract Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the left-sided heart structures. The prenatal diagnosis of this congenital heart disease is crucial because a newborn with undiagnosed HLHS often presents with clinical signs of low cardiac output once the ductus arteriosus begins to close. With that in mind, the aim of this article was to perform a non-systematic review focusing on the key ultrasound features that can be used in the prenatal diagnosis of HLHS. Severe forms of HLHS are characterized by a markedly abnormal four-chamber view of the fetal heart (small left atrium, hypoplastic left ventricle, or abnormal mitral valve). The left ventricular outflow tract view allows the degree of hypoplasia in the tract to be evaluated and the diameter of the ascending aorta to be measured. The Z-scores are intended to aid in the diagnosis and follow-up of HLHS. In mild forms of HLHS, a right ventricle/left ventricle length ratio > 1.28 was the strongest predictor of a univentricular outcome.

Resumo A síndrome do coração esquerdo hipoplásico (SCEH) é caracterizada pelo subdesenvolvimento das estruturas cardíacas do lado esquerdo. O diagnóstico pré-natal dessa cardiopatia congênita é crucial, uma vez que recém-nascido com SCEH não diagnosticado apresenta, frequentemente, sinais clínicos de baixo débito cardíaco, quando o canal arterial começa a se fechar. Por isso, o objetivo deste artigo foi realizar uma revisão não sistemática sobre as principais características ultrassonográficas que podem ser usadas no diagnóstico pré-natal da SCEH. As formas graves de SCEH são caracterizadas por plano de quatro câmaras marcadamente anormal (átrio esquerdo pequeno, ventrículo esquerdo hipoplásico, válvula mitral anormal) do coração fetal. A visualização da via de saída do ventrículo esquerdo permite a avaliação do grau de hipoplasia dessa via e a mensuração da aorta ascendente. Os escores Z têm como objetivo auxiliar no diagnóstico e acompanhamento da SCEH. Nas formas leves da SCEH, a relação comprimento do ventrículo direito/comprimento do ventrículo esquerdo > 1,28 foi a variável mais forte para identificar o desfecho univentricular.

Use of Extracorporeal Membrane Oxygenation and Cerebral Oximetry in a Stage 1 Norwood Repair for Hypoplastic Left Heart Syndrome

The role of extracorporeal membrane oxygenation (ECMO) and its indications in stage I Norwood palliation are controversial. The decision to initiate ECMO and its timing remains difficult with no definitive cut?off points or evidence?based guidelines. It varies on a case?to?case basis. We report a case where the use of ECMO was beneficial after stage I Norwood palliation with severe ventricular dysfunction. The systemic?to?pulmonary artery shunt was kept open to balance the systemic and pulmonary circulations. Cerebral oximetry can be useful as an additional monitoring modality to guide management, monitor cerebral perfusion, and help detect cerebral steal.

Associated Factors with Congenital Heart Disease in the Most Populated State of Brazil Between 2010 and 2018

Abstract Background Cardiac anomalies are the most prevalent congenital malformations among live births in the world. In Brazil, it is estimated that nearly 25,757 new cases occur each year, and the Southeast region presents the highest prevalence, with 10 new cases/1,000 live births. Objective The aim of this study is to evaluate the epidemiology of congenital heart disease (CHD) in the state of São Paulo. Methods This is a cross-sectional and time-series observational study with data from the Brazilian Information System on Live Births. Heart-related cardiac malformation cases — International Classification of Diseases (ICD) 10th revision Q20.0 to Q24.9 — were selected from January 2010 to December 2018, in the state of São Paulo, Brazil. This study analyzed rates of malformations per year and investigated associated factors, using single and multiple logistic regression models. The significance level adopted in this study was 5%. Results The highest cardiac malformation rate was in the São Paulo metropolitan region (2.84:1000), while the lowest was found in the region of Franca (0.3:1000). The most frequent defect was interatrial communication (38.2%). The main associated factors observed in this study were prematurity of 22 to 27 weeks (OR=4,401 95% CI: 3,796-5,104) — CI: Confidence Interval; OR: Odds ratio —, mother's age between 35 and 49 years of age (OR=1,602 95% CI: 1,525-1,682), yellow race (OR=1,481 95% CI: 1,235-1,775), triple or more pregnancy (OR=1,438 95% CI: 1,004-2,060), and history of a dead child (OR=1,213 95% CI: 1,152-1,277). Conclusion The main factors associated with this outcome, which are part of the obstetric history of mothers, should be addressed and considered when pregnancy is a planned event.

A Systematic Review of Benefits and Risks of Fetal Surgery for Congenital Cardiac Defects Such as Pulmonary Valve Stenosis and Critical Aortic Stenosis

ABSTRACT Introduction: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. Methods: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. Results: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. Conclusion: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.

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.

Spinal Anesthesia for Open Gastrostomy in an Infant after Stage I Norwood for Hypoplastic Left Heart

Infants with hypoplastic left heart are at increased risk of adverse events including mortality when they undergo procedures with general anesthesia in the inter?stage period after stage I Norwood. This is primarily caused by an imbalance between pulmonary and systemic blood flows augmented by decreased function of the single ventricle. These factors can be aggravated by general anesthesia, hence the increased risk. Many of these infants experience feeding dysfunction and require a gastrostomy to optimize nutrition. We report a case of open gastrostomy in an infant with Norwood physiology under spinal anesthesia with an excellent outcome.

Manejo anestésico en síndrome corazón izquierdo hipoplásico: Reporte de un caso / Anesthetic management in hypoplastic left heart syndrome: A case report

Introducción: El síndrome corazón izquierdo hipoplásico puede presentarse con signos clínicos inmediato al nacimiento, siendo responsable de las muertes neonatales en la primera semana de vida. El reconocimiento clínico y los hallazgos ecocardiográficos son claves para un diagnóstico y tratamiento oportuno. Reporte de caso: describe el manejo anestésico de un neonato con inestabilidad hemodinámica que requirió cirugía de emergencia con banding pulmonar bilateral para mantener el equilibrio entre la relación del flujo sanguíneo pulmonar y sistémico cercano a la unidad, junto a maniobras de ventilación mecánica, medicamentos anestésicos, inotrópicos y adyuvantes con el objetivo de manipular las resistencias vasculares y el flujo sanguíneo logrando estabilidad hemodinámica del paciente que permitió al quinto día ser sometido a una cirugía electiva de Norwood Sano, tolerando procedimiento con extubación a los 15 días de la cirugía. Conclusión: el momento oportuno de la indicación quirúrgica aumenta la sobrevida de los pacientes síndrome corazón izquierdo hipoplásico.

Background: Hypoplastic left heart syndrome may appear with clinical signs immediately at birth, being responsible for neonatal deaths within the first week of life. Clinical recognition and echocardiographic findings are key to timely diagnosis and treatment. Case report: describes the anesthetic procedure of a neonate with hemodynamic instability who required emergency surgery for bilateral pulmonary banding to maintain the balance between pulmonary and near-unit systemic flow ratio, along with mechanical ventilation maneuvers, anesthetic, inotropic and adjuvant drugs to control vascular resistance and blood flow, thus achieving hemodynamic stability of the patient, which allowed him to undergo an elective Norwood Sano procedure on the fifth day, and which was tolerated with extubation 15 days after surgery. Conclusion: The right timing of the surgical indication increases the survival of patients with hypoplastic left heart syndrome.

The study on brain biometry and cerebral hemodynamics in fetuses with hypoplastic left heart syndrome in the second trimester of gestation / 中华超声影像学杂志

Objective:To explore the changes of biparietal diameter, head circumference and cerebrovascular hemodynamics in fetuses with hypoplastic left heart syndrome (HLHS) during middle pregnancy.Methods:The biparietal diameter, head circumference, middle cerebral artery pulsatility index (MCA-PI), umbilical artery pulsatility index (UA-PI) and MCA-PI/UA-PI (CPR) of 41 fetuses with HLHS(HLHS group) were retrospectively analyzed from January 2015 to December 2019 in Beijing Anzhen Hospital, and were compared with those of 82 normal fetuses matched for gestational age at the same period (control group).Results:The Z-scores of head circumference, MCA-PI and CPR in with HLHS group were lower than in control group(all P<0.05); Head circumference in HLHS group were weakly and positively correlated with the MCA-PI and CPR ( r=0.385, 0.416; all P<0.05). Conclusions:There are some changes in the head circumference and cerebral hemodynamics in fetuses with HLHS during mid-gestational age, and the head circumference is weakly and positively correlated with MCA-PI and CPR, which has clinical significance.

Diagnóstico Prenatal de Fibroelastosis Endocardica

Resumen La fibroelastosis endocárdica es una cardiopatía congénita caracterizada por presentar un engrosamiento difuso del endocardio, esto condiciona una miocardiopatía dilatada en la mayoría de los casos o menos frecuente una miocardiopatía restrictiva. Puede ser una patología primaria o secundaria a otras malformaciones cardíacas, sobre todo a una estenosis o atresia de la aorta. Se estima que la incidencia global al nacimiento es de 1 de cada 5 000 y de 4 por cada 100 cardiopatías congénitas. Se presenta el caso clínico de una gestante de 37 años de edad, quien fuera ingresada a las 19.1 semanas de gravidez en el Hospital Obrero N°2 de Cochabamba-Bolivia, por el diagnóstico ecográfico prenatal de cardiopatía fetal estructural, fibroelastosis endocárdica.

Abstract Endocardial fibroelastosis is a congenital heart disease characterized by diffuse thickening of the endocardium, this conditions a dilated cardiomyopathy in most cases or less frequent restrictive cardiomyopathy. It can be a primary pathology or secondary to other cardiac malformations, especially a stenosis or atresia of the aorta. The global incidence at birth is estimated to be 1 in 5 000 and 4 in 100 congenital heart disease. The clinical case of a 37-year-old pregnant woman is presented, who was admitted at 19.1 weeks of pregnancy in Hospital Obrero N ° 2 in Cochabamba-Bolivia, due to the prenatal ultrasound diagnosis of structural fetal heart disease, endocardial fibroelastosis.

Extubación fallida postoperación de Norwood en pacientes con síndrome de hipoplasia de ventrículo izquierdo / Extubation failure after Norwood procedure in hypoplastic left heart syndrome patients

Resumen: Introducción: En pacientes con Síndrome de Hipoplasia de Ventrículo Izquierdo (SHVI) la primera etapa hacia una fisiología univentricular de Fontan es la operación de Norwood, cuya mortalidad es 10-30%. En estos pacientes la extubación fallida se presenta en un 18% y se ha asociado a aumento de la mortali dad. Objetivo: Describir la frecuencia de extubación fallida y sus factores de riesgo en pacientes con SHVI operados (Norwood). Pacientes y Método: Estudio de casos y controles que incluyó a todos los pacientes con SHVI manejados con cirugía de Norwood en el Hospital Clínico de la Pontificia Universidad Católica (enero-2000 a febrero-2018). Se define como casos a pacientes con falla en la extubación y como controles a los pacientes sin esta complicación. Se registraron variables demográficas, quirúrgicas, y post-quirúrgicas, y se realizó análisis univariado y multivariado (regresión logística) para determinar los factores de riesgo asociados a la falla en la extubación. Resultados: De un total de 107 pacientes, 26 pacientes tuvieron extubación fallida (24,3%). En el análisis univariado los factores asociados a extubación fallida fueron: mayor tiempo de ventilación mecánica postquirúrgico, desarrollar atelectasias, derrame pleural, quilotórax, tener otras comorbilidades respiratorias (apnea y traqueítis), y mayor tiempo de uso de morfina y midazolam. En el análisis multivariado, la presencia de quilotórax, otras comorbilidades respiratorias, y mayor tiempo de uso de midazolam fueron variables asociadas a extubación fallida (p<0,03). La extubación fallida no se asoció a mayor mortalidad. Conclusiones: La presencia de quilotórax, complicaciones respiratorias y uso de mida zolam prolongado deben considerarse para definir el momento de la extubación, con el objetivo de evitar su fracaso.

Abstract: Introduction: Hypoplastic left heart syndrome (HLHS) is the main cause of mortality due to congenital heart disea se. The Norwood surgery is the first procedure of the surgical staging process towards a single ventri cle physiology or Fontan-type operation and has a mortality rate of 10% to 30%. Extubation failure during the postoperative period occurs in up to 18% of these patients and is associated with increased mortality. Objective: To describe extubation failure rates and risk factors in pediatric patients with HLHS who underwent Norwood procedure. Patients and Method: Case-control study that included all the patients with HLHS managed with Norwood surgery at the Hospital Clínico de la Pontificia Universidad Catolica between January 2000 and February 2018. Cases and controls were defined as patients with extubation failure and as patients without this complication, respectively. The following variables were recorded demographic, surgical, and post-surgical ones, and univariate and multivariate analyses (logistic regression) were performed to determine risk factors associated with extubation failure. Results: Out of 107 patients, 26 of them presented extubation failure (24.3%). In the univariate analysis, longer mechanical ventilation time during the postsurgical period, atelectasis, pleural effusion, chylothorax, other respiratory morbidities (i.e. apneas and tracheitis), and longer infusion times of morphine and midazolam, all were associated with a higher extubation failure rate in this population. In the multivariable analysis, chylothorax, other respiratory comorbidities, and longer infusion time of midazolam remained associated with this complication, however, it was not associated with higher mortality. Conclusions: Chylothorax, respiratory comorbidities, and longer use of Midazolam should be addressed before planning airway extubation in order to avoid failure.

Emergency Pulmonary Artery–to-Systemic Artery Shunt to Break the Positive Feedback Loop of a Pulmonary Hypertensive Crisis after Neonatal Coarctation Repair / 대한흉부외과학회지

A 2.5-kg neonate with coarctation of the aorta and a small left ventricle experienced a severe pulmonary hypertensive crisis. An emergency pulmonary artery-to-systemic artery shunt was placed to break the positive feedback loop caused by pulmonary hypertension and functional mitral stenosis. This shunt provided immediate relief of suprasystemic pulmonary hypertension and the resultant low cardiac output.

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.

Modified hybrid procedure in hypoplastic left heart syndrome: initial experience of a center in northeastern Brazil

Abstract Introduction: Although it only corresponds to 2.5% of congenital heart defects, hypoplastic left heart syndrome (HLHS) is responsible for more than 25% of cardiac deaths in the first week of life. Palliative surgery performed after the second week of life is considered an important risk factor in the treatment of HLHS. Objective: The aim of this study is to describe the initial experience of a medical center in Northeastern Brazil with a modified off-pump hybrid approach for palliation of HLHS. Methods: From November 2012 through November 2015, the medical records of 8 patients with HLHS undergoing hybrid procedure were retrospectively evaluated in a tertiary private hospital in Northeastern Brazil. The modified off-pump hybrid palliation consisted of stenting of the ductus arteriosus guided by fluoroscopy without contrast and banding of the main pulmonary artery branches. Demographic and clinical variables were recorded for descriptive analysis. Results: Eight patients were included in this study, of whom 37.5% were female. The median age and weight at the time of the procedure was 2 days (p25% and p75% = 2 and 4.5 days, respectively) and 3150 g (p25% and p75% = 3077.5 g and 3400 g, respectively), respectively. The median length in intensive care unit stay was 6 days (p25% and p75% = 3.5% and 8 days, respectively). There were no in-hospital deaths. Four patients have undergone to the second stage of the surgical treatment of HLHS. Conclusion: In this series, the initial experience with the modified off-pump hybrid procedure showed to be safe, allowing a low early mortality rate among children presenting HLHS.

Stent Placement in a Neonate with Sano Modification of the Norwood using Semi-Elective Extracorporeal Membrane Oxygenation / Colocação de Stent em um Recém-Nascido Submetido à Modificação de Sano da Operação de Norwood Usando Oxigenação por Membrana Extracorpórea Semi-Eletiva

Abstract Extracorporeal membrane oxygenation (ECMO) is a well-established tool of cardiopulmonary circulatory support for cardiopulmonary failure in children and adults. It has been used as a supportive strategy during interventional procedures in neonates with congenital heart disease. Herein, we describe a neonate with hypoplastic left heart syndrome who underwent stenting of the Sano shunt and left pulmonary artery after Norwood Sano operation using intra-procedural ECMO support. The use of ECMO as a bridge to recovery might be a feasible and reasonably safe adjunctive approach in the treatment of complications in selective case of neonates having undergone the Norwood Sano procedure.

Resumo A oxigenação por membrana extracorpórea (ECMO) é uma ferramenta bem estabelecida de suporte circulatório em casos de insuficiência em crianças e adultos. A ECMO tem sido utilizada como uma estratégia de suporte durante procedimentos interventistas em recém-nascidos com doença cardíaca congênita. Descrevemos o caso de um recém-nascido com síndrome do coração esquerdo hipoplásico que foi submetido à colocação de um stent em shunt de Sano e artéria pulmonar esquerda após procedimento de Norwood-Sano utilizando-se a ECMO como suporte. O uso da ECMO pode ser uma estratégia adjunta viável e segura no tratamento de complicações em casos eletivos de recém-nascidos submetidos à operação de Norwood-Sano.

Resultados comparativos de cirugía de Fontan en pacientes con y sin hipoplasia de corazón izquierdo / Comparative results of Fontan surgery in patients with and without hypoplastic left heart syndrome

Introducción: Durante los últimos años múltiples pacientes con diagnóstico de corazón univentricular han sido tratados quirúrgicamente en nuestra institución mediante una anastomosis cavopulmonar total, de acuerdo al protocolo de corrección quirúrgica por etapas. Objetivo: Evaluar los resultados postoperatorios y la sobrevida de pacientes con síndrome de hipoplasia de corazón izquierdo (SHCI) sometidos a la operación de Fontan en comparación con pacientes con otras formas de corazón univentricular. Pacientes y método: Ciento dos pacientes fueron sometidos a la operación de Fontan entre abril de 1996 y marzo de 2014, 25 con SHCI (grupo I) y 77 con otras formas de corazón univentricular (grupo II). Se analizó la sobrevida, variables demográficas, estudio hemodinámico, morbimortalidad quirúrgica, ventilación mecánica, uso de drenajes, estancia postoperatoria, score de isótropos, necesidad de marcapasos y necesidad de revertir el Fontan take down. Resultados: La mortalidad perioperatoria fue del 4% (n = 1) para el grupo I y del 7,8% (n = 6) para el grupo II (p = 0,451). La única diferencia encontrada fue la estancia hospitalaria, siendo de 17 días (6-47) para el grupo I y 12 (5-103) para el grupo II(p = 0,017). El seguimiento promedio fue de 4,24 ± 2,08 años para el grupo I y de 8,7 ± 4,67 para el grupo II. La sobrevida a 8 años para ambos grupos fue de 88% y de 81% a 10 años para el grupo II. Conclusiones: La cirugía de Fontan tuvo una mortalidad similar en pacientes con SHCI en comparación con aquellos con otras formas de corazón univentricular, mientras que el primer grupo tuvo una mayor estancia hospitalaria. La sobrevida a largo plazo fue similar para ambos grupos.

Introduction: During the last few years, numerous patients with univentricular heart disease have been treated surgically with total cavopulmonary anastomosis according to a staged surgery protocol in our institution. Objective: To evaluate the perioperative outcomes and survival of patients with hypoplastic left heart syndrome (HLHS) after the Fontan procedure and compare them with other types of univentricular heart disease. Patients and method: A total of 102 patients underwent a Fontan procedure between April 1996 and March 2014, 25 with HLHS (group I), and 77 patients with other types of univentricular heart disease (group II). Groups survival, demographics, hemodinamic studies, morbimortality, mechanical ventilation, surgical drains, post-operative stay, isotopes score, pacemaker use, and requiriment of Fontan takedown were analyzed. Results: Intraoperative mortality was 4% (n = 1) for group I, and 7.8% (n = 6) for group II (P = .451). A difference was only found in hospital length of stay (LOS), being 17 days (6-47) for group I and 12 days (5-103) for group II (P = .017). Mean follow-up was 4.24 ± 2.08 years for group I, and 8.7 ± 4.67 for group II. Survival rate at 8 years for both groups was 88%, and 81% at 10 years for group II. Conclusions: The Fontan procedure had similar mortality, but longer LOS, in patients with HLHS compared to those with another types of single ventricle anatomy. Long term survival was comparable between both groups.

Estenosis aórtica crítica y síndrome de corazón izquierdo hipoplásico de diagnóstico precoz en un hospital de segundo nivel / Critical aortic stenosis and hypoplastic left heart syndrome of early diagnosis in a second-level hospital

Las cardiopatías son las malformaciones congénitas más frecuentes del desarrollo prenatal, en las que se produce un defecto en la arquitectura normal del corazón fetal interfiriendo en su buen funcionamiento en la vida postnatal. La proporción entre las diferentes cardiopatías varía mucho, sin embargo el Síndrome de Corazón Izquierdo Hipoplásico representa el 2-3% del total de cardiopatías congénitas constituyendo la forma más severa de lesiones obstructivas del corazón izquierdo y la de peor pronóstico. Su diagnóstico en etapas precoces de la gestación es hoy un reto para el obstetra, ya que su carácter evolutivo dificulta este hecho, no siendo en ocasiones, hasta etapas avanzadas de la gestación cuando se produce la alteración anatómica y la subsiguiente expresión ecográfica. Es en este punto donde se debe hacer hincapié, intentando aumentar el número de casos detectados precozmente, lo cual permitiría un adecuado aseso-ramiento familiar y la toma de decisiones oportuna. En los últimos años la introducción de terapias en patología cardíaca fetal, ha despertado un gran interés por tratar de modificar la evolución de esta enfermedad, siendo la valvuloplastía con balón aórtico la técnica de elección en pacientes seleccionados.

Congenital heart defects are the most frecuently congenital malformations in the prenatal development and consist of defect in the normal architecture of the fetal heart which interfering with its proper functioning. The proportion between the different heart disease varies widely, however Hypoplastic Left Heart Syndrome (HLHS) represent 2-3% of all congenital heart diseases and constitute the most severe obstructive lesions of the left heart and worse prognosis. The quickly diagnosis in early stages of the disease is an obstetrician challenge, however, it s evolutional feature hinders this proposal even in advanced stages of pregnancy when the anatomical alteration and subsequent ultrasound expression can be occur. At this point that should be emphasized to try increasing the number of cases detected early, which would allow a proper family counseling and timely decision. Recent years the introduction of therapies on fetal cardiac pathology, has aroused great interest in trying to change the course of the disease and the fetal aortic valvuloplasty with aortic balloon would be the choice technique in selected patients.

Síndrome de hipoplasia de corazón izquierdo: experiencia de 10 años de un programa de etapificación quirúrgica / Hypoplastic left heart syndrome: 10 year experience with staged surgical management

Resumen El síndrome de hipoplasia de corazón izquierdo (SHCI) es una cardiopatía congénita con letalidad superior al 95%. La etapificación quirúrgica es la principal vía de tratamiento, y se inicia con la operación de Norwood; la sobrevida a largo plazo de los pacientes tratados es desconocida en nuestro medio. Objetivos 1) Revisar nuestra experiencia en el manejo de todos los pacientes con SHCI evaluados entre enero 2000 y junio 2010. 2) Identificar factores de riesgo de mortalidad quirúrgica. Pacientes y método Estudio retrospectivo de una única institución con una cohorte de pacientes con SHCI. Se revisan antecedentes clínicos, quirúrgicos, y registros de seguimiento. Resultados Se evaluaron 76 pacientes con SHCI; 9/76 tenían comunicación interauricular (CIA) restrictiva, y 8/76, aorta ascendente de < 2 mm; 65/76 fueron tratados: 77% tuvieron operación de Norwood con conducto entre ventrículo derecho y ramas pulmonares como fuente de flujo pulmonar, 17% Norwood con shunt de Blalock-Taussig, y 6% otra cirugía. La mortalidad en la primera etapa quirúrgica fue del 23%, y en operación de Norwood, del 21,3%. En el período 2000-2005 la mortalidad en la primera etapa quirúrgica fue del 36%, y entre 2005-2010, del 15% (p = 0,05). La sobrevida global fue del 64% a un año y del 57% a 5 años. Por análisis multivariado fueron factores de riesgo para mortalidad la presencia de aorta ascendente diminuta y CIA restrictiva. Conclusiones Nuestros resultados inmediatos y a largo plazo en la etapificación quirúrgica de SHCI son similares a la experiencia de grandes centros. Hay una mejoría en mortalidad operatoria en la segunda mitad de la serie. Se identifican factores de riesgo de mortalidad.

Abstract Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. Objectives 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. Patients and method Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. Results Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤ 2 mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P = .05). Actuarial survival was 64% at one year, and 57% at 5 years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. Conclusions Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.

Biventricular Repair after Bilateral Pulmonary Artery Banding as a Rescue Procedure for a Neonate with Hypoplastic Left Heart Complex

Hypoplastic left heart complex (HLHC) consists of less severe underdevelopment of the left ventricle without intrinsic left valvular stenosis, i.e., a subset of hypoplastic left heart syndrome (HLHS). HLHC patients may be able to undergo biventricular repair, while HLHS requires single ventricle palliation (or transplant). However, there is no consensus regarding the likelihood of favorable outcomes in neonates with HLHC selected to undergo this surgical approach. This case report describes a neonate with HLHC, co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA) who was initially palliated using bilateral pulmonary artery banding due to unstable ductus-dependent circulation. A postoperative echocardiogram showed newly appearing CoA and progressively narrowing PDA, which resulted in the need for biventricular repair 21 days following the palliation surgery. The patient was discharged on postoperative day 13 without complications and is doing clinically well seven months after surgery.