A propósito del artículo Síndrome del corazón izquierdo hipoplásico / A Propos of the Article Síndrome del corazón izquierdo hipoplásico [Hypoplastic Left Heart Syndrome]

Con gran interés leí el artículo: Síndrome del corazón izquierdo hipoplásico, de la autoría de Crespo Vallejo y otros investigadores ecuatorianos. Este síndrome (ICD10CM: Q23.4) representa entre el 7 y el 9 por ciento de las malformaciones congénitas cardiacas y constituye la causa más frecuente de insuficiencia cardiaca y de mortalidad neonatal por cardiopatías congénitas (CC) en la primera semana de vida.1 Los autores describen muy adecuadamente al síndrome de cavidades izquierdas hipoplásicas (SCIH)…como una combinación de varias malformaciones que, en su conjunto, son los responsables de todas las manifestaciones clínicas de la enfermedad y citando a Laranjo y otros, plantean que: …"Se conoce que todas estas afecciones tienen un componente genético; sin embargo, en el caso del SCIH no se ha identificado aún el tipo o localización del trastorno responsable de la aparición del...(AU)

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.

Síndrome del corazón izquierdo hipoplásico / Hypoplastic Left Heart Syndrome

Introducción: El síndrome del corazón izquierdo hipoplásico es una rara anormalidad que se presenta en el periodo prenatal. Sus manifestaciones clínicas se relacionan con la presencia de hipoxemia a nivel sistémico. Objetivo: Socializar las manifestaciones clínicas y elementos diagnósticos del síndrome del corazón izquierdo hipoplásico. Caso clínico: Recién nacido masculino, a término, que presenta manifestaciones clínicas y ecosonográficas que permiten llegar al diagnóstico de un síndrome del corazón izquierdo hipoplásico. Conclusiones: A pesar de los avances de la ciencia y la técnica, el síndrome del corazón izquierdo hipoplásico presenta una elevada mortalidad. Una adecuada planificación de la gestación, el seguimiento preciso del embarazo, un correcto asesoramiento genético y la utilización de técnicas quirúrgicas para corregir las deformidades resultantes parecen ser las herramientas más eficaces para enfrentar esta mortal afectación(AU)

Introduction: Hypoplastic left heart syndrome is a rare abnormality that occurs in the prenatal period. Its clinical manifestations are related to the presence of hypoxemia at the systemic level. Objective: To describe the clinical manifestations and diagnostic elements of hypoplastic left heart syndrome. Clinical case: A male newborn, at term, who presented clinical and echographic manifestations that permitted the diagnosis of hypoplastic left heart syndrome. Conclusions: Despite advances in science and technology, hypoplastic left heart syndrome presents a high mortality. Proper planning for pregnancy, accurate pregnancy monitoring, proper genetic counseling, and the use of surgical techniques to correct the resulting deformities seem to be the most effective tools to deal with this deadly condition(AU)

Sindrome de corazón izquierdo hipoplásico: análisis de las características de una población en un hospital público / Hipoplastic left heart syndrome: analysis of the characteristics of a population in a public hospital

The authors present a study on prenatal diagnosis of hipoplastic left heart syndrome, and an analysis of the characteristics of a population in a public hospital. The hypoplastic left heart syndrome, and an analysis of the characteristics of a population in a public hospital. The hypoplastic left heart syndrome (HLHS) is a rare congenital heart deficit consisting in the hypodevelopment of the left ventricle, aortic valve, mitral valve and the ascendent aorta. Its incidence is 0,016% to 0.034% of the total of newborns alive. It represents between 1 - 3% of the whole congenital cardiopaties (cc). Its natural evolution is severe and it represents the greater cause of death by CC during the neonatal period. There is a predominance in males between the 55 to 67%. The etiology is multifactorial. Presents risk of recurrency between brothers, and because of that circumstance it could have genetical basis. The ais of this report were to analyze the clinical variables, electrocardiographis, echocardiographic and therapeutical, of a population with diagnosis fo HLHS. The incidence of HLHS in the population studied was 0.06% of life births. The results obtained are detailed (AU)

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.

The Effect of Multidisciplinary Approach on the Birth Rate of Fetuses with Prenatally Diagnosed Congenital Heart Disease

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.

Síndrome de hipoplasia del ventrículo izquierdo / Left ventricular hypoplasia syndrome

Antecedentes: el síndrome de hipoplasia del ventrículo izquierdo es un conjunto de alteraciones del corazón fetal que condicionan un hipodesarrollo del corazón izquierdo, que es insuficiente para mantener la circulación sistémica. El corazón derecho se encuentra dilatado e hipertrofiado y soporta la circulación pulmonar y la circulación sistémica fetal a través del conducto arterioso permeable. Representa el 2 a 3% de todas las cardiopatías congénitas. Sin embargo, la incidencia real está subestimada ya que es responsable de abortos espontáneos y otras muertes fetales intrauterinas no diagnosticadas. Reporte de un caso: paciente de sexo femenino, 32 años de edad, con antecedente familiar de importancia (padre con fibrilación auricular). Antecedentes gíneco-obstétricos: un parto céfalo-vaginal, sin complicaciones, de un neonato de sexo masculino que actualmente tiene 6 años de edad. Durante su segunda gesta el feto es diagnosticado de hipoplasia del ventrículo izquierdo y atresia de la válvula mitral a las 34 semanas. Acude al Hospital Metropolitano a las 39 semanas de gestación para terminación de su embarazo mediante parto céfalo-vaginal. El parto no tuvo complicaciones; al nacimiento se confirma el diagnóstico prenatal y el recién nacido fallece en las primeras 72 horas. Conclusiones: el síndrome de hipoplasia del corazón izquierdo es una combinación compleja de malformaciones cardíaca;, debe ser detectado durante la evaluación ecográfica prenatal para planificar su manejo al nacimiento en centros especializados donde sea posible realizar procedimientos con el propósito de mejorar la supervivencia de estos pacientes.

Background: It is a set of alterations of the fetal heart that condition a hypodevelopment of the left heart, which is insufficient to maintain the systemic circulation. The right heart is dilated and hypertrophied and it supports the pulmonary circulation and fetal systemic circulation through patent ductus arteriosus. It represents 2 - 3% of all congenital heart diseases. However, current incidence is underestimated because it produces spontaneous abortions and other undiagnosed intra- uterine fetal deaths. Report of a case: a 32-year-old female patient with an importance family history: father with atrial fibrillation. Obstetric-gynecological antecedents: a cephalo- vaginal delivery without complications who was a male product of 6 years. During her second pregnancy, fetus was diagnosed with left ventricular hypoplasia and mitral valve agenesis at 34 weeks. She went to Metropolitan Hospital at 39.1 weeks to finish her pregnancy due to cephalo-vaginal birth. Delivery occurred without complications, however, the prenatal diagnosis was confirmed and the newborn died in 72 hours. Conclusions: The left heart hypoplasia syndrome is a complex combination of cardiac malformations; it can be detected with a prenatal evaluation with ultrasound which provides an option to pose treatment and also it can guide physicians to prepare for postnatal interventions when it is necessary.

The efficacy of thoracic ultrasonography in postoperative newborn patients after cardiac surgery

Abstract Objective: In this study, the efficacy of thoracic ultrasonography during echocardiography was evaluated in newborns. Methods: Sixty newborns who had undergone pediatric cardiac surgery were successively evaluated between March 1, 2015, and September 1, 2015. Patients were evaluated for effusion, pulmonary atelectasis, and pneumothorax by ultrasonography, and results were compared with X-ray findings. Results: Sixty percent (n=42) of the cases were male, the median age was 14 days (2-30 days), and the median body weight was 3.3 kg (2.8-4.5 kg). The median RACHS-1 score was 4 (2-6). Atelectasis was demonstrated in 66% (n=40) of the cases. Five of them were determined solely by X-ray, 10 of them only by ultrasonography, and 25 of them by both ultrasonography and X-ray. Pneumothorax was determined in 20% (n=12) of the cases. Excluding one case determined by both methods, all of the 11 cases were diagnosed by X-ray. Pleural effusion was diagnosed in 26% (n=16) of the cases. Four of the cases were demonstrated solely by ultrasonography, three of them solely by X-ray, and nine of the cases by both methods. Pericardial effusion was demonstrated in 10% (n=6) of the cases. Except for one of the cases determined by both methods, five of the cases were diagnosed by ultrasonography. There was a moderate correlation when all pathologies evaluated together (k=0.51). Conclusion: Thoracic ultrasonography might be a beneficial non-invasive method to evaluate postoperative respiratory problems in newborns who had congenital cardiac surgery.

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.

First reported case of fetal aortic valvuloplasty in Asia

Prenatal intervention of severe fetal aortic valve stenosis by ultrasound-guided percutaneous balloon valvuloplasty has been performed to prevent the progression to hypoplastic left heart syndrome, and achieve biventricular circulation in neonates. Here we report a case of fetal aortic valvuloplasty prenatally diagnosed with aortic stenosis at 24 weeks of gestation and showed worsening features on a follow-up echocardiography. Prenatal aortic valvuloplasty was performed at 29 weeks of gestation, and was a technical success. However, fetal bradycardia sustained, and an emergency cesarean delivery was performed. To the best of our knowledge, this is the first reported case of fetal aortic valvuloplasty which was performed in Asia.

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.

Three-Dimensional Endo-Cardiovascular Volume-Rendered Cine Computed Tomography of Isolated Left Ventricular Apical Hypoplasia: A Case Report and Literature Review

We report multidetector computed tomography (MDCT) and cardiac magnetic resonance (CMR) findings of a 34-year-old female with isolated left ventricular apical hypoplasia. The MDCT and CMR scans displayed a spherical left ventricle (LV) with extensive fatty infiltration within the myocardium at the apex, interventricular septum and inferior wall, anteroapical origin of the papillary muscle, right ventricle wrapping around the deficient LV apex, and impaired systolic function. MDCT visualized morphologic and also functional findings of this unique cardiomyopathy.

The strong association of left-side heart anomalies with Kabuki syndrome / 소아과

PURPOSE: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. METHODS: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. RESULTS: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. CONCLUSION: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.

Cirugía de Fontan: complicaciones posquirúrgicas: Medellín, Colombia / Fontan surgery: complications postsurgical: Medellin, Colombia

El manejo del corazón univentricular ha mejorado laexpectativa de vida de los pacientes con la cirugía deFontan.Objetivo: identificar las complicacionesposquirúrgicas más frecuentes de la cirugía de Fontanen pacientes de una institución privada de la ciudadde Medellín.Metodología: estudio descriptivo del tipo de seriesclínicas. La población estuvo compuesta porpacientes sometidos a la cirugía de Fontan. Lainformación sobre las variables sociodemográficas yclínicas fue tomada directamente de las historiasclínicas. La investigación contó con la aprobación delComité de ética. Se empleó el programa SPSS®versión 17.0 (SPSS Inc; Chicago, Illinois, EE.UU.) parael procesamiento y análisis de la información. Para elmanejo estadístico de los datos se empleó laestadística descriptiva.Resultados: participaron 21 pacientes, la mediana deedad fue de 5 años (mínimo 3 y máximo 10); el 66,7%fueron de sexo masculino. El 100% de los niños tenían antecedentes de cirugía paliativa previa, la másfrecuente fue el Glenn bidireccional. En cuanto a lascomplicaciones más frecuentes fueron derramepleural (52,4%), disfunción miocárdica e infección enherida (19%).Conclusiones: las complicaciones que sepresentaron coinciden con lo reportado en la literatura, cabe resaltar que ningún paciente refirió estenosissubaórtica ni hemorragias.

Univentricular heart management has improved thelife expectancy of patients with Fontan surgery.Objective: identify the most common postoperative complications of the Fontan surgery in patientsfrom a private institution from Medellin.Methodology: descriptive clinical series. Thepopulation was patients undergoing the Fontansurgery. Information about sociodemographic andclinical variables was taken directly from themedical records. The research was approved bythe Ethics Committee. We used SPSS ® version17.0 (SPSS Inc, Chicago, Illinois, USA) forprocessing and analysis of information. For statistical handling of data is used for descriptiv estatistics. Results: 21 patients participated, the median agewas 5 years (minimum 3, maximum 10), 66.7% were male. 100% of the children had a history of priorpalliative surgery, the most common was the bidirectional Glenn. As for the most common complications were pleural effusion (52.4%), myocardial dysfunction and wound infection (19%). Conclusions: the complications presented agree with those reported in the literature, it is notable that no patient reported sub aortic stenosis and bleeding.

Successful Mechanical Thrombectomy in a 2-Year-Old Male Through a 4-French Guide Catheter / 신경중재치료의학

A 2-year-old boy with hypoplastic left heart syndrome that required multiple cardiovascular surgeries and a heterozygous prothrombin G20210A mutation with resulting thrombophilia maintained on warfarin presented with acute right middle cerebral artery (MCA) infarction manifesting as a left hemiplegia. An MRI revealed a complete occlusion of the right M1 segment with an area of restricted diffusion in the right basal ganglia representing only a small area of acute infarction. Patchy areas of subacute infarction were also present in the right MCA territory. He underwent endovascular mechanical thrombectomy with a stent retriever. This is an account of a successful mechanical thrombectomy performed in the youngest patient reported in the English literature to date.