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.

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.

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.

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.

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.

Nova técnica: operação de Norwood com perfusão regional cerebral e coronariana / New technique: Norwood operation with regional cerebral and coronary perfusion

Objetivo: Avaliar o resultado imediato da operação de Norwood modificado com nova técnica de perfusão regional cerebral (PRCeA) anterógrada associado a perfusão regional coronariana (PRCoR) retrógrada em substituição à parada circulatória total com hipotermia profunda em crianças portadoras da Síndrome da Hipoplasia do Coração Esquerdo (SHCE) com aorta ascendente extremamente hipoplásica (AH). Métodos: No período de dezembro de 2006 a fevereiro de 2008, a operação de Norwood modificado com tubo entre o ventrículo direito e as artérias pulmonares ou shunt tipo Sano foi realizada em oito crianças portadoras de SHCE e aorta ascendente com diâmetro inferior a 3 mm, (quatro do sexo masculino e quatro do sexo feminino) com idade média de 9,2 dias (variando de 1 a 29 dias) e peso médio de 3,3 kg (variando de 2,7 a 3,8 kg). Utilizada CEC e hipotermia a 25ºC com duas cânulas venosas e anastomose de um enxerto de politetrafluoretileno com a artéria inominada utilizado como linha arterial e para PRCeA. A PRCoR foi realizada por meio de um desvio na linha arterial e colocação de um cateter na aorta ascendente. Foram analisados o resultado cirúrgico imediato e a presença de alterações neurológicas nesse período. Resultados: O resultado cirúrgico imediato revelou mortalidade de 25 por cento e ausência de comprometimento neurológico ao exame clínico. Conclusão: A operação de Norwood modificado pode ser realizada com PRCeA e PRCoR em crianças com SHCE e AH com resultado cirúrgico imediato satisfatório e ausência de complicações neurológicas.

Objective: To assess the immediate result of the modified Norwood procedure with new technique of anterograde regional cerebral perfusion (ARCeP) and retrograde regional coronary perfusion (RRCoP) in substitution of profound hypothermia and circulatory arrest in children with hypoplastic left heart syndrome (HLHS) with extremely hypoplastic ascending aorta (HA). Methods: In the period of December of 2006 to February 2008 the modified Norwood procedure with tube between the right ventricle and pulmonary arteries or Sano shunt was performed in eight children with HLHS and with ascending aorta diameter less than 3 mm, (four male and four female) with median age of 9.2 days (ranging from 1 to 29 days) and median weigh of 3.3 kg (ranging from 2.7 to 3.8 kg). We used cardiopulmonary bypass and hypothermia at 25ºC with two venous cannulas and suture with a polytetrafluoroethylene graft sutured to the innominate artery as an arterial line and used to ARCeP. The RRCoP was performed by a deviation in the arterial line and placement of a catheter in the ascending aorta. The immediate surgical results and the clinical neurological manifestation were assessed. Results: The immediate surgical results revealed mortality of 25 percent and there were absence of neurological injury at clinical examination. Conclusions: The modified Norwood procedure can be accomplished with ARCeP and RRCoP in children with HLHS with HA with satisfactory immediate surgical result and without neurological complications.

Experiência inicial de um novo centro no Brasil na abordagem híbrida para a síndrome de hipoplasia do coração esquerdo / Hybrid approach for treatment of the hypoplastic left heart syndrome: initial experience of a new center in Brazil

Objetivo: Apresentar os resultados iniciais com o emprego do procedimento híbrido na síndrome de hipoplasia do coração esquerdo (SHCE). Método: Oito pacientes com SHCE foram submetidos ao primeiro estágio do procedimento. As indicações foram: preferência do grupo em quatro (50%) pacientes, infecção sistêmica em evolução em dois (25%), disfunção ventricular grave em um (12,5%) e lesão encefálica grave com hipertensão pulmonar em um (12,5%). Resultados: Três (37,5%) pacientes morreram no primeiro estágio, dois (25%) foram submetidos ao segundo estágio, e nenhum foi submetido ao terceiro estágio. Três (37,5%) crianças aguardam o segundo estágio, e duas delas estão internadas, uma por insuficiência respiratória grave e infecção sistêmica e outra pós-atriosseptostomia com colocação de stent, evoluindo com infecção pulmonar. Reintervenções foram necessárias em 50% dos casos, em dois deles mais de uma vez (atriosseptostomias por balão em três e colocação de stent na comunicação interatrial em um). O tempo médio de ventilação no primeiro estágio foi de 585 horas e de internação na unidade de terapia intensiva, de 32 dias. Duas crianças já oram submetidas ao segundo estágio e ambas morreram: morte súbita em uma e durante dilatação da artéria pulmonar esquerda por estenose residual em outra...

Objective: Our objective was to review the early results of hybrid procedures in hypoplastic left heart syndrome (HLHS) at our institution. Methods: Eight HLHS patients were submitted to the first stage of the procedure and the indications were the surgeon's preference in 4 (50%), sepsis in 2 (25%), severe ventricular dysfunction in 1 (12.5%) and severe brain damage with pulmonary hypertension in the latter (12.5%). Results: Three (37.5%) patients died after the intervention, 2 (25%) underwent the second stage and none underwent the third stage. Three (37.5%) patients are currently waiting for the second stage and 2 of these are currently hospitalized: one with pneumonia-related sepsis and the other with pneumonia post atrial septal defect stenting. Reinterventions were necessary in 50% of the cases, more than once in 2 patients (balloon atrioseptostomy in 3 and atrial septal defect stenting in 1). Mean mechanical ventilation duration in the first stage was 585 hours and mean hospitalization time in the intensive care unit was 32 days. Both patients undergoing the second stage died: sudden death in one and residual left pulmonary artery stenosis after percutaneous dilatation in the other...

Neonatal cardiac interventions: an Indian perspective.

Palliative interventions in newborn have been performed ever since balloon atrial septostomy was started. The scope and potential of these interventions have been extended to therapeutic interventions and also to replace palliative surgeries. The advancements have happened over the last two decades mainly due to the advancements in cardiac anesthesia of the newborn and primarily due to available hardware improving. The outcomes of these interventions have also improved significantly. The advantage of neonatal interventions is especially relevant in developing countries where limited resources, cost of procedure (lower with reused consumables), lower morbidity due to shorter ICU stay work in favor of the patient. Not only have newborn interventions become well established in centers specializing in pediatric cardiac care, but also premature babies requiring interventions have become successful. This article focuses on the spectrum of neonatal interventions in a contemporary pediatric cardiac centre in India.

Nova bandagem ajustável das artérias pulmonares na Síndrome de Hipoplasia de Câmaras Esquerdas / A novel adjustable pulmonary artery banding system for hypoplastic left heart syndrome

OBJETIVO: A Síndrome de Hipoplasia de Câmaras Esquerdas representa um grande desafio para cirurgiões do mundo inteiro. Atualmente, tem sido proposto procedimento paliativo alternativo, por meio da bandagem bilateral das artérias pulmonares associada à colocação de stent no canal arterial e atrioseptostomia. No entanto, as bandagens utilizadas são fixas, podendo tornar-se inadequadas após o fechamento do esterno ou com o rápido crescimento somático do paciente. Descrevemos a primeira aplicação clínica do novo dispositivo miniaturizado de bandagem ajustável das artérias pulmonares em neonato portador da síndrome de hipoplasia de câmaras esquerdas, o qual permitiu ajustes percutâneos precisos do fluxo sangüíneo pulmonar. MÉTODO: Através de esternotomia mediana, neonato de 5 dias de vida foi submetido à bandagem pulmonar bilateral, usando este novo dispositivo, combinada com interposição de tubo de PTFE entre o tronco pulmonar e o tronco braquiocefálico. RESULTADOS: O paciente apresentou boa evolução pós-operatória. Três ajustes percutâneos das bandagens foram necessários para manter a saturação arterial de oxigênio entre 75-85 por cento. No 48° dia de vida, o paciente foi submetido a atrioseptostomia com colocação de stent (6 mm) para tratamento de comunicação interatrial restritiva. No 106° dia de vida, realizou-se operação de Norwood associada à anastomose cavopulmonar bilateral. As bandagens foram removidas, sem distorção das artérias pulmonares. CONCLUSÕES: O uso clínico deste sistema inovador de bandagem ajustável das artérias pulmonares mostrou-se factível, seguro e eficaz. Permitiu o ajuste fino do fluxo pulmonar de acordo com as necessidades clínicas, proporcionando um equilíbrio preciso entre as circulações pulmonar e sistêmica.

OBJECTIVE: Hypoplastic left heart syndrome remains a challenge for worldwide surgeons. Initial palliation employing bilateral pulmonary artery banding along with ductal stent implantation and atrial septostomy has been proposed as an alternative approach. However, the surgically placed bands are fixed and may become inadequate after sternum closure or with somatic growth of the patient. We describe the first case in which a neonate with hypoplastic left heart syndrome was initially managed using a mini banding system that allows for fine percutaneous adjustments of pulmonary blood flow. METHOD: Through a mid sternotomy, a 5 day-old neonate underwent bilateral pulmonary artery banding using this new system combined with placement of a main pulmonary artery to innominate artery shunt. RESULTS: The patient had an uneventful postoperative course. Three percutaneous adjustments of the banding system were necessary to keep the arterial oxygen saturation in the 75 percent-85 percent range. On the 48th day of life, she was submitted to stent placement (6 mm) within the atrial septum to treat a restrictive atrial septal defect. The Norwood operation and the bidirectional Glenn shunt were carried out on the 106th day of life. The bands were removed with no distortion of the pulmonary arteries. CONCLUSIONS: The clinical use of this innovative pulmonary artery banding system was feasible, safe and effective. It allowed for customization of the pulmonary blood flow according to the underlying clinical needs, resulting in a more precise balance between the pulmonary and systemic circulations.

Síndrome da hipoplasia do coração esquerdo: a influência da estratégia cirúrgica nos resultados / Hypoplastic left heart syndrome: the influence of surgical strategy on outcomes

OBJETIVOS: Relatar uma estratégia cirúrgica para a operação de Norwood na síndrome de hipoplasia do coração esquerdo (SHCE), que possibilite tempo curto de parada circulatória hipotérmica e reconstrução do arco aórtico com pericárdio autólogo. Comparar os resultados das técnicas anastomose de Blalock-Taussig modificado (B-Tm) e tubo ventrículo direito para artéria pulmonar (VD-AP) no restabelecimento da circulação pulmonar. MÉTODOS: Estudo retrospectivo compreendendo 71 neonatos portadores de SHCE, consecutivamente operados entre março de 1999 e fevereiro de 2006. Foi usada a mesma técnica de reconstrução da neo-aorta e duas técnicas diferentes de restabelecimento da circulação pulmonar: anastomose de B-Tm nos primeiros 37 neonatos e tubo VD-AP nos últimos 34. A canulação do canal arterial para a perfusão arterial foi a parte principal da estratégia cirúrgica para diminuir o tempo de parada circulatória hipotérmica. RESULTADOS: A sobrevida geral foi de 74,64 por cento, sendo de 67,57 por cento no grupo B-Tm e de 82,35 por cento no grupo tubo VD-AP (p = 0,1808). Os índices de mortalidade entre o primeiro e o segundo estágios foram de 40 por cento e de 4,4 por cento, respectivamente, nos grupo B-Tm e tubo VD-AP (p = 0,0054). Os tempos de parada circulatória hipotérmica foram, respectivamente, de 45,79 + 1,99 minutos e de 36,62 + 1,62 minutos (p = 0,0012). Coarctação tardia da aorta ocorreu em cinco pacientes (7,2 por cento). CONCLUSÃO: Essa estratégia cirúrgica resultou em tempo curto de parada circulatória, baixa mortalidade e boa morfologia da neo-aorta, com baixa incidência tardia de coarctação aórtica. A maior sobrevida ao primeiro estágio com o tubo VD-AP não foi significante, mas a mortalidade interestágios foi estatisticamente menor na comparação com o procedimento B-Tm.

OBJECTIVES: To report a surgical strategy for the Norwood procedure in the hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest time and aortic arch reconstruction with autologous pericardium patch, and to compare the results of the modified Blalock-Taussig (mBT) shunt with the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow. METHODS: Retrospective study of 71 newborns with HLHS consecutively operated between March, 1999 and February, 2006. One technique for reconstruction of the neoaorta and two different techniques for reestablishment of the pulmonary blood flow were used: the mBT shunt in the first 37 newborns and RV-PA conduit in the last 34. Cannulation of the ductus arteriosus for arterial perfusion was the main part of the surgical strategy to reduce the hypothermic circulatory arrest time. RESULTS: In-hospital survival for the entire cohort was 74.64 percent, or 67.57 percent and 82.35 percent for the mBT shunt and RV-PA conduit groups, respectively (p=0.1808). Mortality rates between the first and second palliation stages were 40 percent and 4.4 percent for the mBT shunt and RV-PA conduit groups, respectively (p=0.0054). Hypothermic circulatory arrest times were 45.79±1.99 min and 36.62±1.62min (p=0.0012), respectively. Late coarctation of the aorta occurred in five patients (7.2 percent). CONCLUSION: This surgical strategy resulted in short circulatory arrest time, low mortality and favorable morphology of the neoaorta, with low incidence of late coarctation of the aorta. The higher rate of survival to first palliation stage with the RV-PA conduit was not significant, but interstage mortality was statistically lower when compared with the modified Blalock-Taussig shunt procedure.

Univentricular heart: management options.

The term ''Univentricular Heart'' encompasses a wide variety of heart defects that functionally and physiologically constitute a single ventricular chamber. The terminology ''univentricular repair'' is frequently used in the surgical literature to include those biventricular hearts that are not amenable for a final two ventricle repair and need to go through the same surgical stages as with a functionally univentricular heart, culminating finally in a total cavo-pulmonary connection. Broadly, treatment is focused on controlling the pulmonary blood flow in early infancy, by means of aorto-pulmonary shunting in pulmonary atresia or stenosis, and pulmonary artery banding or pulmonary artery disconnection with aorto-pulmonary shunt placement in high pulmonary blood flow situations. Concomitant repair of other associated conditions is required. Babies with hypoplastic left heart physiology undergo staged "Norwood" repair resulting in an eventual total cavo-pulmonary connection with the RV functioning as the systemic ventricle. In this review, medical and surgical management of these patients will be discussed, after a brief discussion of nomenclature, anatomic and physiologic considerations.

Operação de Glenn bidirecional no tratamento estagiado da síndrome de hipoplasia do coração esquerdo: resultados imediatos e tardios / Bidirectional Glenn procedure in the staged treatment of hypoplastic left heart syndrome: early and late results

Objetivo: Relatar os resultados imediatos e tardios da operação de Glenn bidirecional como segundo estágio do tratamento da Síndrome de Hipoplasia do Coração Esquerdo (SHCE) e descrever a técnica de miniesternotomia utilizada.Método: Entre março de 1998 e fevereiro de 2004, 15 pacientes com operação de Norwood prévia foram submetidos eletivamente à derivação cavopulmonar. As idades variaram de 2 a 6 meses (média 3,46 meses), sendo seis pacientes do sexo masculino. Foram realizadas miniesternotomias em 11(77,3 por cento)casos. Para adequada oxigenação sanguínea inicial foi associado enxerto sistêmico-pulmonar de 3 mm em nove casos e manutenção do enxerto VD-TP em um caso. Acompanhamento clínico e ecocardiográfico foi realizado em todos os pacientes.Resultados: A sobrevida hospitalar foi de 86,6 por cento, ocorrendo um óbito por sangramento e outro por hipóxia. O ecocardiograma imediato mostrava fluxo pelo enxerto de PTFE nos dez pacientes em que foi utilizado, ocorrendo seu fechamento no controle tardio. Ocorreram dois (13,3 por cento) óbitos tardios, um por complicação de traqueostomia e outro por meningite bacteriana. Sete pacientes aguardam o terceiro estágio, estando assintomáticos. Quatro foram submetidos ao terceiro estágio com sucesso. O ecocardiograma dos 11 pacientes sobreviventes tardios mostra boa função do ventrículo direito, sem insuficiência tricúspide e bom fluxo pela anastomose cavo-pulmonar, num seguimento médio de 2 anos e 5 meses.Conclusões: A operação de Glenn na SHCE apresenta baixa mortalidade hospitalar, com resultados satisfatórios em longo prazo, podendo ser realizada através de miniesternotomia. A associação de fluxo sistêmico-pulmonar acessório em crianças de baixa idade parece melhorar a saturação de oxigênio

A operação de Norwood modificada para tratamento da síndrome de hipoplasia do coração esquerdo / Modified Norwood procedure for hypoplastic left heart syndrome

OBJETIVO: Apresentar os resultados do tratamento da síndrome de hipoplasia do coração esquerdo (SHCE) com técnica de Norwood modificada, na qual somente são usados tecidos autólogos para a reconstrução do arco aórtico. MÉTODO: De janeiro a dezembro de 2002, cinco recém-nascidos com idade variando de dois a nove dias (média 5,0 dias) foram submetidos a operação de Norwood modificada. O diâmetro da aorta ascendente variou de 5 a 8 mm (média 6,2 mm). Foi empregada técnica na qual o arco aórtico e seus ramos foram amplamente dissecados, permitindo a reconstrução de um novo arco aórtico tendo a artéria pulmonar como via de saída, e com a utilização exclusiva de tecidos do próprio paciente. Anastomose sistêmico-pulmonar com politetrafluoretileno expandido de 3,0 mm foi utilizada em três pacientes e de 3,5 mm em dois pacientes. Todos foram operados com parada cardiocirculatória total. RESULTADOS: O tempo de parada cardiocirculatória variou de 41 a 60 minutos (média 52,8 minutos). Todas as crianças sobreviveram ao ato operatório e foram encaminhadas para a unidade de terapia intensiva com o esterno aberto. Ocorreu um óbito no 9º dia de pós-operatório por sepse, o que resultou numa taxa de sobrevivência imediata de 80 por cento. Ocorreu um óbito tardio por pneumonia de aspiração aos dois meses. Uma criança foi submetida ao segundo estágio da operação (cavo pulmonar) e encontra-se bem. Duas crianças estão em acompanhamento aguardando o próximo estágio. Em nenhuma delas existem evidências de obstrução do novo arco aórtico. CONCLUSAO: A técnica de Norwood modificada mostrou-se eficaz e com risco cirúrgico aceitável para o tratamento da SHCE, sem evidências de obstrução do arco aórtico reconstruído.