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.

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.

A síndrome do coração esquerdo hipoplásico não constitui fator de risco para operação de Fontan / The hypoplastic left heart syndrome is not a risk factor for Fontan operation

OBJETIVO: Demonstrar a mortalidade hospitalar de crianças submetidas à operação de Fontan e determinar se a síndrome do coração esquerdo hipoplásico (SCEH) constitui fator de risco para mortalidade. MÉTODOS: De agosto 2001 a junho 2008, 76 pacientes foram submetidos à operação de Fontan, sendo divididos em dois grupos: grupo A com 54 pacientes, sendo 31 (40,7 por cento) portadores de atresia tricúspide e variantes, seis (7,8 por cento) de dupla via de entrada ventrículo esquerdo, quatro (5,3 por cento) de defeito do septo atrioventricular total e 13 (17,1 por cento) de outras cardiopatias congênitas complexas; e grupo B constituído por portadores de SCEH, num total de 22 (28,9 por cento) pacientes. RESULTADOS: Os pacientes do grupo A tiveram média de idade de 6,47 anos ± 4,83 e do grupo B de 2,08 anos ± 0,24 P<0,001; a média de peso foi de 22,42 ± 11,04 contra 12,99 ± 1,2 P=0,016; o tempo médio de CEC foi de 119,5 min contra 113,3 min P=0,0, com tempo médio de pinçamento aórtico de 74,8 min e 73,5 min P= 0,75. O tempo médio de permanência em UTI foi 4,1 dias para o grupo A contra 7,52 dias para o grupo B P= 0,0003. No total (grupo A + B), três pacientes foram a óbito, com mortalidade hospitalar de 3,9 por cento, sendo um paciente portador de SHCE (1,3 por cento) (P<0,001; IC95 por cento 0,001 - 0,228). CONCLUSÃO: Nosso estudo evidencia que, apesar de maior morbidade, a SCEH não constitui um fator de risco para mortalidade hospitalar.

OBJECTIVE: To show the mortality rate of children undergoing to Fontan operation and determine whether the hypoplastic left heart syndrome (HLHS) is a risk factor for hospital mortality. METHODS: From August 2001 to June 2008, 76 patients underwent Fontan operation and were divided into two groups: group A with 54 patients, 31 (40.7 percent) patients with tricuspid atresia and variants, six (7.8 percent) of double-inlet left ventricle, four (5.3 percent) and atrioventricular septal defect and total 13 (17.1 percent) of other complex congenital heart disease and group B all patients with HLHS a total of 22 (28.9 percent) patients. RESULTS: Group A patients had a mean age of 6.47 years ± 4.83 and group B of 2.08 years ± 0.24 P <0.001, the average weight was 22.42 ± 11.04 against 12.99 ± 1.2 P = 0.016, the mean CPB time was 119.5 min versus 113.3 min P = 0.0, with a mean clamping time of 74.8 min and 73.5 min p = 0.75. The mean ICU stay was 4.1 days for group A versus 7.52 days for group B p = 0.0003. In total (group A + B) three patients died, with hospital mortality of 3.9 percent, and one patient with HLHS (4.54 percent) (P <0.001, 95 percent CI 0.001 to 0.228). CONCLUSION: Our study shows that despite higher morbidity in HLHS is not a risk factor for hospital mortality.

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.