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.

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 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.

Cardiac catheter angioplasty for patients after Norwood procedure / 中华胸心血管外科杂志

Objective To evaluate the outcome of the cardiac catheter angiograplasty for patients after Norwood procedure. Methods 13 patients, who had undergone Norwood procedure (prior to Glenn procedure)received cardiac catheterization and angiography. lnterventional therapy was performed in 12 patients as indicated. Results Angioplasty was carried out for re-coarctation in 6 of the13 patients. 1 patient with left pulmonary artery stenosis received balloon dilation and 3 months later a stent implantation. A stent was implanted to improve the pulmonary blood flow in 2 patients with Sano-Shunt stenosis and 1 patient with BT-Shunt stenosis. Transcatheter coil embolization was performed in 3 of the 4 patients with systemic to pulmonary collateral vessels. The other one was scheduled for Glenn procedure the next day. Conclusion The incidence of hemodynamic restrictions after Norwood procedure is high (12 of 13 patients). Postoperative angiography and hemodynamic assessment for diagnosis and interventional treatment for new pulmonary artery or aortic arch stenosis is necessary after Norwood procedure.

Outcome of the Modified Norwood Procedure: 7 Years of Experience from a Single Institution / 대한흉부외과학회지

BACKGROUND: We assessed the early and mid-term results of the modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome (HLHS) and its variants to identify the risk factors for hospital mortality. MATERIAL AND METHOD: Between March, 2003, and December, 2009, 23 patients (18 males and 5 females) with HLHS or variants underwent the modified Norwood procedure. The age at operation ranged from 3 to 60 days (mean, 11.7+/-13.2 days) and weight at operation ranged from 2.2 to 4.8 kg (mean, 3.17+/-0.52 kg). We used a modified technique that spared the anterior wall of the main pulmonary artery in 20 patients. The sources of pulmonary blood flow were RV-PA conduit in 15 patients (group I) and RMBTS in 8 (group II). Follow-up was completed in 19 patients (19/20, 95%) in our hospital (mean 26.0+/-22.8 months). RESULT: Early death occurred in 3 patients (3/23, 13%), of whom 2 had TAPVC. Fourteen patients underwent subsequent bidirectional cavopulmonary connection (BCPC, stage 2) and seven underwent the Fontan operation (stage 3). Three patients died between stages, 2 before stage 2 and one before stage 3. The estimated 1-year and 5-year survival rates were 78% and 69%, respectively. On multivariate regression analysis, aberrant right subclavian artery (RSCA) and associated total anomalous pulmonary venous connection (TAPVC) were risk factors for hospital mortality after stage 1 Norwood procedure. CONCLUSION: HLHS and its variants can be palliated by the modified Norwood procedure with low operative mortality. Total anomalous pulmonary venous connection adversely affects the survival after a stage 1 Norwood procedure, and interstage mortality rates need to be improved.

The Surgical Management of Hypoplastic Left Heart Syndrome andthe Results of a Fontan Operation / 대한흉부외과학회지

BACKGROUND: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. MATERIAL AND METHOD: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was 17.3+/-10.8 days (range: 9~36 days) at the 1st staged operation, 8.9+/-7.1 months (4.6~23.3 months) at the 2nd staged operation (the Bidirectional Glenn procedure) and 32.4+/-9.8 months at the final staged operation (the Fontan procedure). During the 2nd staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. RESULT: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was 19.6+/-14.9 months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I). CONCLUSION: All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.

The Norwood-Rastelli Procedure for Left Ventricular Outflow Tarct Obstruction with a Ventricular Septal Defect: Three case report / 대한흉부외과학회지

Between 2001 and 2006, 3 neonates that had multilevel left ventricular outflow tract obstruction and a ventricular septal defect underwent the Norwood-Rastelli procedure. The body weights ranged from 2.9 to 3.1 kg. The patients had a near normal sized mitral valve and left ventricle. We simultaneously performed a modified Norwood procedure with native tissues-to-tissue anastomosis without circulatory arrest, and a Rastelli type procedure using a non-valved conduit from the right ventricle to the pulmonary artery and intracardiac patch baffling from the left ventricle to the pulmonary valve via the ventricular septal defect. The postoperative courses were uneventful. During follow-up, there was one late mortality caused by a cardiac catheterization related complication at 7 months after surgery. One patient required a Rastelli conduit change. Two patients are doing well during a follow-up period of 1 and 5 years, respectively.

Norwood Procedure on Beating Heart / 대한흉부외과학회지

Modified Norwood procedure with maintaining cardiac beat was done in a 30-day-old neonate. Procedure was done with regional perfusion of innominate and coronary artery. Postoperative course was uneventful. Second-stage operation (bi-directional cavopulmonary shunt) was done 4 months later. The diameter of ascending aorta was more than 5 mm, Norwood procedure can be done in beating hearts.

Norwood Procedure with Home-made Bovine Pericardial Patch: A report of 2 cases / 대한흉부외과학회지

A patch design using bovine pericardial patch for aortic enlargement in the Norwood procedure has been introduced to avoid problems related to homograft availability. We report 2 successful cases of Norwood procedure with home-made bovine pericardial patch. The first case was a 23-day-old (2.2 kg) patient with multi-level left ventricular outflow tract obstruction with ductal-dependent systemic circulation. The other case was a 9-day-old (3 kg) patient with hypoplastic left heart syndrome. This technique was relatively easy to perform, reproducible and quite effective like homograft patch.

Modified Norwood Procedure without Circulatory Arrest and Myocardial Ischemia: Report of 2 cases / 대한흉부외과학회지

The effects of deep hypothermia and circulatory arrest during aortic arch reconstruction are associated with potential neurologic and myocardial injury. We describe a surgical technique that two patients underwent a modified Norwood procedure without circulatory arrest and myocardial ischemia. One was 13-day-old female patient, weighing 3.1kg, having a variant of hypoplastic left heart syndrome and another was 38-day-old male patient, weighing 3.4 kg, diagnosed Taussig-Bing anomaly with severe aortic arch hypoplasia, coarctation of the aorta, and subaortic stenosis. The arterial cannula was inserted in innominate artery directly. During Norwood reconstruction, regional high-flow perfusion into the inominate artery and coronary perfusion were maintained and there were no neurologic, cardiac, and renal complications in two patients. This technique may help protect the brain and myocardium from ischemic injury in patients with hypoplastic left heart syndrome or other arch anomalies including coarctation or interruption.