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.

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)

Extracardiac Conduit Fontan Operation with Reduction Aortoplasty for Left Pulmonary Artery Compression after a Norwood Procedure in a Patient with Double-Inlet Left Ventricle / 대한흉부외과학회지

Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure of the neoaorta to systemic pressure, which may induce adverse effects on adjacent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure.

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.

Translocation of the Aortic Arch with Norwood Procedure for Hypoplastic Left Heart Syndrome Variant with Circumflex Retroesophageal Aortic Arch

Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.

vitro flow visualization of the pulmonary circulation

Flow field of the pulmonary circulation has been investigated by in vitro pulsatile and steady flow visualization in simulation models. A couple of counter-rotating secondary flows were symmetric about the centerline in the normal valve. As the pulmonic valve became more stenotic, the two counter-rotating secondary flows in both the left pulmonary arteries [LPA] and right pulmonary arteries [RPA] were no longer symmetric. With a normal Hancock porcine aortic valve inside the extracardiac conduit, the flow of the proximal conduit was spiral, and that of the distal portion was axial. In stenosed Hancock porcine aortic valve loaded conduit, the flow was a continuous spiral. Studies on cavopulmonary connection models showed that energy savings were more evident at the 50:50 right / left pulmonary artery ratio, and the energy losses increased in proportion to total flow rates. A 60° to 90° anastomotic angle between the subclavian artery and the graft of Blalock-Taussig shunt could result in favorable pulmonary artery flow distribution and peak pressure. Simulations in the Norwood circulation model showed that larger shunts rendered an increased cardiac output to the lungs. In order to determine the idealistic cardiac surgical technical conditions, in vitro flow visualization study is a primarily useful tool in optimizing the flow and diminishing the energy losses

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.

Low Cardiac Output after Division of the Left Superior Vena Cava during a Norwood Operation for Hypoplastic Left Heart Syndrome in a Patient with Coronary Sinus Orifice Atresia: A case report / 대한흉부외과학회지

A 12-day-old female baby underwent a Norwood procedure for hypoplastic left heart syndrome. The left superior vena cava (LSVC), which was found incidentally during the operation, was divided to facilitate surgical exposure. After the operation, she developed signs of low cardiac output and died 7 hours afterward. Autopsy findings showed that the coronary sinus was atretic at the orifice without unroofing into both atria, rendering the LSVC the sole route of coronary sinus drainage. In patients with incidentally-found LSVC during surgery, special care should be taken to leave the LSVC intact because the LSVC may be the exclusive drainage vein of the coronary venous system.

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.

Successful Application of Vacuum Assisted Closure in the Case of Neonatal Mediastinitis after Modified Norwood Operation / 대한흉부외과학회지

Mediastinitis and sternal wound infection frequently occurred after corrective surgery for complex heart anomalies. Most of the patients are neonate or infant and they have showed low oxygen tension. For that poor condition, application of invasive treatment of mediastinitis is not appropriate and recovery takes a long time. We successfully treated a mediastinitis after Norwood operation using vacuum assisted closure. So we introduce the technique and the modification of neonatal vacuum assisted closure.

Postoperative Care after Palliative Surgery: Blalock-Taussig Shunt, Pulmonary artery Banding, Norwood Procedure

No abstract available.

Decision of Surgical Treatment Strategy for Hypoplastic Left Ventricle with Coarctation of Aorta

PURPOSE: The purpose of this study is to determine the feasibility of biventricular repair for hypoplastic left ventricle combined with coarctation of aorta and to provide strategy of surgical treatment. METHODS: The preoperative and postoperative dimension of mitral valve and left ventricle was compared for three patients with hypoplastic left ventricle combined with coarctation of aorta. Ventricular septal defect, atrial septal defect and patent ductus arteriosus were also present in all patients, and the age at the operation was 36, 15 and 11 days. The preoperative end-diastolic left ventricular volume was 13, 28, 24 mL/m2 respectively, and antegrade flow was observed in ascending aorta in all patients. RESULTS: All patients underwent repair of coarctation of aorta. In addition to coarctation repair, pulmonary arterial banding and atrial septal defect closure was performed in Case 1 and 2 respectively, leaving the ventricular septal defect unclosed. In case 3, the ventricular septal defect was closed with the coarctation repair. Case 1 had to undergo Norwood procedure due to inadequate growth of left ventricle and is waiting for univentricular repair. In Case 2 and 3, the left ventricular size increased and biventricular repair could be performed successfully. CONCLUSION: Left ventricular growth can be obtained by repair of coarctation and biventricular repair was feasible in selected cases of hypoplastic left ventricle combined with coarctation of aorta. Size and volume measurement of mitral valve and left ventricle with echocardiography or magnetic resonance image provide useful information to decide the surgical strategy for this group of patients.

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.

The Norwood Operation in Infants with Complex Congenital Heart Disease / 대한흉부외과학회지

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome(n=7), mitral stenosis with small VSD and subaortic stenosis(n=1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis(n=1), interrupted aortic arch with ventricular septal defect and subaortic stenosis(n=1), tricuspid atresia with transposition of the great arteries(n=1), and complex double-inlet left ventricle(n=2). All patients without hypoplastic left heart syndrome were associated with hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months(mean 60.6+/-71.6 days, median 39 days). The operative mortality(<30 days) was 46%(6 patients). Late mortality was 15%(2 patients). All operative deaths occured during the first 24 hours after the operation as a result of cardiopulmonary bypass weaning failure(5 patients) and sudden hemodynamic instability postoperatively(1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals(39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and late death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.