Assessment of microcirculatory alteration by a vascular occlusion test using near-infrared spectroscopy in pediatric cardiac surgery: effect of cardiopulmonary bypass.

OBJECTIVES: Cardiopulmonary bypass cause microcirculatory alterations. Near infrared spectroscopic measurement of tissue oxygen saturation and vascular occlusion test are novel technologies for assessing the microcirculatory function of peripheral tissue specifically in patients undergoing cardiac surgery with cardiopulmonary bypass.Our study aimed to evaluate dynamic microcirculatory function using the vascular occlusion testing during cardiac surgery in pediatric patients. METHODS: 120 pediatric patients were scheduled. Children had continuous regional oxygen saturation monitoring using near infrared spectroscopy and vascular occlusion test. Vascular occlusion test was performed five times; before induction (T1), after induction (T2), then during cardiopulmonary bypass with full flow (T3), after the termination of CPB (T4) and after sternum closure (T5). RESULTS: Basal value was the lowest at T3 and this value was significantly different among measurements (p < 0,01).Values for maximum and minimum tissue oxygen saturation were the lowest at T3 (83,4 and 52,9%).The occlusion slope varied significantly among measurements (p < 0,01).Reperfusion slopes were significantly different among measurements (p < 0,01) with a further progressive decrease in reperfusion slope with duration of cardiopulmonary bypass. CONCLUSION: Microcirculatory function can assessed using VOT with forearm Near-infrared spectroscopy derived variables during cardiopulmonary bypass in pediatric cardiac surgery. Noninvasive assessment of microcirculatory perfusion during cardiopulmonary bypass can further help evaluate and improve circulatory support techniques. TRIAL REGISTRATION: The research Project was registered at ClinicalTrials.gov (NCT06191913).

The prognostic significance of early troponin levels in patients undergoing aortic ridge surgery.

Subaortic stenosis is a CHD that can lead to left ventricular hypertrophy, heart failure, and aortic valve damage if left untreated. The gold standard treatment for subaortic stenosis is septal myectomy. However, there is no clear consensus on the surgical margins required for adequate muscle resection. In this retrospective study, we reviewed the records of 83 patients who underwent subaortic stenosis surgery between 2012 and 2020 to investigate the effect of early troponin levels on prognosis. We excluded patients with additional cardiac pathologies, hypertrophic obstructive cardiomyopathy, and valvular aortic stenosis.Troponin levels were recorded in the early post-operative period, and patients were monitored for complications such as ventricular arrhythmia, left ventricular systolic dysfunction, infective endocarditis, and pacemaker implantation. The troponin levels were significantly higher in the patients who had septal myectomy. The degree of myectomy affected the risk of complications in the early post-operative period and recurrence in the later period. However, when the gradient was substantially or completely removed by myectomy, patients experienced significant symptom improvement in the early post-operative period, and their late survival was equivalent to that of healthy individuals of the same age.Our findings suggest that monitoring troponin levels in patients undergoing septal myectomy may be beneficial in predicting the risk of complications. However, further studies are needed to establish the optimal surgical technique and extent of muscle resection required for subaortic stenosis treatment. Our study adds to the existing knowledge of the benefits and risks associated with septal myectomy as a treatment option for subaortic stenosis.

The effect of albumin and total protein values as nutritional markers after Fontan surgery on prognosis.

INTRODUCTION: Children with CHD develop heart failure due to increased pulmonary blood flow, cyanosis, and pulmonary hypertension. The metabolic needs of these children differ from those of healthy children, and malnutrition is common. Protein-losing enteropathy has been reported in 5 to 13% of patients after the Fontan procedure. Serum albumin and total protein levels, which are indicators of the quality of post-operative care, can be useful tools for monitoring and examining the intensive care treatment strategies of these patients. In our retrospective study, the effects of albumin and total protein values, which are two of the markers that give us an idea about diet, nutritional status, and inflammation, on the prognosis of children who underwent the Fontan procedure were investigated. METHOD: In our study, 127 patients who underwent Fontan procedure in our clinic between 2012 and 2021 were analysed retrospectively. Of the patients, 52.7% (n = 67) were male and 47.3% (n = 60) were female. The mean age is 5.83 ± 4.63 years. Patients who underwent albumin replacement were not included in the study. RESULTS: Although the relationship between pre-operative albumin and total protein values and post-operative mortality was not statistically significant, the inverse correlation of post-operative albumin 1st, 2nd, and 3rd-day values and post-operative total protein 1st, 2nd, and 3rd-day values with mortality was found to be statistically significant. In addition, we found that mortality was statistically high in patients whose total protein amount was below 6.65 mg/dl in the early post-operative period. CONCLUSION: Albumin and total protein, whose blood levels can vary with diet, can be used as predictors in the early post-operative prognosis of Fontan patients. In addition, when we examined the exitus patients, it was observed that the total protein amount was below 6.65 mg/dl on the post-operative 1st day. Based on this, we think that a diet with high protein content before surgery will help reduce post-operative early mortality.

Effects of patient blood management in pediatric heart surgery.

OBJECTIVE: Multidisciplinary patient blood management practices reduce costs of blood products and transfusion-related complications in hospitals. Hospital costs are high in pediatric cardiac surgery patients because high hematocrit levels are usually needed; the need for blood products is high due to complex and long surgical procedures; the length of stay in the intensive care unit (ICU) is long, and the use of treatment modalities such as dialysis and ECMO is common in this patient population. METHODS: In this retrospective study, we investigated the effects of the use of the multidisciplinary patient blood management protocols on the use of blood products and associated costs by comparing the outcomes of the protocol implemented in the year 2019 to the blood product use and costs of the previous year. In our clinic, 414 patients were operated on in 2019; 230 of them were males and 184 of them were females. RESULTS: Transfusions carried out in adherence to such protocols have reduced mortality rates along with a decline in hospital costs. CONCLUSIONS: We, too, achieved a 10% reduction in blood product costs per patient after the implementation of the multidisciplinary patient blood management protocol.

OBJETIVO: Las prácticas multidisciplinarias de manejo de la sangre del paciente reducen los costos de los productos sanguíneos y las complicaciones relacionadas con las transfusiones en los hospitales. Los costos hospitalarios son altos en pacientes pediátricos de cirugía cardíaca porque generalmente se necesitan niveles altos de hematocrito; la necesidad de hemoderivados es alta debido a los procedimientos quirúrgicos largos y complejos; la estancia en la unidad de cuidados intensivos (UCI) es larga y el uso de modalidades de tratamiento como la diálisis y la ECMO es común en esta población de pacientes. MÉTODOS: En este estudio retrospectivo; Investigamos los efectos del uso de los protocolos multidisciplinarios de manejo sanguíneo del paciente sobre el uso de hemoderivados y los costos asociados comparando los resultados del protocolo implementado en el año 2019 con el uso de hemoderivados y los costos del año anterior. En nuestra clínica se operaron 414 pacientes en 2019; 230 de ellos eran varones y 184 mujeres. RESULTADOS: Las transfusiones realizadas en cumplimiento de dichos protocolos han reducido las tasas de mortalidad junto con una disminución de los costos hospitalarios. CONCLUSIONES: Nosotros también logramos una reducción del 10% en los costos de los productos sanguíneos por paciente después de la implementación del protocolo multidisciplinario de manejo de sangre del paciente.

Surgical Approach and Management Strategies in a Pediatric Cardiovascular Surgery Clinic During the COVID-19 Outbreak

ABSTRACT Introduction: The coronavirus disease 2019 (COVID-19) pandemic has required changes in the management of pediatric cardiac surgery. We would like to share the patient treatment and surgical management strategies employed in our Pediatric Cardiovascular Surgery Clinic during the COVID-19 pandemic. Methods: A total of 112 patients were followed up in our clinic between 11.03.2020 and 02.07.2020. Their mean age was 1,118 (4-5,740) days. Management and treatment were performed by our pediatric heart team (pediatric cardiac anesthetists, general pediatricians, pediatric cardiologists, pediatric cardiac surgeons, and an infectious diseases specialist). We prepared new protocols and a surveillance system specific to the pandemic to prevent in-hospital transmission and reduce postoperative mortality and morbidity; our operations were performed according to these protocols. All decisions pertaining to the operation timing and treatment strategy of our COVID-19-positive patients were made by the same team. Results: During the study period, a total of 112 patients, 69 boys and 43 girls, were hospitalized in our clinic. A total of 333 COVID-19 real-time polymerase chain reaction tests were performed on patients and accompanying persons; positive results were found in three patients and two accompanying individuals. Conclusion: By employing new protocols and a surveillance system throughout the healthcare system, we think that early diagnosis and treatment of the pediatric congenital heart disease population, which is susceptible to infections, can continue unperturbed. This and similar approaches can increase postoperative success and prevent transmission in the pediatric population - which are frequently COVID-19 asymptomatic.

Surgical Approach and Management Strategies in a Pediatric Cardiovascular Surgery Clinic During the COVID-19 Outbreak.

INTRODUCTION: The coronavirus disease 2019 (COVID-19) pandemic has required changes in the management of pediatric cardiac surgery. We would like to share the patient treatment and surgical management strategies employed in our Pediatric Cardiovascular Surgery Clinic during the COVID-19 pandemic. METHODS: A total of 112 patients were followed up in our clinic between 11.03.2020 and 02.07.2020. Their mean age was 1,118 (4-5,740) days. Management and treatment were performed by our pediatric heart team (pediatric cardiac anesthetists, general pediatricians, pediatric cardiologists, pediatric cardiac surgeons, and an infectious diseases specialist). We prepared new protocols and a surveillance system specific to the pandemic to prevent in-hospital transmission and reduce postoperative mortality and morbidity; our operations were performed according to these protocols. All decisions pertaining to the operation timing and treatment strategy of our COVID-19-positive patients were made by the same team. RESULTS: During the study period, a total of 112 patients, 69 boys and 43 girls, were hospitalized in our clinic. A total of 333 COVID-19 real-time polymerase chain reaction tests were performed on patients and accompanying persons; positive results were found in three patients and two accompanying individuals. CONCLUSION: By employing new protocols and a surveillance system throughout the healthcare system, we think that early diagnosis and treatment of the pediatric congenital heart disease population, which is susceptible to infections, can continue unperturbed. This and similar approaches can increase postoperative success and prevent transmission in the pediatric population - which are frequently COVID-19 asymptomatic.

New trend: Surgical correction of Ebstein's anomaly using modified cone repair and a combination of different techniques.

BACKGROUND: Ebstein's anomaly,a rare cardiac disease that accounts for <1% of all congenital heart diseases, is a right ventricular myopathy with varying degrees of delamination in the endocardium under the tricuspid valve. Cone reconstruction, first described by da Silva in 2004, is a modification of the Carpentier technique. An alternative surgical treatment for Ebstein's anomaly, it is now the preferred surgical corrective technique. AIMS: This study demonstrates that our modifications of the cone repair procedure to correct Ebstein's anomaly improve valve coaptation and stabilization in the early and midterm. MATERIALS AND METHODS: Of the 134 patients diagnosed with Ebstein's anomaly in our clinic between January 2012 and October 2020, 10 underwent a cone procedure and its modifications and were thus included in the study. The mean age of these patients was 28.6 ± 18.79 years and ranged from 1 to 61 years. Thirty percent of the patients were male and 70% were female. RESULTS: The additional procedures performed were an isolated cone procedure (two patients), anomalous pulmonary venous return repair in addition to a cone procedure (one patient), single-vessel coronary artery bypass (one patient), Glenn procedure (two patients), and tricuspid ring annuloplasty (four patients). CONCLUSION: Based on the results of this study, which assesses the outcomes of patients who underwent cone repairs with surgical modifications, we argue that right ventricular oblique plication, tricuspid valve delamination, surgical valve rotation, and tricuspid ring annuloplasty protect valve coaptation and function in the early and midterm, thereby having beneficial effects on right ventricle remodeling.

Preoperative monocytosis is predictable at diagnosis for RT-PCR negative COVID-19 paediatric cardiac surgery patients.

Other markers investigated in this population have gained importance in the diagnosis of the disease since the course of COVID-19 disease is atypical in the paediatric population and PCR may be misleading. The leukocyte profile is one of these biochemical tests. Children did not have lymphopenia in hemogram count, whereas relatively neutropenia and monocytosis were detected, unlike the adult population. The reason why children do not have lymphopenia is thought to be due to the fact that the thymus is more active in the first years of life.Two-hundred and four patients operated in our paediatric cardiac surgery clinic from 11March, 2020 to 1 April, 2021 were retrospectively examined and 11 patients with preoperative asymptomatic and PCR (-), but with PCR (+) in the post-operative period (patients with incubation period or false PCR negativity) were included in our study. Patients requiring emergency operation and operated from PCR (+) patients in the preoperative period were excluded from the study.The neutrophil ratio in the lymphocytic series of 7 patients out of 11 patients was slightly below the normal range in the preoperative period, the lymphocyte ratio of 3 patients was slightly above the normal range, and the relative monocyte ratio of 10 patients was slightly above the normal range.We think that evaluating the leukocyte profile combined with RT-PCR will give more accurate results in the diagnosis of incubation period and false RT-PCR negative patients. In addition, we believe that the algorithms for non-complex paediatric cardiac surgery procedures and timing in the paediatric population with a better course of COVID-19 disease with a positive post-operative course.

A gray zone in the literature: indications, options, and outcomes for surgical repair of pulmonary artery aneurysms.

Pulmonary artery aneurysms are rare. They are characterised by an aneurysmatic dilatation of the pulmonary vascular bed, including the main pulmonary artery or the accompanying pulmonary artery branches. Increases in pulmonary flow and pulmonary artery pressure increase the risk of rupture: when these conditions are detected, surgical intervention is necessary.This study is a retrospective analysis of 33 patients treated in our paediatric cardiac surgery clinic from 2012 to 2020. Aneurysms and pseudoaneurysms in patients who were patched for right ventricular outflow tract reconstruction and corrected with a conduit were excluded from the study. Seventeen (51.5%) of the patients included in the study were female and 16 (48.5%) were male. The patients were aged between 23 and 61 years (mean 30.66 ± 12.72 years). Graft interpositions were performed in 10 patients (30.3%) and pulmonary artery plications were performed in 23 patients (69.7%) to repair aneurysms. There was no significant difference in mortality between the two groups (p > 0.05).Pulmonary artery aneurysm interventions are safe, life-saving treatments that prevent fatal complications such as ruptures, but at present there is no clear guidance regarding surgical timing or treatment strategies. Pulmonary artery interventions should be performed in symptomatic patients with dilations ≥5 cm or asymptomatic patients with dilations ≥8 cm; pulmonary artery pressure, right ventricular systolic pressure, and pulmonary artery aneurysm diameter must be considered when planning surgeries, their timing, and making decisions regarding indications. Experienced surgical teams can achieve satisfactory results using one of the following surgical techniques: reduction pulmonary arterioplasty, plication, or graft replacement.

Do platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR) have a predictive value on pediatric extracorporeal membrane oxygenation (ECMO) results?

BACKGROUND: It is difficult to predict the complications and prognosis of ECMO, which is gaining widespread use in patients with pediatric surgery. Platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR) are emerging inflammatory markers that can be calculated from complete blood count, which is a cheap and easily accessible laboratory analysis. The ratios between cellular elements in peripheral blood have been demonstrated to provide information on inflammation, infection, and immune response. METHODS: Sixty-seven patients who needed ECMO application after undergoing pediatric cardiovascular surgery in our clinic, between May 2005 and April 2020, were included in this study. The age of patients varied between 4 days and 17 years with a mean of 30.59 ± 147.26 months. RESULTS: The relationships between PLR or NLR values and various blood parameters and blood gas results were found to be statistically nonsignificant in our group of pediatric ECMO recipients. Even if the effect of PLR and NLR values on mortality and prognosis is statistically nonsignificant in patients who need ECMO after congenital heart surgery, PLR and NLR are typically elevated in the postoperative period. An increase in these values above a certain threshold may be a statistically significant indicator for the prediction of mortality. CONCLUSIONS: There are few studies in the literature concerning PLR and NLR values in patients with pediatric heart surgery. We consider this study will make way for new studies in the future.

Impact of platelet population behaviours in patients undergoing the Glenn procedure.

Pulmonary pressure is one of the most important parameters in the postoperative follow-up of patients who have undergone the Glenn procedure. Platelet activation markers, which are inexpensive and easily accessible blood count parameters, have been shown to be associated with the aetiology and pathogenesis of primary pulmonary artery hypertension. We examined the relationship between platelet activation markers and pulmonary pressures in the early postoperative period of patients who underwent the Glenn procedure.Eighty-five patients who underwent the Glenn procedure in our clinic between January 2011 and March 2020 were included in the study retrospectively. Fifty-one patients were male and 34 were female, and age varied from 4 to 416 months, with a mean of 28.64 ± 51 months.Patients with increased pulmonary blood flow on palliation before Glenn surgery had higher mean platelet volume values. However, no correlation was found between pulmonary pressures and platelet activation markers in the early postoperative period.There was not similar study evaluating platelet activation markers in the paediatric age group before and after postoperative Glenn surgery in the literature. Therefore, even if platelet activation markers provide information about the pulmonary bed, they may be misleading due to other reasons that trigger bleeding and inflammatory processes in the early postoperative period.

Temperate approach to ASD closure in pulmonary arterial hypertension: the fenestrated patch technique.

The most common CHD in the adult patient population is an atrial septal defect due to its asymptomatic nature in early life. However, when diagnosis and treatment are delayed, pulmonary arterial hypertension may develop as a long-term complication, sometimes before adulthood. The presence of PAH adversely affects the results of surgical treatment and may even eliminate the feasibility of surgery in some patients. In such patients who have high pulmonary artery pressure and pulmonary vascular resistance at the margin of inoperability, the response to the acute vasoreactivity test determines the treatment modality.In our retrospective study, a total of 906 patients who underwent ASD closure between January, 2011 and November, 2020, seven of which had undergone the fenestrated patch procedure after they were identified to have high PAP, but positive AVT test response were included. Short-term follow-up of patients with fenestrated ASD patches revealed decreased pulmonary pressure, regression in NYHA classification, and improvement in symptoms.The fenestrated patch technique should be kept in mind as an option to expand the surgical spectrum in the treatment of patients with high pulmonary pressures, where complete closure is risky. It appears that the fenestrated patch technique is a safe approach in the management of pulmonary hypertension in patients with ASD who have pulmonary hypertension, according to short- and midterm follow-up findings.

Neurological complications after pediatric cardiac surgery.

Background: The number of pediatric patients who survive open-heart surgery has increased in recent years and the complications seen in this patient group continue to decrease with each technological advance, including new surgical and neuroprotective techniques and the improvement in surgeons' experience with this patient population. However, neurological complications, which are the most feared and difficult to manage, require long-term follow-up, and increase hospital costs remain a leading cause of mortality and morbidity in this cohort. Results: We evaluated the neurological physical examination, cranial computed tomography (CT), and magnetic resonance (MRI) records of 162 pediatric patients with neurological symptoms lasting more than 24 h after undergoing heart surgery in our clinic between June 2012 and May 2020. The patients' ages ranged from 0 to 205 months, with a mean of 60.59 ± 46.44 months.Of the 3849 pediatric cardiac surgery patients we screened, 162 had neurological complications in the early period (the first 10 days after surgery). The incidence was calculated as 4.2%; 69 patients (42.6%) experienced seizures, 17 (10.5%) experienced confusion, 39 (24.1%) had stupor, and 37 (22.8%) had hemiparesis. Of the patients who developed neurological complications, 54 (33.3%) died. Patients with neurological complications were divided into 3 groups: strokes (n = 90), intracranial bleeding (n = 37), and no radiological results (n = 35). Thirty-four patients (37.8%) in the stroke group died, as did 15 (40%) in the bleeding group, and 5 (14.3%) in the no radiological results group. Conclusions: Studies on neurological complications after pediatric heart surgery in the literature are currently insufficient. We think that this study will contribute to a more detailed discussion of the issue. Responses to neurological events and treatment in the pediatric group may differ compared to the adult age group. Primary prevention methods should be the main approach in combating neurological complications; their formation mechanisms should be carefully monitored and preventive treatment strategies should be developed.

The use of neonatal extracorporeal life support in pediatric cardiac intensive care unit.

AIM: The aim of the study is to evaluate extracorporeal life support system (ECLS) employed in neonates in pediatric cardiac intensive care unit. MATERIAL AND METHODS: Twenty-five neonates that required ECLS in between November 2010 and November 2015 were evaluated. RESULTS: The median age was 12 days (range 3-28 days) and the median body weight was 3 kg (range 2.5-5 kg). Venoarterial ECLS was performed in all of the cases. Ascendan aorta-right atrial cannulation in 22 patients and neck cannulation in three patients were performed. The reason for ECLS was E-CPR in two patients, inability to wean from cardiopulmonary bypass (CPB) in seven patients, respiratory insufficiency and hypoxia in nine patients, low cardiac output (LCOS) in seven patients. Median duration of ECLS was four days (range 1-15). Hemorrhagic complications developed in 15, renal complications in 13, pulmonary complications in 12, infectious complications in 11, neurologic complications in three and mechanical complications in two of the patients. Weaning was successful in 15 of the patients. Eleven patients were successfully discharged. CONCLUSION: ECLS is an important treatment option that is performed successfully in many centers around the world to maintain life support in patients unresponsive to medical treatment. The utilization of this modality especially in newborns with congenital heart disease should be taken into consideration.

Analysis of the Early Results of 693 Patients Undergoing Valvular Reoperation Between 1993 and 2011.

BACKGROUND: The study aim was to identify factors affecting early mortality in valvular reoperations. METHODS: Between January 1993 and December 2011, a total of 693 patients who had undergone valvular reoperations due to problems with previously implanted mechanical and biological valves, new valve degeneration or valve failure after a reconstructive procedure was included in the study. Factors affecting early mortality were identified by the examination of preoperative and perioperative data, using multivariate analysis. RESULTS: The average age of the patients was 44.9 years. For all patients, overall hospital mortality was 15.9%, while hospital mortality rates were 12.9% and 35.3% for elective operation and urgent/emergency treatment, respectively. Factors affecting early mortality in the multivariate analysis were longer total perfusion time (>120 min, p = 0.001), emergency or urgent treatment (p = 0.001), and the presence of preoperative renal failure (p = 0.001). CONCLUSIONS: Mortality for elective patients in valvular reoperations was within an acceptable range. Total mortality was dependent on a high mortality level of emergency/urgency of the cases. The use of a well-defined protocol in valvular reoperations may decrease hospital mortality.

Impact of Coronary Artery Anatomy in Arterial Switch Procedure on Early Mortality and Morbidity.

OBJECTIVE: Arterial switch operation has become the treatment of choice for neonates with transposition of the great arteries. The most important step of the procedure is transferring the coronary arteries to the neoaorta successfully. This study shows the impact of coronary anatomy on early mortality and morbidity after arterial switch operation. METHODS: Ninety-two patients with transposition of the great arteries who underwent arterial switch operation between October 2010 and September 2014 were included in this retrospective study. The patients were classified into two groups: group I (n = 68, patients with usual coronary artery anatomy) and group II (n = 24, patients with unusual coronary artery anatomy). Median age was 10 days (6-25 days) in group I and 14 days (7-29 days) in group II. In group I, 25 patients had ventricular septal defect, nine patients had coarctation of the aorta or distal aortic arch hypoplasia, seven patients had Taussig Bing anomaly. In group II, nine patients had ventricular septal defect, one patient had coarctation of aorta, and one patient had Taussig Bing anomaly. RESULTS: Regarding the postoperative variables, no significant statistical difference was found between two groups. But cardiopulmonary bypass time is significantly longer in group II (P = .004). There was no difference in complications including the mortality (P = .265). It is statistically found that associated anomalies did not affect the mortality and complication rates. All mortality cases (n = 4) were related to ventricular dysfunction in group II, whereas only four of the eight patients died because of ventricular dysfunction in group I. CONCLUSION: Coronary artery pattern was not a predictor of mortality in early postoperative period. There may be an impact of unusual coronary artery pattern on the development of ventricular dysfunction as a cause of mortality.

Outcomes of the extracardiac fontan procedure using cardiopulmonary bypass: early results.

BACKGROUND: Although the avoidance of cardiopulmonary bypass during the Fontan procedure has potential advantages, using cardiopulmonary bypass during this procedure has no adverse effects in terms of morbidity and mortality rates. In this study, we assessed the postoperative outcomes of our first 9 patients who have undergone extracardiac Fontan operation by the same surgeon using cardiopulmonary bypass. METHODS: Between September 2011 and April 2013, 9 consecutive patients (3 males and 6 females) underwent extracardiac Fontan operation. All operations were performed under cardiopulmonary bypass at normothermia by the same surgeon. The age of patients ranged between 4 and 17 (9.8 ± 4.2) years. Previous operations performed on these patients were modified Blalock-Taussig shunt procedure in 2 patients, bidirectional cavopulmonary shunt operation in 6 patients, and pulmonary arterial banding in 1 patient. Except 2 patients who required intracardiac intervention, cross-clamping was not applied. In all patients, the extracardiac Fontan procedure was carried out by interposing an appropriately sized tube graft between the inferior vena cava and right pulmonary artery. RESULTS: The mean intraoperative Fontan pressure and transpulmonary gradient were 12.3 ± 2.5 and 6.9 ± 2.2 mm Hg, respectively. Intraoperative fenestration was not required. There was no mortality and 7 patients were discharged without complications. Complications included persistent pleural effusion in 1 patient and a transient neurological event in 1 patient. All patients were weaned off mechanical ventilation within 24 hours. The mean arterial oxygen saturation increased from 76.1% ± 5.3% to 93.5% ± 2.2%. All patients were in sinus rhythm postoperatively. Five patients required blood and blood-product transfusions. The mean intensive care unit and hospital stay periods were 2.9 ± 1.7 and 8.2 ± 1.9 days, respectively. CONCLUSIONS: The extracardiac Fontan operation performed using cardiopulmonary bypass provides satisfactory results in short-term follow-up and is associated with favorable postoperative hemodynamics and morbidity rates.

Introduction to a Norwood program in an emerging economy: learning curve of a single center.

BACKGROUND: There has been a notable improvement in the outcome of stage 1 palliation for hypoplastic left heart syndrome (HLHS) in recent years. Nevertheless, developing a new Norwood program requires a steep learning curve, especially in emerging economies where rapid population growth brings a high volume of patients but, on the other hand, resources are limited. In this paper we aimed to summarize the initial results of a single center. METHODS: Hospital records of 21 patients were reviewed for all patients having a stage 1 palliation procedure for HLHS between May 2011 and May 2013. There were 13 male (62%) and 8 female (38%) patients. Median age was 14 days (range, 4-74 days) and median weight was 3030 g (2600-3900 g). HLHS was defined as mitral or aortic stenosis or atresia (or both) in the presence of normally related great vessels and a hypoplastic left ventricle. Transthoracic echocardiography was the diagnostic modality used in all patients. All procedures but one were performed using an antegrade selective cerebral perfusion method and moderate hypothermia. Cerebral perfusion was monitored with cerebral oximetry in all patients. Modified ultrafiltration was routinely used in all patients. RESULTS: Overall hospital mortality was 47.6% (n = 10). Mortality rates considerably decreased from the first year to second year (69% and 12.5% respectively). No risk factors were identified for mortality. CONCLUSIONS: Surgical palliation of neonates with hypoplastic left heart syndrome continues to be a challenge. To decrease the overall mortality nationwide and improve outcomes, a referral center with a dedicated team is necessary in emerging economies.

Importance of adhesion molecules for children with congenital heart disease.

BACKGROUND: The aim of our study was to compare the blood levels of adhesion molecules in children with different heart diseases and pulmonary flow rates. METHODS: In this study, we evaluated the levels of soluble intercellular adhesion molecule-1 and soluble vascular cellular adhesion molecule-1 in blood samples of 65 children with different congenital heart diseases. The patients were divided into four groups according to their pulmonary blood flow. The first group had increased pulmonary blood flow with pulmonary hypertension and left-to-right shunt. The second group had increased pulmonary blood flow without pulmonary hypertension and left-to-right shunt. The third group had decreased pulmonary blood flow with cyanotic congenital heart disease and the fourth group had normal pulmonary blood flow with left ventricle outflow tract obstruction and aortic stenosis. RESULT: The highest soluble intercellular and vascular cellular adhesion molecule-1 levels with the mean values of 420.2 nanograms per millilitre and 1382.1 nanograms per millilitre, respectively, were measured in the first group and the lowest levels with the mean values of 104.4 and 358.6 nanograms per millilitre, respectively, were measured in the fourth group. The highest pulmonary blood pressure levels were found in the first group. CONCLUSION: Endothelial activity is influenced not only by left-to-right shunt with pulmonary hypertension, but also by decreased pulmonary blood flow in cyanotic heart diseases. Adhesion molecules are valuable markers of endothelial activity in congenital heart diseases, and they are influenced by pulmonary blood flow rate.

Modified Nikaidoh operation in a patient with dextro-transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction.

The optimal surgical management for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) remains controversial. We describe the case of a patient with TGA, a remote restrictive VSD, and LVOTO who underwent a successful modified Nikaidoh operation.