Bidirectional Cavopulmonary Anastomosis Research Articles

Heart Transplantation for Ebstein Anomaly due to Late Surgical Presentation.

Ebstein anomaly is a myopathy of the right ventricle characterized by failure of tricuspid valve delamination with accompanying tricuspid regurgitation. Its rarity, considerable anatomic variability, and frequent absence of symptoms can make the timing of surgery challenging. Contemporary tricuspid repair techniques can be performed with low mortality and bidirectional cavopulmonary anastomosis reduces the risk of operation when right ventricular function is poor. Here, we present a patient who presented late for surgery, failed high-risk conventional surgery and required heart transplantation.

Bilateral bidirectional cavopulmonary connection: a review of surgical techniques and clinical implications.

The presence of bilateral superior caval veins (bSCVs) could negatively influence the outcome of Fontan patients. In the setting of a bilateral bidirectional Glenn, the selective blood flow to the ipsilateral long with consequent flow stagnation in the connecting portion could lead to poor growth of the central portion of the pulmonary artery, potentially affecting the eligibility for Fontan completion and being associated with a higher incidence of thrombotic complications. Alternative surgical techniques have been described to perform a bidirectional cavopulmonary anastomosis in the presence of bSCVs aiming to achieve a balanced growth of the pulmonary bifurcation. The short-term results of these techniques such as the V- or Y-shape seem to be excellent; however, some anatomical settings could affect the feasibility of these techniques. The so-called "unifocalization" creates a configuration comparable to a "normal" bidirectional Glenn and could be a feasible alternative. However, the long-term results of this technique are not published yet. The positive effect of additional pulsatile pulmonary flow on pulmonary artery growth should be considered in case of bilateral bidirectional Glenn, despite the higher incidence of postoperative complications reported and the difficult calibration of the amount of additional flow. The role of computational fluid dynamic to simulate the surgical strategy in single ventricle patients is promising and could be worthwhile in the setting of bSCVs. In fact, the surgical techniques of bilateral bidirectional Glenn could be simulated testing their feasibility and allowing to identify the more favorable hemodynamic pattern, patient specific. This review article highlights the critical issues related to the presence of bSCVs in univentricular physiology, analyzing pros and cons of the different surgical techniques. Besides reviewing the literature, this manuscript focuses on the role of computational fluid simulation in identifying the most favorable surgical technique with an individualized approach, which could potentially improve the clinical outcome.

Surgical pulmonary arterioplasty at bidirectional cavopulmonary anastomosis leads to favorable pulmonary hemodynamics at final stage palliation

ObjectivePulmonary arterioplasty (PA plasty) at bidirectional cavopulmonary anastomosis (BDCA) is associated with increased morbidity, but outcomes to final stage palliation are unknown. We sought to determine the influence of PA plasty on pulmonary artery growth and hemodyamics at Fontan. MethodsWe retrospectively reviewed clinical data and outcomes for BDCA patients from 2006 to 2018. PA plasty was categorized by extent (type 1-4), as previously described. Outcomes included pulmonary artery reintervention and mortality before final palliation. ResultsFive hundred eighty-eight patients underwent BDCA. One hundred seventy-nine patients (30.0%) underwent concomitant PA plasty. Five hundred seventy (97%) patients (169 [94%] PA plasty) survived to BDCA discharge. One hundred forty out of 570 survivors (25%) required PA/Glenn reintervention before final stage palliation (59 out of 169 [35%]) PA plasty; 81 out of 401 (20%) non-PA plasty; P < .001). Twelve-, 24-, and 36-month freedom from reintervention after BDCA was 80% (95% CI, 74-86%), 75% (95% CI, 69-82%), and 64% (95% CI, 57-73%) for PA plasty, and 95% (95% CI, 93-97%), 91% (95% CI, 88-94%), and 81% (95% CI, 76-85%) for non-PA plasty (P < .001). Prefinal stage mortality was 37 (6.3%) (14 out of 169 PA plasty; 23 out of 401 non-PA plasty; P = .4). Five hundred four (144 PA plasty and 360 non-PA plasty) patients reached final stage palliation (471 Fontan, 26 1.5-ventricle, and 7 2-ventricular repair). Pre-Fontan PA pressure and pulmonary vascular resistance were 10 mm Hg (range, 9-12 mm Hg) and 1.6 mm Hg (range, 1.3-1.9 mm Hg) in PA plasty and 10 mm Hg (range, 8-12 mm Hg) and 1.5 mm Hg (range, 1.3-1.9 mm Hg) in non-PA plasty patients, respectively (P = .29, .6). Fontan hospital mortality, length of stay, and morbidity were similar. ConclusionsPA plasty at BDCA does not confer additional mortality risk leading to final palliation. Despite increased pulmonary artery reintervention, there was reliable pulmonary artery growth and favorable pulmonary hemodynamics at final stage palliation.

Superior cavopulmonary shunt via right vertical axillary mini-thoracotomy.

We present 2 cases of patients with univentricular heart physiology of different anatomic variants who successfully underwent minimally invasive bidirectional cavopulmonary anastomosis via the right vertical axillary mini-thoracotomy.

Towards Zero Phrenic Nerve Injury in Reoperative Pediatric Cardiac Surgery: The Value of Intraoperative Phrenic Nerve Stimulation.

Phrenic nerve injury is a devastating complication that results in significant morbidity and mortality. We developed a novel technique to localize the phrenic nerve and evaluate its success. Two groups of children underwent repeat sternotomy for a variety of indications. Group I (69 patients, nerve stimulator) and Group II (78 patients, no nerve stimulator). There was no significant difference in the mean age and weight between the two groups: (6.4 ± 6.5 years vs. 5.6 ± 6.4 years; p = 0.65) and (25.2 ± 24.1 vs. 22.6 ± 22.1; p = 0.69), respectively. The two groups were comparable in the following procedures: pulmonary conduit replacement, bidirectional cavopulmonary anastomosis, aortic arch repair, and Fontan, while Group I had more pulmonary arterial branch reconstruction (p = 0.009) and Group II had more heart transplant patients (p = 0.001). There was no phrenic nerve injury in Group I, while there were 13 patients who suffered phrenic nerve injury in Group II (p < 0.001). No early mortality in Group I, while five patients died prior to discharge in Group II. Eleven patients underwent diaphragm plication in Group II (p = 0.001). The mean number of hours on the ventilator was significantly higher in Group II (137.3 ± 324.9) compared to Group I (17 ± 66.9), p < 0.001. Group II had a significantly longer length of ICU and hospital stays compared to Group I (p = 0.007 and p = 0.006 respectively). Phrenic nerve injury in children continues to be associated with significant morbidities and increased length of stay. The use of intraoperative phrenic nerve stimulator can be an effective way to localize the phrenic nerve and avoid its injury.

Intraoperative transit-time flow measurement of caval veins before and after bidirectional cavopulmonary anastomosis.

Haemodynamic changes in caval venous flow distribution occurring during bidirectional cavopulmonary anastomosis operation are still largely unknown. Transit time flow measurements were performed in 15 cavopulmonary anastomosis operations. Superior and inferior caval vein flows were measured before and after the cavopulmonary anastomosis. Ratio of superior caval vein to overall caval veins flow was calculated. Mean superior caval vein flow ratio before cavopulmonary anastomosis was higher than previously reported for healthy children. Superior caval vein flow ratio decreased in 14/15 patients after cavopulmonary anastomosis: mean 0.63 ± 0.12 before versus 0.43 ± 0.14 after. No linear correlation between intraoperative superior caval vein pressure and superior caval vein flow after cavopulmonary anastomosis was found. Neither Nakata index nor pulmonary vascular resistance measured at preoperative cardiac catheterisation correlated with intraoperative flows. None of patients died or required a take down. The higher mean superior caval vein flow ratio before cavopulmonary anastomosis compared to healthy children suggests flow redistribution in univentricular physiology to protect brain and neurodevelopment. The decrease of superior caval vein flow ratio after cavopulmonary anastomosis may reflect the flow redistribution related to trans-pulmonary gradient. The lack of correlation between superior caval vein pressure and superior caval vein flow could be explained by limited sample size and multifactorial determinants of caval veins flow, although pressure remain essential. Larger sample of measurements are needed to find flow range potentially predictive for clinical failure. To authors' knowledge, this is the first intraoperative flow measurement of both caval veins during cavopulmonary operations.

Severe form of Ebstein’s anomaly associated with ventricular septal defect

Although the ventricular septal defect (VSD) that coexists with the severe form of Ebstein’s anomaly was thought to be beneficial for hemodynamics, it is unclear whether biventricular repair is always possible. Ebstein’s anomaly with VSD was diagnosed at 4 days of age based on a heart murmur and mild cyanosis. The cardiothoracic ratio was 78% and the Celermajer index was 1.6 (grade 4), but the hemodynamics were stable and tricuspid valve regurgitation was less than mild. Pulmonary overcirculation developed, and therefore, biventricular repair was attempted at 4 months of age, consisting of VSD patch closure, atrial septal defect partial closure, and the cone reconstruction of the tricuspid valve. Due to persistent cyanosis, however, nitric oxide inhalation and high flow nasal oxygen inhalation could not be discontinued. Catheter examination showed a cardiac index of 2.1 L/min/m2, moderate tricuspid regurgitation, and a pulmonary-to-systemic blood flow ratio of 0.64, and thus, takedown to one and one-half ventricular circulation using bidirectional superior cavopulmonary anastomosis, atrial communication closure, and right pulmonary artery banding was performed. The patient was discharged on postoperative day 25. Four months after discharge, the patient is doing well with home oxygen therapy.

The use of a modified blelock taussig anastomosis in the surgical treatment of complex congenital heart defects with univentricular hemodynamics and depleted pulmonary blood flow

Aim . To study the five-year experience of using a modified subclavian-pulmonary anastomosis according to BlelokTaussig in the surgical treatment of complex congenital heart disease in patients with univentricular hemodynamics and depleted pulmonary blood flow. Material and methods . From 2012 to 2020, 32 children with complex CHD, with duct-dependent blood flow, underwent operations to impose a modified subclavian-pulmonary Blelock-Taussig anastomosis (MBTA). The average age of the children was 13±6.7 months, and the average weight was 8.8± 5.2 kg. Results and discussion . Fatal outcome in the early postoperative period was observed in 3.13% of cases. Bleeding in the early postoperative period was observed in 6.25% of patients. In 28.1% of patients with Fallo’s tetrada thereafter radical correction of the defect was performed. Bidirectional cavopulmonary anastomosis was performed in 40.6% of patients with a single ventricle. Postoperative follow-up results were studied in 96.8% of patients. Conclusion . The use of a modified left subclavian-pulmonary anastomosis according to Blalock-Taussig is the optimal palliative procedure in children with depleted pulmonary blood flow. This surgery is performed to prepare the patient for subsequent correction of a complex heart defect and allows the right pulmonary artery to be preserved intact for future use during radical or hemodynamic correction of the defect.

Ebstein anomaly. Retrospective study.

Ebstein anomaly is a congenital heart malformation of the tricuspid valve and right ventricle. Surgical correction of Ebstein’s anomaly improves functional class and exercise tolerance, eliminates intracardiac right-to-left shunting (if present), and reduces the incidence of supraventricular tachyarrhythmias [3, 4, 6]. Due to the degree of variability of Ebstein’s Anomaly, correction is based on anatomical factors. In the case of unfavorable anatomical conditions, the replacement of the tricuspid valve is resorted to [3, 8]. The application of bidirectional cavopulmonary anastomosis is reserved for patients with poor right ventricular function [6, 9, 10]. Currently, overall early mortality after surgical correction in children and adults has decreased to less than 3% in experienced centers [4]. Surgical treatment of the symptomatic neonate remains a significant challenge, with approaches including either a biventricular correction or a univentricular correction. The given study is retrospective and reports the data on the incidence of the types according to the Carpentier classification, the severity and the surgical procedure applied for the correction of the anomaly [1, 9].

Intramyocardial cell-based therapy with Lomecel-B during bidirectional cavopulmonary anastomosis for hypoplastic left heart syndrome: the ELPIS phase I trial.

Hypoplastic left heart syndrome (HLHS) survival relies on surgical reconstruction of the right ventricle (RV) to provide systemic circulation. This substantially increases the RV load, wall stress, maladaptive remodelling, and dysfunction, which in turn increases the risk of death or transplantation. We conducted a phase 1 open-label multicentre trial to assess the safety and feasibility of Lomecel-B as an adjunct to second-stage HLHS surgical palliation. Lomecel-B, an investigational cell therapy consisting of allogeneic medicinal signalling cells (MSCs), was delivered via intramyocardial injections. The primary endpoint was safety, and measures of RV function for potential efficacy were obtained. Ten patients were treated. None experienced major adverse cardiac events. All were alive and transplant-free at 1-year post-treatment, and experienced growth comparable to healthy historical data. Cardiac magnetic resonance imaging (CMR) suggested improved tricuspid regurgitant fraction (TR RF) via qualitative rater assessment, and via significant quantitative improvements from baseline at 6 and 12 months post-treatment (P < 0.05). Global longitudinal strain (GLS) and RV ejection fraction (EF) showed no declines. To understand potential mechanisms of action, circulating exosomes from intramyocardially transplanted MSCs were examined. Computational modelling identified 54 MSC-specific exosome ribonucleic acids (RNAs) corresponding to changes in TR RF, including miR-215-3p, miR-374b-3p, and RNAs related to cell metabolism and MAPK signalling. Intramyocardially delivered Lomecel-B appears safe in HLHS patients and may favourably affect RV performance. Circulating exosomes of transplanted MSC-specific provide novel insight into bioactivity. Conduct of a controlled phase trial is warranted and is underway.Trial registration number NCT03525418.

Innominate vein reconstruction in patients with bilateral superior vena cava undergoing bilateral bidirectional superior cavopulmonary anastomosis.

A 4.5-year-old patient with tricuspid atresia, pulmonary stenosis, bilateral superior vena cava veins with absent innominate vein, and hypoplasia of the left pulmonary artery required a bidirectional superior cavopulmonary anastomosis. An innominate vein was fashioned out of a 6 mm polytetrafluoroethylene graft. The technique is briefly discussed.

Comparison of the Methods of Surgical Treatment of Complex Congenital Heart Defects Combined with Right Ventricular Hypoplasia

Surgical treatment of complex congenital heart defects (CHD) in patients who cannot undergo radical correction due to pronounced hypoplasia or dysfunction of the right ventricle (RV) remains one of the most urgent problems in pediatric cardiac surgery. In 1989, Billingsley et al. first reported the addition of bidirectional cavopulmonary anastomosis to definitive biventricular repair in patients with RV hypoplasia, pulmonary artery atresia, and intact interventricular septum in four patients, and introduced the term one and half ventricle repair (1.5VR). The method of 1.5VR consists of bidirectional cavopulmonary anastomosis, which reduces the volume load on the RV, in addition to the complete repair of other CHDs.&#x0D; The aim. To assess perioperative characteristics of patients, immediate and long-term results after surgical correction of complex CHD through the method of 1.5VR.&#x0D; Materials and methods. In the period from 1996 to 2022, surgical correction was performed in 33 patients with complex CHD combined with hypoplasia and/or dysfunction of the RV at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. There were 21 male patients (64%) and 12 female patients (36%). The mean age of the patients at the time of surgery was 57.9 months. Me = 34 [3.5; 312] months. The main method of diagnosis in determining the defect and assessing the immediate and long-term results was echocardiographic examination and probing of the heart cavities, which made it possible to assess the morphology, kinetics of the heart structures, and hemodynamic state. Depending on the selected tactics of surgical treatment, all the patients were divided into two groups: group І (n=21) with patients operated through the method of 1.5VR, and group ІІ (n = 12) including those operated through the single ventricle pathway.&#x0D; Results. In the early postoperative period, there were 3 (9%) deaths in both groups: one (4.7%) death in group I, and 2 (16%) deaths among patients of group II. In all the deceased patients, the cause of death was acute heart failure, which subsequently led to multiple organ failure. In one patient of the group II, in addition to heart failure, acute cerebrovascular accident occurred. In the group I, the average indicator of systemic saturation (92±6.2%) at discharge from the hospital was higher, than that in group II (87±4.7%). Average duration of mechanical ventilation (10±7.3 vs. 15±7.8 hours), total exudation (65±34 vs. 88.8±39.9 hours), total duration of sympathomimetic support (71±27.5 vs. 108±75.5 hours) and its doses (4.8±2 vs. 6.5±3.8 μkg/kg/hour) were significantly lower in the group I, therefore, the patients of the group II stayed almost twice as long in the intensive care department (146±56 vs. 96±49.8 hours), although the total length of hospital stay did not differ significantly (25±3.8 vs. 26±12.3 days). An uncomplicated course of the early postoperative period was observed in 15 (46%) patients. Other 18 (54%) patients had complications: 8 patients (24%) of group I had 9 complications, and 10 patients (30%) of group II had 14 complications. The mean observation period was 48±29.8 months, (6 to 190 months). No deaths were observed during the observation period in 26 patients (79%) of both groups, 4 (12%) patients were lost to follow-up. Total cavоpulmonary anastomosis (Fontan procedure) was performed in 7 patients (58%) of the group II. Hemodynamic indicators in the examined 17 patients (81%) of the group I showed good long-term results that did not require other interventions. In 15% (3 of 20) of the patients of the group I, a high mean pressure in the right atrium of 14±1.8 mmHg attracts attention with dilatation of the inferior vena cava and hepatic veins, and 5 (20%) patients had high mean pressure of 16±2.7 mmHg in the superior vena cava. Transplantation-free survival during the observation period in patients of both groups was 100%.&#x0D; Conclusions. 1.5VR is a reasonable alternative to the palliative Fontan strategy in children in whom the anatomic or functional status of the RV is between biventricular reconstruction and the single ventricle pathway and shows good immediate and long-term results.

Contemporary risk factors for a longer hospital stay following bidirectional cavopulmonary anastomosis.

Despite high survival after bidirectional cavopulmonary anastomosis, a considerable number of patients suffer significant post-operative morbidities related to prolonged length of stay. A single-center retrospective cohort study of all consecutive patients undergoing a first-time bidirectional cavopulmonary anastomosis from 2006 to 2019. Prolonged length of stay was defined as hospital stay greater than the 75th percentile for our cohort. Of 195 patients who met inclusion criteria, the median post-operative length of stay was 8 days (interquartile range, 4-15 days). Prolonged length of stay was defined as greater than 15 days. In multivariate analysis, greater than mild systemic atrioventricular valve regurgitation (odds ratio 3.7, 95% CI 1.05-13.068, p = 0.04), longer length of stay after the initial palliative procedure (odds ratio 1.028, 95% CI 1.004-1.05, p = 0.02), and pre-operative higher superior vena cava oxygen saturation (odds ratio 0.922, 95% CI 0.85-0.99, p = 0.04) maintained statistical significance as independent risk and protective factors for prolonged length of stay. A one-level increase in the severity of pre-operative systemic atrioventricular valve regurgitation was associated with a multiplicative change in the odds ratio of prolonged length of stay of 5.45 (p = 0.005) independent of the severity of systemic ventricular dysfunction. Pre-operative characteristics with greater than mild systemic atrioventricular valve regurgitation, longer length of stay after the initial palliative procedure, and lower superior vena cava oxygen saturation were associated with prolonged length of stay after a first-time bidirectional cavopulmonary anastomosis.

Experience in Performing Bidirectional Cavopulmonary Anastomosis in the Surgical Treatment of Tricuspid Atresia

Tricuspid atresia (TA) is a rare cyanotic congenital heart disease (CHD) first described by Kreysig (1817). Bidirectional cavopulmonary anastomosis (BCPA) is part of the hemodynamic correction of such a complex combined CHD. In patients after BCPA, blood flow through the superior vena cava, providing efficient pulmonary blood flow, is the most important factor influencing blood oxygen saturation.&#x0D; The aim. To evaluate perioperative characteristics of the patients, immediate and long-term results after BCPA with TA.&#x0D; Materials and methods. In the period from January 1996 to April 2022, 50 patients with TA and multiple concomitant CHD underwent hemodynamic correction at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. There were 27 male patients (54%) and 23 female patients (46%). The mean age of the patients at the time of surgery was 31.6 ± 17.2 months (from 6 to 144 months). The main method of diagnosis in determining the defect and assessing the immediate and long-term results was echocardiographic examination and probing of the heart cavities. Among the 50 patients surveyed, 17 children were older than 2.5 years, so all the subjects were divided into 2 age groups: group I (n = 33) up to 2.5 years, group II (n = 17) older than 2.5 years to determine the optimal time of hemodynamic correction based on immediate and long-term results.&#x0D; Results. In the early postoperative period, there were 2 (4%) deaths among the patients of group II. The cause of death was acute heart failure in one case and infectious factor which led to the development of sepsis in another. There was a very interesting trend of increase in the duration of the operation (min): 239 ± 73.3 in group I, 251 ± 68.5 in group II; duration of mechanical ventilation (hours): 5.3 ± 1.4 in group I, 11 ± 3.7 in group II; duration of exudation (hours): 67 ± 22.9 in group I, 76 ± 26.8 in group II. There was significantly higher average dose of sympathomimetic (μg/kg/hour): 5 ± 2.1 for 60 ± 21.4 (hours) in group I; 7 ± 4.5 for 84 ± 29 (hours) in group II, hospital stay (days): 18 ± 5.7 in group I, 22 ± 11.3 in group II. Significantly lower mean systemic saturation at discharge 81 ± 4.6 (%) which was reported in children of group II compared to those of group I 88 ± 3.4 (%) clearly shows a decrease in the contribution of superior vena cava flow into the systemic circulation depending on the patient’s age, therefore, the clinical effect of performing BCPA as a stage of single-ventricular correction in patients with TA is much better when performing surgery in early childhood. Early postoperative period was uneventful in 27 (54%) patients. The other 23 (46%) patients of both groups had 16 and 18 complications in 10 (20%) patients of group I and 13 (26%) patients of group II, respectively, due to adverse factors of preoperative condition and specific changes in blood circulation characteristic of the BCPA. During the observation period lasting 49 ± 23.5 months, from 6 to 180 months, the final stage of hemodynamic correction was examined and performed: total cavopulmonary anastomosis was created in 31 (65%) patients. No deaths were reported after the final stage of hemodynamic correction.&#x0D; Conclusions. The contribution of superior vena cava flow to total cardiac output is directly related to the patient’s age and gradually decreases in the elderly, which indicates a decrease in systemic saturation, so the clinical effect of BCPA as a stage of single ventricular correction in patients with TA is much better when performing surgery in early childhood.

Risk factors for death or mechanical ventilation time after bidirectional cavopulmonary anastomosis in a developing country.

Glenn procedure is performed for patients with cyanotic CHD and univentricular physiology and has a survival rate above 90%. To evaluate the risk factors associated with a poor outcome after Glenn procedure. The data for this retrospective analysis were collected from a regional Brazilian registry of congenital heart surgeries (ASSIST initiative) from 2014 to 2019. Data from 97 patients who underwent the Glenn procedure were considered. The primary outcomes were prolonged mechanical ventilation (>24 hours post-operatively) or in-hospital death, alone and combined. The overall in-hospital mortality was 13.4% (n = 13). Prolonged mechanical ventilation occurred in 52% (n = 51) of the patients. Our analysis found that risk factors for death or prolonged post-operative mechanical ventilation were cardiopulmonary bypass duration and post-operative lactate, whereas weight-for-age z-score and age at surgery were risk factors for prolonged mechanical ventilation. Cardiopulmonary bypass duration increased (adjusted odds ratio: 1.02; 95 % CI: 1.01, 1.03) and age at surgery decreased (adjusted odds ratio: 0.96; 95 % CI: 0.93, 0.99) the odds of the combined outcome. Age at surgery, post-operative lactate, and low-weight-for-age z-score are associated with prolonged mechanical ventilation and death following the Glenn procedure.

Clinical Impact of Autologous Cell Therapy on Hypoplastic Left Heart Syndrome After Bidirectional Cavopulmonary Anastomosis

Preservation of right ventricle function (RV) is a key to favorable outcome in Hypoplastic Left Heart Syndrome (HLHS), but methods to preserve or improve RV function are limited. Our goal was to assess the clinical and functional impact of autologous umbilical cord blood-derived mononuclear cells (UCB-MNC) therapy when given to patients with HLHS at Stage II surgery. UCB-MNC patients were enrolled prospectively in a phase I, FDA monitored trial as previously described (Burkhart etal., 2019). Matched retrospective controls were identified by review of clinical databases. Growth and RV echocardiographic variables were assessed in both groups prestage II through the first 6 months postoperatively. Statistical comparisons between the groups at similar postoperative time points were made to define potential impact of the cell therapy. There were 7 UCB-MNC patients and 17 controls. Prestage II, most parameters showed no differences between groups, although median fractional area change (FAC) was slightly greater in the controls (FAC: controls = 45% vs UCB-MNC = 41% P= 0.02). At dismissal, FAC and estimated Ejection Fraction (EF) decreased in controls, while both were unchanged from baseline in UCB-MNC patients (ΔFAC: -5% vs -1%, P < 0.01; ΔEF: -8% vs 0%, P = 0.03, respectively). Subsequently, median FAC increased slightly in UCB-MNC patients over the 6 month follow-up period, while it decreased in controls (ΔFAC: UCB-MNC +3% vs control -5%, P = 0.03). Preoperative weight percentiles were similar in both groups (UCB-MNC 34%ile vs controls 22%ile, P = 0.93). However, by 6 months postoperative, median weight percentile improved to 63% in the UCB-MNC treated group, but declined to 8% in controls (P = 0.02). UCB-MNC therapy appears to limit the initial negative impact on RV FAC and EF seen after stage II surgery. During early follow up, FAC and weight percentile improved in UCB-MNC patients relative to controls, suggesting a beneficial effect of UCB-MNC therapy.

In Hospital Outcomes after Bidirectional Cavopulmonary Anastomosis: 18 Year Experience from a Single Center

Background: Bidirectional cavopulmonary shunt (BCPS) is used for the interim palliation of a variety of cyanotic cardiac lesions. It is a standard palliative step for patients with functionally univentricular hearts in hope of achieving Fontan completion. At our center, first case of BCPS was performed on February 7, 2002. Here, we share our experience with BCPS over last 18 years.&#x0D; Methods: This is a retrospective analysis of all patients undergoing BCPS from February 2002 to July 2019. Patients who underwent BCPS as a part of one-and-half ventricular repair for Ebstein’s anomaly were excluded. Baseline, intraoperative, and postoperative variables were collected from hospital records.&#x0D; Results: A total of 326 patients with mean age of 5.7±5.8 years (median 3.5 years; range: 1.5 months-32 years) underwent BCPS over last 18 years. Majority (61%) were males. Double outlet right ventricle (DORV) was the most common primary cardiac lesion (30%) followed by tricuspid atresia (23%). Mean ICU stay was 3.9±4.8 days, with hospital stay of 7.8±5.9 days. In-hospital mortality was 15%. Patients who died had low body weight (11.8±10.7 kg vs 16.0±11.6 kg; p=0.019), and longer cardiopulmonary bypass time (101±64 min vs 76±42 min; p=0.001). Oxygen saturation improved significantly at the time of discharge (79.2±9.5% vs 68.6±13.7%; p=0.000).&#x0D; Conclusion: This is the first report of its kind to analyze the outcomes of BCPS in our center. We have discussed the evolution of BCPS surgery in our center and presented our outcomes. Our in-hospital mortality remains high, and we need to strive towards reducing the mortality.

The assessment of patients with situs inversus and corrected transposition of the great arteries

IntroductionCongenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect. There are different subgroups according to the location of the heart in the thorax, apical position and situs. ObjectivesThe purpose of this study was to assess pediatric patients with situs inversus (SI) ccTGA (SI-ccTGA), a rare subgroup of this condition, in detail. MethodsThe records of patients with SI-ccTGA followed between January 1, 2010 and January 1, 2019 in our clinic were analyzed retrospectively. Demographic features, associated cardiac defects, arrhythmias and follow-up data were recorded. ResultsTwenty-one out of 120 ccTGA patients had SI. The median age was 30 months (4 days-18 years). There were hemodynamically significant associated lesions in 85.7% (n=18) of the patients. A large ventricular septal defect was found in 16 patients (76.2%), severe pulmonary stenosis in 11 (52.4%), pulmonary atresia in six (28.5%), and severe tricuspid regurgitation in two (9.5%). Eleven out of 21 patients had biventricular physiology and eight had single-ventricle physiology. Bidirectional cavopulmonary anastomosis followed by a hemi-Mustard-Rastelli operation were planned for the remaining two patients. Twelve out of 18 patients with associated defects (66.6%) were operated and surgery was planned for three more patients (16.6%). The remaining three patients were scheduled for clinical follow-up. Arrhythmias developed in two (9.5%) patients on follow-up; ablation was performed in one of them and pacemaker implantation followed by cardiac resynchronization therapy was performed in the other. Two patients died during follow-up, one after a central shunt operation and the other preoperatively due to pneumonia and sepsis. ConclusionSI-ccTGA is not a mirror image of situs solitus ccTGA (SS-ccTGA) due to important anatomic and physiologic differences between them. SI-ccTGA patients have a lower risk of tricuspid valve regurgitation than SS-ccTGA patients. The timing of clinical presentation of these patients mainly depends on the type and severity of the associated lesions, as in all subtypes of ccTGA.

Post-Glenn Shunt Systemic Desaturation—Is Venovenous Collateral the Culprit?

AbstractAfter classic Glenn shunt or bidirectional cavopulmonary anastomosis, the reappearance or deepening of cyanosis may be due to systemic venous collateral channels. There are only few case reports on this issue in the present literature. Here, we present two cases that underwent bidirectional Glenn shunt, who later presented with cyanosis and desaturation and both of them were found to have venovenous collaterals.

Early and long-term results of surgical correction for Ebstein anomaly by cone reconstruction

Objective. To evaluate the results of surgical correction of Ebstein anomaly by cone reconstruction in the early and late postoperative periods of observation. Material and Methods. The study included 35 patients with Ebstein anomaly aged 18 years and younger. The average age of patients was 5.5 [2; 10] years; weight was 23.4 [12.5; 27.5] kg; and height was 112.7 [90; 137] cm. All patients underwent surgical treatment for Ebstein anomaly by cone reconstruction, including four cases (11.4%) requiring the creation of a bidirectional cavopulmonary anastomosis. The echocardiographic parameters of the heart were studied in the preoperative, early postoperative, and long-term observation periods. The main emphasis was on the evaluation of tricuspid valve (TV) function indicators, including TV regurgitation degree, pressure gradient, Z-score, and size of the valve ring expressed as percentages of reference stature-weight values. The electrocardiographic records of patients were evaluated to analyze cardiac rhythm disturbances. The maximum period of observation was eight years. Results . The indexed sizes of the right atrium and right ventricle significantly decreased and the left ventricular end-diastolic index and stroke volume increased in the early postoperative period. In the long-term period of observation, there was an increase in the fibrous ring of TV ( p &lt; 0.05), whereas the tricuspid pressure gradient and regurgitation did not change over time. Atrioventricular conduction disorders were not detected among patients in the early postoperative period. The bidirectional cavopulmonary anastomosis was created in the presence of the following conditions: the TV mean pressure gradient was more than 8 mmHg; the pressure gradient between the right and left atriums was above 1.5.