Cavopulmonary Anastomosis Research Articles

Congenital Heart Surgery Outcomes in Turner Syndrome: The Society of Thoracic Surgeons Database Analysis

Turner syndrome (TS) is a genetic syndrome characterized by monosomy X (45,XO) in phenotypic females and is commonly associated with congenital heart disease. We sought to describe the distribution, mortality, and morbidity of congenital heart surgery in TS and compare outcomes to individuals without genetic syndromes. The Society of Thoracic Surgeons Congenital Heart Surgery Database was used to evaluate index cardiovascular operations performed from 2000 to 2017 in pediatric patients (aged 0-18 years) with and without TS. Analyses were stratified by the most common operations, including coarctation repair, aortic arch repair, partial anomalous pulmonary venous return repair, Norwood, superior cavopulmonary anastomosis (Glenn), and Fontan. Included were 780 operations in TS and 62,659 operations in controls. The most common TS operations were coarctation repair in 274 (35%), aortic arch repair in 116 (15%), and Norwood in 59 (8%). Compared with controls, TS patients had lower weight-for-age Z-scores across all operations (P < .01 for all); however, operative mortality rates did not differ significantly. The chylothorax rate was higher in TS after coarctation repair (8.8% vs 2.8%, P < .001) and Norwood (22% vs 8.1%, P< .001). The median (interquartile range) postoperative length of stay was longer in TS for coarctation repair (6.5 [5.0-15.5] days vs 5.0 [4.0-9.0] days, P < .001), aortic arch repair (15.0 [8.0-27.5] days vs 11.0 [7.0-21.0] days, P= .004), and Glenn (9.0 [6.0-16.0] days vs 6.0 [5.0-11.0] days, P= .013). Turner syndrome patients most commonly underwent operations for left-sided obstructive lesions. Despite increased morbidity for select operations, TS was not associated with increased operative mortality.

Results of Hemodynamic Correction in Patients with Double Outlet Right Ventricle

Aim. To analyze results of hemodynamic correction in surgical treatment of double outlet right ventricle (DORV).&#x0D; Маterials and methods. For the period from January 1996 to September 2017, 31 (6.03 % of total number of patients with DORV) patients underwent hemodynamic correction of DORV. The age of the patients ranged from 1 to 19 years (71.2 ± 50.5 months on the average). The weight of the patients ranged from 9 to 41 kg (19.6 ± 11.3 kg on the average). Of these, 19 (61.3 %) were male patients and 12 (38.7 %) were female patients. The overwhelming majority (25 (80.6 %)) of the patients were diagnosed with transposition-type DORV. The anatomy of DORV with non-committed ventricular septal defect was observed in 5 (16.1 %) patients. In one patient (3.1 %), the anatomy of DORV (in the form of tetralogy of Fallot) was combined with tricuspid valve atresia.&#x0D; Results. The main reasons of hemodynamic correction in 16 (51.6 %) cases was LV hypoplasia. In 2 cases it was combined with tricuspid valve (TV) straddling, and in 2 cases it was an integral part of the unbalanced form of complete atrio-ventricular communication (AVC). In one case (3.1 %), the unbalanced form of complete AVC was combined with a mixed form of the common ventricle. The mixed form of the common ventricle was the reason of hemodynamic correction in 9 (29 %) patients. In 2 (6.2 %) cases, hemodynamic correction was performed due to the anatomy of the RV hypoplasia. In the remaining 2 patients, anatomy of the common ventricle was not diagnosed, but a combination of other concomitant defects was a contraindication to biventricular correction. Palliative operations (Blalock-Taussig shunt, BTS) as the first stage of correction were performed in 16 (51.6 %) patients. In 2 patients with LV outflow tract obstruction, systemic-pulmonary anastomosis was applied in combination with plastic repair of the great vessel roots using the proprietary technique for elimination of the left ventricular outflow tract (LVOT) stenosis. Bidirectional cavopulmonary anastomosis (BCPA) was applied in 29 (93.5 %) cases. Of these, 4 (13.8 %) patients subsequently underwent total cavopulmonary anastomosis (TCPA) procedure. Two patients with good hemodynamic parameters underwent TCPA without prior palliative procedures.&#x0D; Conclusion. Palliative surgery as the first stage to hemodynamic correction is accompanied by significant improvement in hemodynamic parameters of patients. Application of BCPA as the second stage of hemodynamic correction provides good results and is required to prepare the patient for TCPA. The long-term period is characterized by improvement in the quality of life in patients with complex DORV. In the long-term period, 85.2 % of patients are classified as NYHA FC I.

Risk associated with anesthesia for noncardiac surgery in children with congenital heart disease.

Database analysis has indicated that perioperative cardiac arrest occurs with increased frequency in children with congenital heart disease. Several case series and large datasets from ACS NSQIP have identified subgroups at the highest risk. Consistently, patients with single ventricle physiology (especially prior to cavopulmonary anastomosis), severe/supra-systemic pulmonary hypertension, complex lesions, and cardiomyopathy with significantly reduced ventricular function have been shown to be at increased risk for adverse events. Based on these results, algorithms for assessing risk have been proposed. How hospitals and health care systems apply these guidelines to provide safe care for these challenging patient groups requires the application of modern quality improvement techniques. Each institution should develop a system which reflects local expertise and resources.

Early results of ring-reinforced conduit and curved porcine patch in Sano-Norwood procedure.

Patch augmentation of the aortic arch as well as construction of an unobstructed pulmonary blood supply are two important surgical targets in patients with hypoplastic left heart syndrome. In this report, we aimed to present our preliminary results with a combination of two relatively new approaches in Norwood-Sano procedure. A retrospective analysis was performed in 10 newborns with the diagnosis of hypoplastic left heart syndrome. Our surgical approach incorporated the interposition of a 6.0-mm ring-reinforced tube graft with the "dunked technique" between the right ventricle and the pulmonary artery; and reconstruction of the aortic arch using a curved porcine pericardial patch which is specifically designed for the Norwood procedure. Mean age and body weight at the time of the Sano-Norwood operation were 7.3 ± 2.4 days and 3164 ± 406 g, respectively. We encountered 1 (10%) early and 1 (10%) late mortality. All of the patients were discharged without any residual gradients at the aortic arch. Four out of eight patients underwent stage 2 bidirectional cavopulmonary anastomosis at a median age of 5 months (range, 4-6 months). Pericardial patch augmentation of the left pulmonary artery was deemed mandatory in one of our patients. The dunked technique of interposing a ring-reinforced conduit between the right ventricle and pulmonary artery along with the utilization of a curved porcine pericardial patch specifically designed for aortic arch reconstruction are promising modifications of the Sano-Norwood procedure in newborns with hypoplastic left heart syndrome.

Younger age remains a risk factor for prolonged length of stay after bidirectional cavopulmonary anastomosis

This study sets out to determine the influence of age at the time of surgery as a risk factor for post-operative length of stay after bidirectional cavopulmonary anastomosis. All patients undergoing a Glenn procedure between January 2010 and July 2015 were included in this retrospective cohort study. Demographic data were examined. Standard descriptive statistics was used. A univariable analysis was conducted using the appropriate test based on data distribution. A propensity score for balancing the group difference was included in the multi-variable analysis, which was then completed using predictors from the univariable analysis that achieved significance of p&lt;0.1. Over the study period, 50 patients met the inclusion criteria. Patients were separated into two cohorts of ⩾4 months (28 patients) and &lt;4 months (22 patients). Other than height and weight, the two cohorts were indistinguishable in their pre-operative saturation, medications, catheterisation haemodynamics, atrioventricular valve regurgitation, and ventricular function. After adjusting group differences, younger age was associated with longer post-operative length of hospitalisation - adjusted mean 15 (±2.53) versus 8 (±2.15) days (p=0.03). In a multi-variable regression analysis, in addition to ventricular dysfunction (β coefficient=8.8, p=0.05), Glenn procedures performed before 4 months were independently associated with longer length of stay (β coefficient=-6.9, p=0.03). We found that Glenn procedures performed after 4 months of age had shorter post-operative length of stay when compared to a younger cohort. These findings suggest that balancing timing of surgery to decrease the inter-stage period should take into consideration differences in post-operative recovery with earlier operations.

Corrigendum to "Indications for and results of axillary arterio-venous fistula in patients with a functionally single ventricle after cavopulmonary anastomosis" [Eur J Cardiothorac Surg 2019;55:572-578]†.

Corrigendum to "Indications for and results of axillary arterio-venous fistula in patients with a functionally single ventricle after cavopulmonary anastomosis" [Eur J Cardiothorac Surg 2019;55:572-578]†.

A better approach for left ventricular training in transposition of the great arteries and intact interventricular septum: Bidirectional cavopulmonary anastomosis and pulmonary artery banding.

Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients. Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage-1: palliation and ventricular training) in our center. In four out of these six patients, arterial switch operation was performed with takedown and direct re-anastomosis of the superior vena cava to right atrium after an interstage period of 21-30months (stage-2: anatomical repair). Any mortality was not encountered. The left ventricular mass indices increased from 18-32to 44-74g/m2 in patients undergoing the anatomical repair. All of the patients were uneventfully discharged following the second stage. The mean follow-up period was 20months (9-32months) following stage 2. All of the patients are doing well with trivial neoaortic regurgitation and normal biventricular function. Bidirectional cavopulmonary anastomosis with pulmonary artery banding may be a promising left ventricle training approach in ventriculoarterial discordance when compared to the traditional pulmonary artery banding with concomitant systemic-to-pulmonary artery shunt procedures which still carry a significant interstage morbidity and mortality.

Impact of Ventricular Morphology on Myocardial Deformation in Patients with Single Ventricle

Introduction: Functional single ventricle represent a heterogeneous group of anomalies sharing a common feature which is a functional single cardiac chamber. (1) In different series of congenital heart disease patients, cases of UVH represent about 1–2% of total congenital heart diseases. (2) Two-dimensional speckle-tracking echocardiography has recently emerged as a novel technique for objective and quantitative evaluation of global and regional myocardial function. Objectives: To determine impact of ventricular morphology on myocardial deformation in patients with single ventricle. Patients and Methods: The study included 47 patients with functional single ventricle whether they underwent cavopulmonary anastomosis or not. All patients were referred for elective cardiac catheterization or follow up echocardiography in Ain Shams university hospitals, Cardiology department, from November 2017 to July 2018. All patients underwent full echocardiographic assessment including assessment of the dominant ventricular function by STE. Results: The study included 47 patients, 30 males (63.8%) and 17 females (36.2%). Their age ranged between 6-12 years, with mean age of 8.22 ± 1.94. The morphology of the dominant ventricle determined ventricular function by both 2D parameters and STE. Patients with a dominant LV had higher strain values. Patients who underwent a cavopulmonary shunt also had better ventricular functions. All patients included in the study showed impaired dominant ventricular function by STE even those who had normal EF by standard 2D TTE suggesting that subtle ventricular dysfunction in patients with single ventricle can be unmasked by STE.

Unilateral unidirectional superior cavopulmonary anastomosis in a patient with bilateral superior caval veins and atretic left pulmonary artery.

A unidirectional superior cavopulmonary anastomosis was performed on the right side in a patient with a functionally univentricular heart, atresia of main and left pulmonary artery, bilateral superior caval veins, and a patent arterial duct in the right pulmonary artery. Anastomosis of the left superior caval vein to the right superior caval vein created a neo-innominate vein without using prosthetic material.

Dacron Conduit for Extracardiac Total Cavopulmonary Anastomosis: A Word of Caution

The extracardiac conduit technique is a valid option for completing total cavopulmonary anastomosis (TCPC) in patients with a single ventricle. The technique allows for beating heart surgery, optimal flow dynamics, and reduced postoperative atrial arrhythmia. Different types of conduit have been proposed. This study reported a single-centre experience with two different types of conduit. Consecutive patients referred for TCPC at the current institution between January 2001 and September 2013 were included. Retrospective extraction of pertinent variables was accomplished through electronic patient chart review. Patients were stratified based on the type of conduit used to perform the TCPC: polytetrafluoroethylene (PTFE) conduit (Group A) and polyethylene-terephthalate (Dacron) conduit (Group B). The patient population included 105 patients: Group A had 80 patients, and Group B had 25 patients. The two groups were similar in major clinical and procedural variables, including conduit size and Nakata index. Eighteen patients (Group A: one [1.25%]; Group B: 17 [68%]) had conduit occlusion or severe stenosis requiring intervention after a mean 46.9±35months after the operation. The percentage of patients in Group A who were free of conduit obstruction at 3, 5, and 10 years was 100%, 100%, and 96%, respectively, whereas these figures were 68%, 52%, and 35% in Group B (log-rank <0.000). Conduit re-intervention was associated with an increased risk of overall mortality after primary intervention (p<0.004). Dacron tube was found to be an independent risk factor for mid-term stenosis or obstruction (hazard ratio, 62.9; 95% CI, 8.2-482.2; p=0.000). Dacron conduit for TCPC surgery was associated with a higher risk of obstruction and need for early re-intervention compared with PTFE conduit. Surgical or percutaneous re-interventions for conduit obstruction increased the risk of late mortality.

RF24 HETEROTAXY SYNDROME WITH SIGNLE VENTRICLE PHYSIOLOGY: SINGLE CENTER EXPERIENCE

Objective: Treatment of infants with functional single-ventricle and heterotaxy syndrome is challenging due to multistage palliation with high operative mortality and morbidity. We report our experience. Methods: Between 1996 and 2017, 20 patients with heterotaxy syndrome underwent their first palliative surgery and form the study population. All relevant preoperative, operative and follow-up data were analyzed retrospectively. Results: Fourteen (70%) patients required neonatal palliation including a modified Blalock-Taussig shunt (n = 11; 55%), Norwood procedure (n = 1; 5%) or pulmonary artery banding (n = 2; 10%); two patients underwent TAPVC repair during first-stage palliative operation. In the remaining 6 patients (30%), primary bidirectional cavopulmonary anastomosis (BCPA) was the initial palliative surgery. 13 patients underwent BCPA operation as second-stage palliation, whereas one died in the interstage period. Nowadays, 12 of these patients have already undergone total cavopulmonary connection (TCPC) as a third-stage operation after BCPA. In case of Kawashima operation, median time interval before TCPC was 7 years (min 4 years; max 8 years). Mean follow-up time was 9,6 ± 7,9 years. One patient died during long-term follow-up after TCPC. Overall survival for the entire cohort following first surgery were 94,4% at 1 and 5 years and 82,6% at 10 years. Conclusions: Despite recent advances, the management of infants with heterotaxy syndrome with single-ventricle physiology is still a challenge for pediatric cardiac surgeons with high described morbidity and mortality. Conversely, in our experience overall mortality is inferior compared to other single-center reports and these patients may expect satisfying mid-to-long term survival. Further studies are needed to assess long-term morbidity.

Extracardiac Fontan With Direct Inferior Vena Cava to Main Pulmonary Artery Connection Without Cardiopulmonary Bypass

We report the case of a six-year-old patient who underwent an extracardiac Fontan operation including bilateral bidirectional superior cavopulmonary anastomosis and direct inferior vena cava to main pulmonary artery connection that was performed without cardiopulmonary bypass.

Pulmonary Arterial Stenting Complication: A Case Report about the Retrieval of a Stent Lost in a Child with Bilateral Glenn Shunt

Percutaneous stenting of the pulmonary artery is a diffuse procedure, above all in patients with congenital heart disease. The effectiveness of the procedure is associated with potential and feared complications. This case describes the embolization of a premounted stent into the left lower lobe pulmonary artery in a 3-years old patient with univentricular heart and left-side superior vena cava undergone to Norwood procedure modified according to Sano (stage I) and then bilateral cavo-pulmonary anastomosis according to Glenn (stage II). The retrieval procedures were complicated by the severe hypoplasia of the left pulmonary artery and by the complex anatomy of the patient. This case shows how to pull-back an embolized stent in a child with a complex congenital heart disease despite the limitations related to the low weight and the complex vascular anatomy.

MRI measured liver stiffness does not predict focal liver lesions after the Fontan operation.

Focal liver lesions are common in children and adults after Fontan procedures. To explore the relationship between liver shear stiffness, using magnetic resonance (MR) elastography, and the presence of focal liver lesions in patients after Fontan procedures (total cavopulmonary anastomosis). The retrospective study was approved by the institutional review board and the requirement for informed consent was waived. By searching institutional electronic medical records, we identified all patients with a history of Fontan palliation of congenital heart disease who had undergone same-day liver MR elastography and liver MRI without and with intravenous contrast material between January 2012 and December 2017. Using imaging reports, patients were placed into two groups: 1) no focal liver lesions and 2) one or more focal liver lesions. Patient age, sex, mean liver shear stiffness (kPa) and maximum single anatomical level liver shear stiffness (kPa) were recorded. The Mann-Whitney U test was used to compare age and liver stiffness between groups, while the Fisher exact test was used to assess the impact of gender on liver lesions. Forty-eight patients met study inclusion criteria; 33 (69%) had one or more focal liver lesions. The median age was 20.0years (IQR [interquartile range]: 10.8-29.1years) for patients without liver lesions and 19.9years (IQR: 17.2-27.0years) for patients with liver lesions (P=0.49). Eleven of 21 male patients (52.4%) had liver lesions compared to 22 of 27 female patients (81.5%) (P=0.058). Mean (4.62kPa [IQR: 4.10-5.59kPa] vs. 4.10kPa [IQR: 3.44-4.80kPa]; P=0.02) and maximum (5.53kPa [IQR: 4.64-6.56kPa] vs. 4.50kPa [IQR: 3.82-5.35kPa]; P=0.009) liver stiffness were significantly higher in patients without liver lesions as compared to patients with liver lesions. Our study demonstrated a significant negative association between focal liver lesions and increased liver stiffness in patients following Fontan procedures.

Pathophysiology, adaptation, and imaging of the right ventricle in Fontan circulation.

The Fontan procedure, which creates a total cavopulmonary anastomosis and represents the final stage of palliation for hypoplastic left heart syndrome, generates a unique circulation relying on a functionally single right ventricle (RV). The RV pumps blood in series around the systemic and pulmonary circulation, which requires adaptations to the abnormal volume and pressure loads. Here, we provide a complete review of RV adaptations as the RV assumes the role of the systemic ventricle, the progression of RV dysfunction to a distinct pattern of heart failure unique to this disease process, and the assessment and management strategies used to protect and rehabilitate the failing RV of Fontan circulation.

Alterations in cerebral ventricle size in children with congenital heart disease.

Children with tetralogy of Fallot (TOF) and superior cavopulmonary anastomoses (SCPA) can have chronically elevated central venous pressure (CVP), which has been postulated to put patients at risk for cerebral ventriculomegaly. We aimed to examine cerebral ventricle size in children with these congenital heart lesions before and after surgery to determine how changes in CVP affect ventricle size. We reviewed the records of patients who underwent SCPA or TOF repair between 2006 and 2015. Patients with pre- or post-operative cranial imaging were included. Frontal-occipital (FO) horn ratios were calculated as measures of cerebral ventricle volume. Reported normal mean FO ratio is 0.37 ± 0.03. Patient characteristics including occipito-fronto circumference (OFC) and available CVP measurements were recorded. CVP, FO ratios, and OFC percentiles were compared using paired and unpaired t tests and Wilcoxon matched pairs signed-rank test as appropriate. We reviewed 44 patients who underwent SCPA and 31 patients who underwent TOF repair who had cranial imaging studies available. In the 22 patients who underwent SCPA and had pre- and post-operative imaging, mean FO ratios significantly increased from 0.37 ± 0.03 to 0.40 ± 0.04 (P< 0.001). In contrast, in the seven patients with TOF with pre- and post-operative imaging, FO ratio was elevated at baseline and remains so after surgical repair, 0.43 ± 0.08 to 0.42 ± 0.08 (P= 0.65). Similar patterns were noted with OFC percentiles, which were significantly increased as compared to baseline after SCPA (P< 0.001) but were not significantly changed after TOF repair (P= 0.58). Finally, when available, preoperative and postoperative CVP measurements of all patients were examined, CVP increased in patients who underwent SCPA, from 6.5 ± 2mmHg preoperatively to 9.1 ± 2.3mmHg postoperatively (P< 0.001), while CVP remained statistically unchanged in patients who underwent TOF repair, 12.9 ± 3.3mmHg preoperatively to 14.4 ± 3.1mmHg postoperatively (P= 0.2). Cerebral ventriculomegaly was observed in patients with SCPA and TOF, and the observed changes in FO ratio and OFC may be related, at least in part, to CVP.

Cost Savings Analysis of Early Extubation Following Congenital Heart Surgery.

The clinical benefit of early extubation following congenital heart surgery has been demonstrated; however, its effect on resource utilization has not been rigorously evaluated. We sought to determine the cost savings of implementing an early extubation pathway for children undergoing surgery for congenital heart disease. We performed a cost savings analysis after implementation of an early extubation strategy among children undergoing congenital heart surgery at British Columbia Children's Hospital (BCCH) over a 2.5-year period. All patients undergoing one of the eight Society of Thoracic Surgeons (STS) benchmark operations, ASD repair, or bidirectional cavopulmonary anastomosis were included in the analysis (n = 370). We compared our data to aggregate STS multi-institutional data from a contemporary cohort. We estimated daily costs for ICU care, ward care, medications, imaging, additional procedures, and allied health care using an administrative database. Direct costs, indirect costs, and cost savings were estimated. Simulation methods, Monte Carlo, and bootstrapping were used to calculate the 95% credible intervals for all estimates. The mean cost savings per procedure was $12,976 and the total estimated cost savings over the study period at BCCH was $4.8million with direct costs accounting for 91% of cost savings. Sensitivity analysis demonstrated a mean cost savings range of $11,934-$14,059 per procedure. Early extubation is associated with substantial cost savings due to reduced hospital resource utilization. Implementation of an early extubation strategy following congenital heart surgery may contribute to improved resource utilization.

Vasoactive Inotropic Score and Outcome Assessment in Cyanotic Infants after Cardiovascular Surgery

Abstract Objective Prospective assessment of vasoactive inotropic score (VIS) for predicting postoperative outcomes in cyanotic infants following open-heart surgery. Methods In this prospective observational study, postoperative outcomes were analyzed in 100 cyanotic infants operated between September 2014 and April 2016. Inotropic score and VIS were calculated at 1 hour, 24 hours, and 48 hours following surgery. Results Mean age at surgery was 7.39 ± 4.4 months (3 days–12 months, median 9 months). Surgical procedures included repair of total anomalous pulmonary venous connection (n = 40), tetralogy of Fallot (n = 24), double outlet right ventricle with pulmonary stenosis (n = 4), bidirectional superior cavopulmonary anastomosis (n = 19), and miscellaneous (n = 13). On regression analysis, prolonged intensive care unit and hospital stay correlated best with VIS at 48-hour interval (R 2 = 0.67 and 0.50 respectively, p &gt; 0.001). Low birth weight (&lt; 2.5 kg), preoperative ventilation and high first-hour VIS score (&gt; 25) were predictive of higher postoperative mortality. Maximum VIS at 24 and 48 hours were positively correlated with adverse postoperative outcomes (p &lt; 0.001). Conclusion In cyanotic infants undergoing open-heart surgery, higher 48-hour VIS was associated with poor postoperative outcome indicating its utility as a valuable tool in predicting postoperative outcomes.

Surgical Management and Outcomes of Ebstein Anomaly in Neonates and Infants: A Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis

Ebstein anomaly (EA) encompasses a broad spectrum of morphology and clinical presentation. Those who are symptomatic early in infancy are generally at highest risk, but there are limited data regarding multicentric practice patterns and outcomes. We analyzed multiinstitutional data concerning operations and outcomes in neonates and infants with EA. Index operations reported in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2016) were potentially eligible for inclusion. Analysis was limited to patients with diagnosis of EA and less than 1 year of age at time of surgery (neonates ≤30 days, infants 31 to 365 days). The study population included 255 neonates and 239 infants (at 95 centers). Among neonates, median age at operation was 7 days (interquartile range, 4 to 13 days) and the majority required preoperative ventilation (61.6%, n= 157). The most common primary operation performed among neonates was Ebstein repair (39.6%, n=101), followed by systemic-to-pulmonary shunt (20.4%, n= 52) and tricuspid valve closure (9.4%, n= 24). Overall neonatal operative mortality was 27.4% (n= 70), with composite morbidity-mortality of 51.4% (n= 48). For infants, median age at operation was 179 days (interquartile range, 108-234 days); the most common primary operation for infants was superior cavopulmonary anastomosis (38.1%, n= 91) followed by Ebstein repair (15.5%, n= 37). Overall operative mortality for infants was 9.2% (n= 22) with composite morbidity-mortality of 20.1% (48). Symptomatic EA in early infancy is very high risk and a variety of operative procedures were performed. A dedicated prospective study is required to more fully understand optimal selection of treatment pathways to guide a systematic approach to operative management.

Velocity vector imaging for the assessment of segmental ventricular function in children with a single right ventricle after cavopulmonary anastomosis

Objective: Ventricular function assessment is very important for the treatment and prognostic classification of children with a single right ventricle (SRV) after cavopulmonary anastomosis (CPA). However, unusual ventricular shapes can result in inaccurate measurements. The aim of this study was to evaluate velocity vector imaging (VVI) for assessing segmental ventricular function in children with SRV after CPA.Methods: Twenty-one children with SRV after CPA and 21 age- and sex-matched children with normal biventricular anatomy and function were included. The longitudinal velocity, displacement, strain and strain rate were measured in the two groups in six segments by VVI. The velocity, displacement, strain and strain rate of the SRVs were compared with max(dp/dt) measured during simultaneous cardiac catheterization in the SRV subjects.Results: The control group consisted of 13 males and 8 females (69% males) with a mean age of 6.7 ± 3.5 years and mean weight of 20.5 ± 6.5 kg, and the study group consisted of 13 males and 8 females with a mean age 6.7 ± 3.7 years and mean weight of 20.6 ± 6.8 kg. Age, weight and sex distribution were similar between the groups (all, p > .05). Strain and strain rate values in all six segments were significantly lower in the study group than in the control group (all, p < .05). The max(dp/dt) of the SRV was 522.84 ± 158.32 mmHg/s, and the strain rate of the basal segment at the rudimentary chamber correlated best with max(dp/dt) (r = 0.74, p < .01).Conclusions: Segmental ventricular dysfunction was present in children with SRV after CPA, and it could be assessed using VVI.