Functional Single Ventricle Research Articles

Results of surgery on adults with functional single ventricle without prior cardiac surgery in childhood.

Patients with unoperated functional single ventricle (FSV) rarely survive into adulthood with good functional status and there are few reports about surgical results of adult patients with FSV. This study retrospectively reviews our experience with surgery in adult patients with FSV. From January 2008 to September 2017, 65 adult patients with FSV underwent surgery in our hospital. Twenty underwent Blalock-Taussig shunt or bidirectional Glenn procedures in other hospitals prior, and four were lost to follow-up. Finally, 41 patients were included in this study. The early postoperative mortality was 7.3% (3/41). Postoperative systemic arterial oxygen saturation (SpO2 , 83.7% ± 4.8%) was significantly higher than preoperative SpO2 (77.9% ± 10.1%, P < .01). The mean follow-up time was 3.9 ± 3.1 years (range 1-11 years). There was only one case of late mortality and massive hemoptysis was the cause of death. Right ventricular morphology and severe atrioventricular valve regurgitation (AVVR) were the risk factors of postoperative death for patients with pulmonary stenosis(PS). There was no death case in patients who received Fontan procedure. In a follow-up, we found the grade of AVVR was reduced and the grade of heart function (New York Heart Association) was improved. Adult patients with FSV can also undergo surgery. The mortality was acceptable and late results were satisfactory. After surgery, oxygen saturations increased, grades of AVVR decreased and the heart functions improved. Right ventricular morphology and severe AVVR were risk factors for patients with PS.

Dual-Propeller Cavopulmonary Pump for Assisting Patients with Hypoplastic Right Ventricle

Various congenital heart defects (CHDs) are characterized by the existence of a single functional ventricle, which perfuses both the systemic and pulmonary circulation. A three-stage palliation procedure, including the final Fontan completion, is often adopted by surgeons to treat patients with such CHDs. The completion Fontan involves the creation of a total cavopulmonary connection (TCPC), commonly accomplished with an extracardiac conduit. This TCPC results in nonphysiologic flow conditions that can lead to systemic venous hypertension, reduced cardiac output, and ultimately the need for heart transplantation. A modest pressure rise of 5-6 mm Hg could correct the abnormal flow dynamics in these patients. To achieve this, we propose a novel conceptual design of a dual-propeller pump inside a flared TCPC. The TCPC dual-propeller conjunction was examined for hydraulic performance, blood flow pattern, and potential for hemolysis inside the TCPC using computational fluid dynamics (CFD). The effect of axial distance between the two propellers on the blood flow interference and energy loss was studied to determine the optimal separation distance. Both the inferior vena cava (IVC) and superior vena cava (SVC) propellers provided a pressure rise of 1-20 mm Hg at flow rates ranging from 0.4 to 7 lpm while rotating at speeds of 6,000-12,000 rpm. Larger separation distance provided favorable performance in terms of flow interference, energy loss, and blood damage potential. The ability of a dual-propeller micropump to provide the required pressure rise would help to augment the cavopulmonary flow and mimic flows seen in normal biventricular circulation.

Improved outcomes in neonates who require venoarterial extracorporeal membrane oxygenation after the Norwood procedure.

Extracorporeal membrane oxygenation after the Norwood procedure has historically been associated with poor outcomes, with reported hospital survival rates of 13%-48%. We hypothesized that contemporary outcomes in this population have improved. We aimed to compare clinical outcomes of contemporary cohorts of patients with functional single ventricle physiology who did and did not receive extracorporeal membrane oxygenation after the Norwood procedure. Single-center retrospective cohort study of patients with single ventricle anatomy who underwent the Norwood procedure between 2009 and 2017 was performed. Kaplan-Meier survival curves were constructed, and Cox proportional hazard regression analyses were performed to compare transplant-free survival in patients who did and did not receive venoarterial extracorporeal membrane oxygenation. In total, 85 patients met inclusion criteria. Venoarterial extracorporeal membrane oxygenation was utilized in 25 patients (29%). A total of 18 patients (72%) who received venoarterial extracorporeal membrane oxygenation survived to hospital discharge, compared to 54 patients (92%) who did not receive venoarterial extracorporeal membrane oxygenation (p = 0.013). Post-discharge transplant-free survival was not significantly different between patients who did and did not receive venoarterial extracorporeal membrane oxygenation (log-rank p value = 0.28). Cox proportional hazard regression analysis revealed that the occurrence of cardiac arrest requiring cardiopulmonary resuscitation (hazard ratio = 4.5; 95% confidence interval = 2.0-10.1) during the perioperative period was independently associated with death or transplantation, whereas venoarterial extracorporeal membrane oxygenation was not an independent risk factor for death or transplantation (hazard ratio = 2.0; 95% confidence interval = 0.8-4.9). In our cohort of children who received venoarterial extracorporeal membrane oxygenation after the Norwood procedure, hospital survival was improved compared to historical data. In addition, venoarterial extracorporeal membrane oxygenation utilization was not independently associated with worse outcomes.

CRT Implantation in a Patient with Congenital Heart Malformation

Abstract Treatment of patients with functional single-ventricle physiology is achieved using surgical corrections known as the “Fontan Procedure”. Two separate blood circulations are created from the single-ventricle function. These are non-cyanogenic patients, but at high risk of developing HF. CRT implantation is an option to improve the functional class and EF.

Retraction Note to: Primary Draining Vein Stenting for Obstructive Total Anomalous Pulmonary Venous Connection in Neonates with Right Atrial Isomerism and Functional Single Ventricle Improves Outcome.

The authors have retracted this article [1] because, contrary to the statement in the article, this research did not obtain ethics approval from the National Cerebral and Cardiovascular Center Institutional Review Board prior to submission of the manuscript to Pediatric Cardiology. All authors agree to this retraction.

Midterm results and risk factors of functional single ventricles with extracardiac total anomalous pulmonary venous connection.

To evaluate the clinical outcomes of surgical repair of an extracardiac total anomalous pulmonary venous connection (TAPVC) in a functional single-ventricle (f-SV) strategy. This was a retrospective analysis of 48 consecutive cases of extracardiac TAPVC repair, from 1998 to 2015. Demographic and clinical variables were as follows: median age, 24 (range 0-744) days; median weight, 3.1 (range 2.0-9.6) kg; type of TAPVC-supracardiac, 21 patients, infracardiac, 8, and mixed, 19; right atrial isomerism, 45 patients; pulmonary atresia, 24 patients; and obstructed TAPVC, 30 patients. Concomitant procedures included systemic-to-pulmonary shunting in 15 patients, pulmonary artery banding in 12, ventricle-to-pulmonary artery shunting in 2, a Norwood procedure in one, a bidirectional Glenn procedure in 16, and a Fontan procedure in 1. The 1- and 5-year cumulative survival rates were 66.0% and 58.0%, respectively. Of the 28 survivors, 22 (78.6%) underwent Fontan completion and 4 (14.3%) a bidirectional Glenn procedure, and 1 (3.6%) was awaiting a bidirectional Glenn procedure. Recurrent pulmonary venous stenosis (PVS) was observed in 17 patients, with a 1- and 5-year rates of freedom from recurrent PVS 59.8% and 53.5%, respectively. Of the 20 post-operative deaths, only 4 were PVS related. Only pre-operative pulmonary atresia was identified as an independent risk factor of mortality after TAPVC repair. The midterm surgical outcomes of f-SV with extracardiac TAPVC were acceptable. Moreover, among survivors, Fontan completion can be sufficiently expected. Further improvement, with the development of a comprehensive treatment strategy, is required for this patient group.

Risk factors of thrombotic complications in patients with single functional ventricle

Objective: to analyze the parameters of the hemostasis system and the results of molecular genetic testing in patients with a single functional ventricle. The study included 102 patients. All the patients underwent a staged surgical hemodynamic correction of a single functional ventricle. The authors performed a retrospective analysis of patient records in order to identify the episodes of thrombosis. The incidence of thrombotic complications at different stages of hemodynamic correction in the examined patients with a single functional ventricle was 12.7%. The indicators of plasma link hemostasis in the observed patients have been characterized by a balance of hemostatic reactions in the group of children with thrombosis and without. The results of a molecular genetic study demonstrated that the carrier of the heterozygous genotype of 20210GA factor II gene in patients with a single functional ventricle increased the risk of thrombotic complications 16 times (15.4% in patients with thrombosis versus 1.1% in the group without thrombosis; odds ratio 16.0; 95% confidence interval 1.34–191.24; p=0.028). All patients with thrombosis in the history revealed a homozygous condition according to variant 10976GG factor VII gene (p=0.017). Conclusion: molecular genetic analysis of polymorphic variants of the hemostatic system in patients with a single functional ventricle is required to predict the risk, timely prevention and correction of thrombotic complications during the surgical treatment of congenital heart disease.

Challenges of Caring for Functionally Single Ventricle Patients in Africa.

Surgical palliation has remarkably improved survival of functionally single ventricle (FSV) patients born in developed nations but such outcomes have not occurred in Africa. The poor care coverage for FSV patients in Africa exists within the larger sphere of deficient health care for children born with congenital heart defects (CHDs) in Africa generally. This review takes the position that to improve health-care coverage for CHD patients on the continent, political priority is paramount. This can be attained with cohesive leadership for the CHD agenda, a guiding institution, and the mobilization of civil society to drive advocacy at national and international levels.

The Superior Cavopulmonary Connection: History and Current Perspectives.

The development of the superior cavopulmonary connection is a rich illustration of international influences in congenital cardiac surgery. The bidirectional Glenn and hemi-Fontan procedures have improved survival as both definitive and staged functional single ventricle palliation. The optimal timing of the second-stage superior cavopulmonary procedures varies by center but for low- and intermediate-risk patients, this may be within three to six months after the Norwood procedure. The list of risk factors continues to grow but the most frequently cited factors include atrioventricular valve regurgitation, decreased ventricular function, need for reintervention, and failure to attain nutritional and growth goals. Ongoing prospective, multi-institutional studies, particularly those fostered internationally by the World Society for Pediatric and Congenital Heart Surgery and other associations, will hopefully provide further clarification of the complex management issues related to patients with functional single ventricle physiology.

Effect of multistage surgery in patients with functional single ventricle and risk factors of postoperative death

Objective: To investigate the efficacy of multistage surgery in patients with functional single ventricle (FSV) and risk factors of postoperative death. Methods: The clinical data of all consecutive patients with FSV undergoing multistage single ventricle palliation surgery in Guangzhou women and children's medical center from January 2008 to December 2017 were retrospectively reviewed. The study included 289 patients. The age was 10.0 (6.0, 35.4) months,and there were 198 male and 91 female patients. The patients were followed up at outpatient clinic. Survival rates were calculated with Kaplan-Meier. Multivariate Cox regression analysis was made to determine the risk factors of postoperative death. Results: Seventy-nine patients required the first stage palliation surgery, 232 patients received the Glenn shunt surgery, and 162 patients completed the Fontan procedure. Overall, postoperative death occurred in 39 patients including 21 after the first stage palliation surgery (early stage 13 cases, late stage 8 cases) , 10 after the Glenn shunt surgery (early stage 5 cases, late stage 5 cases) , and 8 following the Fontan procedure (early stage 6 cases, late stage 2 cases) . Kaplan-Meier analysis showed that survival rate of the entire cohort was 90.2% (95%CI 86.7%-93.7%) , 85.9% (95%CI 81.8%-90.0%) ,and 84.6% (95%CI 79.7%-89.5%) at 1 year, 5 years and 10 years post operation. Survival rate was 74.4% (95%CI 64.8%-84.0%) , 73.0% (95%CI 63.2%-82.8%) , and 73.0% (95%CI 63.2%-82.8%) at 1 year, 5 years, and 10 years post the first stage palliation surgery, 97.8% (95%CI 95.8%-99.7%) , 95.2% (95%CI 92.3%-98.1%) , and 95.2% (95%CI 92.3%-98.1%) at 1 year, 5 years, and 10 years post Glenn shunt surgery, 95.6% (95%CI 92.5%-98.7%) and 93.7% (95%CI 88.8%-98.6%) at 1 year and 5 years post Fontan surgery. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (HR=5.47, 95%CI 2.71-11.04, P<0.001), atrioventricular valve regurgitation more than moderate (HR=2.52, 95%CI 1.32-4.79, P=0.005), systemic ventricular outflow tract obstruction (HR=3.47, 95%CI 1.30-9.29, P=0.013), and required the first palliation surgery (HR=3.12, 95%CI 1.59-6.15, P=0.001) were risk factors of postoperative death. Conclusions: The multistage surgery can effectively improve the survival of patientswith functional single ventricle and is associated with satisfactory long-term prognosis. Total anomalous pulmonary venous connection, atrioventricular valve regurgitation more than moderate, systemic ventricular outflow tract obstruction, and required the first palliation surgery are risk factors of postoperative death in these patients.

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.

The Effect of Multidisciplinary Approach on the Birth Rate of Fetuses with Prenatally Diagnosed Congenital Heart Disease.

BackgroundThis study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs).MethodsAmong the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results.ResultsAmong the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035).ConclusionWhen a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.

Predictors of Bidirectional Glenn Shunt Failure and Fontan Completion Rate in Patients with Single Ventricle

Background: The introduction of bidirectional Glenn (BDG) procedure in patients with functional single ventricles improved the clinical outcomes for all candidates undergoing total cavopulmonary connection (TCPC). We tried to evaluate the obstacles that prevent patients to achieve TCPC after BDG. Methods: All patients who underwent BDG at Leipzig Heart Centre from April 2003 to November 2013 were included in this study, except cases preceded by Norwood procedure. Results: 82 patients were included in this study. 59 patients underwent TCPC (72%), while 12 patients were waiting for TCPC (14.6%). There were two in-hospital deaths (2.4%) after BDG. In two patients (2.4%), completion of TCPC was not possible, while 6 patients were lost during follow-up (7.3%). One patient had his BDG taken down (1.3%). Therefore the survival rate till completion of Fontan was 72% with another 14.6% waiting for TCPC. A univariate analysis revealed that patients with unbalanced atrioventricular septal defect (AVSD), previous repair of total anomalous pulmonary venous connection (TAPVD), elevated mean pulmonary arterial pressure and long operation time were significant predictors of death, take-down, or failure to progress to Fontan Operation. Thrombosis was found to be the main mechanism for morbidity and mortality. Conclusion: The staged plan for single ventricle candidates provides excellent clinical results. The main risk factors for death, take-down, or failure to progress to Fontan Operation were elevated pulmonary arterial pressure, unbalanced AVSD, TAPVD and long operation time.

918: Variable phenotypic presentation of PLD1 associated congenial heart disease in a set of identical triplets

To investigate the underlying etiology of three variable phenotypic congenital heart defects (CHD) in a set of identical triplets. The initial diagnosis of fetal CHD was made during an anatomy scan performed at 20 weeks and 2 days of gestation. Further analysis of the triplet hearts were performed via interval echocardiograms throughout the pregnancy. Post-natal evaluations included neonatal echocardiograms and familial microarray comparative genomic hybridization (aCGH) assays and next generation sequencing (NGS) gene panels. Final diagnoses of cardiac status of infant A: patent foramen ovale; infant B: bicuspid pulmonary valve, pulmonary stenosis, ventricular septal defect, atrial septal defect, and bilateral superior vena cava without evidence of bridge; infant C: functional single ventricle (tricuspid atresia, absent pulmonary valve, right ventricle hypoplasia due to right ventricular septal mass, left ventricle non compaction cardiomyopathy), dual atrioventricular node physiology, and multi-factorial pulmonary hypertension. Postnatal aCGH and NGS were performed on all family members. This allowed for the detection of compound heterozygous changes in the PLD1 gene in all three of the infants. The mother was heterozygous for the missense mutation, c.3142C>G on chromosome 3 in the region of the PLD1 gene. The father was heterozygous for a partial duplication of exon 12-19 in the PLD1 gene. All three infants were found to have compound heterozygous mutations of PLD1, including a partial duplication and a missense mutation. While PLD1 pathogenic variants have been associated with cardiac changes mainly in the valves, this report shows that the same genetic changes in identical siblings can have a dramatic variation in phenotype. It also reveals a new association between left ventricular non-compaction and the PLD1 gene.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Surgical management of atrioventricular valve regurgitation in functional single ventricle

Objective: To evaluate the early- and mid-term outcomes of surgical atrioventricular valve (AVV) intervention in patients with functional single ventricle (FSV). Methods: The clinical data of 40 consecutive FSV patients who underwent surgical AVV intervention between January 2008 and December 2017 at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University were reviewed retrospectively. There were 25 male and 15 female patients. The median age at AVV intervention was 4.5 to 204.0 months (M(Q(R)): 42.5 (59.7) months), and the median body weight was 6.0 to 55.4 kg (M(Q(R)): 13.8 (8.1) kg). The systemic AVV morphology included common AVV in 21 patients, mitral valve in 7 patients, tricuspid valve in 3 patients, and both mitral and tricuspid valve in 9 patients. At first surgical intervention, 15 patients had severe, 25 patients had moderate AVV regurgitation. The timings of the initial surgical intervention were at the first palliative, Glenn, between Glenn and Fontan, and Fontan stage in 5, 23, 4, and 8 patients, respectively. The methods of the initial surgical intervention were AVV repair in 31 patients, AVV replacement in 9 patients. Actuarial survivals were estimated by the Kaplan-Meier method. Prognosis factors for atrioventricular valve reoperation were analyzed using the Cox regression hazard model. Results: There were 6 early deaths, with a mortality of 15.0%. Thirty-four survival patients received a follow-up of 1 to 117 months (M(Q(R)): 44 (34) months). Survival of patients after the initial surgical intervention were 85.0% (95% CI: 74.0% to 95.9%), 79.3% (95% CI: 66.6% to 92.0%), and 79.3% (95% CI: 66.6% to 92.0%) at 1 year, 5 years and 10 years, respectively. In all, only 13 patients completed the Fontan procedure. Six patients underwent 8 reoperations, including AVV replacement in 5 patients, redo AVV repair in 3 patients. Freedom from AVV reoperation at 1 and 5 years was 89.8% (95% CI: 78.8% to 100%) and 79.4% (95% CI: 64.7% to 94.1%), respectively. Multivariate Cox regression analysis revealed that common AVV (HR=3.53, 95% CI: 1.63-7.67, P=0.020) was the prognosis factors for AVV reoperation. Conclusions: The early- and mid-term outcomes of surgical AVV intervention in FSV patients are not satisfactory. The mortality and reoperation rate are still high, and common AVV predicts the AVV reoperation after the initial surgical intervention.

Surgical palliation of univentricular heart disease in children with Down's syndrome: A systematic review.

ObjectivesNo standard protocol is available for the management of children with Down's syndrome (DS) and a functional single ventricle. This review attempts to determine the outcomes of the single ventricular surgical palliation pathway in high-risk children with DS.MethodsSeveral databases were searched using the following MeSH terms: ‘Congenital heart disease’, ‘Atrioventricular septal defect’, ‘Balanced AVSD’, ‘Unbalanced AVSD’, ‘Down's syndrome’, ‘Univentricular repair’, ‘bidirectional Glenn procedure’, and ‘Fontan procedure’. A structured algorithm was used for the selection of studies for an in-depth analysis.ResultsThere was no universal agreement on the best surgical approach for unbalanced atrioventricular septal defect in DS. The majority of paediatric cardiac surgeons did not recommend the complete Fontan procedure; conversely, the use of a Glenn shunt (superior cavopulmonary connection) was preferred.ConclusionsCareful assessment of the suitability for Fontan surgery, including the absence of elevated pulmonary vascular resistance, pulmonary arterial anatomy, and function of the dominant ventricle, is mandatory. A staged surgical procedure ending with complete Fontan repair provides acceptable medium-term results.

Abstract 17326: The Effect of Maintenance of Antegrade Pulmonary Blood Flow Upon Bidirectional Glenn on Pre- and Post-Fontan Outcomes

Introduction: Maintaining pulsatility at the time of bidirectional Glenn (BDG) has theoretical advantages and disadvantages. The practice is diverse throughout the centers and individual surgeons without clear rationale behind its application. We sought to investigate the impact of antegrade pulmonary blood flow on pre- and post-Fontan outcomes. Methods: From 2003 through June 2014, among 237 patients who underwent BDG as an interim palliation for functional single ventricle (FSV), patients with preexisting pulmonary blood flow from the ventricle before BDG were included and patients undergoing Kawashima operation or having history of Norwood operation or bilateral pulmonary arterial band were excluded (n=151). Patients were grouped by their presence or absence of antegrade pulmonary blood flow following BDG: group 1 (pulsatile) (n=73) or group 2 (non-pulsatile) (n=78). The pre- and post-Fontan outcomes were compared between two groups. Results: Age and weight at BDG were 7 months and 7.8kg. Oxygen saturation was higher in group 1 (84±4%) compared to group 2 (82±4%) before Fontan (p&lt;0.001). The level of brain natriuretic peptide (BNP) was similar before Fontan (p=0.966). In pre-Fontan cardiac catheterization, mean pulmonary arterial pressure was similar (Group 1: 11±4mmHg vs. Group 2: 10±4mmHg, p=0.146), McGoon ratio seemed higher in group 1(2.6±0.6) compared to group 2(2.3±0.4) (p=0.057), and pulmonary vascular resistance was lower in group 1(1.3±0.7) compared to group 2(1.6±0.7)(p=0.038). A total of 136 patients (90%) underwent Fontan operation. Duration of stay in the intensive care unit (p=0.766), stay in hospital (p=0.142), and indwelling chest tube (p=0.128) was not different between groups. Overall survival after BDG was better in group 2 (p=0.046) [Figure]. In multivariable analysis, maintenance of pulsatility was identified as a risk factor for survival after BDG (p=0.012, HR 4.1 [CI 1.4-12.4]). Conclusions: Maintaining pulsatility at the time of BDG in FSV might be beneficial for pulmonary arterial growth and oxygen saturation without increasing BNP. However, such beneficial effect did not have a positive effect on subsequent Fontan outcome. Attention must be paid to the negative effect of pulsatility on overall post-BDG survival.

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.

Psychosocial functioning of parents of children with heart disease-describing the landscape.

The aim was to describe the psychological functioning of parents of school-age children with heart disease (HD) in a large-scale, transnational evaluation of parent dyads across the spectrum of cardiac diagnoses and a range of psychosocial domains. Parents of children with HD attending routine out-patient cardiology follow-up visits completed questionnaires assessing their mental health, coping, and family functioning. Parents (1197 mothers and 1053 fathers) of 1214 children (mean age: 12.6years; S.D. 3.0years; median time since last surgery: 8.9years) with congenital or acquired HD from three centers each in the UK and the USA participated (80% response rate). Parents of children with milder HD demonstrated few differences from healthy norms and had significantly lower scores on measures of illness-related stress and post-traumatic stress than parents of children with single ventricle conditions or cardiomyopathy. Parents in these latter two diagnostic sub-groups had significantly higher levels of anxiety and depression than healthy norms but did not differ on other measures of family functioning and coping skills. There were few differences between parents from the UK and the USA. Agreement between mothers and fathers within a dyad was highest for the measure of frequency of illness-related stressors (ICC = 0.67) and lowest for anxiety (ICC = 0.12).Conclusion: Our results suggest two different pathways for the long-term psychological well-being of parents of children with HD: on the one hand, more complex HD is associated with poorer long-term psychosocial outcomes; in contrast, there are also grounds for optimism, particularly for parents of children with less complex conditions, with better psychological outcomes noted for some groups of parents compared to previously reported early psychosocial outcomes. Future work needs to identify factors other than disease severity which might explain poorer (or better) functioning in some parents of children with more complex HD. What is Known: • Parents of children with congenital heart disease report elevated levels of anxiety, depression, and stress after cardiac surgery in infancy. • Maternal mental health problems can have an adverse impact on the psychological adjustment of the child with congenital heart disease. What is New: • Parents of children with milder forms of heart disease do not differ from healthy norms in the longer term and psychological outcomes are better than might be expected from early findings. • More complex diagnoses, particularly functional single ventricle conditions and cardiomyopathy, are associated with poorer long-term psychosocial outcomes for parents.

The experience of surgical treatment for Ebstein anomaly in 141 children

Objective: To explore the surgical strategy for Ebstein anomaly in children. Methods: From January 2003 to December 2015, a total of 141 cases of Ebstein anomaly were treated at Department of Pediatric Cardiothoracic Surgery, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiaotong University. There were 65 male and 76 female patients, with age of (6.9±1.6) years (ranging from 10 months to 15 years), weight of (19.6±4.7) kg (ranging from 6.5 to 59.0 kg). All patients were diagnosed by 2 dimensional Doppler echocardiography and the septal leaflet and posterior leaflet displaced downward from 1.0 to 5.0 cm. The tricuspid valve regurgitation (TR) were mild in 26 cases, moderate in 46 cases and severe in 69 cases. Tricuspid valvuloplasty were performed in 131 cases (94 cone reconstruction, 37 valve hoist), tricuspid valve replacement in 2 cases and tricuspid valve closed in 8 cases. Surgical strategy were divided into biventricular heart function in 77 cases, one and a half ventricular heart function in 56 cases, and single ventricular heart function in 8 cases. Results: Three patients were changed to one and a half ventricular repair from biventricular repair due to unstable hemodynamics in the early postoperative period. One case died in biventricular group. The complete atrioventricular block were occurred in 3 patients and pacemaker were applied. One hundred and forty cases discharged from hospital. There were mild TR in 118 cases, moderate in 14 cases and closed in 8 cases. One hundred and thirty-seven cases were followed up regularly in 18 to 172 months. Ninety-one cases were treated by cone reconstruction (mild TR in 75 cases, moderate in 15 cases and severe in 1 case). Thirty-six cases were operated by tricuspid valve hoist (mild TR in 21 cases, moderate in 12 cases and severe in 3 cases). In the patients with severe TR (4 cases), 3 cases were reoperated by cone reconstruction. One case's valve was closed because of the dysplasia of the anterior valve and then from one and a half ventricular heart function to single ventricular function, the oxygen saturation was increased. Two patients underwent tricuspid valve replacement, 1 died and the other's mechanical valve was removed, and changed to single ventricular function repair. Conclusions: Although tricuspid cone reconstruction can achieve good results, the stable hemodynamic of early postoperative can be effectively maintained by using the surgical strategy of one and a half ventricular repair. To the patients with severe tricuspid regurgitation and hypoxemia due to severe tricuspid valve dysplasia, transforming to a functional single ventricle may be the only choice when there comes to the unstable hemodynamic.