Textbook Outcome for Superior Cavopulmonary Connection: A Metric for Single Ventricle Heart Surgery.

Background: To develop a more holistic measure of congenital heart center performance beyond mortality, we created a composite "textbook outcome" (TO) for the Glenn operation. We hypothesized that meeting TO would have a positive prognostic and financial impact. Methods: This was a single center retrospective study of patients undergoing superior cavopulmonary connection (bidirectional Glenn or Kawashima ± concomitant procedures) from 2005 to 2021. Textbook outcome was defined as freedom from operative mortality, reintervention, 30-day readmission, extracorporeal membrane oxygenation, major thrombotic complication, length of stay (LOS) >75th percentile (17d), and mechanical ventilation duration >75th percentile (2d). Multivariable logistic regression and Cox proportional hazards modeling were used. Results: Fifty-one percent (137/269) of patients met TO. Common reasons for TO failure were prolonged LOS (78/132, 59%) and ventilator duration (67/132, 51%). In multivariable analysis, higher weight [odds ratio, OR: 1.44 (95% confidence interval, CI: 1.15-1.84), P = .002] was a positive predictor of TO achievement while right ventricular dominance [OR 0.47 (0.27-0.81), P = .007] and higher preoperative pulmonary vascular resistance [OR 0.58 (0.40-0.82), P = .003] were negative predictors. After controlling for preoperative factors and excluding operative mortalities, TO achievement was independently associated with a decreased risk of death over long-term follow-up [hazard ratio: 0.50 (0.25-0.99), P = .049]. Textbook outcome achievement was also associated with lower direct cost of care [$137,626 (59,333-167,523) vs $262,299 (114,200-358,844), P < .0001]. Conclusion: Achievement of the Glenn TO is associated with long-term survival and lower costs and can be predicted by certain risk factors. As outcomes continue to improve within congenital heart surgery, operative mortality will become a less informative metric. Textbook outcome analysis may represent a more balanced measure of a successful outcome.

Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study.

OBJECTIVE: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. METHODS: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. RESULTS: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. CONCLUSIONS: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.

The impact of additional antegrade pulmonary blood flow at bidirectional Glenn shunt on long-term outcomes.

OBJECTIVES: We investigated the impact of additional antegrade pulmonary blood flow on the long-term outcomes after bidirectional Glenn shunt. METHODS: From 2001 to 2015, 279 patients underwent bidirectional Glenn shunt as an interim palliation for a functionally single ventricle. After excluding patients with a previous Kawashima or Norwood operation, 202 patients with preexisting antegrade pulmonary blood flow before bidirectional Glenn shunt were included in this study. Antegrade pulmonary blood flow was eliminated in 110 patients (no antegrade pulmonary blood flow group) and maintained in 92 patients (antegrade pulmonary blood flow group). The impact of antegrade pulmonary blood flow at bidirectional Glenn shunt on long-term outcome was analyzed using inverse probability of treatment weighting. RESULTS: Median age and body weight at bidirectional Glenn shunt were 8 months and 7.8 kg, respectively. Prolonged chest tube drainage or readmission for effusion after bidirectional Glenn shunt was more frequent in the antegrade pulmonary blood flow group (odds ratio, 3.067; 95% confidence interval, 1.036-9.073; P = .043). In the no antegrade pulmonary blood flow group, B-type natriuretic peptide level was decreased further until the Fontan operation (P = .012). In the no antegrade pulmonary blood flow group, oxygen saturation was lower just after bidirectional Glenn shunt, although it was increased further until Fontan operation (P < .001), despite still lower oxygen saturation before Fontan operation compared with antegrade pulmonary blood flow group (P < .001). The McGoon ratio was decreased in both groups without intergroup difference, although the McGoon ratio before Fontan operation was higher in the antegrade pulmonary blood flow group (2.3 ± 0.4 vs 2.1 ± 0.4, P = .008). Overall transplant-free survival was worse in the antegrade pulmonary blood flow group (hazard ratio, 2.37; confidence interval, 1.089-5.152; P = .030). CONCLUSIONS: Maintaining antegrade pulmonary blood flow at bidirectional Glenn shunt was beneficial for higher oxygen saturation and larger pulmonary artery size before Fontan operation. However, it was unfavorable for overall transplant-free survival with a sustained higher risk of death or transplant until the elimination of antegrade pulmonary blood flow.

Atrioventricular Valve Regurgitation in Single Ventricle Heart Disease: A Common Problem Associated With Progressive Deterioration and Mortality.

The Fontan procedure has provided patients with single ventricle physiology extended survival into adulthood and in many cases has improved their quality of life. Atrioventricular valve regurgitation (AVVR) is common in single ventricle patients and is associated with increased risk of mortality. AVVR is more common in patients with a systemic tricuspid or common atrioventricular valve but is generally progressive irrespective of underlying valve morphology. AVVR can be attributable to diverse structural and functional abnormalities at multiple levels of the valvar apparatus, as well as ventricular dysfunction and dilation. Multiple imaging modalities including recent advances in 3-dimensional echocardiography and cross-sectional imaging have been used to further understand AVVR. Surgery to address AVVR must be tailored to the underlying mechanism and the timing of surgical repair should be chosen carefully. In this review, we discuss the etiologies, treatment options, surgical timing, and outcomes of valve repair or replacement for AVVR in patients with single ventricle congenital heart disease, with a focus on those with a Fontan circulation as AVVR is associated with increased risk for Fontan failure and mortality. In-depth understanding of the current literature will help guide clinicians in their approach and management of AVVR in this population.

Red Blood Cell Transfusion After Stage I Palliation Is Associated With Worse Clinical Outcomes.

Background Packed red blood cell transfusion may improve oxygen content in single-ventricle neonates, but its effect on clinical outcomes after Stage 1 palliation is unknown. Methods and Results Retrospective multicenter analysis of packed red blood cell transfusion exposures in neonates after Stage 1 palliation, excluding those with intraoperative mortality or need for extracorporeal membrane oxygenation. Transfusion practice variability was assessed, and multivariable regression used to identify transfusion risk factors. After propensity score adjustment for severity of illness, clinical outcomes were compared between transfused and nontransfused subjects. Of 396 subjects, 323 (82%) received 930 postoperative red blood cell transfusions. Packed red blood cell volume (median 9-42 mL/kg [P<0.0001]), donor exposures (1-2 [P<0.0001]), transfusion number (1-3 [P<0.0001]), and pretransfusion hemoglobin (12.1-13 g/dL, P=0.0049) varied between sites. Cyanosis (P=0.02), chest tube output (P=0.0003), and delayed sternal closure (P=0.0033) increased transfusion risk. Transfusion was associated with prolonged mechanical ventilation (6 [interquartile range 4, 12] versus 3 [1, 5] days, P=0.02) and intensive care unit stay (19 [12, 33] versus 9 [6, 19] days, P=0.016). When stratified by number of transfusions (0, 1, or >1), duration of mechanical ventilation (3 [1, 5] versus 4 [3, 6] versus 9 [5, 16] days [P<0.0001]) and intensive care unit stay (9 [6, 19] versus 13 [8, 25] versus 21 [13, 38] days [P<0.0001]) increased for those transfused more than once. Most subjects who died were transfused, though the association with mortality was not significant. Conclusions Packed red blood cell transfusion after Stage 1 palliation is common, and transfusion practice is variable. Transfusion is a significant predictor of longer intensive care unit stay and mechanical ventilation. Further studies to define evidence-based transfusion thresholds are warranted.

Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease.

OBJECTIVE: To develop a risk score to predict mortality or transplant in the interstage period. BACKGROUND: The "interstage" period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease. METHODS: This was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015. The primary composite endpoint was interstage mortality or transplant. Multivariable logistic regression and classification and regression tree analysis were performed on two-thirds of the patients ("learning cohort") to build a risk score for the composite endpoint, that was validated in the remaining patients ("validation cohort"). RESULTS: In the 2128 patients analyzed in the registry, the overall event rate was 9% (153 [7%] deaths, 42 [2%] transplants). In the learning cohort, factors independently associated with the composite endpoint were (1) type of Norwood; (2) postoperative ECMO; (3) discharge with Opiates; (4) No Digoxin at discharge; (5) postoperative Arch obstruction, (6) moderate-to-severe Tricuspid regurgitation without an oxygen requirement, and (7) Extra Oxygen required at discharge in patients with moderate-to-severe tricuspid regurgitation. This model was used to create a weighted risk score ("NEONATE" score; 0-76 points), with >75% accuracy in the learning and validation cohorts. In the validation cohort, the event rate in patients with a score >17 was nearly three times those with a score ≤17. CONCLUSIONS: We introduce a risk score that can be used post-stage 1 palliation to predict freedom from interstage mortality or transplant.

Early Integration of Palliative Care in Families of Children with Single Ventricle Congenital Heart Defects: A Quality Improvement Project to Enhance Family Support.

Children with single ventricle congenital heart defects (SVCHD) experience a significant risk of early mortality throughout their lifespan, particularly during their first year of life. Due to the intense care needed for these children and families, pediatric palliative care (PPC) team consults should be routine; however, medical staff are often reluctant to broach the idea of PPC to families. The involvement of PPC for many carries with it an association to end-of-life (EOL) care. Setting the standard of PPC involvement from the time of admission for the first palliative surgery led to increased family support, decreased days to consult, improved acceptance and communication. The purpose of this article is to describe a quality improvement project of early integration of PPC with families of children with SVCHD. Lessons learned will be presented, including the resources needed and the barriers encountered in assimilating PPC into the standard of care for all patients with SVCHD. The single ventricle (SV) and PPC teams collaborated to enhance the support given to SV families. Education was initiated with cardiology and PPC providers to understand the goal of consistent PPC consults beginning after birth for patients with SVCHD. Parents were educated during fetal consultation regarding the involvement of the PPC team. The SV team ensured compliance with the PPC initiative by identifying eligible patients and requesting consult orders from the primary providers. PPC consultation increased significantly over the 40 month study period to nearly 100% compliance for children with SVCHD who are undergoing pre-Fontan surgery. In addition, mean days to consult decreased dramatically during the study to a current average of 3 days into the patient's hospitalization; the data likely suggest that more PPC consults were routinely ordered versus urgently placed for unexpected complications. Data indicate that patients are being followed by the PPC team at an earlier age and stage in their SV journey which allows for more opportunity to provide meaningful support to these patients and families. The early involvement of the PPC team for children with SV physiology was operationally feasible and was accepted by families, thus allowing PPC providers to establish a therapeutic relationship early in the disease trajectory with the family. It allowed more continuity throughout the SV journey in a proactive fashion rather than a reactive manner.

Outcomes of Multistage Palliation of Infants With Single Ventricle and Atrioventricular Septal Defect.

BACKGROUND: Published palliation outcomes of infants with functional single ventricle (SV) and common atrioventricular septal defect (AVSD) are poor due to associated cardiac and extracardiac anomalies and development of atrioventricular valve (AVV) regurgitation. We report current palliation results. METHODS: From 2002 to 2012, 80 infants with functional SV with AVSD underwent multistage palliation. Competing-risks analyses modeled events after first-stage surgery and Glenn (death/transplantation vs next palliation surgery) and examined factors associated with survival and AVV intervention. RESULTS: Sixty-eight (80%) patients received neonatal palliation: modified Blalock-Taussig shunt (n = 33, 41%), Norwood (n = 20, 25%), and pulmonary artery band (n = 15, 19%), whereas 12 (15%) received primary Glenn. On competing-risks analysis, one-year following first-stage surgery, 29% of patients had died or received transplantation and 62% had undergone Glenn. Five years following Glenn, 9% of patients had died or received transplantation and 68% had undergone Fontan. Overall eight-year survival was 64% and was lower in patients with genetic syndromes (53% vs 82%), patients requiring concomitant total anomalous pulmonary venous connection repair (53% vs 69%), and those requiring neonatal palliation (48% vs 100%). Factors associated with mortality were unplanned reoperation (hazard ratio [HR]: 3.7 [1.7-8.0], P = .001) and extracorporeal membrane oxygenation use (HR: 7.1 [3.0-16.6], P < .001). Initial AVV regurgitation ≥ moderate was associated with AVV intervention (HR: 6.2 [2.4-16.1], P = .002) with eight-year freedom from death or AVV intervention of 25% in those patients. CONCLUSIONS: Patients with SV with AVSD are a distinct group and commonly have associated cardiac and extracardiac malformations that complicate care and affect survival. The development of AVV regurgitation requiring intervention is common but does not affect survival.

Atrioventricular Valve Repair in Single Ventricle Physiology: Timing Matters.

OBJECTIVES: Atrioventricular valve (AVV) regurgitation in patients with single ventricle (SV) physiology severely impacts prognosis; the appropriate timing for surgical treatment is unknown. We sought to study the results of surgical treatment of AVV regurgitation in SV patients and evaluate risk factors for mortality. METHODS: Medical records of 81 consecutive patients with moderate or severe AAV regurgitation who were submitted to AVV repair or replacement during any stage of univentricular palliation between January 2013 and May 2017 were examined. We studied demographic data and perioperative factors looking for predictors that might have influenced the results. Binary logistic regression was used to assess the impact on postoperative ventricular dysfunction and mortality. RESULTS: Median age and weight were seven months (interquartile range [IQR]: 3-24) and 5.2 kg (IQR: 3.7-11.2), respectively. Seventy (86.4%) patients underwent AVV repair, and 11 (13.6%) patients underwent AVV replacement. There was an association between AVV repair effectiveness and timing of intervention (P = .004). Atrioventricular valve intervention at the time of initial surgical palliation was associated with more ineffective repairs (P = .001), while AVV replacement was more common between Glenn and Fontan procedures (P = .004). Overall 30-day mortality was 30.5% (25 patients). In-hospital mortality was 49.4%, and it was higher when AVV repair was performed concomitant with initial (stage 1) palliation (64.1% vs 35.7%; P = .01) and when an effective repair was not achieved (75% vs 41%; P = .008). Multivariable analysis identified timing concomitant with stage 1 palliation as an independent risk factor for mortality (P = .01); meanwhile, an effective repair was a protective factor against in-hospital mortality (P = .05). CONCLUSION: Univentricular physiology with AVV regurgitation is a high-risk group of patients. Surgery for AVV regurgitation at stage 1 palliation was associated with less effective repair and higher mortality in this initial experience. On the other hand, effective repair determined better outcomes, highlighting the importance of experience and the learning curve in the management of such patients.

Outcomes of Common Atrioventricular Valve Repair in Patients With Single-Ventricle Physiology　- Indication, Timing and Repair Techniques.

BACKGROUND: Common atrioventricular valve (CAVV) repair in patients with a single ventricle remains a great challenge and a refractory issue for pediatric cardiac surgeons. Methods and Results: From January 2007 to April 2018, 37 consecutive patients with a single ventricle who underwent CAVV repair were included in the study group. Patients were divided into 2 groups based on the repair technique: patients in Group A were treated using the bivalvation technique, and patients in Group B underwent conventional repair techniques; baseline data were similar between groups. The inhospital and follow-up mortality were 5.4% (2/37) and 11.4% (4/35), respectively. After a follow-up of 65.5±29.3 months, the estimated 1-, 5-, and 10-year overall survival rates were 94.6%, 83.4%, and 77.0%, respectively. The rates of freedom from CAVV failure were 94.3%, 72.7%, and 62.9% after 1, 5, and 10 years, respectively. In the multivariate analysis, the independent factors for CAVV repair failure were repair technique (P=0.004) and heterotaxy syndrome (P=0.003). A total of 30 patients (81.1%) completed total cavopulmonary connection (TCPC); 3 patients required re-intervention; 24 of 31 patients (77.4%) were in New York Heart Association classes II and I at the latest follow-up. CONCLUSIONS: Outcomes of CAVV repair in patients palliated by single-ventricular surgery are acceptable. The bivalvation technique is a simple and effective technique.

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation / Resultados da Conversão da Cirurgia de Fontan-Kreutzer para Cavo-Pulmonar Total Devido Falência da Circulação Univentricular

Abstract Background: The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment. Objective: To evaluate the results of conversion from FK to TCPC. Methods: A retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05. Results: Fontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured. Conclusions: The conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia.

Resumo Fundamento: O procedimento de Fontan-Kreutzer (FK) foi amplamente realizado no passado, mas a longo prazo gerou muitas complicações, resultando em falha na circulação univentricular. A conversão para conexão cavopulmonar total (CCPT) é uma das opções de tratamento. Objetivo: Avaliar os resultados da conversão de FK para CCPT. Métodos: Revisão retrospectiva de prontuários de pacientes submetidos à conversão de FK para CCPT no período de 1985 a 2016. Significância p < 0,05. Resultados: Operações do tipo Fontan foram realizadas em 420 pacientes durante este período: CCPT foi realizada em 320, técnica de túnel lateral em 82 e FK em 18. Dez casos do grupo FK foram eleitos para conversão em CCPT. Todos os pacientes submetidos à conversão de Fontan foram incluídos neste estudo. Em nove pacientes, a indicação deveu-se a arritmia não controlada e em um devido à enteropatia perdedora de proteínas. A morte foi observada nos dois primeiros casos. O tempo médio de internação na unidade de terapia intensiva (UTI) foi de 13 dias e o tempo médio de internação hospitalar foi de 37 dias. Uma classe funcional pela melhora da New York Heart Association (NYHA) foi observada em 80% dos pacientes em NYHA I ou II. Cinquenta e sete por cento das conversões devido a arritmias tiveram melhora das arritmias; quatro casos foram curados. Conclusões: A conversão é um procedimento complexo e requer que um hospital terciário experiente seja realizado. A conversão melhorou a classe funcional da NYHA, apesar de uma resolução insatisfatória da arritmia.