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.

Factors associated with mortality or transplantation versus Fontan completion after cavopulmonary shunt for patients with tricuspid atresia.

OBJECTIVE: Tricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS-Fontan interstage death/transplantation versus transition to Fontan in TA. METHODS: A total of 417 infants younger than 3 months of age with TA were enrolled (January 1999 to February 2020) from 40 institutions into the Congenital Heart Surgeons' Society TA cohort. Parametric competing risk methodology was used to determine factors associated with the competing end points of death/transplantation without Fontan completion, and transition to Fontan. RESULTS: CPS was performed in 382 patients with TA; of those, 5% died or underwent transplantation without transition to Fontan and 91% transitioned to Fontan by 5 years after CPS. Prenatal diagnosis (hazard ratio [HR], 0.74; P < .001) and pulmonary artery band (PAB) at CPS (HR, 0.50; P < .001) were negatively associated with Fontan completion. Preoperative moderate or greater mitral valve regurgitation (HR, 3.0; P < .001), concomitant mitral valve repair (HR, 11.0; P < .001), PAB at CPS (HR, 3.0; P < .001), postoperative superior vena cava interventions (HR, 9.0; P < .001), and CPS takedown (HR, 40.0; P < .001) were associated with death/transplantation. CONCLUSIONS: The mortality rate after CPS in patients with TA is notable. Those with preoperative mitral valve regurgitation remain a high-risk group. PAB at the time of CPS being associated with both increased risk of death and decreased Fontan completion may represent a deleterious effect of antegrade pulmonary blood flow in the CPS circulation.

Attrition between the superior cavopulmonary connection and the Fontan procedure in hypoplastic left heart syndrome.

OBJECTIVE: We investigated the incidence and predictors of failure to undergo the Fontan in children with hypoplastic left heart syndrome who survived superior cavopulmonary connection. METHODS: The cohort consists of all patients with hypoplastic left heart syndrome who survived to hospital discharge after superior cavopulmonary connection between 1988 and 2017. The primary outcome was attrition, which was defined as death, nonsuitability for the Fontan, or cardiac transplantation before the Fontan. Subjects were excluded if they were awaiting the Fontan, were lost to follow-up, or underwent biventricular repair. The study period was divided into 4 eras based on changes in operative or medical management. Attrition was estimated with 95% confidence intervals, and predictors were identified using adjusted, logistic regression models. RESULTS: Of the 856 hospital survivors after superior cavopulmonary connection, 52 died, 7 were deemed unsuitable for Fontan, and 12 underwent or were awaiting heart transplant. Overall attrition was 8.3% (71/856). Attrition rate did not change significantly across eras. A best-fitting multiple logistic regression model was used, adjusting for superior cavopulmonary connection year and other influential covariates: right ventricle to pulmonary artery shunt at Norwood (P < .01), total support time at superior cavopulmonary connection (P < .01), atrioventricular valve reconstruction at superior cavopulmonary connection (P = .02), performance of other procedures at superior cavopulmonary connection (P = .01), and length of stay after superior cavopulmonary connection (P < .01). CONCLUSIONS: In this study spanning more than 3 decades, 8.3% of children with hypoplastic left heart syndrome failed to undergo the Fontan after superior cavopulmonary connection. This attrition rate has not decreased over 30 years. Use of a right ventricle to pulmonary artery shunt at the Norwood procedure was associated with increased attrition.

Arteriovenous fistula creation for hypoxia after single ventricle palliation: A single-institution experience and literature review.

BACKGROUND: Hypoxia is a common and sometimes severe morbidity of single ventricle congenital heart disease (CHD). Creation of an arteriovenous fistula (AVF) is occasionally performed for patients after superior or total cavopulmonary connection (SCPC or TCPC) in an attempt to improve oxygen saturations. Despite previous reports, AVF creation is a rare palliation with inadequately defined benefits and risks. We sought to determine changes in peripheral oxygen saturation (SpO2 ) and risk of adverse event after AVF creation in children with single ventricle CHD at our institution. METHODS: We conducted a retrospective chart review of patients with a history of single ventricle palliation and history of surgical AVF creation who were seen at our tertiary care center from 1996 to 2017. RESULTS: A total of seven patients were included in our study. SpO2 for the overall cohort did not significantly increase after AVF creation (pre-AVF 79.1 ± 6.9%, post-AVF 82.7 ± 6.0% [P = .23]). SpO2 trended up for large shunts (>5 mm) (pre-AVF 75.0 ± 7.6%, post-AVF 84.0 ± 5.3% [P = .25]). SpO2 did not improve for small shunts (≤5 mm) (pre-AVF 82.3 ± 6.5%, post-AVF 81.0 ± 8.5% [P = .50]). The 12-month overall and transplant-free survival were 85.7% and 71.4%, respectively. Freedom from AVF-related complication (cephalic edema, thrombotic occlusion) was 51.4% at 12 months. CONCLUSION: Palliative AVF creation for patients with single ventricle CHD and hypoxia does not universally improve SpO2 and is prone to early complications. Despite a lack of durable benefit and known risks, AVF creation remains a reasonable palliation for a subset of patients after SCPC who are not candidates for TCPC, or potentially as a bridge to heart transplantation.

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation.

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.

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.

Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts.

OBJECTIVE: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. METHODS: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Baseline characteristics and clinical outcomes were retrieved from the database. The survival analysis was performed at the end of 2016. Prognostic factors were identified using multivariate analysis. RESULTS: A total of 366 consecutive patients (24.5 ± 17.6 years of age, 40% male) with PAH-CHD were analyzed. Most had simple shunts (85 pre-tricuspid, 105 post-tricuspid, 102 combined shunts). Patients with pre-tricuspid shunts were significantly older at diagnosis in comparison to post-tricuspid, combined, and complex shunts. Clinical classifications identified patients as having Eisenmenger syndrome (ES, 26.8%), prevalent left to right shunt (66.7%), PAH with small defect (3%), or PAH following defect correction (3.5%). At follow-up (median = 5.9 years; 0.1-20.7 years), no statistically significant differences in survival rate were seen among the anatomical-pathophysiological shunts (p = 0.1). Conversely, the clinical classifications revealed that patients with PAH-small defect had inferior survival compared to patients with ES, PAH post-corrective surgery, or PAH with prevalent left to right shunt (p = 0.01). Significant mortality risks were functional class III, age < 10 years, PAH-small defect, elevated right atrial pressure > 15 mmHg, and baseline PVR > 8 WU•m.2. CONCLUSION: Patients with PAH-CHD had a modest long-term survival. Different anatomical-pathophysiological shunts affect the natural presentation, while clinical classifications indicate treatment strategies and survival. Contemporary therapy improves survival in deliberately selected patients.

Atrioventricular valve regurgitation in patients undergoing total cavopulmonary connection: Impact of valve morphology and underlying mechanisms on survival and reintervention.

OBJECTIVE: The study objective was to determine the mechanisms of atrioventricular valve regurgitation in single-ventricle physiology and their influence on outcomes after total cavopulmonary connection. METHODS: Among 460 patients who underwent a total cavopulmonary connection, 101 (22%) had atrioventricular valve surgery before or coincident with total cavopulmonary connection. RESULTS: Atrioventricular valve morphology showed 2 separated in 33 patients, mitral in 11 patients, tricuspid in 41 patients, and common in 16 patients. Patients with a tricuspid and a common atrioventricular valve underwent atrioventricular valve surgery frequently, 27% and 36%, respectively. Atrioventricular valve regurgitation was due to 1 or more of the following mechanisms: dysplastic leaflet (62), prolapse (53), annular dilation (27), cleft (22), and chordal anomaly (14). Structural anomalies were observed in 89 patients (88%). The procedure was atrioventricular valve repair in 81 patients, atrioventricular valve closure in 16 patients, and atrioventricular valve replacement in 4 patients. Among 81 patients who underwent initial repair, repeat repair was required in 20 patients, atrioventricular valve replacement was required in 7 patients, and atrioventricular valve closure was required in 3 patients. Among patients undergoing atrioventricular valve surgery, overall survival after total cavopulmonary connection (88% vs 95% at 15 years, P = .01), freedom from atrioventricular valve reoperation after total cavopulmonary connection (75% vs 99% at 15 years, P < .01), and grade of atrioventricular valve regurgitation at a median follow-up of 6.6 years (P < .01) were worse than in those who did not require atrioventricular valve surgery. CONCLUSIONS: Atrioventricular valve regurgitation in univentricular heart is more frequently associated with a tricuspid or a common atrioventricular valve, and structural anomalies are the primary cause. Significant atrioventricular valve regurgitation requiring surgery influences survival after total cavopulmonary connection, especially when atrioventricular valve replacement was needed. Surgical management based on mechanisms of regurgitation is mandatory.

When is the right time for Fontan conversion? The role of cardiopulmonary exercise test.

BACKGROUND: To determine if Fontan conversion (FC) resulted in improvement in exercise capacity (EC), and to determine the role of cardiopulmonary exercise test (CPET) in risk stratification of patients undergoing FC. METHODS: A retrospective review of patients who underwent CPET prior to FC at Mayo Clinic from 1994 to 2014. The patients who also underwent post-operative CPET were selected for the analysis of improvement in EC defined as 10% increase in baseline peak oxygen consumption (VO2). RESULTS: 75 patients CPET prior to FC; mean age 24±6years; 44 males (59%); and 51 (68%) were in NYHA III/IV prior to FC. Pre-operative peak VO2 was 15.5±3.4ml/kg/min. A comparison of pre- and post-FC CPET data was performed using 42 patients (56%) that underwent CPET after FC. Improvement in EC occurred in 18 of 42 patients (43%). Baseline peak VO2 >14ml/kg/min was associated with improved EC (hazard ratio [HR] 1.85; P=.02). Improvement in New York Heart Association (NYHA) class occurred in 12 (67%) patients with improved EC vs 2 (8%) without improved EC. Improvement in NYHA class was more likely to occur in patients with improved EC compared to those without improvement EC (odds ratio 4.11, P=.01). There were 10 (13%) perioperative deaths, and baseline peak VO2 ≤14ml/kg/min was predictive of perioperative mortality (HR 3.74; P<.001). CONCLUSIONS: Baseline peak VO2 was predictive of perioperative survival, and improvement in EC. Performance on CPET in failing Fontan patients might be a useful clinical parameter in determining appropriate timing of FC.

Current outcomes of the bi-directional cavopulmonary anastomosis in single ventricle patients: analysis of risk factors for morbidity and mortality, and suitability for Fontan completion.

OBJECTIVES: The bi-directional cavopulmonary anastomosis forms an essential staging procedure for univentricular hearts. This review aims to identify risk factors for morbidity, mortality, and suitability for Fontan completion. METHODS: A total of 114 patients undergoing cavopulmonary anastomosis between 1992 and 2012 were reviewed to assess primary - mortality and survival to Fontan completion - and secondary outcome endpoints - re-intubation, new drain, and ICU stay. Median age and weight were 8 months and 6.9 kg, respectively. In 83% of patients, 1-3 interventions had preceded. Norwood-type procedures became more prevalent over time. RESULTS: Extubation occurred after a median of 4 hours, median ICU stay was 2 days; 10 patients (8.8%) needed re-intubation and 18 received a new drain. Higher central venous pressure and transpulmonary gradient were risk factors for new drain insertion (p<0.01). Higher pre-operative pulmonary pressure correlated with increased inotropic support and prolonged intubation (p=0.01). Need for re-intubation was significantly affected by younger age at operation (p=0.01). Hospital and pre-Fontan mortality were 11.4 and 5.3%, respectively. Operative mortality was independently affected by younger age (p=0.013), lower weight (p=0.02), longer bypass time (p=0.04), and re-intubation (p=0.004). Interstage mortality was mainly influenced by moderate ventricular function (p=0.03); 82% of survivors underwent or are candidates for Fontan completion. CONCLUSION: The cavopulmonary anastomosis remains associated with adverse outcomes. Age at operation decreases with rising prevalence of complex univentricular hearts. Considering the important impact of re-intubation on hospital mortality, peri-operative management should focus on optimising cardio-respiratory status. Once this selection step is taken, successful Fontan completion can be expected, provided that ventricular function is maintained.

Second stage after initial hybrid palliation for hypoplastic left heart syndrome: Arterial or venous shunt?

OBJECTIVE: Hybrid palliation for hypoplastic left heart syndrome has been developed as an alternative to neonatal Norwood surgery. At the second stage, a source of pulmonary blood flow has to be established. Options include an arterial modified Blalock-Taussig or a venous superior cavopulmonary shunt. METHODS: We retrospectively reviewed patients who received second-stage palliation after the initial hybrid. Patients were stratified according to the source of pulmonary blood supply into the arterial shunt (n = 17 patients) or venous shunt (n = 26 patients). RESULTS: Age and weight at second stage were lower in the arterial group (85 [45-268] days vs 152.5 [61-496] days, P = .001 and 3.6 [2.7-9.4] kg vs 5.1 [2.97-9.4] kg, P = .001, respectively). All recorded surgical times were shorter in the arterial group. Mechanical ventilation and intensive care stay were shorter in the venous group (5.82 [2.01-14.9] days vs 2.42 [0.56-13.67] days, P = .005 and 8.5 [3.6-23.7] vs 5.75 [0.8-17.6] days, P = .036, respectively) There was no difference in mortality (2/17 vs 5/26; P = .685) or incidence of complications between the 2 groups. There was a tendency toward a higher need for intervention in the immediate postoperative period in the venous group, but this did not reach significance (6/17 vs 13/26, P = .342). The arterial group has shown better development of the branch pulmonary arteries with a higher lower lobe index (158.38 ± 39.43 mm(2)/m(2) vs 113.33 ± 43.96 mm(2)/m(2), respectively, P = .037). CONCLUSIONS: Both arterial and venous shunts are viable options with mortality and morbidity results comparable to those in the literature. The arterial shunt pathway (2-stage Norwood I) may offer better pulmonary arterial growth than the venous shunt (comprehensive/combined Norwood I and II).

Outcomes of Damus-Kaye-Stansel anastomosis at time of cavopulmonary connection in single ventricle patients at risk of developing systemic ventricular outflow tract obstruction.

OBJECTIVES: Children with various single ventricle anomalies are at risk of developing systemic ventricular outflow tract obstruction (SVOTO) following volume unloading with cavopulmonary connection (CPC). We aim to evaluate the value of Damus-Kaye-Stansel (DKS) anastomosis at the time of CPC in eliminating late SVOTO risk. METHODS: Retrospective review of single ventricle patients who underwent DKS concurrent with CPC between 1997 and 2012 was performed. Clinical, echocardiographic and angiographic outcomes were analysed. RESULTS: Thirty-six children with single ventricle underwent DKS at the time of Glenn bidirectional CPC (n = 29) or Fontan total CPC (n = 7). The underlying anatomy was double inlet left ventricle (n = 18), double outlet right ventricle (n = 8), unbalanced atrioventricular septal defect (n = 4) and other (n = 6). Prior palliation included pulmonary artery band (n = 35), coarctation/arch repair (n = 11) and atrial septectomy (n = 8). Median age at the time of DKS was 8.9 months (range 3.6 months-9.1 years) and the median weight was 6.7 kg (range 5-27 kg). At the time of DKS, 17 patients (47%) had no SVOT gradient and 19 (53%) had SVOT gradient (mean 23.4 ± 18.7 mmHg). Overall survival was 89 and 83% at 1 month and 5 years, respectively. None of the deaths were related to SVOTO or DKS complications. When present, SVOT gradient decreased from 23.4 ± 18.7 mmHg preoperatively to 0 after DKS (P < 0.001). At the last follow-up, none of the patients developed any SVOT gradient; 78% of them had zero or trivial aortic/neoaortic valve regurgitation while 22% had mild regurgitation. None of the patients had evidence of compression of the left pulmonary artery or bronchus. Eighty-one percent of patients have reached or are suitable candidates awaiting final palliative surgery. CONCLUSIONS: DKS can be safely performed in conjunction with CPC without added mortality risk. It is very effective in mitigating SVOTO risk, with sustainable good semilunar valves function. Our data support an aggressive approach to performing DKS concurrent with CPC in children with single ventricle pathologies at risk of developing SVOTO.

Cavopulmonary anastomosis without cardiopulmonary bypass.

OBJECTIVES: There is an increasing trend to perform the bidirectional superior cavopulmonary (Glenn) anastomosis without cardiopulmonary bypass. In this report, we present our results of off-pump bidirectional Glenn operation done without using a venoatrial shunt to decompress the superior vena cava during clamping. [corrected]. METHODS: A prospective, non-randomized comparative study was conducted in 50 patients with functional single ventricle anomalies who underwent bidirectional Glenn anastomosis without cardiopulmonary bypass. The patients were divided into two groups: Group I (n = 25), where it was done without a veno-atrial shunt, and Group II (n = 25), where it was done with a veno-atrial shunt. Two patients in Group I and 4 patients in Group II had a bilateral bidirectional Glenn shunt. Five patients in Group I and three patients in Group II had a previous left modified Blalock-Taussig shunt. All patients underwent a complete neurological examination both preoperatively as well as postoperatively. RESULTS: The early hospital mortality was 4% (2/50), one in each group. The median follow-up was 14 months. The mean internal venous pressure on clamping the superior vena cava was 37.07 ± 7.12 mmHg in Group I and 24 ± 4.4 mmHg in Group II. The mean clamp time was 9.85 ± 3.52 min in Group I and 21.3 ± 4.4 min in Group II. The transcranial pressure gradient was 62.37 ± 15.01 mmHg in Group I, while 65.08 ± 13.89 in Group II. The mean intensive care unit stay was 2.57 ± 75 days in Group I, 3.3 ± 1.09 in Group II. There were no major neurological complications apart from treatable convulsions in one case in Group I (4%), 2 cases in Group II (8%), and delayed recovery in one case (4%) in Group I. CONCLUSIONS: Off-pump bidirectional Glenn operation without caval decompression is a safe, simple and more economic procedure.

Superior cavopulmonary anastomosis timing and outcomes in infants with single ventricle.

OBJECTIVES: We sought to identify factors associated with the timing and surgical outcomes of the superior cavopulmonary anastomosis. METHODS: The Pediatric Heart Network's Infant Single Ventricle trial database identified participants who underwent superior cavopulmonary anastomosis. Factors potentially associated with age at superior cavopulmonary anastomosis, length of stay and death by 14 months of age were evaluated. Factors included subject demographics, cardiac anatomy, measures from neonatal hospitalization and pre-superior cavopulmonary anastomosis visit, adverse events, echocardiographic variables, intraoperative variables, superior cavopulmonary anastomosis type, and number of concurrent cardiac surgical procedures. Age at superior cavopulmonary anastomosis was analyzed using Cox proportional hazards regression. Natural log length of stay was analyzed by multiple linear regression. RESULTS: Superior cavopulmonary anastomosis was performed in 193 subjects at 5.2 months of age (interquartile range, 4.2, 6.2) and weight of 5.9 kg (interquartile range, 5.3, 6.6). The median length of stay was 7 days (interquartile range, 6, 10). There were 3 deaths and 1 transplant during the superior cavopulmonary anastomosis hospitalization, and 3 deaths and 3 transplants between discharge and 14 months of age. Age at superior cavopulmonary anastomosis was associated with center and interstage adverse events. A longer length of stay was associated with younger age and greater case complexity. Superior cavopulmonary anastomosis type, valve regurgitation, ventricular ejection fraction, and ventricular end-diastolic pressure were not independently associated with age at superior cavopulmonary anastomosis or the length of stay. CONCLUSIONS: Greater case complexity and more frequent interstage adverse events are associated with an earlier age at superior cavopulmonary anastomosis. Significant variation in age at superior cavopulmonary anastomosis among centers, independent of subject factors, highlights a lack of consensus regarding the optimal timing. Factors associated with length of stay could offer insights for improving presuperior cavopulmonary anastomosis care and surgical outcome.

Endostatin, an inhibitor of angiogenesis, decreases after bidirectional superior cavopulmonary anastamosis.

Pulmonary arteriovenous malformations (PAVMs) are a common source of morbidity after bidirectional superior cavopulmonary anastomosis (Glenn). The diversion of hepatic venous effluent away from the pulmonary circulation after Glenn appears to play a significant role in the pathogenesis of PAVMs. Although the liver is known to produce factors that regulate vascular development, specific hepatic inhibitors of angiogenesis have not been described in the post-Glenn population. Endostatin, produced from its precursor collagen XVIII, is a potent inhibitor of angiogenesis produced by the liver. This study aimed to investigate the hypothesis that endostatin levels decrease in patients after Glenn. Levels of endostatin and its precursor, long-type collagen XVIII, were determined by enzyme-linked immunoassay and immunoprecipitation, respectively, for serum samples from 38 patients undergoing Glenn, total cavopulmonary anastomosis (Fontan), or biventricular repair of cardiac defects. Samples were obtained before surgery and 24 h afterward. In patients undergoing a bidirectional Glenn procedure, endostatin levels decreased after surgery (n = 17; 4.42 vs 3.34 ng/ml; p < 0.001), and long type-collagen XVIII levels increased by 200 % (n = 10; p = 0.0001). However, endostatin levels did not change after surgery in patients undergoing Fontan (n = 13) or biventricular repair (n = 8). In patients undergoing Fontan, long-type collagen XVIII increased by 18 % (p < 0.01), whereas in control subjects, the levels were unchanged. These data suggest that the diversion of hepatic blood flow away from the pulmonary circulation in patients after the Glenn procedure inhibits endostatin production from collagen XVIII, resulting in decreased circulating serum endostatin levels. A decrease in endostatin may promote angiogenesis. The mechanism whereby the pulmonary circulation processes endostatin and its potential role in the pathogenesis of PAVMs warrant further study.

Mid-term results of bidirectional cavopulmonary anastomosis and hemi-Mustard procedure in anatomical correction of congenitally corrected transposition of the great arteries.

OBJECTIVES: The Senning or Mustard procedure combined with the arterial switch operation (ASO) (± VSD and no left ventricular (LV) outflow tract obstruction) or the Rastelli operation (VSD and LV outflow tract obstruction) has become the preferred strategy over conventional repair as it is thought to prevent long-term dysfunction of the right ventricle (RV). More recently, hemi-Mustard rerouting of blood from the inferior vena cava to the RV in combination with bidirectional cavopulmonary anastomosis (BCPA) has been adopted by some centres for potential benefits over the classic atrial switch procedure. The aim of this study was to analyse our experience with hemi-Mustard and BCPA as part of an anatomical repair of congenitally corrected transposition of the great arteries (CCTGA) in selected patients. METHODS: Between 2004 and 2011, eight patients underwent hemi-Mustard/BCPA with the Rastelli operation (n = 6) or ASO (n = 2). The median age was 2.9 (range: 1.2-9.1) years. Positional anomalies were present in 75% of the patients. Both patients with ASO had dysplastic and insufficient tricuspid valves. In the Rastelli group, four patients had previously received shunts followed by BCPA in one patient. In the ASO group, both patients underwent pulmonary artery banding initially. RESULTS: There was one in-hospital death and no late mortality. Two patients received a pacemaker. One patient from the Rastelli group required conduit change 6 years later. At the mean follow-up of 4.5 years, six and one patients are in NYHA classes I and II, respectively; six patients showed good biventricular function, while one had LV dysfunction. Systemic venous obstruction and sinus node dysfunction were not observed, and BCPA was functioning well in all patients. CONCLUSIONS: Hemi-Mustard/BCPA is useful in anatomical repair of CCTGA in selected patients. When compared with the classic atrial switch operation, it is technically easier which makes it especially helpful in atrio-apical discordance; it unloads an RV with limited size or function, and avoids complications related to the upper limb of the classic atrial switch procedure. Mid-term results of this approach are favourable. Further follow-up is needed to prove long-term benefits.

Immediate results of bidirectional cavopulmonary anastomosis and Fontan operations in adults.

The purpose of this study is to analyze the immediate results of bidirectional cavopulmonary anastomosis (BCPA) and Fontan operations performed in adults, and to reveal the risk factors. During the years 1983-2010, 681 consecutive patients underwent BCPA or a Fontan operation. Fifty-three of 681 patients were more than 18 years of age. Twenty-nine adults underwent BCPA and 24 underwent a Fontan operation. Immediate results of surgical treatment were followed during the hospital period. The average number of exceeded 'operability' criteria by Choussat et al. [Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H. Selection criteria for Fontan procedure. In: Anderson RH, Shinebourne EA, editors. Pediatric Cardiology. Edinburgh: Churchhill Livingstone, 1977:559-566] was significantly higher in patients from the BCPA group compared to the Fontan group (1.3±0.8 vs. 0.9±0.7, P=0.034). Hospital mortality after BCPA in adults was 6.9% (2/29) and did not differ from children (7.1%, 19/268), P=0.634. Hospital mortality after Fontan operation in adults was 8.3% (2/24) and did not differ from children (11.9%, 43/360), P=0.419. The frequency of non-lethal hospital complications was higher in patients after a Fontan operation. Patients from the Fontan group more frequently developed arrhythmias and prolonged pleural effusions. Preoperative regurgitation at atrioventricular valves was an independent risk factor for hospital mortality and morbidity after a Fontan operation. BCPA and Fontan operations performed in adults are accompanied by good immediate results and considerably improves patients' condition.

A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at Fontan completion.

OBJECTIVE: We previously reported no difference in morbidity or mortality in a cohort of infants undergoing stage 1 and 2 reconstructions for hypoplastic left heart syndrome with either a modified Blalock-Taussig shunt or a right ventricular to pulmonary artery conduit. This article compares the hemodynamics and perioperative course at the time of the Fontan completion and reports longer-term survival for this cohort. METHODS: We retrospectively reviewed the hospital records of all patients who underwent stage 1 reconstruction between January 2002 and May 2005 and subsequent surgical procedures, as well as cross-sectional analysis of hospital survivors. RESULTS: A total of 176 patients with hypoplastic left heart syndrome or a variant underwent stage 1 reconstruction with either modified Blalock-Taussig shunt (n = 114) or right ventricular to pulmonary artery conduit (n = 62). Shunt selection was at the discretion of the surgeon. The median duration of follow-up was 58 months (range 1-87 months). By Kaplan-Meier analysis, shunt type did not influence survival or freedom from transplant at 5 years (right ventricular to pulmonary artery conduit 61%; 95% confidence limit, 47-72 vs modified Blalock-Taussig shunt 70%; 95% confidence limit, 60-77; P = .55). A total of 107 patients underwent Fontan (69 modified Blalock-Taussig shunts and 38 right ventricular to pulmonary artery conduits) with 98% (105/107) early survival. Patients with a right ventricular to pulmonary artery conduit shunt pre-Fontan had higher pulmonary artery (13 +/- 8 mm Hg vs 11 +/- 3 mm Hg, P = .026) and common atrial (8 +/- 2.3 mm Hg vs 6.8 +/- 2.7 mm Hg, P = .039) pressures. By echocardiography evaluation, there was more qualitative moderate to severe ventricular dysfunction (right ventricular to pulmonary artery conduit 31% [12/36] vs modified Blalock-Taussig shunt 17% [11/67], P = .05) and moderate to severe atrioventricular valve regurgitation (right ventricular to pulmonary artery conduit 40% [14/35] vs modified Blalock-Taussig shunt 16% [11/67], P = .01) in the right ventricular to pulmonary artery conduit group. Use of diuretic therapy, angiotensin-converting enzyme inhibition, reflux medications, and tube feedings were not different between groups. Overall, 5 patients underwent heart transplantation (right ventricular to pulmonary artery conduit 4 vs modified Blalock-Taussig shunt 1, P = .1) before Fontan. There was no difference in age or weight at Fontan, bypass time, intensive care unit or hospital length of stay, postoperative pleural effusions, or need for reoperation between groups. CONCLUSIONS: Interim analyses continue to suggest there is no survival advantage of one shunt type compared with the other. Longer-term follow-up of a randomized patient population remains of utmost importance.

Impact of evolving strategy on clinical outcomes and central pulmonary artery growth in patients with bilateral superior vena cava undergoing a bilateral bidirectional cavopulmonary shunt.

OBJECTIVE: We reported a high incidence of thrombosis, central pulmonary artery hypoplasia, and mortality for bilateral bidirectional cavopulmonary shunts. We hypothesized that technical modifications in the cavopulmonary anastomosis and anticoagulation would limit thrombus and central pulmonary artery hypoplasia, and thereby improve outcomes. METHODS: Sixty-one patients (median age, 8.4 months; weight, 6.6 kg) underwent bilateral bidirectional cavopulmonary shunt from 1990 to 2007. The cohort was divided into 2 groups: 1) the conventional group (1990-1999, n = 37) and 2) the V-shaped group, with a hemi-Fontan or modification in which the cavae were anastomosed to the pulmonary artery adjacent to each other so they formed the appearance of a V (1999-2007, n = 24). Central and branch pulmonary artery growth, survival, and reinterventions were determined. RESULTS: The pre-Fontan study showed equivalent superior venae cavae and Nakata indices. The central pulmonary artery index and central pulmonary artery/Nakata index ratio were significantly higher in the V-shaped group (P < .05). There were no differences in freedom from death or transplant (conventional 69% vs V-shaped 75% at 3 years, P = .5), and a nonsignificant trend toward improving freedom from reinterventions (63% vs 81% at 3 years, P = .15) and thrombosis (82% vs 95% at 1 year, P = .11) was observed in the V-shaped group. Multivariate analysis showed anastomotic strategy, low saturation, and thrombosis were predictors for death. Anastomotic strategy, lack of anticoagulation, thrombosis, and small superior venae cavae were predictors for reintervention (P < .05). Predictors for thrombus included small superior venae cavae, Nakata index, and low saturation (P < .03). CONCLUSIONS: Surgical modifications for bilateral bidirectional cavopulmonary shunts were associated with the larger central pulmonary artery size. Lack of anticoagulation and anastomotic strategy affected reintervention. Anastomotic strategy and postoperative thrombus affected mortality.