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.

Tricuspid atresia: Where are we now?

Tricuspid atresia (TA) is a complex congenital heart disease that presents with cyanosis in the neonatal period. It is invariably fatal if left untreated and requires multiple stages of palliation. Early recognition and timely surgical intervention are therefore pivotal in the management of these infants. This literature review considers the pathophysiology, presentation, investigations, and classification of TA. Moreover, it discusses the evidence upon which the latest medical and surgical treatments are based, as well as numerous recent case reports. Further work is needed to elucidate the etiology of TA, clarify the role of pharmacotherapy, and optimize the surgical management that these patients receive.

Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): A systematic review and meta-analysis.

BACKGROUND: In this study we sought to evaluate risk factors (RFs) for death or heart transplantation (D-HT) in single-ventricle (SV) physiology due to tricuspid atresia (TA), pulmonary atresia‒intact ventricular septum (PA-IVS), and heterotaxy with SV (HX), clinical conditions for which outcome data are limited. METHODS: To conduct a systematic review, we included citations that evaluated occurrence of D-HT in SV physiology of TA, PA-IVS, and HX in English articles published between January 1998 and December 2017 based on inclusion and exclusion criteria, following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The Cochrane Risk of Bias in Non-Randomized Studies-Interventions (ROBINS-I) tool for non-randomized studies was used to assess the risk of bias. Meta-analysis was performed if RF data were available in more than 3 studies. RESULTS: Of 11,629 citations reviewed, 30 met inclusion criteria. All 30 were observational, retrospective studies. In all, 1,770 patients were included, 481 died and 21 underwent HT (63 lost to follow-up); 723 patients reached Fontan completion. We found that systemic ventricular dysfunction (odds ratio [OR] 20.7, confidence interval [CI] 10.0-42.5, I2 = 0%) and atrioventricular valve regurgitation (AVR) were associated with risk of D-HT (OR 3.7, CI 1.9-6.9, I2 = 14%). RF associations with D-HT could not be derived for right ventricle‒dependent coronary circulation, pulmonary arteriovenous malformations, total anomalous pulmonary venous return, arrhythmias, and pulmonary atresia. CONCLUSIONS: This systematic review and meta-analysis has identified a high mortality rate in children born with non-HLHS SV heart disease and points to potential under-utilization of HT. Systemic ventricular dysfunction and AVR were identified as RFs for D-HT in this subset of patients SV with TA, PA-IVS, and HX.

Revisitation of Double-Inlet Left Ventricle or Tricuspid Atresia With Transposed Great Arteries.

BACKGROUND: Patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries are predisposed to systemic outflow tract obstruction necessitating systemic outflow relief operations (SORO). METHODS: Between January 2000 and February 2018, 30 patients with double-inlet left ventricle (n = 20) or tricuspid atresia (n = 10) with transposition of the great arteries underwent single-ventricle palliation. Arch obstruction was observed in 14 patients. Initial palliative procedures comprised pulmonary artery banding with (n = 4) or without (n = 14) arch repair, bilateral pulmonary artery banding with ductal stenting (n = 5), primary Norwood operation (n = 4), and palliative arterial switch operation (n = 1). Cox proportional hazards model was fitted in 15 patients with initial postnatal echocardiography to identify risk factors for decreased time to SORO. RESULTS: One early and one late death occurred during the median follow-up period of 66 months (10-year survival rate, 93.3%). Various types of SORO were required in 20 of 30 patients (66.7%): Damus-Kaye-Stansel procedure (n = 12), primary Norwood-type palliation (n = 4), palliative arterial switch operation (n = 1), and bulboventricular foramen extension (n = 3). Freedom from SORO at 5 years was 34.5% in all patients (N = 30). Cox regression for the subgroup (n = 15) revealed that arch obstruction (hazard ratio, 20.6; 95% confidence interval, 2.9 to 148.2; p = 0.003) and smaller systemic outflow tract area index at end-systolic phase (hazard ratio, 1.5 at 10 mm2/m2 decrease; 95% confidence interval, 1.0 to 2.1; p = 0.033) were identified as risk factors for decreased time to SORO. CONCLUSIONS: Arch obstruction and a smaller systemic outflow tract area index at end-systolic phase at initial presentation are predictors of subsequent need for SORO in patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries.

Improved long-term outcomes in double-inlet left ventricle and tricuspid atresia with transposed great arteries: systemic outflow tract obstruction present at birth defines long-term outcome.

OBJECTIVES: In 2 subtypes of functional single ventricle, double inlet left ventricle (DILV) and tricuspid atresia with transposed great arteries (TA-TGA), systemic output passes through an outflow chamber before entering the aorta. Intracardiac obstruction to this pathway causing systemic outflow tract obstruction (SOTO) may be present at birth or develop over time. Long-term survival after Fontan has not been defined. We defined outcomes utilizing records from 2 centres that were cross-checked with data from a bi-national Fontan Registry for completeness and accuracy. METHODS: Two hundred and eleven patients were identified, 59 TA-TGA,152 DILV. Median follow-up was 17 years (range 4 days to 49.8 years). The Kaplan-Meier method was used for all of the time to event analyses and the log-rank test was used to compare the time-to-events. Cox proportional hazard models were used to test the association between potential predictors and time-to-event end-points. RESULTS: TA-TGA had reduced survival compared to DILV (cumulative risk of death 28.8% vs 11%, hazard ratio (HR) 3.1 (95% confidence interval (CI) 1.6-6.1), P = 0.001). In both groups, SOTO at birth carried a worse prognosis HR 3.54 (1.36-9.2, P = 0.01). SOTO was not more common in either morphology at birth ( P = 0.20). Periprocedural mortality accounted for 40% of deaths. Fontan was achieved in 82%, DILV were more likely to achieve Fontan than TA-TGA (91% vs 60%, P <0.001). After Fontan there were 9 deaths (4%) with no difference according to morphology. CONCLUSIONS: Patients with TA-TGA have poorer outcomes than those with DILV, affecting survival and likelihood of achieving Fontan. SOTO at birth carries a high risk of mortality suggesting that, when present, initial surgical management should address this.

Risk Factors for Increased Hospital Resource Utilization and In-Hospital Mortality in Adults With Single Ventricle Congenital Heart Disease.

Most patients with single ventricle congenital heart disease are now expected to survive to adulthood. Co-morbid medical conditions (CMCs) are common. We sought to identify risk factors for increased hospital resource utilization and in-hospital mortality in adults with single ventricle. We analyzed data from the 2001 to 2011 Nationwide Inpatient Sample database in patients aged ≥18 years admitted to nonteaching general hospitals (NTGHs), TGHs, and pediatric hospitals (PHs) with either hypoplastic left heart syndrome, tricuspid atresia or common ventricle. National estimates of hospitalizations were calculated. Elixhauser CMCs were identified. Length of stay (LOS), total hospital costs, and effect of CMCs were determined. Age was greater in NTGH (41.5 ± 1.3 years) than in TGH (32.8 ± 0.5) and PH (25.0 ± 0.6; p <0.0001). Adjusted LOS was shorter in NTGH (5.6 days) than in PH (9.7 days; p <0.0001). Adjusted costs were higher in PH ($56,671) than in TGH ($31,934) and NTGH ($18,255; p <0.0001). CMCs are associated with increased LOS (p <0.0001) and costs (p <0.0001). Risk factors for in-hospital mortality included increasing age (odds ratio [OR] 5.250, CI 2.825 to 9.758 for 45- to 64-year old vs 18- to 30-year old), male gender (OR 2.72, CI 1.804 to 4.103]), and the presence of CMC (OR 4.55, CI 2.193 to 9.436) for 2 vs none). No differences in mortality were found among NTGH, TGH, and PH. Cardiovascular procedures were more common in PH hospitalizations and were associated with higher costs and LOS. CMCs increase costs and mortality. In-hospital mortality is increased with age, male gender, and the presence of hypoplastic left heart syndrome.

Prenatal diagnosis of functionally univentricular heart, associations and perinatal outcomes.

OBJECTIVE: Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH. METHODS: We retrospectively evaluated patients who presented between 2008 and June 2015 in our centre and in prenatal practice praenatal.de in Cologne. We included double inlet left ventricle (DILV), tricuspid valve atresia (TA), pulmonary valve atresia and intact ventricular septum (PA:IVS), unbalanced atrioventricular septal defect (AVSD), heterotaxy, hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC). RESULTS: Of initially 155 patients, 128 were liveborn (82.6%). Ten neonates (7.8%) were lost to follow-up, in three (2.5%) neonates, parents decided for compassionate care. Overall survival after prenatal diagnosis of functionally UVH was 67.1%, and 90.4% on an intention-to-treat basis. Survival after surgery reached 93.7%. The majority of deaths occurred within the group of dominant RV (10/74, 13.5%). High risk HLHS with restrictive foramen ovale was associated with the lowest survival rate (13/17, 76.5%) with significant difference compared to survival rate in dominant LV (40/41, 97.6%, p < 0.05). CONCLUSION: These results should be explained to parents to ensure informed decisions and counselling. © 2016 John Wiley & Sons, Ltd.

Influence of Morphology and Initial Surgical Strategy on Survival of Infants With Tricuspid Atresia.

BACKGROUND: Tricuspid atresia (TA) is a heterogeneous single-ventricle anomaly in which initial presentation and, consequently, timing and mode of palliation vary based on morphology and degree of pulmonary or systemic outflow obstruction. We report current era palliation outcomes and examine whether morphologic and, subsequently, surgical factors influence survival. METHODS: From 2002 to 2012, 105 infants with TA underwent surgical palliation. Competing risks analyses modeled events after first-stage surgery (Glenn versus death) and after Glenn (Fontan versus death) and examined risk factors affecting outcomes. RESULTS: Seventy-eight patients (74%) required neonatal first-stage palliation, including modified Blalock-Taussig shunt (n = 46, 44%), Norwood (n = 18, 17%), and pulmonary artery band (n = 14, 13%), whereas 27 (26%) received primary Glenn as their initial surgery. Hospital mortality was 5 patients (4.8%). Competing risks models showed that by 1 year after first-stage surgery, 15% of patients had died and 83% had undergone Glenn. By 5 years after Glenn, 2% of patients had died and 80% had undergone Fontan. Overall 8-year survival was 84%. On multivariable analysis, risk factors for mortality were genetic/extracardiac anomalies (hazard ratio 7.0, 95% confidence interval: 2.4 to 20.6, p < 0.001) and pulmonary atresia (hazard ratio 4.4, 95% confidence interval: 1.6 to 12.2, p = 0.004). Survival was not affected by initial palliation type (p = 0.36), ventriculoarterial discordance (p = 0.25), systemic outflow obstruction (p = 0.84), or arch obstruction (p = 0.62). CONCLUSIONS: Despite morphologic and physiologic variations necessitating different palliative sequences, multistage palliation outcomes of various TA subtypes are comparable and generally good, with the exception of patients with associated genetic/extracardiac anomalies. The bulk of mortality is interstage, indicating continued opportunity for improvement in monitoring and managing patients during this critical period.

Surgical management of competing pulmonary blood flow affects survival before Fontan/Kreutzer completion in patients with tricuspid atresia type I.

OBJECTIVES: To determine the association between surgical management of pulmonary blood flow (PBF) at initial and staged procedures with survival to Fontan/Kreutzer operation (Fontan) in patients with tricuspid atresia. METHODS: Infants aged <3 months with tricuspid atresia type I (n = 303) were enrolled from 34 institutions (1999-2013). Among those who underwent surgical intervention (n = 302), initial procedures were: systemic to pulmonary artery shunt (SPS; n = 189; 62%); pulmonary artery banding (PAB; n = 50; 17%); and superior cavopulmonary connection (SCPC; n = 63; 21%). Multiphase parametric-hazard models were used to analyze competing outcomes. RESULTS: Risk-adjusted 6-year survival was lower after SPS (85%; P = .04) versus PAB (93%) or SCPC (93%). Survival after SPS when the main pulmonary artery (MPA) was closed (n = 21) or banded (n = 4) was 60%, versus 93% without MPA intervention (P = .02). After SPS, survival before SCPC was lower with an open ductus arteriosus (n = 7; 76% vs 97%; P = .02). Similarly, after SPS, risk-adjusted survival was similar to that for patients who had an initial PAB or SCPC when MPA intervention was avoided and the ductus arteriosus either closed spontaneously before SPS, or was closed during SPS. For all patients reaching SCPC (n = 277), survival to Fontan was not significantly influenced by whether PBF persisted through the MPA. CONCLUSIONS: Tricuspid atresia patients with SPS represent a high-risk subgroup. Avoiding an open ductus arteriosus and concomitant MPA intervention during SPS may help mitigate the risk associated with SPS. The presence of antegrade PBF through the MPA, at initial and staged operations, did not significantly influence survival to Fontan operation.

Outcomes of adolescents and adults undergoing primary Fontan procedure.

Patients who have undergone the Fontan procedure in later adolescence and adulthood represent a unique population at risk for significant morbidity and mortality. The optimal strategy for long-term management of such patients is unknown. The aim of this study was to evaluate outcomes of patients who had undergone Fontan surgery later in life, focusing on late survivorship, mode of death, and predictors of mortality. Eighty-eight patients were identified who had their initial Fontan operation from 1973 to 2007 at ≥15 years of age. A standardized tiered contact protocol was followed to capture the recent health status of each patient; the probability of survival was 83%, 71%, and 66% at 5, 10, and 15 years of follow-up, respectively. Despite focused efforts, the modes of death were not available in 48% of the patients. A prolonged intensive care unit stay at the time of operation was the single predictor of mortality (p = 0.0123). In conclusion, this investigation highlights the significant mortality that exists in patients who undergo a Fontan procedure later in life and the difficulties in achieving standardized medical follow-up for this high-risk group of patients.

Morbilidad y mortalidad posoperatoria en niños con corazón univentricular según diagnóstico morfológico / Postoperative mortality and morbidity found in children with univentricular heart according to the morphological diagnosis

Introducción: la atresia tricuspídea es la cardiopatía congénita cianótica que motiva mayor indicación de cirugía univentricular. El tratamiento quirúrgico consiste en separar las circulaciones sistémica y pulmonar a través de una derivación cavopulmonar total (proceder de Fontan). Objetivo: comparar la evolución posoperatoria del proceder de Fontan entre enero de 1990 y diciembre de 2010 en relación con el diagnóstico morfológico. Métodos: se ordenaron en 2 grupos de estudio (AT: atresia tricuspídea y No AT: con diagnóstico diferente a atresia tricuspídea). Las variables cualitativas y cuantitativas se analizaron a través de las pruebas chi cuadrado y t de student para un nivel de significación £ 0,05. Resultados: presentaron atresia tricuspídea 31 pacientes y 61 no atresia tricuspídea. En el posoperatorio 68 pacientes presentaron complicaciones, 23 con atresia tricúspidea (74,2 por ciento) y 45 con diagnóstico diferente a atresia tricuspídea (73,7 por ciento). Treinta pacientes presentaron efusiones pleurales persistentes durante el posoperatorio, 11 (35,5 por ciento) y 19 (31,4 por ciento) para la atresia tricúspidea y con diagnóstico diferente a atresia tricuspídea respectivamente. Fallecieron 18 enfermos, sin diferencia significativa entre ambos grupos. Durante el seguimiento, la causa de morbilidad más frecuentes fue por capacidad funcional disminuida de forma moderada o grave, en 13 pacientes con atresia tricúspidea (44,8 `por ciento) y en 20 (44,4 por ciento) en el otro grupo (p= 1). Conclusiones: el proceder de Fontan es una opción quirúrgica que permite la supervivencia de los pacientes con corazón univentricular asociada a morbilidad posoperatoria a largo plazo. El diagnóstico morfológico de atresia tricuspídea no se relaciona con la morbilidad y mortalidad(AU)

Introduction: tricuspid atresia is the cyanotic congenital heart disease that more frequently involves the indication of univentricular heart surgery. The surgical treatment consists of separating the systemic circulation from the pulmonary one through total cavopulmonary shunt (Fontan procedure). Objective: to compare the evolution of Fontan procedure postoperatively from January 1990 to December 2010 with the morphological diagnosis. Methods: the children were divided into 2 study groups (Triscupid atresia and Triscupid atresia-free with diagnosis different from that of tricuspid atresia). Chi square and Student's tests helped to analyze qualitative and quantitative variables for significance level £ 0.05. Results: tricuspid atresia was observed in 31 patients and 61 tricuspid atresia-free. In the postoperative period, 68 patients suffered complications, 23 with tricuspid atresia (74.2 percent) and 45 diagnosed with a disease other than tricuspid atresia (73.7 percent). Thirty patients had persistent pleural effusions during the postoperative period, 11 (35.5 percent) and 19 (31.4) in the tricuspid atresia group and in the tricuspid atresia-free group respectively. Eighteen patients died and no significant association was found in neither of the groups. During the follow-up, the most frequent cause of morbidity was reduced functional capacity either moderate or severe in 13 patients with tricuspid atresia (44.8 percent) and in 20 children (44.4 percent)from the other group (p= 1). Conclusions: Fontan procedure is a surgical option that allows the survival of patients with univentricular heart associated to long-term postoperative morbidity. The morphological diagnosis of tricuspid atresia is not related to morbility and mortality(AU)

Morbilidad y mortalidad posoperatoria en niños con corazón univentricular según diagnóstico morfológico / Postoperative mortality and morbidity found in children with univentricular heart according to the morphological diagnosis

Introducción: la atresia tricuspídea es la cardiopatía congénita cianótica que motiva mayor indicación de cirugía univentricular. El tratamiento quirúrgico consiste en separar las circulaciones sistémica y pulmonar a través de una derivación cavopulmonar total (proceder de Fontan). Objetivo: comparar la evolución posoperatoria del proceder de Fontan entre enero de 1990 y diciembre de 2010 en relación con el diagnóstico morfológico. Métodos: se ordenaron en 2 grupos de estudio (AT: atresia tricuspídea y No AT: con diagnóstico diferente a atresia tricuspídea). Las variables cualitativas y cuantitativas se analizaron a través de las pruebas chi cuadrado y t de student para un nivel de significación £ 0,05. Resultados: presentaron atresia tricuspídea 31 pacientes y 61 no atresia tricuspídea. En el posoperatorio 68 pacientes presentaron complicaciones, 23 con atresia tricúspidea (74,2 por ciento) y 45 con diagnóstico diferente a atresia tricuspídea (73,7 por ciento). Treinta pacientes presentaron efusiones pleurales persistentes durante el posoperatorio, 11 (35,5 por ciento) y 19 (31,4 por ciento) para la atresia tricúspidea y con diagnóstico diferente a atresia tricuspídea respectivamente. Fallecieron 18 enfermos, sin diferencia significativa entre ambos grupos. Durante el seguimiento, la causa de morbilidad más frecuentes fue por capacidad funcional disminuida de forma moderada o grave, en 13 pacientes con atresia tricúspidea (44,8 `por ciento) y en 20 (44,4 por ciento) en el otro grupo (p= 1). Conclusiones: el proceder de Fontan es una opción quirúrgica que permite la supervivencia de los pacientes con corazón univentricular asociada a morbilidad posoperatoria a largo plazo. El diagnóstico morfológico de atresia tricuspídea no se relaciona con la morbilidad y mortalidad

Introduction: tricuspid atresia is the cyanotic congenital heart disease that more frequently involves the indication of univentricular heart surgery. The surgical treatment consists of separating the systemic circulation from the pulmonary one through total cavopulmonary shunt (Fontan procedure). Objective: to compare the evolution of Fontan procedure postoperatively from January 1990 to December 2010 with the morphological diagnosis. Methods: the children were divided into 2 study groups (Triscupid atresia and Triscupid atresia-free with diagnosis different from that of tricuspid atresia). Chi square and Student's tests helped to analyze qualitative and quantitative variables for significance level £ 0.05. Results: tricuspid atresia was observed in 31 patients and 61 tricuspid atresia-free. In the postoperative period, 68 patients suffered complications, 23 with tricuspid atresia (74.2 percent) and 45 diagnosed with a disease other than tricuspid atresia (73.7 percent). Thirty patients had persistent pleural effusions during the postoperative period, 11 (35.5 percent) and 19 (31.4) in the tricuspid atresia group and in the tricuspid atresia-free group respectively. Eighteen patients died and no significant association was found in neither of the groups. During the follow-up, the most frequent cause of morbidity was reduced functional capacity either moderate or severe in 13 patients with tricuspid atresia (44.8 percent) and in 20 children (44.4 percent)from the other group (p= 1). Conclusions: Fontan procedure is a surgical option that allows the survival of patients with univentricular heart associated to long-term postoperative morbidity. The morphological diagnosis of tricuspid atresia is not related to morbility and mortality

Univentricular hearts in Denmark 1977 to 2009: incidence and survival.

BACKGROUND: The incidence of children born with functional univentricular heart (UVH) and their prognosis presumably changed substantially in recent years. This is due to introduction of fetal echocardiography and potential termination of pregnancy (TOP) when UVH is diagnosed (UVH TOP), and to improvements in treatment. We aimed to explore changes in incidence, to estimate changes in survival, and to describe predictors of mortality in UVH patients. METHODS: Using a population-based design we identified all UVH cases in Denmark from 1977 to 2009. RESULTS: 703 UVH live births and 106 UVH TOP were identified. A dramatic decrease in birth incidence of UVH patients and a corresponding increase in UVH TOP was observed in recent years. Mean incidence rate of UVH (live births and UVH TOP) was 0.39 per 1000 births. In adjusted analysis survival improved significantly from birth era 1977-1989 to 1990-1999 (HR 2.65, 95% confidence interval (CI), 2.06-3.42) but not significantly from 1990-1999 to 2000-2009 (HR 0.77, 95% CI, 0.57-1.05). In the birth era 2000-2009, the lowest five-year survival was seen with hypoplastic left heart syndrome (HLHS) (18.8%), whereas the best survival was seen with tricuspid atresia (79.8%). Adjusted risk of death was 7.3 times higher in the HLHS group compared to the tricuspid atresia group (95% CI, 3.94-13.47). CONCLUSIONS: This study demonstrates a dramatic decrease in birth incidence of UVH patients most probably due to a corresponding increase in UVH TOP. Despite survival improved after introduction of Fontan surgery, survival has not improved significantly during the last 20years.

Fontan conversion with concomitant arrhythmia surgery for the failing atriopulmonary connections: mid-term results from a single centre.

OBJECTIVES: Classical Atriopulmonary Fontan connections tend to fail in the long term due to progressive anastomotic site obstruction, right atrial enlargement, and refractory atrial arrhythmias. Conversion to total cavopulmonary connection with concomitant arrhythmia surgery is a promising treatment but optimal timing of the procedure remains controversial. METHODS: Between the years 2002 and 2009, 15 patients with a median age of 26.2 (12-43) years underwent Fontan conversion operation with concomitant arrhythmia surgery. All were symptomatic and 14 out of the 15 patients had refractory arrhythmias. The duration of pre-operative arrhythmia and the outcome of surgery were correlated to study the impact of delay in surgical intervention on post-operative survival and arrhythmia control. RESULTS: There were two patients who died in the early post-operative period (13.3%). At the mid-term follow-up, 53 (20-86) months, late atrial arrhythmias had recurred in two of the 13 surviving patients (15.30%) and one patient developed late sinus node dysfunction. The need for anti-arrhythmic drugs decreased considerably from 93.5% to 15.3% on mid-term follow-up. There was no late death or need for cardiac transplantation. The duration of arrhythmia before surgery was prolonged for more than 10 years in patients who died as well as in those who had complications like late recurrence of arrhythmias, dependence on anti-arrhythmic medications, and worsening of ventricular dysfunction. CONCLUSIONS: Fontan conversion is a well-established treatment option for salvaging the failing atriopulmonary connections. Concomitant arrhythmia surgery effectively resolves the refractory atrial arrhythmias and improves survival, but we need to optimise the timing of Fontan conversion to improve the long-term outcome.

Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome.

OBJECTIVE: To evaluate the intrauterine course and outcome of tricuspid atresia detected in the fetus. METHODS: This was a retrospective review of all confirmed cases of tricuspid atresia detected prenatally between 1998 and 2006 in three tertiary referral centers in Germany. RESULTS: Fifty-four cases of tricuspid atresia were detected prenatally during the study period and confirmed postnatally: 28 (51.9%) cases had a concordant ventriculoarterial connection of which 14 also had pulmonary outflow obstruction; 25 (46.3%) cases had a discordant ventriculoarterial connection of which 14 also had aortic outflow obstruction, six had pulmonary outflow tract obstruction and two had other associated intracardiac anomalies; and one (1.9%) had a common arterial trunk. The peak velocity index for veins in the ductus venosus was significantly elevated in 19 of the 37 (51.4%) cases assessed; however, this finding did not correlate with adverse intrauterine outcome. There were associated extracardiac anomalies in 12 cases: five with chromosomal anomalies, two with VACTERL association, one with unilateral renal agenesis, one with hypospadia, one with hydrothorax, one with megacystis and one with agenesis of the ductus venosus. Seventeen of the 54 (31.5%) cases underwent termination of pregnancy, two (3.7%) died in utero, two (3.7%) died in infancy and 33 (61.1%) children survived with a median follow-up of 26 (range, 12-120) months. Prenatal echocardiography correctly anticipated the postnatal course and the need for neonatal intervention in 29/35 (82.9%) continued pregnancies; in the remaining six (17.1%) cases the right outflow tract obstruction had been underestimated. CONCLUSIONS: Tricuspid atresia and the frequently associated intracardiac anomalies can be diagnosed in the fetus with considerable accuracy. A thorough search for extracardiac malformations should be performed in order to rule out chromosomal anomalies and multiple malformation syndromes. Elevated pulsatility in the ductus venosus does not indicate cardiac failure. The short-term overall survival in continued pregnancies in our study exceeded 89%, with the greatest rate of loss being in the first year of postnatal life.

Survival and prognostic factors in patients with an absent atrioventricular connection.

INTRODUCTION AND OBJECTIVES: To identify anatomical and functional characteristics associated with survival in adult patients with an absent atrioventricular connection and to highlight the diagnostic importance of echocardiography. METHODS: The clinical histories and echocardiographic and hemodynamic test results of 24 patients were recorded. RESULTS: Some 87.5% of patients were in New York Heart Association (NYHA) functional class I/II. In 92%, the ECG demonstrated sinus rhythm and left ventricular dilatation. Chest X-ray showed grade-II cardiomegaly in 83%. Situs solitus and an absent right atrioventricular connection were found on echocardiography in 92%. The ventriculoarterial connection was most frequently concordant (in 71%). All patients had an atrial septal defect, 21 had a ventricular septal defect and 21 had decreased pulmonary flow. The ejection fraction of the main ventricle in the whole patient group was 55% +/- 10%; 52% +/- 12% in those who did not undergo surgery and 58% +/- 8% in those who did (P=NS). Factors associated with poor survival were an ostium secundum atrial septal defect, hemoglobin <16 g/dL and a main ventricle ejection fraction <50%. Of the 54% of patients who underwent surgery, 85% are alive and the majority are in NYHA functional class I/II. Among those who did not, 82% are alive and 73% are in NYHA functional class I/II. CONCLUSIONS: The presence of a wide atrial septal defect, a normal hemoglobin level and a normal main ventricle ejection fraction were associated with the survival of these patients into adulthood. Echocardiography can provide clinicians and surgeons with information that is valuable for selecting treatment and monitoring follow-up.

Impact of age at Fontan completion on postoperative hemodynamics and long-term aerobic exercise capacity in patients with dominant left ventricle.

BACKGROUND: Although the Fontan procedure is now being applied to younger patients, the influence of patients' age at the time of surgery on long-term results remains unclear. We investigated whether age at Fontan completion affects subsequent hemodynamics and exercise capacity in patients with a dominant left ventricle followed up for more than 5 years. METHODS: Between January 1990 and December 2001, 68 patients with a dominant left ventricle underwent total cavopulmonary connection (TCPC) at our institute. The survivors routinely underwent postoperative exercise tests and catheterization at 1 year and then every 5 years after TCPC, and were divided into group A (< 3 years; n = 32) and group B (> or = 3 years; n = 33), according to age at time of the Fontan procedure and retrospectively reviewed. We also examined the correlation between age at Fontan and data from postoperative catheterization or exercise tests. RESULTS: None of the patients died during a follow-up period of 91.2 +/- 46.7 months. The postoperative exercise capacity (peak oxygen consumption; % of normal [61.3 +/- 11.5 versus 51.9 +/- 9.1, p = 0.0001]), cardiac index at 5 years (3.31 +/- 0.62 versus 2.86 +/- 0.69, p = 0.0133) and at 10 years after TCPC (3.24 +/- 0.46 versus 2.61 +/- 0.65, p = 0.0194), and ventricular ejection fraction (%) at 10 years after TCPC (62.1 +/- 7.6 versus 53.0 +/- 8.0, p = 0.0131) were significantly higher in group A. Age at Fontan completion inversely correlated with postoperative peak oxygen consumption (R = -0.374, p = 0.0006) and cardiac index at 5 years (R = -0.452, p = 0.0003) and 10 years after TCPC (R = -0.491, p = 0.0072). CONCLUSIONS: Earlier TCPC is beneficial in the long term from the viewpoint of exercise capacity and hemodynamics among patients with a dominant left ventricle.

Matching procedure to morphology improves outcomes in neonates with tricuspid atresia.

OBJECTIVE: This study was undertaken to characterize morphologic substrate of tricuspid atresia with ventriculoarterial concordance and discriminate management strategies that lead to successful definitive repair. METHODS: From 1999 to 2004, a total of 150 babies with type I tricuspid atresia were enrolled from first diagnosis at 26 institutions. Antegrade pulmonary blood flow was absent in 19%, restricted in 54%, and unrestricted in 28%. Competing-risk methodology determined the time-related prevalence and risk factors for death versus cavopulmonary anastomosis and subsequent death versus Fontan completion. RESULTS: Overall 5-year survival was 86%. Initial palliation included systemic-pulmonary arterial shunt in 64%, pulmonary artery banding in 11%, and cavopulmonary anastomosis in 24%. Median age at cavopulmonary anastomosis was 6 months, with 83% undergoing bidirectional Glenn shunt and 17% undergoing hemi-Fontan procedure. By the age of 2 years, 89% had cavopulmonary anastomosis, 6% were dead, and 4% remained alive without cavopulmonary anastomosis. Risk factors for death without cavopulmonary anastomosis included presence of mitral regurgitation (P = .03) and palliation with systemic-pulmonary arterial shunts not originating from the innominate artery (P = .04). Factors associated with decreased transition rate to cavopulmonary connection included patient variables (younger admission age to a participating institution, noncardiac anomalies) and procedural variables (larger systemic-pulmonary arterial shunt diameter, previous palliation). Of patients undergoing cavopulmonary anastomosis, 75% had undergone a Fontan operation within 3 years. CONCLUSION: Smaller shunt size and decreased pulmonary blood flow decrease mortality after initial palliation and increase the rate of successful transition to cavopulmonary anastomosis. Outcomes can be improved by placing smaller shunts from the innominate artery, especially in patients with any mitral regurgitation.

Long-term outcome and quality of life in adult patients after the Fontan operation.

The first successful Fontan operation was performed in 1971, and this first cohort of Fontan patients is reaching adulthood with unclear outcome of this palliative procedure. We studied the mortality, morbidity, and quality of life in our adult Fontan patients. We examined all patients (n = 36) who underwent a Fontan procedure and were being seen in an adult outpatient clinic by using electrocardiography, exercise testing, and echocardiography. Quality of life was assessed by the Short Form 36 questionnaire. The mean follow-up period was 15 years (range 0 to 23). Of the initial 36 patients, 10 died (28%) at a mean of 10 years (range 0 to 21) after the Fontan operation and 1 patient underwent cardiac transplantation. Reoperations were performed in 21 patients (58%), and the most common reason was revision of the Fontan connection. Sustained supraventricular tachycardia was observed in 20 patients (56%) with an increased incidence of arrhythmias with longer follow-up. Thromboembolic events were detected in 9 patients (25%), 5 of whom had adequate anticoagulant levels at the time of event. The thromboembolic event was fatal for 3 patients. A total of 195 hospital admissions (mean 3.8 +/- 2.7, range 1 to 13) was recorded. Quality-of-life assessment showed physical functioning, mental health, and general health perception to be significantly lower for Fontan patients than for the normal Dutch population. Thus, we found high mortality and very high morbidity in adult patients after the Fontan operation. In particular, reoperations, arrhythmias, and thromboembolic events compromised quality of life.