RESUMO
OBJECTIVE: A high risk of morbidity and mortality is well documented in adults with a Fontan circulation. The difference in outcomes between those with and without significant morbidity at the time of transition to adult care has not been well characterised. METHODS: We analysed clinical outcomes in patients enrolled in the Australian and New Zealand Fontan Registry ≥16 years of age. Low risk (LR) Fontan patients were defined as those without history of sustained arrhythmia, thromboembolic event, transplantation, Fontan conversion, protein-losing enteropathy, plastic bronchitis, New York Heart Association class III/IV and/or moderate/severe atrioventricular valve regurgitation or ventricular dysfunction. Increased risk (IR) patients had one or more risk factor. RESULTS: Inclusion criteria were met in 822 patients; mean age 26±8 years, median follow-up from age 16 was 9 years, 203 had atriopulmonary connection (APC) and 619 had total cavopulmonary connection (TCPC). Survival at 30 years was higher in the LR versus IR; 94% versus 82% (p=0.005), 89% versus 77% (p=0.07) for APC and 96% versus 89% (p=0.05) for TCPC. LR patients experienced less Fontan failure (HR 0.34, 95% CI 0.23 to 0.49, p<0.001) and ventricular dysfunction (HR 0.46, 95% CI 0.29 to 0.71, p=0.001) compared with IR patients. For LR TCPC patients, modelled survival projections at 60 years were 49%-67%. CONCLUSIONS: Clinical outcomes for adolescents LR at transition to adult care are markedly superior to those who have established risk factors for Fontan failure, which is an important consideration when formulating individualised long-term risk estimates and counselling patients.
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OBJECTIVES: The mechanisms of attrition of the Fontan population have been poorly characterised and it is unclear whether some of the deaths are potentially preventable. We analysed the circumstances of late death in patients with a Fontan circulation, with a special focus on identifying lesions amenable to intervention that may have contributed to the decline of their circulation. METHODS: Between 1975 and 2018, a total of 105 patients from a Bi-National Registry died beyond 1 year after Fontan completion, at a median age of 18.6 (IQR 13.8-26.0) years old, 12.7 (IQR 6.0-19.3) years after Fontan completion. RESULTS: A total of 105 patients died-63 patients (60%) with an atriopulmonary (AP) Fontan, 21 patients (20%) with a lateral tunnel (LT) and 21 patients (20%) with an extracardiac conduit (ECC). 72 patients (69%) were reviewed within 2 years preceding death, with 32% (23/72) deemed to be clinically well. Fontan circulatory failure was the most common cause of death in 42 patients (45%). Other causes of death included sudden death/arrhythmia (19%), perioperative death (12%), neurological complication (7%) and thromboembolism (7%). All patients with an LT or ECC who died from Fontan failure had at least one surgical defect that was amenable to intervention at time of death. CONCLUSIONS: Conventional clinical surveillance has been insensitive in detecting a significant proportion of patients at risk of late death. Fontan circulatory failure contributes to half of the late deaths. Patients with an LT or ECC Fontan who died with a clinical picture of circulation failure may have potentially correctable lesions.
Assuntos
Técnica de Fontan/mortalidade , Cardiopatias Congênitas/cirurgia , Complicações Pós-Operatórias/mortalidade , Adolescente , Austrália/epidemiologia , Causas de Morte , Feminino , Técnica de Fontan/efeitos adversos , Nível de Saúde , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Nova Zelândia/epidemiologia , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/cirurgia , Sistema de Registros , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: Patients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients. METHODS: A retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival. RESULTS: Of the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%. CONCLUSIONS: A substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention.
Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Efeitos Adversos de Longa Duração , Adulto , Austrália/epidemiologia , Efeitos Psicossociais da Doença , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/métodos , Técnica de Fontan/estatística & dados numéricos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Humanos , Efeitos Adversos de Longa Duração/epidemiologia , Efeitos Adversos de Longa Duração/cirurgia , Estudos Longitudinais , Masculino , Mortalidade , Avaliação das Necessidades , Nova Zelândia/epidemiologia , Marca-Passo Artificial/estatística & dados numéricos , Sistema de Registros/estatística & dados numéricos , Reoperação/estatística & dados numéricosRESUMO
BACKGROUND: The need for permanent pacing has been identified as a predictor of poor outcomes in the late survivors of Fontan surgery. However, it is not clear if the need for a pacemaker is a surrogate marker of a declining Fontan state, or if pacing is deleterious to the Fontan circulation. OBJECTIVES: We sought to compare the long-term outcomes of propensity-matched Fontan patients with and without a permanent pacemaker. METHODS: Patients who have survived Fontan completion with a documented history of cardiac arrhythmia were identified from the Australia and New Zealand Fontan Registry. Pacemaker insertion details, cardiac function and electrophysiological data were obtained for the patients with a permanent pacemaker. Survival analysis was performed with propensity score matching to compare late survival and outcomes in patients with versus without a pacemaker. RESULTS: There was a total of 310 patients with a history of cardiac arrhythmia, of which 126 (41%) had a permanent pacemaker. After propensity-score matching, 99 pairs were generated (nâ¯=â¯198). Patients with a permanent pacemaker had a higher risk of death (HR 3.32 95% CI 1.60-6.90, pâ¯=â¯0.001) and death or transplantation (HR 3.55 95% CI 1.87-6.73, pâ¯<â¯0.001). Patients who were only paced atrially were not at a significantly increased risk of death or transplantation. However, patients who were ventricular paced >50% of the time were much more likely to encounter late death or transplantation (HR 3.82 95% CI 1.64-8.95, pâ¯=â¯0.002). CONCLUSIONS: Having a permanent pacemaker and needing ventricular pacing is likely associated with an increased risk of death and transplantation in patients with a Fontan circulation.
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Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/cirurgia , Técnica de Fontan/mortalidade , Transplante de Coração/mortalidade , Marca-Passo Artificial/efeitos adversos , Adolescente , Austrália/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Seguimentos , Técnica de Fontan/tendências , Transplante de Coração/tendências , Humanos , Masculino , Mortalidade/tendências , Nova Zelândia/epidemiologia , Marca-Passo Artificial/tendências , Sistema de Registros , Fatores de RiscoRESUMO
BACKGROUND: Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. METHODS: A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99m DTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR). RESULTS: Mean age and time since Fontan were 19.8⯱â¯9.3 and 14.1⯱â¯7.6â¯years, respectively. Features suggestive of hepatic fibrosis were observed on ultrasound in 87/143 (61%) and no patient was diagnosed with hepatocellular carcinoma. FibroScan median kPa was ≥10 in 117/133 (88%), ≥15 in 75/133 (56%), and ≥20 in 41/133 (31%). Fifty-four patients (54/118, 46%) had a FibroTest score ≥0.49 (equivalent to ≥F2 fibrosis). FibroTest score correlated with FibroScan value (râ¯=â¯0.24, pâ¯=â¯0.015) and ACR (râ¯=â¯0.29, pâ¯=â¯0.002), and patients with ultrasound features of hepatic fibrosis had a higher FibroScan median kPa (19.5 vs 15.4, pâ¯=â¯0.002). Renal impairment was mild (mGFR 60-89â¯ml/min/1.73â¯m2) in 46/131 (35%) and moderate (mGFR 30-59â¯ml/min/1.73â¯m2) in 3/131 (2%). Microalbuminuria was detected in 52/139 participants (37%). By multivariable analysis, time since Fontan was associated with increased FibroScan median kPa (ßâ¯=â¯0.89, 95% CI 0.54-1.25, pâ¯=â¯0.002) and decreased mGFR (ßâ¯=â¯-0.77, 95% CI -1.29-0.24, pâ¯=â¯0.005). CONCLUSIONS: In the second decade after Fontan hepatic and renal structure and function are abnormal in a significant number of patients: close to 60% have ultrasonographic evidence of structural hepatic abnormalities, 46% have elevated serum hepatic fibrosis scores, and 57% have either reduced glomerular filtration rate or microalbuminuria. Hepatic and renal function should be monitored for potential impacts on outcomes after Fontan completion.
Assuntos
Técnica de Fontan/tendências , Rim/diagnóstico por imagem , Fígado/diagnóstico por imagem , Sistema de Registros , Relatório de Pesquisa , Adolescente , Adulto , Austrália/epidemiologia , Estudos Transversais , Ecocardiografia/tendências , Feminino , Técnica de Fontan/efeitos adversos , Taxa de Filtração Glomerular/fisiologia , Humanos , Rim/fisiologia , Fígado/fisiologia , Masculino , Nova Zelândia/epidemiologia , Adulto JovemRESUMO
OBJECTIVES: We aimed to compare the long-term performance of pulmonary homografts and stented bioprosthetic valves in the pulmonary position in patients aged 10-20 years. METHODS: Between January 1995 and December 2015, 188 patients aged 10-20 years undergoing pulmonary valve replacement were identified retrospectively from hospital databases in both congenital cardiac centres in Brisbane. Valve performance was evaluated using previously described standard criteria. Propensity score matching was used to balance the 2 treatment groups. RESULTS: Freedom from structural valve degeneration in homografts (n = 131) was 97%, 92% and 85% at 3, 5 and 10 years, respectively, and 91% and 53% at 3 and 5 years, respectively, in the bioprosthesis group (n = 57). Freedom from reintervention in homografts was 96%, 93% and 88% at 3, 5 and 10 years, respectively, and 93% and 68% at 3 and 5 years, respectively, in the bioprosthesis group. The unadjusted Cox regression analysis demonstrated that a bioprosthesis was at 5.64 times the risk of structural valve degeneration and 3.89 times the risk of reintervention. The Cox regression analysis performed on the propensity matched sample (45 pairs of patients) revealed that a bioprosthesis was at almost 10 times the risk of experiencing structural valve degeneration [hazard ratio (HR) = 9.18] and at more than 8 times the risk of undergoing a reintervention (HR = 8.34). CONCLUSIONS: In our patient population, pulmonary homografts outperformed stented bioprosthetic valves within 5 years when implanted in the pulmonary position in patients aged 10-20 years. We recommend the use of a pulmonary homograft for pulmonary valve replacement in this age group in patients undergoing surgery for congenital heart disease.
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Bioprótese , Próteses Valvulares Cardíacas , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Adolescente , Criança , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/métodos , Xenoenxertos , Humanos , Estimativa de Kaplan-Meier , Complicações Pós-Operatórias , Falha de Prótese , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Stents , Adulto JovemRESUMO
BACKGROUND: Long-term outcomes of Fontan patients who survive to age ≥16 years have not been well characterized. The Australian and New Zealand Fontan Registry (ANZFR) provides a unique opportunity to understand survival and complication rates in Fontan patients who transition to adult congenital heart disease centers. OBJECTIVES: This study sought to describe the survival and complications of adult patients who have had a Fontan procedure. METHODS: The study analyzed outcomes in patients ≥16 years of age who were prospectively enrolled in the ANZFR. RESULTS: Data from all 683 adult survivors from the ANZFR were analyzed. Mortality status was confirmed from the National Death Index. There were 201 atriopulmonary (AP) connections and 482 total cavopulmonary connections (249 lateral tunnels and 233 extracardiac conduits). For these subjects, the survival rate at age 30 years was 90% (95% CI: 87% to 93%), and it was 80% (95% CI: 75% to 87%) at 40 years of age. Survival at age 30 years was significantly worse for the patients with AP connections (p = 0.03). At latest follow-up, only 53% of patients were in New York Heart Association functional class I. After the age of 16 years, 136 (20%) had experienced at least 1 new arrhythmia, 42 (6%) required a permanent pacemaker, 45 (7%) had a thromboembolic event, and 135 (21%) required a surgical reintervention. Only 41% (95% CI: 33% to 51%) of Fontan patients were free of serious adverse events at 40 years of age. CONCLUSIONS: This comprehensively followed cohort showed that a variety of morbid complications is common in Fontan adults, and that there is a substantial incidence of premature death, particularly in patients with AP connections.
Assuntos
Técnica de Fontan/tendências , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Complicações Pós-Operatórias/mortalidade , Adolescente , Adulto , Idoso , Austrália/epidemiologia , Estudos de Coortes , Feminino , Seguimentos , Técnica de Fontan/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Nova Zelândia/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos , Sistema de Registros , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVES: The specific outcomes of patients with pulmonary atresia with intact ventricular septum late after Fontan palliation are unknown. Patients with smaller right ventricles and myocardial sinusoids are known to have worse survival in the first years of life. Whether the potential for coronary ischaemia affects the long-term outcomes of these patients after Fontan palliation is still unknown. METHODS: All patients with pulmonary atresia with intact ventricular septum who underwent the Fontan procedure from 1984 to 2016 in Australia and New Zealand were identified, and preoperative, perioperative and follow-up data were collected. RESULTS: Late follow-up data were available for 120 patients. The median length of follow-up after the Fontan procedure was 9.1 years (interquartile range 4.2-15.4 years). Late death occurred in 9% of patients (11/120). Six were sudden, unexpected deaths; 4 of those occurred in patients known to have right ventricle-dependent coronary circulation (RVDCC). Those with RVDCC had a higher incidence of sudden death (4/20 vs 2/100; P = 0.007). RVDCC was associated with late death (P = 0.01) and the development of myocardial ischaemia after Fontan completion (P < 0.001). The 10-year survival rate was 77% (95% confidence interval 56-100%) for patients with RVDCC vs 96% (95% confidence interval 92-100%) for patients without RVDCC. CONCLUSIONS: Long-term survival of patients with pulmonary atresia with intact ventricular septum after the Fontan procedure is excellent, but patients with RVDCC remain susceptible to coronary ischaemia and sudden death. Closer surveillance and investigation for exercise-induced ischaemia may be necessary.
Assuntos
Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Isquemia Miocárdica/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Atresia Pulmonar/cirurgia , Adolescente , Criança , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Técnica de Fontan/estatística & dados numéricos , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: Partial atrioventricular septal defect (pAVSD) is usually repaired between 2 and 4 years of age with excellent results. Repair during infancy has been associated with poorer outcomes. However, most infants in reported series had heart failure or significant left atrioventricular valve (LAVV) regurgitation. The impact of surgery during infancy on outcomes remains unclear. METHODS: All children at three institutions who underwent repair of pAVSD from 1975 to 2015 were included. Infants (aged <1 year) were compared with older children in a propensity score matched analysis. Variables used to generate propensity scores were: failure to thrive, congestive heart failure, preoperative LAVV regurgitation, associated congenital heart disease, sex and the presence of trisomy 21. RESULTS: pAVSD repair was performed on 430 children, 17.4% (75/430) were infants. Infants (mean age 0.5±0.3 years) had higher rates of LAVV regurgitation, heart failure and additional cardiac malformations than older children (mean age 4.7±3.5 years). At 30 years, survival for infants was 82.1% (95% CI 70.1% to 89.6%) compared with 95.7% (95% CI 91.3% to 97.9%) in older children (P<0.001).Propensity score matching yielded 52 well-matched pairs. Survival at 30 years was 87.9% (95% CI 75.0% to 94.4%) for infants compared with 98.1% (95% CI 87.1% to 99.7%) for older children (P=0.04). There was no significant difference in freedom from reoperation between the groups. CONCLUSIONS: Despite matching for risk factors, survival after repair of pAVSD during infancy is lower than that when repair is performed in older children, with no difference in reoperation rates. This suggests that elective repair of pAVSD should be deferred until after infancy.
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Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Comunicação Interventricular/cirurgia , Complicações Pós-Operatórias/etiologia , Fatores Etários , Procedimentos Cirúrgicos Cardíacos/mortalidade , Criança , Pré-Escolar , Feminino , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/mortalidade , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Intervalo Livre de Progressão , Pontuação de Propensão , Queensland , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , VitóriaRESUMO
The objective of this study was to characterize late outcomes of the lateral tunnel (LT) Fontan procedure. The outcomes of all patients who underwent an LT Fontan procedure in Australia and in New Zealand were analyzed. Original files were reviewed and outcomes data were obtained through a binational registry. Between 1980 and 2014, a total of 301 patients underwent an LT Fontan procedure across 6 major centers. There were 13 hospital mortalities, 21 late deaths, 8 Fontan conversions and revisions, 8 Fontan takedowns, and 4 heart transplantations. Overall survival at 15 and 25 years was 90% (95% confidence interval [CI]: 86%-93%) and 80% (95% CI: 69%-91%), respectively. Protein-losing enteropathy or plastic bronchitis was observed in 14 patients (5%). Freedom from late failure at 15 and 25 years was 88% (95% CI: 84%-92%) and 82% (95% CI: 76%-87%), respectively. Independent predictors of late Fontan failure were prolonged pleural effusions post Fontan operations (hazard ratio [HR] 3.06, 1.05-8.95, P = 0.041), age >7 years at Fontan (vs 3-5 years, HR 9.7, 2.46-38.21, P = 0.001) and development of supraventricular tachycardia (HR 4.67, 2.07-10.58, P < 0.001). Freedom from tachy- or bradyarrhythmias at 10 and 20 years was 87% (95% CI: 83%-91%) and 72% (95% CI: 66%-79%), respectively. Thromboembolic events occurred in 45 patients (16%, 26 strokes), and freedom from symptomatic thromboembolism at 10 and 20 years was 93% (95% CI: 89%-96%) and 80% (95% CI: 74%-86%), respectively. Over a 25-year period, the LT technique has achieved excellent late survival. As this population ages, it is at an increasing risk of failure and adverse events. We are likely to see an increasing proportion requiring heart transplantation and late reintervention.
Assuntos
Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Austrália , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Cardiopatias Congênitas/mortalidade , Transplante de Coração , Ventrículos do Coração/anormalidades , Mortalidade Hospitalar , Humanos , Masculino , Nova Zelândia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Sistema de Registros , Reoperação , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Falha de TratamentoRESUMO
OBJECTIVE: Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT). METHODS: From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand. Demographic, medical and outcome data were collected and analysed prospectively and retrospectively. RESULTS: The patients with ES were predominantly female (60%), aged 31 (SD 12) years. At diagnosis of ES, 64% were WHO functional class ≥3. The most common underlying lesion was ventricular septal defect (33%) with 21% having 'complex' anatomy. Over a median follow-up time of 9.1 years, the majority (72%) had been prescribed at least one AT (49% single agent), mostly bosentan (66%, 168 patients). The mean time on AT was 6 (SD 3.6) years. Those on AT were more functionally impaired at presentation (69% WHO ≥3 vs 51%, p=0.007) and more likely to have been prescribed anticoagulation (47% vs 27%, p=0.003). The risk of death/transplant was 4.8 %/year in AT exposed versus 8.4% in those never exposed. On multivariable analysis, exposure to AT was independently associated with greater survival (survival HR 2.27, 95% CI 1.49 to 3.45; p<0.001). WHO ≥3 at presentation was associated with a worse prognosis (mortality HR 1.82, 95% CI 1.19 to 2.78; p=0.006). CONCLUSION: Treatment with AT was independently associated with greater survival in patients with ES, even though they were comparatively sicker prior to treatment.
RESUMO
We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.7%), tricuspid atresia (19.7%), and double inlet left ventricle (17.1%). The mean hospital cost for a Fontan patient from birth to 18 years of age was estimated to be $390,601 (95% confidence interval [CI] $264,703 to $516,499), corresponding to 164 (95% CI 98 to 231) inpatient days. The cost incurred from birth through to Fontan completion (the staged procedures period) was $219,482 (95% CI $202,410 to $236,553) and the cost thereafter over 15 years was $146,820 (95% CI $44,409 to $249,231), corresponding to 82 (95% CI 72 to 92) and 65 (95% CI 18 to 112) inpatient days, respectively. Costs were higher in male and hypoplastic left heart syndrome patients in the staged procedures period (p <0.001). Having fenestration was associated with higher costs in the staged procedures period (p <0.001) and lower cost after Fontan over 15 years (p = 0.66). In conclusion, patients with single ventricle congenital heart disease continue to demand considerable inpatient resources after the staged procedures period. Over 40% of the pediatric hospital costs for Fontan patients were estimated to occur after the last planned surgery.
Assuntos
Técnica de Fontan/economia , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Custos Hospitalares , Pacientes Internados , Sistema de Registros , Adolescente , Austrália/epidemiologia , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/economia , Cardiopatias Congênitas/mortalidade , Ventrículos do Coração/cirurgia , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Taxa de Sobrevida/tendênciasRESUMO
BACKGROUND: Patients undergoing palliative surgeries for single-ventricle conditions are affected by multiple comorbidities or non-cardiac conditions. The prevalence, costs and the cost implications of these conditions have not been assessed. METHODS: Administrative costing records from four hospitals in Australia and New Zealand were linked with the Fontan registry database to analyze the inpatient resource use for co-morbid or non-cardiac conditions. Inpatient costing records from the birth year through to Fontan completion were available for 156 patients. The most frequent primary diagnoses were hypoplastic left heart syndrome (33%), double inlet left ventricle (13%), and tricuspid atresia (12%). RESULTS: During the staged surgical treatment period, children had a mean of 10±6 inpatient admissions and spent 85±64days in hospital. Among these admissions, 3±5 were for non-cardiac conditions, totaling 21±41 inpatient days. Whilst cardiac surgeries were the major reason for resource use (77% of the total cost), other cardiac care that is not surgical contributed 5% and non-cardiac admissions 18% of the total cost. The three most prevalent non-cardiac diagnostic admission categories were 'Respiratory system', 'Digestive system', and 'Ear, nose, mouth and throat', affecting 28%, 21% and 34% of the patients respectively. Multivariate regression estimated that admissions for each of these categories resulted in an increased cost of $34,563 (P=0.08), $52,438 (P=0.05) and $10,525 (P=0.53) per patient respectively for the staged surgical treatment period. CONCLUSIONS: Non-cardiac admissions for single-ventricle patients are common and have substantial resource implications. Further research assessing the causes of admission and extent to which admissions are preventable is warranted.
Assuntos
Técnica de Fontan/economia , Custos Hospitalares , Hospitalização/economia , Síndrome do Coração Esquerdo Hipoplásico/economia , Atresia Tricúspide/economia , Austrália/epidemiologia , Criança , Pré-Escolar , Comorbidade , Bases de Dados Factuais/tendências , Feminino , Técnica de Fontan/tendências , Custos Hospitalares/tendências , Hospitalização/tendências , Humanos , Síndrome do Coração Esquerdo Hipoplásico/epidemiologia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Masculino , Nova Zelândia/epidemiologia , Sistema de Registros , Atresia Tricúspide/epidemiologia , Atresia Tricúspide/cirurgiaRESUMO
OBJECTIVES: To determine the risk of atrioventricular valve failure (valve intervention or moderate or greater regurgitation) during the lifetime of patients with single ventricle physiology and common atrioventricular valve. METHODS: Patients' data were extracted from an existing bi-national, population based registry. A retrospective review of their medical records was undertaken to determine the incidence of atrioventricular valve repair/replacement or moderate or greater regurgitation. RESULTS: From a registry of 1468 Fontan survivors, 136 patients with common atrioventricular valve were identified. Complete echocardiographic follow-up was available for 114 patients. Median length of follow-up was 10.2 years (interquartile range 5-15 years). Twenty-five year survival and freedom from Fontan failure were 94% [95% confidence interval (CI), 88-100%] and 74% (95% CI, 64-87%), respectively. Twenty-eight patients underwent 24 initial repairs and 4 replacements. The 24 patients undergoing repair subsequently needed 6 re-repairs, 2 replacements and 8 had moderate or greater regurgitation at last follow-up. Four-year freedom from atrioventricular valve repair failure was 50% (95% CI, 34-75%). An additional 30 patients developed moderate or greater atrioventricular valve regurgitation (6 New York Heart Association ≥3, 10 Fontan failures, 0 deaths). Cumulative incidence of the composite endpoint of atrioventricular valve failure at 28 years was 62% (95% CI, 49-74%). CONCLUSIONS: Patients with single ventricle physiology and common atrioventricular valve experience a continuous decline in valve function. The majority of patients experience valve failure in the first 30 years of life.
Assuntos
Técnica de Fontan , Defeitos dos Septos Cardíacos , Ventrículos do Coração , Criança , Pré-Escolar , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Técnica de Fontan/estatística & dados numéricos , Defeitos dos Septos Cardíacos/epidemiologia , Defeitos dos Septos Cardíacos/mortalidade , Defeitos dos Septos Cardíacos/cirurgia , Doenças das Valvas Cardíacas/mortalidade , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/cirurgia , Humanos , Estimativa de Kaplan-Meier , Masculino , Cuidados Paliativos/estatística & dados numéricos , Estudos RetrospectivosRESUMO
BACKGROUND: Transcatheter pulmonary valve implantation (TPVI) with the Melody® transcatheter pulmonary valve (TPV) has demonstrated good haemodynamic and clinical outcomes in the treatment of right ventricular outflow tract (RVOT) conduit dysfunction in patients with repaired congenital heart disease CHD. We present the first Australian single centre experience of patients treated with Melody TPV. METHOD: A prospective, observational registry was developed to monitor clinical and haemodynamic outcomes in patients with RVOT dysfunction treated with the Melody TPV (Medtronic Inc, Minneapolis, United States). RESULTS: Seventeen patients underwent TPVI with Melody TPV at The Prince Charles Hospital between January 2009 and February 2016 with a median (range) age of 34 (R: 15-60). Fifteen (88%) were NYHA Class 2 dyspnoea and 11 (59%) had corrected Tetralogy of Fallot. Indication for TPVI was stenosis in eight (47%), regurgitation in two (12%) and mixed dysfunction in seven (41%). Device implantation was successful in all patients. Peak RVOT gradient was significantly reduced and there was no significant regurgitation post procedure. There was one (6%) major procedural adverse event and two (12%) major adverse events at last recorded follow-up. There were no patient deaths. Follow-up cardiac magnetic resonance imaging revealed a significant reduction in indexed right ventricular end diastolic volume. CONCLUSION: This study confirms the safety and effectiveness of TPVI with Melody TPV for RVOT dysfunction in repaired CHD.
Assuntos
Cateterismo Cardíaco/métodos , Implante de Prótese de Valva Cardíaca/métodos , Hemodinâmica/fisiologia , Valva Pulmonar/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Adolescente , Adulto , Ecocardiografia , Feminino , Seguimentos , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Valva Pulmonar/diagnóstico por imagem , Fatores de Tempo , Tomografia Computadorizada por Raios X , Obstrução do Fluxo Ventricular Externo/complicações , Obstrução do Fluxo Ventricular Externo/fisiopatologia , Adulto JovemRESUMO
OBJECTIVES: Patients living with a Fontan circulation are prone to develop arrhythmias. However, their prognostic impact has been seldom studied. As such, we aimed to determine the incidence and predictors of arrhythmias after the Fontan procedure and the long-term outcomes after the first onset of arrhythmias. METHODS: Of the 1034 patients who have undergone a Fontan procedure as recorded in the Australian and New Zealand Fontan Registry, we identified those in whom a tachyarrhythmia or bradyarrhythmia developed. We evaluated the incidence and predictors of developing arrhythmias and their prognostic impact on late outcomes. RESULTS: Arrhythmia developed in 195 patients. Tachyarrhythmia was present in 162 patients, bradyarrhythmia was present in 74 patients, and both forms were present in 41 patients. At 20 years, freedom from any arrhythmia, tachyarrhythmia, and bradyarrhythmia was 66% (95% confidence interval [CI], 59-72), 69% (95% CI, 62-75), and 85% (95% CI, 80-90), respectively. On multivariable analyses, patients with an extracardiac Fontan (hazard ratio [HR], 0.23; 95% CI, 0.10-0.51; P < .001) were less likely to develop an arrhythmia, whereas those with left atrial (HR, 3.18; 95% CI, 1.45-6.95; P = .004) and right atrial (HR, 4.00; 95% CI, 2.41-6.61; P < .001) isomerism were more likely to have an arrhythmia. After onset of any arrhythmia (tachyarrhythmia or bradyarrhythmia), 10- and 15-year survivals were 74% (65%-83%) and 70% (60%-80%), respectively, and freedom from Fontan failure was 55% (44%-64%) and 44% (32%-56%), respectively. The development of any arrhythmia (HR, 2.20; 95% CI, 1-44-3.34; P < .001), tachyarrhythmia (HR, 2.56; 95% CI, 1.60-4.11; P < .001), and bradyarrhythmia (HR, 1.85; 95% CI, 1.16-2.95; P = .01) were all independent predictors of late Fontan failure on multivariable analyses. CONCLUSIONS: The development of an arrhythmia is associated with a heightened risk of subsequent failure of the Fontan circulation.
Assuntos
Arritmias Cardíacas/epidemiologia , Técnica de Fontan , Complicações Pós-Operatórias/epidemiologia , Austrália/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Masculino , Nova Zelândia/epidemiologia , Prognóstico , Sistema de Registros , Fatores de Risco , Resultado do TratamentoRESUMO
BACKGROUND: The number and age demographic of the future Fontan population is unknown. METHODS: Population projections were calculated probabilistically using microsimulation. Mortality hazard rates for each Fontan recipient were calculated from survivorship of 1353 Fontan recipients in the Australia and New Zealand Fontan Registry, based on Fontan type, age at Fontan, gender and morphology. Projected rates of new Fontan procedures were generated from historical rates of Fontan procedures per population births. RESULTS: At the end of 2014, the living Fontan population of Australia and New Zealand was 1265 people from an Australian and New Zealand regional population of 28 million (4.5 per 100,000 population). Of those, 165 (13%) received an atrio-pulmonary (AP) procedure, 262 (21%) a lateral tunnel (LT) procedure and 838 (66%) an extra-cardiac conduit (ECC) procedure. This population is expected to grow to 1917 (95% CI: 1846: 1986) by 2025 (5.8 per 100,000 population), with 149 (8%) AP procedures, 254 (13%) LT procedures, and 1514 (79%) ECC procedures. By 2045, the living Fontan population is expected to reach 2986 (95% CI: 2877: 3085; 7.2 per 100,000 population). The average age of the Fontan population is expected to increase from 18years in 2014 to 23years (95% CI: 22-23) by 2025, and 31years (95% CI: 30-31) by 2045. CONCLUSION: The Australian and New Zealand population of patients alive after a Fontan procedure will double over the next 20years increasing the demand for heart-failure services and cardiac transplantation. Greater consideration for the needs of this mostly adult Fontan population will be necessary.
Assuntos
Técnica de Fontan/mortalidade , Técnica de Fontan/tendências , Previsões Demográficas/métodos , Sistema de Registros , Adolescente , Adulto , Austrália/epidemiologia , Criança , Feminino , Humanos , Masculino , Nova Zelândia/epidemiologia , Taxa de Sobrevida/tendências , Adulto JovemRESUMO
OBJECTIVES: The most effective method of long-term thromboprophylaxis after the Fontan procedure is not clear. We compared the rates of thromboembolic events between patients receiving aspirin and warfarin after an extracardiac conduit Fontan procedure in a bi-national registry. METHODS: All patients who underwent an extracardiac conduit Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified. Medication status and routine follow-up and echocardiographic data were obtained from all patients. Survival analysis with propensity score matching and adjustment was performed to determine the treatment effect of warfarin compared with that of aspirin beyond the first year of follow-up, after which time patients had settled on their long-term thromboprophylaxis strategy. RESULTS: Of 570 eligible patients, the data of 475 patients who were regularly followed up without mechanical valve replacement were available for analysis. Long-term thromboprophylaxis consisted of warfarin in 301 patients (63%), aspirin in 157 (33%) and none in 17 (4%). The 10-year rate of freedom from all thromboembolic events was 91% [95% confidence interval (CI) 88-94%]. Thromboembolic events beyond the first year of follow-up occurred in 18 patients (6 on aspirin and 12 on warfarin). After (i) propensity score adjustment and (ii) matching yielding 164 pairs, the hazard rates of thromboembolic events beyond the first year were not statistically different between the warfarin and aspirin groups [(i) hazard ratio (HR) 2.3, 95% CI 0.7-7.4, P = 0.2 and (ii) HR 1.5, 95% CI 0.5-4.7, P = 0.5, respectively]. CONCLUSIONS: No difference in the hazard rates of late thromboembolic events was observed between aspirin and warfarin beyond the first year after the extracardiac conduit Fontan procedure.
Assuntos
Anticoagulantes/uso terapêutico , Aspirina/uso terapêutico , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Tromboembolia/prevenção & controle , Varfarina/uso terapêutico , Austrália/epidemiologia , Feminino , Técnica de Fontan/métodos , Cardiopatias Congênitas/epidemiologia , Hemorragia/epidemiologia , Hemorragia/etiologia , Humanos , Assistência de Longa Duração/métodos , Masculino , Nova Zelândia/epidemiologia , Cuidados Pós-Operatórios/métodos , Pontuação de Propensão , Sistema de Registros , Tromboembolia/epidemiologia , Tromboembolia/etiologia , Resultado do TratamentoRESUMO
BACKGROUND: Despite a lack of evidence supporting the use of angiotensin-converting enzyme (ACE) inhibitors in patients with a Fontan circulation, their use is frequent. We decided to identify the rationale for ACE inhibitor therapy in patients within the Australia and New Zealand Fontan Registry. METHODS: All patients in the Registry taking an ACE inhibitor at last follow up were identified, and a review of medical records was undertaken to determine the rationale for treatment initiation and reasons for treatment continuation or dose increase. RESULTS: In 2015, 36% of the surviving patients in the Registry (462/1268) were taking an ACE inhibitor. Indications for initiation of therapy were ventricular systolic or diastolic dysfunction (29%), atrioventricular valve regurgitation (19%), preservation of normal ventricular function (7%), prolonged effusions at Fontan (6%), hypertension (6%), other (6%) and unknown (2%). No indication was stated in the remaining patients (25%). Those with hypoplastic left heart syndrome were more likely to be on an ACE inhibitor than those with an alternative primary morphology (70% vs 32%; p<0.001). Only 36% of the patients treated with an ACE inhibitor at last follow up (166/462) had an indication that would generally justify treatment in a two-ventricle circulation. CONCLUSION: It is likely that the use of ACE inhibitors in patients with a Fontan circulation is excessive within our region. The coordination of prospective, multicentre studies and initiatives such as the Australia and New Zealand Fontan Registry will facilitate further investigations to guide treatment decisions in the growing Fontan population.
Assuntos
Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Técnica de Fontan , Cardiopatias Congênitas/tratamento farmacológico , Cardiopatias Congênitas/cirurgia , Adolescente , Adulto , Austrália/epidemiologia , Criança , Feminino , Seguimentos , Cardiopatias Congênitas/epidemiologia , Humanos , Masculino , Nova Zelândia/epidemiologia , Sistema de Registros , Adulto JovemRESUMO
We evaluated the long-term outcomes following repair of truncus arteriosus (TA) from a single institution. We conducted a retrospective review of children (n = 171) who underwent TA repair between 1979 and 2014. Early mortality rate was 11.7% (20/171). There were 19 late deaths. Most deaths (74%, 29/39) occurred within the first year following surgery. The 1-year mortality rate in 1979-2004 was 18% (25/136) and decreased to 11% (4/35) in 2005-2014. The overall survival rate was 73.6% at 30 years. Multivariate analysis identified postoperative extracorporeal membrane oxygenation (P = 0.003), operative weight <2.5kg (P = 0.012), prior surgical intervention (P = 0.018), and coronary artery anomaly (P = 0.037) as risk factors for early mortality. A Cox regression model identified DiGeorge syndrome (P = 0.008) as a risk factor for late mortality. Freedom from right ventricular outflow tract reoperation was 4.6% at 20 years. Concomitant truncal valve (TV) repair or replacement was undertaken in 20 patients. Additionally, 14 patients underwent late TV repair or replacement. The overall survival rate in patients who underwent TV operation was 76.9% at 20 years. A total of 19 patients had concomitant interrupted aortic arch with a survival rate of 89.5% at 20 years. Median follow-up was 19 years (mean = 17 years, range: 1-34 years). All patients were in New York Heart Association Class I/II at last follow-up. Following repair of TA, patients had good long-term functional status but had high reoperation rates. Repair of interrupted aortic arch and TV were not risk factors for mortality. Postoperative extracorporeal membrane oxygenation, operative weight <2.5kg, prior surgical intervention, and coronary artery anomaly were risk factors for early death. DiGeorge syndrome was associated with late death, most commonly from infection.