Long-term outcomes after first-onset arrhythmia in Fontan physiology Carins, Thomas A., BBiomedSc, BSc, MD; Shi, William Y., MBBS; Iyengar, Ajay J., MBBS, BMedSc ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
11/2016, Letnik:
152, Številka:
5
Journal Article
Recenzirano
Odprti dostop
Abstract Objectives Patients living with a Fontan circulation are prone to developing arrhythmia. 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 of after 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 who developed either a tachyarrhythmia or bradyarrhythmia. We evaluated the incidence and predictors of developing arrhythmias and its prognostic impact on late outcomes. Results A total of 195 patients developed an arrhythmia. Tachyarrhythmia was present in 162, bradyarrhythmia in 74 while both forms were present in 41 patients. At 20 years, freedom from any arrhythmia, tachyarrhythmia and bradyarrhythmia was 68% (95% CI 59-72%), 69% (62-75%), and 85% (80-90%) respectively. On multivariable analyses, patients with an extra-cardiac Fontan (HR 0.23, 0.10-0.51, p<0.001) were less likely to develop an arrhythmia, while those with left atrial (HR 3.18, 1.45-6.95, p=0.004) and right atrial (HR 4.00, 2.41-6.61, p<0.001) isomerism were more likely to have an arrhythmia. After onset of any arrhythmia (tachy- or bradyarrhythmia), 10- and 15-year survival was 74% (65-83%) and 70% (60-80%) respectively and freedom from Fontan failure was 55% (44-64%) and 44% (32-56%). The development of any arrhythmia (HR 2.20, 1-44-3.34, p<0.001), tachyarrhythmia (2.56, 1.60-4.11, p<0.001) and bradyarrhythmia (1.85, 1.16-2.95, p=0.01) 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.
Abstract 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 ...four 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 (HLHS) (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 HLHS 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 post 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.
Twenty-Five Year Outcomes of the Lateral Tunnel Fontan Procedure Wilson, Thomas G, BSc, MD; Shi, William Y, MBBS; Iyengar, Ajay J, MBBS(Hons), BMedSci, PhD ...
Seminars in thoracic and cardiovascular surgery,
2017 Autumn, Letnik:
29, Številka:
3
Journal Article
Recenzirano
Abstract Objective(s) To characterize late outcomes of the lateral tunnel (LT) Fontan procedure. Methods The outcomes of all patients who underwent a LT Fontan procedure in Australia and New Zealand ...were analysed. Original files were reviewed and outcomes data were obtained through a binational Registry. Results Between 1980 and 2014, a total of 301 patients underwent a LT Fontan procedure across 6 major centers. There were 13 hospital mortalities, 21 late deaths, 8 Fontan conversions/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/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 operation (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 (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. Conclusions Over a twenty-five-year period, the LT technique has achieved excellent late survival. As this population ages, they are at increasing risk of failure and adverse events. We are likely to see an increasing proportion requiring heart transplantation and late reintervention.
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.
Abstract Objective To evaluate long-term outcomes following repair of truncus arteriosus (TA) from a single-institution. Methods A retrospective review of children (n=171) who underwent TA repair ...between 1979 and 2014 in a single institution. Results Early mortality was 11.7% (20/171). There were 19 late deaths. Most deaths (74%, 29/39) occurred within the first year following surgery. One-year mortality in 1979–2004 was 18% (25/136) and decreased to 11% (4/35) in 2005–2014. Overall survival was 73.6% at 30 years. Multivariate analysis identified post-operative extra-corporeal membrane oxygenation (ECMO) ( p= 0.003), operative weight <2.5 kg ( p =0.012), p rior surgical intervention ( p =0.018), and coronary artery anomaly (CAA) ( p =0.037) as risk factors for early mortality. Cox-regression 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/replacement was undertaken in 20 patients. Fourteen additional patients underwent late TV repair/replacement. Overall survival in patients who had TV operation was 76.9% at 20 years. Nineteen patients had concomitant interrupted aortic arch (IAA) with survival 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. Conclusions Following repair of TA, patients had good long-term functional status, but high reoperation rate. Repair of IAA and TV were not risk factors for mortality. Postoperative ECMO, operative weight <2.5 kg, prior surgical intervention and CAA were risk factor for early death. DiGeorge syndrome was associated with late death. Ultra-mini abstract Repair of 171 patients with TA had early mortality was 11.7% and 19 late deaths. Surgery has good long-term functional outcomes, but high reoperation rate. Postoperative ECMO, operative weight <2.5 kg, prior surgical intervention and CAA were risk factor for early death. DiGeorge syndrome was associated with late death.
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