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.
Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect with limited data on long-term outcomes after surgical intervention.
We conducted a retrospective ...review of all children (N = 42) who underwent surgical repair of ALCAPA between 1980 and 2014 at the Royal Children's Hospital, Melbourne.
Twenty-nine (69% 29 of 42) patients underwent coronary reimplantation, 12 (29% 12 of 42) had intrapulmonary baffle (Takeuchi) repair, and 1 (2% 1 of 42) patient had ligation of the anomalous coronary artery. Nine (21%, 9 of 42) patients had concomitant mitral valve (MV) repair at the time of ALCAPA repair. A left ventricular assist device (LVAD) was used in 36% (15 of 42) of patients. Early mortality was 2.4% (1 of 42 patients). Median follow-up was 14 years (mean, 13 years; range, 4 months-31 years). There were no late deaths. Survival was 98% at 20 years. Freedom from reoperation was 81%, 81%, and 76% at 5, 10, and 20 years after operation, respectively. Eight patients underwent late MV repair or replacement at a median of 3 years (mean, 8 years; range, 2 months-25 years) after operation. Freedom from late MV repair or replacement was 86% at 5 and 10 years and 81% at 20 years after operation. Eleven (26% 11 of 42) patients had severe mitral regurgitation (MR) preoperatively. Of those 11 patients, 5 (45% 5 of 11) had concomitant MV repair at the time of ALCAPA repair, 3 (27% 3 of 11) had late MV repair or replacement, and the remaining 3 (27% 3 of 11) patients had mild MR at last follow-up. Thirty-six (90% 36 of 41) patients had normal left ventricular function and 4 (10% 4 of 41) patients had mildly reduced left ventricular (LV) function at last follow-up.
ALCAPA can be operated on with good outcomes. Persistent MR and a moderate rate of late MV repair warrants close follow-up.
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.
Objectives This study examined changes in practice and analyzed risk factors for adverse early outcomes after Fontan surgery through use of a binational, population-based registry. Methods ...Demographic, preoperative, and perioperative data were collected from all participating institutions of the Australia and New Zealand Fontan Registry. Patient and operative characteristics were analyzed with multivariable logistic regression for impact on early mortality, early Fontan failure (death, takedown, or mechanical support), effusions (prolonging hospital stay >30 days or requiring surgical reintervention), and stay longer than 30 days. Results Overall mortality was 3.5% (37/1071) and declined throughout the study period, from 8% (1975-1990) to 4% (1991-2000) and 1% (2001-2010). There were no differences between the extracardiac and lateral tunnel modifications for any outcome. After 2006, the extracardiac conduit was performed exclusively, with 1.3% mortality. The proportion of patients with hypoplastic left heart syndrome rose to 17% in the current era, and this group had more effusions (odds ratio, 3.0; 95% confidence interval, 1.4-6.6) and stayed on average 2 days longer in the hospital. Hypoplastic left heart syndrome was also an independent risk factor for composite adverse early outcome (death, failure, prolonged effusions, or prolonged stay >30 days; odds ratio, 2.6; 95% confidence interval 1.4-4.8 respectively). Conclusions The extracardiac conduit is now the exclusive Fontan modification performed in Australia and New Zealand. Even with a higher proportion of high-risk cases, perioperative outcomes are excellent in the modern era. Hypoplastic left heart syndrome confers a higher risk of prolonged pleural effusion and early composite adverse outcome.
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.
Intramural coronary arteries may complicate coronary artery transfer during the arterial switch operation. We sought to determine the long-term outcomes of 28 patients with intramural coronary ...arteries who underwent an arterial switch operation at a single institution.
All patients who had intramural coronary arteries and underwent an arterial switch operation were identified from the hospital database and retrospectively reviewed.
From 1983 to 2009, 720 patients underwent an arterial switch operation at our institution. Twenty-eight (3.9%, 28 of 720) had intramural coronary arteries. Patients with intramural coronary arteries had transposition of the great arteries (96%, n = 27) or Taussig-Bing anomaly (4%, n = 1). There were no deaths. Follow-up was 100% complete. Mean follow-up was 16.3 years (median, 15.5 years; range, 5.6 to 26.9 years). No patient required reoperation or catheter reintervention on the coronary arteries. Freedom from reoperation was 93% at 10 years. No patient had more than mild aortic regurgitation at last follow-up. Nine (32%, 9 of 28) patients had coronary angiograms at median 16 months (range, 14 months to 17 years) after arterial switch operation. All patients were asymptomatic at the time of angiogram. One patient had mild stenosis of the circumflex coronary artery demonstrated on a routine coronary angiogram 14 months postoperatively. All 28 patients were asymptomatic and in New York Heart Association functional class I at last follow-up.
Patients with intramural coronary arteries are not at increased risk of death or coronary reinterventions and have excellent late outcomes after the arterial switch operation.
Absent pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary arteries and compression of the tracheobronchial tree and may lead to significant respiratory compromise. We ...describe the outcomes of surgical correction of absent pulmonary valve syndrome and risk factors for mortality and reoperation.
A review of 52 patients with absent pulmonary valve syndrome who underwent surgical correction between 1975 and 2013 was conducted. The median age and weight at repair were 9 months (range, 4 days to 24.2 years) and 6.9 kg (range, 1.8 to 56 kg). Preoperative intubation was required in 15 patients (29%), and 21 patients (40%) underwent urgent repair. The pulmonary valve was replaced with a valved conduit in 16 patients (31%) or monocusp valve in 16 patients (31%). Valveless repair was performed in 20 patients (38%). Pulmonary artery reduction was performed in 39 patients (75%), and 2 patients (4%) underwent a Lecompte maneuver.
The median follow-up time was 13 years (range, 1 month to 35 years). Early mortality was 18.8% (3 of 16) during 1975 through 1989, 19% (4 of 21) during 1990 through 2000, and 0% (0 of 15) during 2001 through 2013. Late mortality was 6.7% (3 of 45). Overall survival at 5, 10, and 20 years was 81.4%±5.6%. On multivariate analysis, preoperative ventilation (p=0.009) was the only risk factor for overall mortality. Freedom from late reoperation at 5, 10, and 20 years was 79.7%±6.9%, 69.4%±8.2%, and 52.1%±9.8%, respectively. No difference in reoperation rates was found between valved conduit, monocusp, or valveless techniques. Risk factors for late reoperation on multivariate analysis were prematurity (p=0.001) and neonatal primary repair (p=0.007). Longer postoperative ventilation periods were predicted by preoperative ventilation (p<0.001) and surgery during infancy (p=0.01).
Long-term survival for absent pulmonary valve syndrome has improved during the last decade. Preoperative ventilation predicted longer postoperative ventilation and mortality.
Twenty-three years of single-stage end-to-side anastomosis repair of interrupted aortic arches Hussein, Aisyah, BMedSci; Iyengar, Ajay J., MBBS, BMedSci; Jones, Bryn, FRACP ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
04/2010, Letnik:
139, Številka:
4
Journal Article
Recenzirano
Odprti dostop
Objective This study defined long-term results of a policy of single-stage repair of interrupted aortic arch with end-to-side anastomosis. Methods Records of 112 consecutive patients undergoing ...interrupted aortic arch repair between 1985 and 2007 were reviewed. Single-stage repair was performed in 95 patients, with 90 having end-to-side repair. Results There were 11 in-hospital deaths (10%). Twelve patients needed arch reintervention during the same hospital stay: 7 for residual arch obstruction and 5 for left main bronchus obstruction. Nine patients were unavailable for follow-up. After a mean of 10 ± 7 years, 6 late deaths occurred, for 18-year survival of 92% (95% confidence interval CI, 84%–97%). Patients with end-to-side anastomoses had better 18-year survival (97%, 95% CI, 87%–99%, vs 74%, 95% CI, 44%–89%, P < .01). After discharge, 19 patients underwent further aortic arch intervention. The only factors predictive of late arch reintervention were technique other than end-to-side ( P < .001) and reoperation for left outflow tract obstruction. Freedom from arch reintervention after end-to-side repair was 78% at 18 years (95% CI, 59%–89%). Another 16 patients had significant residual obstruction. The 18-year freedom from hypertension was 88% (95% CI, 72%–95%). Conclusions Single-stage repair with end-to-side anastomosis seems the best approach for most neonates with interrupted aortic arch, because it provides relief of the arch obstruction with low early mortality. After 2 decades of experience with this approach, incidence of late hypertension seems minimal. The need for further arch reintervention warrants close follow-up of these patients.
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