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Background Few patients with total anomalous pulmonary venous drainage (TAPVD) and a univentricular circulation survive to Fontan completion. Hence, we sought to determine the long-term outcomes of the Fontan operation in patients with TAPVD. Methods Patients with TAPVD who underwent the Fontan operation and survived to hospital discharge in Australia and New Zealand between 1985 to 2017 were identified (n = 54) from a binational Fontan registry. Results Thirty-two patients (60%) underwent repair of TAPVD at a median age of 0.8 (interquartile range: 0.3-1.6) years. Thirty-seven patients (69%) had heterotaxy. The median age at time of Fontan operation was 5.7 years. There were 4 late deaths and 3 patients required cardiac transplantation for a failing Fontan circulation. On univariate analysis, the concomitant diagnosis of pulmonary stenosis and right ventricular dominance was associated with late death or transplantation (P = .04). Freedom from late death or transplantation at 15 years after the Fontan operation was 88% ± 7% (95% confidence interval [CI], 67%-96%) for the repaired TAPVD group and 90% ± 6% (95% CI, 67%-98%) for the unrepaired TAPVD group (P = .47). Median follow-up after the Fontan procedure was 10.8 (interquartile range, 6.7-16.2) years. The majority of survivors (94%) were in New York Heart Association functional class I or II. The 15-year freedom from death or transplantation was similar for patients with TAPVD (89% ± 5%; 95% CI, 76%-95%) compared with patients without TAPVD in the Fontan registry (n = 1446; 92% ± 1%; 95% CI, 90%-93%) (P = .12). Conclusions Long-term survival of patients with TAPVD who undergo the Fontan operation and survived to hospital discharge is comparable to Fontan survivors without TAPVD.

Remote ischaemic preconditioning (RIPC) has been employed as a non-invasive protective intervention against myocardial ischaemia-reperfusion injury in animal studies. However, the underlying mechanisms are incompletely defined in humans and its clinical efficacy has been inconclusive. As advanced age, disease, and drugs may confound RIPC mechanisms in patients, our aim is to measure whether RIPC evokes release of adenosine, bradykinin, met-enkephalin, nitric oxide, and apolipoproteins in healthy young adults. Healthy subjects (n = 18, 9 males, 23 ± 1.5 years old; 9 females, 23 ± 1.8 years old) participated after informed consent. RIPC was applied using a blood pressure cuff to the dominant arms for four cycles of 5-minute cuff inflation (ischaemia) and 5-minute cuff deflation (reperfusion). Blood was sampled at baseline and immediately after the final cuff deflation (Post-RIPC). Baseline and Post-RIPC plasma levels of adenosine, bradykinin, met-enkephalin, apolipoprotein A-1 (ApoA-1), apolipoprotein D (ApoD), and nitric oxide (as nitrite) were measured via ELISA and high-performance liquid chromatography. Mean (±SD) baseline levels of adenosine, bradykinin, met-enkephalin, ApoA-1, ApoD, and nitrite in healthy young adults were 13.8 ± 6.5 ng/mL, 2.6 ± 1.9 μg/mL, 594.1 ± 197.4 pg/mL, 3.0 ± 0.7 mg/mL, 22.2 ± 4.0 μg/mL, and 49.8 ± 13.4 nmol/L, respectively. Post-RIPC adenosine and nitrite levels increased (59.5 ± 37.9%, P

Mucosal-associated invariant T cells (MAIT cells) detect microbial vitamin B2 derivatives presented by the antigen-presenting molecule MR1. Here we defined three developmental stages and checkpoints for the MAIT cell lineage in humans and mice. Stage 1 and stage 2 MAIT cells predominated in thymus, while stage 3 cells progressively increased in abundance extrathymically. Transition through each checkpoint was regulated by MR1, whereas the final checkpoint that generated mature functional MAIT cells was controlled by multiple factors, including the transcription factor PLZF and microbial colonization. Furthermore, stage 3 MAIT cell populations were expanded in mice deficient in the antigen-presenting molecule CD1d, suggestive of a niche shared by MAIT cells and natural killer T cells (NKT cells). Accordingly, this study maps the developmental pathway and checkpoints that control the generation of functional MAIT cells.

Background 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. Methods All patients who had intramural coronary arteries and underwent an arterial switch operation were identified from the hospital database and retrospectively reviewed. Results 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. Conclusions 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.

Supravalvular aortic stenosis is a rare congenital cardiac anomaly involving stenosis of the sinotubular junction that can extend to the ascending aorta and the aortic arch. It is an elastin arteriopathy that is classically associated with Williams syndrome. Many surgical techniques have been developed over time, most commonly involving pericardial patches to relieve the stenotic area. The 1-patch repair techniques (McGoon and Doty repairs) have low mortality but do not restore the normal morphology of the aortic root. Herein we describe the extended 3-patch technique that can symmetrically relieve supravalvular aortic stenosis.

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

Objectives To evaluate whether the long-term outcomes of modified-single-patch (MSP) repair of complete atrioventricular septal defect are equivalent to double-patch (DP) repair with respect to survival and risk of reoperation for left atrioventricular valve regurgitation or left ventricular outflow tract obstruction. Methods All patients who underwent biventricular repair of complete atrioventricular septal defect in Australia from 1990 to 2015 using either a MSP or DP technique were identified. Demographic characteristic details, operative data, and outcomes were analyzed. A propensity score analysis was performed to balance the 2 treatment groups according to several baseline covariates. Survival and freedom from reintervention between the 2 groups were compared using Kaplan-Meier curves and log-rank tests. Results A total of 819 patients underwent repair of complete atrioventricular septal defect (252 MSP and 567 DP) during the study period. There was no significant difference in unmatched survival (P = .85) and event-free survival (P = .49) between MSP and DP repair. Propensity score matching resulted in a total of 223 matched pairs. Matched analysis found no difference in overall survival (P = .59) or event-free survival (P = .90) between repair techniques, with an estimated event-free survival at 5, 10, and 15 years of 83%, 83%, and 74% for DP and 83%, 80%, and 77% for the MSP group, respectively. There was no significant difference between repair techniques in reoperation for left atrioventricular valve regurgitation or left ventricular outflow tract obstruction or need for permanent pacemaker. Conclusions Overall and event free survival are similar following either MSP or DP repair of complete atrioventricular septal defect. There is no increased risk of reoperation for left ventricular outflow tract obstruction with the MSP technique.

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether the transatrial-transpulmonary approach to tetralogy of Fallot repair in non-neonatal patients provides superior outcomes compared with the transventricular approach. Altogether, 175 papers were found using the reported search, of which 11 represented the best evidence to answer the clinical question. Two randomized controlled trials (RCTs) and 3 observational studies showed that the transatrial approach resulted in better preservation of right ventricular (RV) function, whereas 4 observational studies showed no significant difference. Three observational studies showed better attenuation of RV dilatation, whereas 3 showed no difference. One RCT and 2 observational studies showed lower incidence of postoperative ventricular arrhythmias, while 1 RCT and 4 observational studies showed no difference. Two observational studies demonstrated greater freedom from reoperation, 1 RCT and 2 observational studies showed no difference, while 1 retrospective study observed a higher incidence of residual RV outflow tract obstruction and lower freedom from reoperation in infants. Two observational studies reported lower risk of requiring pulmonary valve replacement, whereas 2 reported no difference. Three observational studies reported superior exercise capacity, while 1 reported no difference. No difference in long-term survival was demonstrated. The results presented suggest that transatrial repair of tetralogy of Fallot confers superior or equivalent outcomes in terms of preservation of RV function and volume, ventricular arrhythmias, need for pulmonary valve replacement, and exercise capacity compared with transventricular repair. However, the incidence of residual RV outflow tract obstruction may be higher in infants undergoing transatrial repair.

Long-term survival, risk of transplantation, and causes of death after repair of total anomalous pulmonary venous connection (TAPVC) remain unknown. By linking the Pediatric Cardiac Care Consortium with the National Death Index and the United Network for Organ Sharing, we evaluated long-term transplant-free survival in children undergoing repair of TAPVC.We identified 777 infants within the Pediatric Cardiac Care Consortium who underwent TAPVC repair (median 21 days; interquartile range, 5 to 80) and had sufficient personal identifiers for linkage with the National Death Index and United Network for Organ Sharing. Sixty-six deaths, ten cardiac transplantations, and one bilateral lung transplantation had occurred by the end of 2014. Data collected included age and weight at time of procedure, TAPVC type, associated cardiac lesions, and postoperative length of stay. The study cohort was divided into simple and complex TAPVC based on the presence of an associated cardiac lesion. Parametric survival plots were constructed, and risk factor analyses were performed to identify demographic and clinical characteristics associated with long-term outcomes.Mortality or need for transplantation was 9.7% with a median follow-up of 18.4 years and a median age of death or transplant of 0.74 years. The risk of mortality and transplant after TAPVC repair was highest during the first 18 months after hospital discharge. Cardiac causes accounted for the majority of deaths. Multivariate regression models for transplant-free survival demonstrated that complex TAPVC, mixed TAPVC, and postoperative length of stay were associated with increased risk of death/transplant.Transplant-free survival after TAPVC repair is excellent, with most deaths or transplant events occurring early. Factors associated with the worst long-term outcomes included complex TAPVC, mixed TAPVC, and prolonged postoperative length of stay.

The study objective was to identify determinants of adverse outcomes in patients undergoing univentricular palliation after the shunt procedure.We performed a retrospective study of patients with univentricular lesions undergoing modified Blalock-Taussig shunt and central shunt placement, with or without concomitant Norwood/Damus-Kaye-Stansel procedures, between 2004 and 2014.Overall, 246 patients were included, with 150 patients undergoing concomitant Norwood/Damus-Kaye-Stansel procedure. The 30-day and in-hospital mortality were 7.3% and 14.6%, respectively. Progression to bidirectional cavopulmonary connection was achieved in 76% of patients. Acute events occurred in 66 patients (27%), with 42 having an acute event in the first 72 hours postoperatively. Of all in-hospital deaths, 75% had preceding acute events. On multivariable analysis, the risk factor for 30-day or in-hospital mortality was the incidence of an acute event (odds ratio [OR], 13.5; 95% confidence interval [CI], 5.51-36.4; P .001). Postoperative pH was associated with fewer acute events (OR, 0.61 per 0.1 unit increase; 95% CI, 0.45-0.82; P = .002). Risk factors for shunt thrombosis associated with an acute event were increased postoperative hematocrit (OR, 2.12 per 0.1 unit increase; 95% CI, 1.01-4.58; P = .049) and 3.0-mm shunt size (OR, 3.78; 95% CI, 1.47-10.6; P = .007). Damus-Kaye-Stansel/Norwood procedure, shunt type, morphology, and extracardiac or genetic anomaly were not risk factors for mortality or acute events.More than one-fifth of patients shunted during univentricular palliation die before the second stage. The majority of these deaths are associated with acute events occurring early after surgery. Strategies to improve survival should focus on prevention and management of acute events.

Surgical treatment of multiple ventricular septal defects (VSDs) has advanced significantly in recent years, yet remains technically challenging. With high rates of complications and reoperations, we sought to assess the outcomes of patients undergoing a variety of management techniques for multiple VSDs. From 1988 to 2015, 157 consecutive patients underwent surgical management of multiple VSDs at a median age of 2.2 months (2 days-16 years). Sixty-nine patients (44%) had exclusively multiple VSDs, 62 patients (39%) had multiple VSDs with concomitant intracardiac anomalies, and 26 patients (17%) had multiple VSDs with aortic arch anomalies. The predominant techniques used at the initial operations were patch closure (84 patients), pulmonary artery band (83 patients), suture closure (37 patients), and sandwich technique (13 patients). Eighteen patients underwent ventriculotomies. There were 3 hospital deaths (2%). Mean follow-up time was 8.6 ± 6 years (1 day-22 years). Four patients died during follow-up, whereas freedom from reoperations was 52% (95% confidence interval 42-61%) at 16 years. Freedom from reoperation was significantly lower in the 1988-2002 era than in the post-2002 era (38% vs 73%, P = 0.016). Pacemaker implantation was ultimately required in 9% (14 of 150) of patients. No deleterious impact of a ventriculotomy could be detected. Surgical treatment of multiple VSDs can be performed with excellent short- and long-term survival, and normal late functional outcome, however, carries a significant rate of reoperation. The recent inclusion of absorbable pulmonary artery bands and the sandwich technique appear safe and are useful adjuncts in these patients.

The arterial switch operation has excellent early outcomes. Although the majority of patients are growing into healthy adults, a small subset require reoperation for pulmonary stenosis as well as neoaortic root dilatation and neoaortic valve regurgitation, especially as follow-up increases. Challenging subgroups include patients with anomalous coronary anatomy, concomitant aortic arch obstruction, Taussig-Bing anomaly, ventricular septal defect, and low operative weight, and patients with an intact interventricular septum who present late.

Chylothorax is a rare but severe complication after pediatric cardiac surgical procedures and is related to significant morbidity and mortality. It is suspected to be more frequent after single-ventricle staged palliation procedures, but focused studies on chylothorax in patients with univentricular heart physiology are scarce.From January 2008 to December 2016, a total of 289 patients underwent 376 cavopulmonary connection (CPC) procedures over 9 years (superior cavopulmonary connection [SCPC], 199; Fontan completion, 177). Patients were classified according to whether they had a chylothorax (group 1) or not (group 2). Chylothorax was confirmed on a pleural fluid test.The rate of chylothorax after a CPC procedure was 19.7% (74 of 376): 15.6% after SCPC and 24.3% after Fontan completion. Mean follow-up was 4.3 ± 0.1 years. Systemic right ventricle was more frequent in group 1 than in group 2 (64.9% vs 46%, respectively; p = 0.003). Chylothorax was associated with a higher rate of early reoperation (p = 0.001) and late failure of the CPC (p0.001). Late mortality was also more frequent in group 1 than in group 2 (17.6% vs 4.3%; p0.001). By multivariate analysis, having a systemic right ventricle was the only identified predictor for the development of chylothorax (odds ratio, 2.49; 95% confidence interval, 1.4 to 4.7; p = 0.004).The incidence of chylothorax in patients undergoing the univentricular pathway procedure is higher than previously suggested. Having a systemic right ventricle is a significant risk factor for developing a chylothorax after a CPC.

The advent of the slide tracheoplasty technique and a multi-disciplinary approach has improved outcomes of congenital tracheal stenosis. However, tracheal surgery in younger patients with pulmonary malformations, especially low birth-weight neonates, has been associated with increased mortality. Patients with very low birth-weight, pulmonary malformations and prematurity may be palliated prior to definitive tracheal surgery due to the poor prognosis. We report a successful and unique approach of delaying tracheal reconstruction to allow growth and development in the premature, very low birth-weight neonate (1046g) with left lung agenesis.

OBJECTIVES: We sought to determine the quality of life after the arterial switch operation (ASO) using the Short Form 36 questionnaire in adult survivors. METHODS: All patients (n = 107) who underwent the ASO and were 18 years of age or older living in the Australian state of Victoria with a contact telephone number were identified from the hospital database. Fifty-one (48%) patients were 18-24 years old and 56 (52%) patients were 25-34 years old. Patients completed the Short Form 36 quality of life questionnaire via telephone. The results of the 8 domains of the Short Form 36 questionnaire and the derived health state summary score (Short Form 6-Dimension) were compared against mean scores from age-matched Australian population data. RESULTS: Compared with the Australian population age-matched data, 18- to 24-year-old ASO patients ranked their health higher in 3 of the 8 domains (P

Background We evaluated our experience with ventricular assist device (VAD) implantation in children and young adults. Methods A total of 64 patients underwent VAD implantation in two centres. The mean age was 15 ± 7.2 years. Thirty-five (55%) patients were under 18 years of age. Devices implanted included the Thoratec Paracorporeal in 30 (47%) patients, Berlin Heart EXCOR in 11 (17%) and VentrAssist in 14 (22%). The diagnosis was cardiomyopathy in 53, congenital heart disease in 11, and graft failure in four patients. Results There were 10 (16%) in-hospital deaths. Mortality was higher in patients

Background To (1) determine the prevalence of hypertension late after coarctation repair in patients with normal-sized transverse arches, and (2) evaluate the potential for end-organ damage related to hypertension after coarctation repair. There are no studies specifically investigating end-organ damage and hypertension after coarctation repair using noninvasive techniques. Methods Eighty-two patients aged 10 years or greater with a coarctation repair and a normal-sized arch operated on between 1978 and 2010, underwent a transthoracic echocardiogram, 24-hour blood pressure (BP) monitoring, and retinal imaging. Median age at repair was 1 year (interquartile range, 0 to 6); 45% (37 of 82) were operated in the first year of life. Results After a follow-up of 24 ± 7 years, 27% (22 of 82) and 50% (41 of 82) suffered resting hypertension and resting prehypertension, respectively. On 24-hour BP monitoring, 61% (49 of 80) and 21% (17 of 80) suffered hypertension and prehypertension, respectively. Arch reobstruction (echo gradient > 25 mm Hg) was present in only 15% (12 of 82), and in only 15% (7 of 47) with 24-hour hypertension. Resting hypertension was associated with a smaller central retinal artery equivalent (average width of arterioles) and central retinal vein equivalent (average width of venules) ( p = 0.0006 and p = 0.003, respectively). Left ventricular hypertrophy on echocardiography was present in 63% (31 of 49) with 24-hour hypertension compared with only 42% (13 of 31) with normal 24-hour BP ( p = 0.06). Conclusions There is a high rate of hypertension late after coarctation repair, even in patients with unobstructed arches. The presence of retinal imaging abnormalities and left ventricular hypertrophy signals the presence of end-organ damage in this young adult population. Regular follow-up with 24-hour BP monitoring is warranted.

To identify determinants of adverse outcomes in patients with atrial isomerism.Determinants of survival were analyzed for the group as a whole as well as separately for left and right atrial isomerism.There were 78 cases with right and 104 with left atrial isomerism. Nineteen patients were not offered surgery; 49 (47%) of those with left atrial isomerism and 60 (77%) with right atrial isomerism were directed to single ventricle palliation. A total of 67 patients died. Survival to 25 years was 62% (95% confidence interval [CI], 53%-69%). Independent predictors of mortality were obstructed total anomalous pulmonary venous drainage (P.001; hazard ratio [HR], 5.2; 95% CI, 2.7-9.7) and asplenia (P = .008; HR, 2; 95% CI, 1.2-3.3). There was no evidence that patients born in the recent era had improved survival (P = .47; HR, 1.1; 95% CI, 0.8-1.5). Survival was better for patients with left than right atrial isomerism: 18 years survival 74% (95% CI, 63%-82%) versus 50% (95% CI, 38%-60%). Independent predictors of mortality for patients with left atrial isomerism were dextrocardia (P = .009; HR, 3.0; 95% CI, 1.3-6.7) and pulmonary stenosis (P = .042; HR, 0.3; 95% CI, 0.1-0.9) and patients with right atrial isomerism, biventricular repair (P.001; HR, 6.0; 95% CI, 2.8-13), and obstructed total anomalous pulmonary venous drainage (P.001; HR, 4.2; 95% CI, 2.0-8.6).A significant proportion of patients with isomerism still die before reaching adulthood. Only a fraction of those with obstructed pulmonary veins survive. Having biventricular repair does not confer a survival advantage to those born with right atrial isomerism.

Background Data on outcomes of atrioventricular (AV) valve surgical procedures in patients with Fontan circulation are limited. Methods We conducted a retrospective review of all children with Fontan circulation who underwent AV valve operations. Results From 1981 to 2014, 581 patients underwent Fontan operations, and 9.3% (54/581) of them required AV valve operations. The first AV valve operation was performed before (n = 32), during (n = 15), or after (n = 7) the Fontan operation. The mean follow-up time was 9.8 ± 7.1 years (range, 6 months to 32 years). Operative mortality for the initial AV valve operation was 1.9% (1/54) and occurred in a patient who had the initial valve operation concomitantly with the Fontan. Late mortality was 5.7% (3/53). Heart transplantation was performed in 13.0% (7/54) of patients. Freedom from death or transplantation after the first AV valve operation was 89.8 ± 4.4% at 5 years (95% confidence interval [CI], 77.1 to 95.6) and 81.0 ± 6.2% at 10 years (95% CI, 65.0 to 90.2). Reoperation on the AV valve was performed in 44.4% (24/54) of patients. The median time to initial valve reoperation was 3.1 years (interquartile range, 0.8 to 7.4 years). Freedom from reoperation or transplantation was 63.4 ± 7.0% at 5 years (95% CI, 48.2 to 75.3) and 48.9 ± 7.9% at 10 years (95% CI, 32.8 to 63.2). Freedom from moderate or more regurgitation in patients who had not undergone reoperation or transplantation was 74.0 ± 6.9% (95% CI, 57.5 to 84.8) at 5 years and 67.5 ± 7.7% (95% CI:,50.0 to 80.0) at 10 years. After initial valve operation, thromboembolic events occurred in 13.0% (7/54) of patients, stroke occurred in 24.1% (13/54) of patients, pacemaker insertion was required in 16.7% (9/54) of patients, and protein-losing enteropathy was diagnosed in 7.4% (4/54) of patients. Of the 43 surviving transplant-free patients, 62.8% (27/43) were in New York Heart Association (NYHA) class I, 34.9% (15/43) were in NYHA class II, and 1 patient was in NYHA class III. Conclusions The AV valve operation done before, during, or after the Fontan operation is associated with low operative mortality but a high reoperation rate with significant risk of late death, transplantation, and persistent AV valve regurgitation.

Background The bovine jugular vein (Contegra) conduit has been described as an alternative to the homograft for right ventricle (RV) to pulmonary artery (PA) connection. We assessed the outcomes of Contegra conduits and homografts at a single institution. Methods We conducted a retrospective review of children (n = 249) who underwent RV-to-PA conduit (Contegra or homograft) implantation from 2001 to 2011. Results Median operation age was 4.8 years (2 days–18 years). Indications for surgery were as follows: primary conduit insertion (n = 131; 53%), previous conduit failure (n = 57; 23%) or Ross procedure (n = 61; 25%). There were 113 (45%) Contegra conduits and 136 (55%) homografts (92 pulmonary, 44 aortic) inserted. Early mortality was 5% (n = 12). Overall survival was 89% (95% confidence interval (CI): 84–92%) at 5 years and 87% (95% CI: 81–92%) at 10 years. Mortality was associated with smaller conduit size (P = 0.044) and syndrome diagnosis (P = 0.012). Freedom from reoperation was 85% (95% CI: 77–91%) and 75% (95% CI: 59–86%) at 5 years for homografts and Contegra conduits, respectively. Patients required conduit replacement (15%) for endocarditis (n = 4; 11%) or graft failure (n = 34; 89%). Eleven patients developed distal conduit stenosis with the majority occurring in Contegra conduits (n = 7; 64%) (P = 0.004). A larger conduit (P = 0.007) was protective against reoperation. There was no difference in reoperation between conduits (P = 0.41). Mean follow-up was 5 ± 3.2 years (96% complete). Majority of survivors (99%) were in New York Heart Association Class II/I. Conclusion The Contegra conduit and homograft demonstrate similar mid-term outcomes in children. Smaller conduit size is associated with higher graft failure and mortality.

An aortopulmonary window (APW) is a rare congenital cardiac anomaly, which involves communication between the ascending aorta and the main pulmonary artery. It may be an isolated anomaly (simple APW) or associated with concomitant cardiovascular anomalies (complex APW). Contemporary repair of simple APW has a mortality approaching zero. Mortality is almost always due to concomitant cardiovascular anomalies. One of the most common concomitant cardiovascular anomalies is an interrupted aortic arch. Rarely, an APW may have a concomitant anomalous coronary artery. Herein we describe the surgical technique to repair APW with associated interrupted aortic arch.

Background Heart transplantation (HT) is established therapy for end-stage heart failure in children with cardiomyopathy or congenital heart disease. Aims This review summarises experience at a national referral centre since the first local transplant. Methods Medical records of children referred for HT between 1 April 1988 and 1 January 2010 were retrospectively reviewed. All patients listed for HT were included. Survival analysis was used to summarise wait-list time to death/transplant, and separately, time to death in HT patients. Results One hundred and thirty-nine children were accepted on to the HT waiting list during the study (median age 7.7 (interquartile range (IQR) 2.5, 13.6) years), of whom 93 underwent HT (median age 10.9 (IQR 4.4, 14.6) years). Wait-list mortality was 32% (45 of 139 patients), lowest among children aged >10 years at listing (P 0.05). Conclusions Results of paediatric HT in Australia are comparable with international results, despite limitations of geographic isolation, small population and low organ donation rate. Increasing case complexity has not impacted on post-transplant survival.

Congenital heart disease (CHD) represents a broad spectrum of conditions, from simple defects with an excellent prognosis, to the complex and severe, which require multiple procedures and have uncertain long-term outcomes. As outcomes in cardiac surgery have improved, research has tended to focus on more complex cardiac lesions, rather than mild defects where results were already excellent (1). Simple defects have been defined as ventricular septal defect (VSD), atrial septal defect (ASD), pulmonary stenosis (PS), patent ductus arteriosus (PDA), aortic stenosis (AS) and aortic coarctation in older children (2). In the case of many simple forms of CHD, survival has been said to be normal (3) and guidelines do not even recommend cardiology follow-up (4,5). Have these recommendations been made on sound evidence? Or has it simply become a truism that these patients have the same life expectancy as the general population?

We herein describe the successful surgical repair of a very rare combination of an aorta-to-left ventricle tunnel with the right coronary artery arising from it. The neonate presented with signs of heart failure due to significant regurgitation of blood via the tunnel. The closure of the tunnel was feasible during neonatal period without patches.

Objectives Preoperative moderate or greater truncal valve (TV) insufficiency is one of the most important factors influencing mortality in children with truncus arteriosus. We therefore sought to determine the impact of TV insufficiency and concomitant TV surgery on children who underwent truncus arteriosus repair at a single institution. Methods We reviewed 180 patients who underwent truncus arteriosus repair between 1979 and 2016. Preoperative echocardiography demonstrated TV insufficiency in 80 patients (mild: 33.9%, 61/180; moderate: 9.4%, 17/180 and severe: 1.1%, 2/180). Results Twenty-one patients had concomitant TV surgery with an early mortality of 19% (4/21) and overall survival of 70.8 ± 10.1% at 25 years. There were 60 neonates, 11 of whom had concomitant TV surgery with an early mortality of 27% (3/11) and overall survival of 62.3 ± 15.0% at 20 years. Concomitant TV repair (P = 0.5) was not a risk factor for death. TV reoperation was common in those who had concomitant TV surgery, with freedom from reoperation of 19.2 ± 14.9% at 20 years. In the remaining 159 patients, 14 required subsequent TV surgery, and the freedom from TV surgery was 84.0 ± 4.6% at 20 years. At a median follow-up of 18.5 years, TV insufficiency was none or trivial in 79.6% (109/137) and mild or less in 98.5% (135/137) of patients. Conclusions Most patients with mild TV insufficiency are free from TV surgery up to 25 years. The durability of TV repair is poor. Most patients with moderate or greater TV insufficiency and a quadricuspid TV will require TV surgery.

Background Outcomes of operations for total anomalous pulmonary venous drainage (TAPVD) have improved. However, operations in the neonatal period and the development of postoperative pulmonary venous obstruction are associated with a high mortality rate. Methods A retrospective review was conducted for all neonates and infants (n = 214) who underwent operations for isolated TAPVD (1973 to 2014). Results Median age was 18 days (1 day to 1 year). There were 17 (7.9%) early deaths. Risk factors for early death were prolonged cardiopulmonary bypass time ( p = 0.005) and neonatal age at the operation ( p = 0.048). Early mortality was 2.5% for infants (n = 81) and 11% for neonates (n = 133; p = 0.021) during the entire study period. Hospital deaths for neonates remained unchanged during the four eras of 1973 to 1988, 1989 to 1998, 1999 to 2008, and 2009 to 2014. Survival at 10 and 20 years was 88% ± 2.2% (95% confidence interval, 82% to 91%). Reoperation for postoperative pulmonary venous obstruction was required in 22 patients (10%). Risk factors for reoperation were prolonged cardiopulmonary bypass time ( p = 0.015), lower operative weight ( p = 0.003), and an episode of postoperative pulmonary hypertensive crisis ( p = 0.005). Freedom from reoperation at 20 years was 86% ± 3.2% (95% confidence interval, 78% to 91%). All survivors were asymptomatic at a mean of 13 ± 9 years (range, 1 month to 42 years) after the operation. Conclusions Although isolated TAPVD repair in infants can be performed without death, the operation is associated with a high mortality rate in neonates that remained unchanged during the long study period. Survival beyond 1 year after the operation is associated with excellent long-term outcomes.

ObjectivePartial 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.MethodsAll children at three institutions who underwent repair of pAVSD from 1975 to 2015 were included. Infants (aged ResultspAVSD 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 (PPropensity 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.ConclusionsDespite 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.

Objectives Late hypertension after coarctation repair is associated with high mortality, but its risk factors remain unclear. This study aims to determine early and late postoperative risk factors for late hypertension after coarctation repair. Methods A cross-sectional study including transthoracic echocardiogram and 24-h blood pressure (BP) monitoring was performed in 144 patients aged ≥10 years with previous coarctation repair. Median age at repair was 39 days (interquartile range 0-3 years). Early postoperative hypertension was evaluated by calculating the mean of BP measurements taken on the same day before hospital discharge or the need for antihypertensives prior to discharge. Multivariable analyses were performed to adjust for gender, surgical age, and follow-up age. Results After a mean follow-up period of 22 ± 7 years, 59% (84/142) of patients were hypertensive: 58% (82/142) on 24-h BP monitoring and 1% (2/142) on antihypertensives. Early postoperative hypertension was present in 58% (73/126): 39% (49/126) on BP measurements and 19% (24/126) on antihypertensives. Early and late arch reobstruction (transthoracic echocardiogram peak gradient ≥25 mmHg) was present in 37% (23/62) and 23% (33/144), respectively. On multivariable logistic analysis, early postoperative hypertension and maximum descending arch velocity on echocardiography were associated with late hypertension on 24-h BP monitoring (odds ratio 2.21, 95% confidence interval 1.05-4.66, P = 0.04; and odds ratio 2.28, 95% confidence interval 1.08-4.81, P = 0.03; respectively). Conclusions There is a high prevalence of late hypertension after coarctation repair. Arch reobstruction may be a major determinant of late hypertension. Early postoperative hypertension may identify very early in life those at risk of developing late hypertension.

Objectives To determine the incidence of vocal cord paresis (VCP) after neonatal aortic arch repair/Norwood-type procedure, and the effectiveness of noninvasive laryngeal ultrasound in detecting VCP compared with gold standard invasive nasoendoscopy. Methods Fifty-two patients who underwent an arch repair (39 of 52; 75%) or Norwood-type procedure (13 of 52; 25%) via sternotomy between April 1, 2015, and April 30, 2017 underwent laryngeal ultrasound (50 of 52; 96%) and/or flexible fiber optic nasoendoscopy (39 of 52; 75%) at 48 to 72 hours after endotracheal extubation. Primary arch diagnoses were coarctation in 56% (29 of 52), hypoplastic left heart syndrome in 17% (9 of 52), isolated hypoplastic arch in 17% (9 of 52), and interrupted aortic arch in 10% (5 of 52). The median patient age at surgery was 5.5 days (interquartile range, 4.0-12.5 days). Fifteen patients (15 of 52; 29%) required preoperative intubation. Results Left VCP was present in 59% (23 of 39) of patients on nasoendoscopy and in 59% (27 of 46) of patients on laryngeal ultrasound, and 4 additional patients had inconclusive ultrasound results. There was agreement between the results of nasoendoscopy and conclusive ultrasound in all cases. The overall sensitivity, specificity, positive and negative predictive values, and Cohen's kappa coefficient of laryngeal ultrasound compared with nasoendoscopy for the detection of left VCP were 95%, 88%, 91%, 93%, and 0.83, respectively. On multivariable analysis, preoperative intubation and arch repair techniques other than the Norwood procedure were associated with left VCP (odds ratio, 12.7; P = .03; and 14.1; P = .03, respectively). Conclusions There is a high incidence of VCP after arch repair via sternotomy. Laryngeal ultrasound seems to be an effective and noninvasive method for detecting VCP in neonates and young children.

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon cardiac defect in children. The mixed subset accounts for 5%-10% of the TAPVD and is variable in its anatomy. The outcomes associated with this subset of patients are rarely reported. A retrospective review of all patients with mixed TAPVD undergoing repair at a single institution (1984-2014) was conducted. A descriptive analysis was performed. Twenty-four patients underwent repair for mixed TAPVD (6 univentricular physiology, 18 biventricular physiology). The mixed TAPVD anatomy included 8 patients in group I (2 + 2 veins), 11 patients in group II (3 + 1 veins), and 5 patients in group III (atypical). Preoperative pulmonary venous obstruction occurred in 8 patients (33%). The median age at repair was 2.2 months (range 2 days to 3 years) and median weight was 4.2 kg (range 1.9 to 12.5 kg). Operative mortality was 13% (3 of 24), 33.3% (2 of 6) for patients with univentricular physiology, and 5.6% (1 of 18) for patients with biventricular physiology. There have been no operative deaths in the biventricular group since 1997 (n = 11). Survival at 30-days was 83% ± 15% (95% confidence interval: 27%-97%) and 94% ± 5% (95% confidence interval: 67%-99%) for the univentricular and biventricular groups, respectively. Reoperation for recurrent pulmonary venous obstruction was required in 2 patients (8.3%) where the sutureless technique was used. The average follow-up after surgery was 9.3 ± 6.4 years (5 months to 21 years), and all surviving patients were asymptomatic. Mixed TAPVD can be repaired with good results in children, particularly in those undergoing biventricular repair.

Background In 2005 the Department of Cardiothoracic Surgery at The Royal Children's Hospital started an early academic mentoring program for medical students and junior doctors with the aim of fostering an interest in academic surgery. Methods Between 2005 and 2015, 37 medical students and junior doctors participated in research in the Department of Cardiothoracic Surgery at The Royal Children's Hospital. Each was given an initial project on which to obtain ethics approval, perform a literature review, data collection, statistical analysis, and prepare a manuscript for publication. A search of the names of these former students and doctors was conducted on PubMed to identify publications. Results A total of 113 journal articles were published in peer-reviewed journals with an average impact factor of 4.1 (range, 1.1 to 19.9). Thirty (30 of 37, 81%) published at least one article. A mean of 4.3 journal articles was published per student or junior doctor (range, 0 to 29). Eleven (11 of 37, 30%) received scholarships for their research. Nine (9 of 37, 24%) have completed or are enrolled in higher research degrees with a cardiothoracic surgery focus. Of these 9, 2 have completed doctoral degrees while in cardiothoracic surgery training. Five will complete their cardiothoracic surgery training with a doctoral degree and the other 2 are pursuing training in cardiology. Conclusions A successful early academic mentoring program in a busy cardiothoracic surgery unit is feasible. Mentoring of motivated individuals in academic surgery benefits not only their medical career, but also helps maintain high academic output of the unit.

Background Systemic-to-pulmonary shunts are useful palliative procedures, although many teams have been deterred by high mortality and morbidity. We aimed to identify predictors of adverse outcomes after shunts in biventricular lesions. Methods From 2004 to 2014, 173 children had shunt procedures. Morphologies included: tetralogy of Fallot, pulmonary atresia with ventricular septal defect (VSD) with and without major aortopulmonary collaterals (MAPCAs), transposition of great arteries with pulmonary stenosis, and double outlet right ventricle. Median age was 22 days (range, 3 to 3,438 days) and median weight 3.2 kg (range, 1.7 to 20 kg). Shunt sizes ranged from 3 to 5 mm with median shunt size/weight ratio 1.03 mm/kg (range, 0.3 to 2.5 mm/kg). Results In-hospital mortality was 5.2% for the initial shunt procedure. Inter-stage mortality was 3.6%. Overall, 86% of patients progressed to corrective surgery. Acute events were observed in 41 patients, leading to 6 deaths. Events included 30 emergency chest openings, 16 shunt thrombosis, and 17 pulmonary overcirculation. Independent predictors of acute events or in-hospital mortality were genetic or extracardiac anomalies (hazard ratio [HR] = 1.9, p = 0.04), and preoperative shock/acidosis (HR = 2.73, p = 0.003). Diagnosis of pulmonary atresia with VSD and MAPCAs was protective (HR = 0.23, p = 0.042). Weight, shunt size, and size/weight ratios were not significant risk factors. Shunt thrombosis occurred at a median 3 hours (range, 0 to 46 hours) postoperatively, leading to 33% of in-hospital deaths. Low postoperative oxygen saturation and higher platelet counts in intensive care were independent predictors of thrombosis ( p = 0.022, p = 0.005 respectively). Conclusions Early adverse events contribute to mortality and morbidity for the shunt procedure. Patients with lower oxygen saturation and higher platelet counts in the postoperative period are at higher risk of shunt thrombosis.

OBJECTIVES: To determine the early and late outcomes of patients undergoing aortic arch reoperations. METHODS: The follow-up of 70 patients undergoing a second arch operation (excluding univentricular physiology) between 1979 and 2015 was reviewed. Median age at initial arch operation and second operation was 9 days (interquartile range: 5-35) and 10 months (interquartile range: 3-64), respectively. The most common indication for initial arch operation was coarctation in 79% (55/70). The most common indication for a second arch operation was arch reobstruction in 90% (63/70). RESULTS: There were 2 hospital deaths (2/70, 3%) and 3 early third arch operations (3/70, 4%). Late follow-up was available in 94% (64/68) of hospital survivors. After a mean of 9 ± 7 years, there were 5 late deaths (5/64, 8%). Fifteen-year survival was 90% (95% confidence interval: 75-96). Arch reobstruction (echocardiogram gradient >25 mmHg/third operation for reobstruction) was present in 28% (18/64) and 16% (10/64) required a third arch operation. Fifteen-year freedom from arch reobstruction and third arch operation was 63% (95% confidence interval: 43-78) and 74% (95% confidence interval: 52-87), respectively. On multivariable analysis, hypoplastic arch at initial arch repair (P = 0.03) and interposition graft at second arch operation (P

We aimed to determine the effect of elective left heart decompression at the time of initiation of central venoarterial extracorporeal membrane oxygenation (VA ECMO) on VA ECMO duration and clinical outcomes in children in a single tertiary ECMO referral center with a large pediatric population from a national referral center for pediatric cardiac surgery. We studied 51 episodes of VA ECMO in a historical cohort of 49 pediatric patients treated between the years 1990 and 2013 in the Paediatric Intensive Care Unit (PICU) of the Royal Children's Hospital, Melbourne. The cases had a variety of diagnoses including congenital cardiac abnormalities, sepsis, myocarditis, and cardiomyopathy. Left heart decompression as an elective treatment or an emergency intervention for left heart distension was effectively achieved by a number of methods, including left atrial venting, blade atrial septostomy, and left ventricular cannulation. Elective left heart decompression was associated with a reduction in time on ECMO (128 h) when compared with emergency decompression (236 h) (P = 0.013). Subgroup analysis showed that ECMO duration was greatest in noncardiac patients (elective 138 h, emergency 295 h; P = 0.02) and in patients who died despite both emergency decompression and ECMO (elective 133 h, emergency 354 h; P = 0.002). As the emergency cases had a lower pH, a higher PaCO2 , and a lower oxygenation index and were treated with a higher mean airway pressure, positive end-expiratory pressure, and respiratory rate prior to receiving VA ECMO, we undertook multivariate linear regression modeling to show that only PaCO2 and the timing of left heart decompression were associated with ECMO duration. However, elective left heart decompression was not associated with a reduction in length of PICU stay, duration of mechanical ventilation, or duration of oxygen therapy. Elective left heart decompression was not associated with improved ECMO survival or survival to PICU discharge. Elective left heart decompression may reduce ECMO duration and has therefore the potential to reduce ECMO-related complications. A prospective, randomized controlled trial is indicated to study this intervention further.

Background Dextrocardia is found in a significant proportion of patients undergoing a single-ventricle repair. Surgical outcomes in this cohort are unclear. Methods The records of 41 consecutive patients with single-ventricle physiology and dextrocardia presenting in a single institution from 1990 to 2008 were reviewed. Of this cohort, 19 patients had heterotaxy syndrome. Twenty-five of the 41 patients had atrioventricular valve regurgitation (AVVR) on presentation (mild, 13; moderate, 9; severe, 3). Results One patient died before intervention. Initial surgical palliation was performed in 31 patients. Four patients died postoperatively and 4 interim deaths occurred between initial palliation and bidirectional cavopulmonary shunt (BCPS). Thirty of the surviving 32 patients underwent BCPS, with 2 perioperative deaths. There were 4 additional deaths before Fontan surgery. Twenty-two of the surviving 25 patients underwent a Fontan procedure. There was 1 postoperative mortality. Survival to the age of 15 years was 56% (95% confidence interval [CI], 39%-70%). Patients with moderate or severe regurgitation had higher mortality if they were managed conservatively rather than by surgery (5 of 6 vs 2 of 6; P = .24). Patients with bilateral BCPS had better operative outcomes and survival compared with peers with unilateral anastomosis (odds ratio, 27; P = .005; 95% CI, 2.7-269). The side of the systemic venous pathway did not seem to influence outcomes. Conclusions Surgical outcomes of single-ventricle palliation seem poor in patients with dextrocardia. Aggressive management of congenital AVVR might improve the long-term prognosis.

Background The purpose of this study was to determine the rate of reinterventions from birth to adulthood after end-to-side anastomosis technique for interrupted and hypoplastic aortic arches. Multicenter data have shown that more than half of patients require arch reintervention in the 2 decades after repair. Methods The follow-up of 170 consecutive patients undergoing end-to-side repair through sternotomy for interrupted aortic arch (95) or coarctation (75) in one institution between 1985 and 2012 was reviewed. Associated lesions included ventricular septal defect (143) and bicuspid aortic valve (50). One-stage repair was performed in 158 patients (93%). Results There were 12 hospital deaths (7%), and 4 early arch reinterventions (2 for bronchial compression). Eleven hospital survivors (7%) were lost to follow-up. After a mean of 10 ± 6 years, there were 9 late deaths. Eighteen-year survival was 93% (95% confidence interval: 87 to 96). Eight patients had bronchial compression, 5 during initial stay and 3 after hospital discharge; 2 of them required surgery. Eighteen-year freedom from arch reoperation was 87% (95% confidence interval: 76 to 93). An additional 10 patients underwent balloon dilation, for an 18-year freedom from reintervention (balloon dilation or surgery) of 77% (95% confidence interval: 65 to 85). At last follow-up, 24 patients (16%) had an echocardiographic gradient greater than 25 mm Hg. Blood pressure was recorded in 105 patients, and only 11 (10%) were hypertensive. Conclusions The end-to-side anastomosis technique for repair of aortic arch lesions results in a low rate of mortality, arch reoperation, and late hypertension. The development of arch obstruction requiring balloon dilation warrants continuous follow-up of these patients.

OBJECTIVES: Partial atrioventricular septal defect (pAVSD) is routinely repaired with a low mortality. However, limited data are available on the long-term follow-up of these patients. The current study was designed to determine long-term survival and morbidity of a large cohort of patients operated on at a single institution. METHODS: From 1975 to 2012, 249 consecutive patients underwent pAVSD repair at the Royal Children’s Hospital. The follow-up data were obtained from hospital records, correspondence with cardiologists and primary care physicians, patient surveys and the state death registry. RESULTS: The early mortality rate was 1.2% (3/249), while the long-term survival rate was 96% (95% CI: 93–98%) at 10 years and 94% (95% CI: 89–97%) at 30 years. Freedom from reoperation was 84% at 10 years and 75% at 30 years. The most common reoperations were left atrioventricular valve surgery (30/249, 12.1%), resection of left ventricular outflow tract obstruction (12/249, 4.8%) and closure of residual atrial septal defects (5/249, 2.0%). Implantation of a permanent pacemaker was required in 3.2% (8/249) of patients. Despite a substantial reoperation rate, only 43% of patients older than 18 years of age were seen by a cardiologist within the most recent 2 years of the study period, compared with 80% of those younger than 18 years (P< 0.001). CONCLUSIONS: Repair of pAVSD is performed with a low mortality and excellent long-term survival. However, a substantial reoperation rate warrants close follow-up into adulthood.