Bilateral remote ischemic conditioning in children: A two-center, double-blind, randomized controlled trial in young children undergoing cardiac surgery.

Objective: The study objective was to determine whether adequately delivered bilateral remote ischemic preconditioning is cardioprotective in young children undergoing surgery for 2 common congenital heart defects with or without cyanosis. Methods: We performed a prospective, double-blind, randomized controlled trial at 2 centers in the United Kingdom. Children aged 3 to 36 months undergoing tetralogy of Fallot repair or ventricular septal defect closure were randomized 1:1 to receive bilateral preconditioning or sham intervention. Participants were followed up until hospital discharge or 30 days. The primary outcome was area under the curve for high-sensitivity troponin-T in the first 24 hours after surgery, analyzed by intention-to-treat. Right atrial biopsies were obtained in selected participants. Results: Between October 2016 and December 2020, 120 eligible children were randomized to receive bilateral preconditioning (n = 60) or sham intervention (n = 60). The primary outcome, area under the curve for high-sensitivity troponin-T, was higher in the preconditioning group (mean: 70.0 ± 50.9 µg/L/h, n = 56) than in controls (mean: 55.6 ± 30.1 µg/L/h, n = 58) (mean difference, 13.2 µg/L/h; 95% CI, 0.5-25.8; P = .04). Subgroup analyses did not show a differential treatment effect by oxygen saturations (pinteraction = .25), but there was evidence of a differential effect by underlying defect (pinteraction = .04). Secondary outcomes and myocardial metabolism, quantified in atrial biopsies, were not different between randomized groups. Conclusions: Bilateral remote ischemic preconditioning does not attenuate myocardial injury in children undergoing surgical repair for congenital heart defects, and there was evidence of potential harm in unstented tetralogy of Fallot. The routine use of remote ischemic preconditioning cannot be recommended for myocardial protection during pediatric cardiac surgery.

Esophago-Vascular Fistulae in Children: Five Survivors, Literature Review, and Proposal for Management.

INTRODUCTION: Esophago-vascular fistulae in children are almost uniformly fatal with death occurring by exsanguination. We present a single centre series of five surviving patients, a proposal for management and literature review. MATERIALS AND METHODS: Patients were identified from surgical logbooks, surgeon recollection and discharge coding data. Demographics, symptoms, co-morbidities, radiology, management and follow up details were recorded. RESULTS: Five patients (1M, 4F) were identified. Four were aorto-esophageal and one caroto-esophageal. Median age at initial presentation was 44 (8-177) months. Four patients had cross sectional imaging prior to surgery. Median time from presentation to combined entero-vascular surgery was 15 (0-419) days. Four patients required repair on cardio-pulmonary bypass with four undergoing staged surgical procedures. All required combined esophageal and cardio-vascular surgery. Length of PICU stay following combined surgery was 4 (2-60) days and overall hospital stay was 53 (15-84) days. Median follow up was 51 (17-61) months. Two patients had esophageal atresia and trachea-esophageal fistula managed as neonates. Three had no co-morbidities. Four had esophageal foreign bodies:1 esophageal stent, 2 button batteries, 1 chicken bone. One patient had a complication following colonic interposition. Four patients required an esophagostomy at the time of definitive surgery. All patients were alive and well at last follow up with one having successful reconnection surgery. CONCLUSION: In this series, outcomes were favourable. Multidisciplinary discussion and surgery are mandatory. If hemorrhage is controlled at presentation, then survival to discharge is possible but the magnitude of surgical intervention is both significant and very high risk. LEVEL OF EVIDENCE: Level 3.

Outcomes of the arterial switch for transposition during infancy using a standardized approach over 30 years.

OBJECTIVES: The aim of this study was to describe the early and late outcomes of the arterial switch for transposition. METHODS: A single-centre retrospective cohort study was conducted to assess the early and late outcomes of arterial switch performed during infancy using a standardized institutional approach between 1988 and 2018, compared by morphological groups. RESULTS: A total of 749 consecutive patients undergoing arterial switch during infancy were included, 464 (61.9%) with intact septum, 163 (21.8%) with isolated ventricular septal defect and 122 (16.3%) with complex transposition with associated lesions, including 67 (8.9%) with Taussig-Bing anomaly. There were 34 early deaths [4.5%, 95% confidence interval (CI) 3.1-6.1] with only 10 (2.6%) early deaths since 2000. Complex morphology (odds ratio 11.44, 95% CI 4.76-27.43) and intramural coronary artery (odds ratio 5.17, 95% CI 1.61-15.91) were identified as the most important risk factors for 90-day mortality. Overall survival was 92.7% (95% CI 90.8-94.6) at 5 years and 91.9% (95% CI 89.9-94.1) at 20 years; in hospital survivors, there were 15 (2.1%) late deaths during a median follow-up of 13.7 years. Cumulative incidence of surgical or catheter reintervention was 16.0% (95% CI 14.5-17.5) at 5 years and 22.7% (95% CI 21.0-24.0) at 20 years; early and late reinterventions were more common in the complex group, with no difference between the other groups. CONCLUSIONS: Using a standardized approach, the arterial switch can be performed with low early mortality, moderate rates of reintervention and excellent long-term survival. Concomitant lesions were the most important risk factor for early death and were associated with increased risk of late reintervention.

Long-term milrinone therapy in children with dilated cardiomyopathy.

AIM: The aim of this study was to assess the safety and efficacy of long-term milrinone therapy in children with acute decompensated heart failure due to dilated cardiomyopathy (DCM). METHODS: A single-centre retrospective study of all children ≤18 years with acute decompensated heart failure and DCM who received continuous long-term (≥7 consecutive days) intravenous milrinone between January 2008 and January 2022. RESULTS: The 47 patients had a median age of 3.3 months [interquartile range (IQR) 1.0-18.1], weight of 5.7 kg [IQR 4.3-10.1] and fractional shortening of 11.9% [±4.7]. Idiopathic DCM (n = 19) and myocarditis (n = 18) were the most common diagnoses. The median milrinone infusion duration was 27 days [IQR 10-50, range 7-290]. No adverse events necessitated milrinone termination. Nine patients required mechanical circulatory support. Median follow-up was 4.2 years [IQR 2.7-8.6]. On initial admission, four patients died, six were transplanted and 79% [37/47] were discharged home. The 18 readmissions resulted in five more deaths and four transplantations. Cardiac function recovered in 60% [28/47], as measured by normalised fractional shortening. CONCLUSION: Long-term intravenous milrinone is safe and effective in paediatric acute decompensated DCM. Combined with conventional heart failure therapies, it can act as a bridge to recovery and thereby potentially reduce the need for mechanical support or heart transplantation.

Common arterial trunk in functionally univentricular hearts: a case series.

INTRODUCTION: The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. METHODS: The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. RESULTS: Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. CONCLUSIONS: Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.

Pulmonary artery banding: still a role for staged bi-ventricular repair of intracardiac shunts?

OBJECTIVES: Although pulmonary artery banding remains a useful palliation in bi-ventricular shunting lesions, single-stage repair holds several advantages. We investigate outcomes of the former approach in high-risk patients. METHODS: Retrospective cohort study including all pulmonary artery banding procedures over 9 years, excluding single ventricle physiology and left ventricular training. RESULTS: Banding was performed in 125 patients at a median age of 41 days (2-294) and weight of 3.4 kg (1.8-7.32). Staged repair was undertaken for significant co-morbidity in 81 (64.8%) and anatomical complexity in 44 (35.2%). The median hospital stay was 14 days (interquartile range 8-33.5) and 14 patients (11.2%) required anatomical repair before discharge. Nine patients died during the initial admission (hospital mortality 7.2 %) and five following discharge (inter-stage mortality 4.8%). Of 105 banded patients who survived, 19 (18.1%) needed inter-stage re-admission and 18 (14.4%) required unplanned re-intervention. Full repair was performed in 93 (74.4%) at a median age of 13 months (3.1-49.9) and weight of 8.5 kg (3.08-16.8). Prior banding, 54% were below the 0.4th weight centile, but only 28% remained so at repair. Post-repair, 5/93 (5.4%) developed heart block requiring permanent pacemaker, and 11/93 (11.8%) required unplanned re-intervention. The post-repair mortality (including repairs during the initial admission) was 6/93 (6.5%), with overall mortality of the staged approach 13.6% (17/125). CONCLUSIONS: In a cohort with a high incidence of co-morbidity, pulmonary artery banding is associated with a significant risk of re-intervention and mortality. Weight gain improves after banding, but heart block, re-intervention, and mortality remain frequent following repair.

Four right ventricle to pulmonary artery conduit types.

OBJECTIVE: The most durable valved right ventricle to pulmonary artery conduit for the repair of congenital heart defects in patients of different ages, sizes, and anatomic substrate remains uncertain. METHODS: We performed a retrospective analysis of 4 common right ventricle to pulmonary artery conduits used in a single institution over 30 years, using univariable and multivariable models of time-to-failure to analyze freedom from conduit dysfunction, reintervention, and replacement. RESULTS: Between 1988 and 2018, 959 right ventricle to pulmonary artery conduits were implanted: 333 aortic homografts, 227 pulmonary homografts, 227 composite porcine valve conduits, and 172 bovine jugular vein conduits. Patients weighed 1.6 to 98.3 kg (median 15.3 kg), and median duration of follow-up was 11.4 years, with 505 (52.2%) conduits developing dysfunction, 165 (17.2%) requiring catheter intervention, and 415 (43.2%) being replaced. Greater patient weight, conduit z-score, type and position, as well as catheter intervention were predictors of freedom from replacement. Multivariable analysis demonstrated inferior durability for smaller composite porcine valve conduits, with excellent durability for larger diameter conduits of the same type. Bovine jugular vein conduit longevity was inferior to that of homografts in all but the smallest patients. Freedom from dysfunction at 8 years was 60.7% for aortic homografts, 72% for pulmonary homografts, 51.2% for composite porcine valve conduits, and 41.3% for bovine jugular vein conduits. Judicious oversizing of the conduit improved conduit durability in all patients, but to the greatest extent in patients weighing 5 to 20 kg. CONCLUSIONS: Pulmonary and aortic homografts had greater durability than xenograft conduits, particularly in patients weighing 5 to 20 kg. Judicious oversizing was the most significant surgeon-modifiable factor affecting conduit longevity.

Stenting of the right ventricular outflow tract as an initial intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries.

OBJECTIVES: To assess the role of right ventricular outflow tract stenting as the primary intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries. BACKGROUND: The management of a subset of infants with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries requires a staged approach including rehabilitation of diminutive native pulmonary arteries, conventionally using an aortopulmonary shunt. We share our experience of pulmonary artery rehabilitation with right ventricular outflow tract stenting. METHODS: Retrospective review of all patients with Tetralogy of Fallot with pulmonary stenosis who underwent right ventricular outflow tract stenting as primary intervention over an 8-year period. RESULTS: Ten patients underwent right ventricular outflow tract stent insertion at a median age of 61 days (interquartile range (IQR) 8.3-155 days). Median weight at stent deployment was 4.2 kg (IQR 3.2-5.7 kg). Oxygen saturations improved from a median of 79% (IQR 76-80%) to 92% (IQR 90-95%), p = 0.012. The median right and left pulmonary artery z score increased from -3.51 (IQR -4.59 to -2.80) and -2.07 (IQR -3.72 to 0.15) to a median of -1.17 (IQR -2.26 to 0.16) p < 0.05, and 0.24 (IQR -1.09 to 1.84) p < 0.05, respectively, at subsequent angiogram. Nine patients underwent further catheterisation. Four patients underwent complete anatomical repair. Only one patient required unifocalisation, as most patients had a native supply to all-important lung segments. CONCLUSION: Right ventricular outflow tract stenting is a useful procedure in the subset of patients with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries, where native pulmonary arterial growth is required to facilitate repair.

Short to medium term outcomes of right ventricular outflow tract stenting as initial palliation for symptomatic infants with complete atrioventricular septal defect with associated tetralogy of Fallot.

OBJECTIVES: To assess the impact of right ventricular outflow tract (RVOT) stenting as the primary palliation in infants with complete atrioventricular septal defect with associated tetralogy of Fallot (cAVSD/TOF). BACKGROUND: Historically, palliation of symptomatic patients with cAVSD/TOF has been achieved through surgical systemic to pulmonary artery shunting. More recently RVOT stenting has evolved as an acceptable alternative in patients with tetralogy of Fallot. METHODS: Retrospective review of all patients with cAVSD/TOF who underwent RVOT stenting as palliation over a 13-year period from two large tertiary referral centers. RESULTS: Twenty-six patients underwent RVOT stenting at a median age of 57 days (interquartile range [IQR] 25.5-106.5). Median weight for stent deployment was 3.7 kg (IQR 2.91-5.5 kg). RVOT stenting improved oxygen saturations from a median of 72% (IQR 70-76%) to 90% (IQR 84-92%), p < .001. There was a significant increase in the median Z-score for both branch pulmonary arteries at median follow-up of 255 days (IQR 60-455). Eight patients required RVOT stent balloon dilatations and 8 patients required re-stenting for progressive desaturation. The median duration between reinterventions was 122 days (IQR 53-294 days). Four patients died during the follow-up period. No deaths resulted from the initial intervention. To date, definitive surgical intervention was achieved in 19 patients (biventricular repair n = 15) at a median age of 369 days (IQR 223-546 days). CONCLUSION: RVOT stenting in cAVSD/TOF is a safe and effective palliative procedure in symptomatic infants, promoting pulmonary artery growth and improving oxygen saturations.

Strategies to Minimise Need for Prosthetic Aortic Valve Replacement in Congenital Aortic Stenosis-Value of the Ross Procedure.

To examine the role and outcomes of all interventions for aortic stenosis in children, with focus on freedom from reintervention and the aim to minimise prosthetic aortic valve replacement (pAVR) during childhood. Retrospective analysis of 194 consecutive children who underwent any aortic valve intervention for a biventricular repair strategy at a single institution between 1995 and 2017. Data were obtained from hospital records and follow-up was 100% complete. Over a 22-year period, 194 children underwent total 313 aortic valve procedures: Primary interventions were surgical valvotomy (SV)/surgical repair (SR) in 94 (48.5%), balloon valvuloplasty (BV) in 60 (30.9%), pAVR in 8 (4.1%) and Ross/Ross-Konno procedure in 32 (16.5%). Median age at first intervention was 1.1 years (interquartile range [IQR] 0.1-9.4) and varied with type of intervention: SV/SR were most common in neonates (33, 75%) and infants (35, 68%), whilst BV was most frequent in older children (42, 42%). Operative survival was 99% (2 early deaths, both in neonates with critical aortic stenosis and poor left ventricular function) and 15-year survival was 95%. A Ross procedure was performed in 79 (40.7%) patients over the 15-year study period, 1 of whom required late pAVR for autograft failure. Freedom from any reintervention after SV/SR and BV was 41% and 40% at 10 years, compared to 90% at 10 years with the Ross procedure (P < 0.001). Among neonatal SV/SR and BV, 98% required reintervention during childhood with no difference between groups. Valve morphology did not influence freedom from ultimate valve replacement. In patients who went on to have a Ross procedure, median time from initial intervention to Ross was 2.8 years (IQR 0.1-11.9) in neonates and 6.0 years (IQR 3.1-7.5) in all other age groups. Overall freedom from pAVR was 97% at 10 years and was similar in the SV/SR and BV groups. A strategy of simple valve repair and primary Ross procedure provides excellent survival and good freedom from pAVR. However, reintervention rates after simple interventions for congenital AS are high, especially in younger age groups. The Ross procedure offers the best freedom from reintervention of any technique and wider use of primary Ross in younger age groups should be considered.

An Unusual Combination of Double Inlet Left Ventricle With Discordant Ventriculoarterial Connections and Bilateral Arterial Ducts.

The presence of bilaterally persistent arterial ducts is an uncommon abnormality. Here, we describe the anatomy and successful management of an unusual patient with bilateral ducts in the setting of double inlet left ventricle, discordant ventriculoarterial connections, aortic atresia, and a severely hypoplastic and serpentine aortic arch.

Unifocalization cannot rely exclusively on native pulmonary arteries: the importance of recruitment of major aortopulmonary collaterals in 249 cases†.

OBJECTIVES: We sought to define the early and late outcomes of unifocalization based on a classification of the native pulmonary artery (nPA) system and major aortopulmonary collateral arteries (MAPCAs) with a policy of combined recruitment and rehabilitation and to analyse the role of unifocalization by leaving the ventricular septal defect (VSD) open with a limiting right ventricle-pulmonary artery (RV-PA) conduit in borderline cases. METHODS: An analysis of 271 consecutive patients assessed for unifocalization at a single institution between 1988 and 2016 was performed. Patients were classified according to the pulmonary blood supply: group A, unifocalization based on nPA only; group B, based on nPA and MAPCAs; group C, MAPCAs only (absent nPAs). RESULTS: Unifocalization was achieved in 249 (91.9%) cases with an early mortality of 2.8%. Group A included 72 (28.9%) patients, group B 119 (47.8%) patients and group C 58 (23.3%) patients with no difference in early survival between groups. Survival at 5, 10 and 15 years was 90.0% (85.9-94.3), 87.2% (83.5-91.2) and 82.3% (75.2-89.9), respectively. Late survival in groups A and B was similar but 10- and 15-year survival in group C decreased to 79.2% (68.2-92.1) and 74.3% (61.1-90.4) (P = 0.02), respectively. A mean of 1.9 (±0.6) MAPCAs were recruited per patient (range 0-6). The VSD was left open with a limiting RV-PA conduit in 97 (39.0%) cases, but subsequently closed in 48 patients, giving a total of 200 (80.3%) patients achieving VSD closure (full repair). Delaying VSD closure was not associated with increased risk for early or late survival. A central shunt to rehabilitate the nPAs was used in 56 (22.5%) cases. This was associated with a reduction in the number of MAPCAs recruited, but still required a mean of 1.8 (±0.5) MAPCAs recruited per patient to achieve unifocalization. In multivariate risk analysis, those suitable for single-stage full repair had the best long-term outcomes. Group C anatomy was associated with poor late survival compared to groups A and B (hazard ratio 2.7). CONCLUSIONS: Survival is maximized by a combined approach of rehabilitation and recruitment. MAPCAs should always be recruited if they supply areas with absent nPA supply. A strategy of leaving the VSD open with a limiting RV-PA conduit is a safe and effective way of managing borderline cases.

Fate of the Left Ventricular Outflow Tract After Rastelli With Selective Infundibular Muscle Resection.

BACKGROUND: The Rastelli procedure has been criticized for a high rate of left ventricular outflow tract obstruction (LVOTO) and translocation procedures have been proposed as an alternative. Infundibular resection can be performed during Rastelli to optimize the outflow tract. This study examines whether a selective policy to enlarge the outflow tract improves Rastelli outcomes across all morphological variants. METHODS: Single institution 29-year experience in 105 patients. Patients were classified into transposition of the great arteries with ventricular septal defect, congenitally corrected transposition of the great arteries with ventricular septal defect, and double outlet right ventricle morphology groups. The infundibular muscle was routinely resected if prominent, in 28 cases. RESULTS: Early mortality was 1.9% (2 of 105) and actuarial survival was 95.4% (95% confidence interval [CI], 89% to 99%) at 1 year, 92.9% (95% CI, 85% to 97%) at 5 years, and 84.5% (95% CI, 74% to 92%) at 10 years. The cumulative freedom from LVOTO was 99% (95% CI, 96% to 100%), 97% (95% CI, 92% to 99%), and 90% (95% CI, 88% to 96%) at 1, 5, and 10 years, respectively. The incidence was similar in all morphological groups and those undergoing infundibular resection were not at higher risk of late LVOTO. Eleven patients required surgical reoperation on the left ventricular outflow tract over a median follow-up period of 8.5 years, with no mortality, although 2 of these patients developed complete heart block. Left ventricular function was well preserved in 98.1% of all cases, including all of those requiring left ventricular outflow tract reoperation. CONCLUSIONS: The Rastelli is a safe procedure that can be applied in a variety of morphological variants. LVOTO remains a late complication of Rastelli, but can be minimized by concomitant infundibular muscle resection. Late reoperation is safe and ventricular function is well preserved in greater than 95% of cases at late follow-up. The operation has stood the test of time and avoids many of the risks of translocation procedures.

Approaches to Pulmonary Atresia With Major Aortopulmonary Collateral Arteries.

Pulmonary atresia with major aortopulmonary collateral arteries (MAPCAs) is one of the most challenging surgical conditions to manage-not only because of the technical complexity of the surgery but also in terms of defining the anatomy of the pulmonary vasculature, the timing of surgery, and decision making on staged vs complete repair. The importance of early definition of pulmonary blood supply is paramount, establishing which areas of the lung are supplied by MAPCAs alone and which have dual supply with the native system (noting that 20% of patients have absent intrapericardial native vessels). Early unifocalization (3-6 months) is ideal, with closure of the ventricular septal defect (VSD) performed if 15 or more out of 20 lung segments can be recruited. Leaving the ventricular septal defect open with a limiting right ventricle-pulmonary artery conduit can be a useful interim or even definitive circulation in patients with borderline vasculature. Rehabilitation of small native vessels with central shunts can be very effective, but best outcomes are achieved by a combination of unifocalization of MAPCAs together with the native vessels (if present). A variety of reconstructive techniques are necessary to be able to effect these complex repairs with careful choice of materials. Ideally, surgery can be completed through sternotomy alone, but separate thoracotomies may be necessary to control and access some MAPCAs.

The importance of age and weight on cavopulmonary shunt (stage II) outcomes after the Norwood procedure: Planned versus unplanned surgery.

OBJECTIVE: The study objective was to evaluate the outcomes of the cavopulmonary shunt after the Norwood procedure with a particular focus on age, weight, and whether surgery was planned or expedited by clinical findings. METHODS: We studied 297 consecutive patients with hypoplastic left heart syndrome undergoing the cavopulmonary shunt operation between 2002 and 2014. All patients underwent the Norwood procedure with a right ventricle to pulmonary artery conduit. Unplanned surgery was any situation in which surgery was expedited because of refractory desaturation, angiographic findings, or failure to discharge. RESULTS: There were 222 planned and 75 unplanned procedures. Planned surgery was performed at median of 5.4 (4.6-6.3) months compared with 3.9 (3.3-5.0) months for the unplanned group (P < .001). Early mortality was 1.3% in the planned group compared with 5.3% in the unplanned group (P = .07). At 5 and 10 years, survival was significantly worse in the unplanned group (73% vs 85%, P = .03). On multivariable analysis, atrioventricular valve regurgitation, the need for atrioventricular valve repair, and impaired ventricular function (graded composite variable) were the predominant risk factors (hazard ratio [HR], 7.49), with an incremental risk within these 3 variables. The planned versus unplanned nature of the surgery was less significant (HR, 1.75) because it was eclipsed by ventricular function and tricuspid intervention. Additional factors were aortic atresia/mitral atresia subgroup (HR, 2.07) and somatic growth (z-score weight) at the time of surgery (HR, 0.60 for z-score change from -2.54 to -1.16). Age and weight at surgery were not significant risk factors for survival. Interstage interventions on the aortic arch or right ventricle to pulmonary artery conduit did not adversely influence outcomes. CONCLUSIONS: The ventricular function, atrioventricular valve regurgitation, and need for tricuspid intervention were the primary risk factors for survival after the cavopulmonary shunt operation. Although unplanned surgery is associated with poor outcomes, this is related to the primary risk factors and not the timing or age of the patient. Somatic growth also has a significant influence on survival. Thus, an earlier cavopulmonary shunt operation is safe in infants who are thriving, but those with poor weight gain are at higher risk.

Extracorporeal membrane oxygenator support in infants with systemic-pulmonary shunts.

BACKGROUND: Management of a patent systemic-pulmonary (SP) shunt and the resulting runoff during extracorporeal membrane oxygenation (ECMO) varies among institutions. We have used a strategy of increased flow without surgical reduction of the shunt diameter, and here report our results with this strategy. METHODS: In this database review of 169 successive veno-arterial ECMO runs performed between 2002 and 2013 in infants and neonates, ECMO flow, time to achieve lactate clearance, normal pH, and negative fluid balance were compared in patients with shunts and those without shunts. RESULTS: Fifty-one of 169 infants (30.2%) had a shunt in situ when ECMO was initiated. Significantly higher ECMO flows were maintained in the shunt group compared with the nonshunt group (161 ± 43 mL/kg/minute vs 134 ± 41 mL/kg/minute; P < .001). Infants with shunts had significantly higher pre-ECMO and peak lactate levels (12.4 ± 5.6 mmol/L vs 10.0 ± 6.3 mmol/L; P < .05 and 13.7 ± 4.9 mmol/L vs 11.6 ± 5.5 mmol/L; P < .02, respectively) and required a longer period of support for clearance (median, 28.8 hours [16.1-63.3 hours] vs 17.5 hours [10.8-34.5 hours]; P < .001). Although the absolute rate of lactate clearance was not significantly different between the 2 groups (median, 0.46 mmol/L/hour [0.12-0.72 mmol/L/hour] vs 0.48 mmol/L/hour [0.22-0.86 mmol/L/hour]; P = .139) the presence of a shunt, neonatal age, peak lactate, extracorporeal cardiopulmonary resuscitation, and the use of hemofiltration on ECMO significantly predicted the rate of clearance. Survival to hospital discharge was similar in the shunt and nonshunt groups (49.0% vs 48.3%; P = .932). CONCLUSIONS: A strategy of increased ECMO flow without surgically restricting shunt diameter appears to be successful in providing circulatory support in the majority of patients with an SP shunt. Equivalent survival suggests that routine surgical reduction of shunt diameter is not indicated.

Late outcomes of infants supported by extracorporeal membrane oxygenation following the Norwood operation.

BACKGROUND: Hospital survival for infants who require extracorporeal membrane oxygenation (ECMO) following the Norwood operation is 30% to 60%. However, little is known about late outcomes of hospital survivors and their ability to progress through subsequent palliative stages. METHODS: Between 2002 and 2012, 38 (13.4%) of the 284 neonates with hypoplastic left heart syndrome or other single ventricle variants received ECMO support following Norwood. We examined factors affecting hospital death and compared postdischarge events between hospital survivors who received postoperative ECMO (n = 16 of 38) and a control of hospital survivors who did not receive ECMO (220 of 246). RESULTS: Unplanned cardiac reoperation was the only predictor of postoperative ECMO requirement. Overall, 22 (58%) of the 38 patients were weaned from ECMO support and 16 (42%) of the 38 survived to hospital discharge. The ECMO duration was a significant factor for hospital mortality (odds ratio = 1.52 per 1-day increase [1.03-2.24], P = .035). Following discharge, 15 (94%) of the 16 underwent Glenn and 1 (6%) of the 16 had interstage mortality. In the control group, 194 (88%) of the 220 underwent Glenn and 26 (12%) of the 220 had interstage mortality or received transplantation (P = .499). Following Glenn, 3 (20%) of the 15 patients had interstage mortality or received transplantation and 12 (80%) of the 15 proceeded to Fontan or were alive awaiting Fontan. In the control group, 23 (12%) of the 194 had interstage mortality or received transplantation and 171 (88%) proceeded to Fontan or were alive awaiting Fontan (P = .357). Overall, 81% of hospital survivors were alive 5 years following discharge in both ECMO and non-ECMO groups. CONCLUSIONS: ECMO support following Norwood is associated with high probability of hospital death. Nonetheless, interstage mortality, progression to subsequent palliative stages, intermediate survival, and freedom from heart transplantation are comparable to those in patients who did not require postoperative ECMO support.

The impact of mechanical circulatory support on outcomes in paediatric heart transplantation.

OBJECTIVES: Internationally, the number of donors for cardiac transplantation has remained static, while the number of patients requiring transplantation for congenital heart disease (CHD) has increased. Although the availability of mechanical circulatory support (MCS) may increase the number of transplants performed by reducing deaths while waiting, it may also lead to increased morbidity post-transplantation. We sought to assess the impact of mechanical support on post-transplant outcomes in a single centre. METHODS: We assessed the outcomes of paediatric (age ≤16 years) heart transplantation in a single unit in the era of mechanical support (1998-2012) by retrospective cohort study. Outcomes before (1998-2005) and after (2005-2012) the routine use of the Berlin Heart EXCOR device were contrasted. RESULTS: A total of 167 patients underwent heart transplantation during this period. The diagnosis was dilated cardiomyopathy in 61.7%, two-ventricle CHD in 11.4%, single ventricle CHD in 16.8% and miscellaneous in 10.1%. Sixty-nine (41%) were bridged to transplant by mechanical support; with extracorporeal membrane oxygenation in 19 (28%), ventricular assist device in 40 (58%) and a combination in 10 (14.0%). Post-transplant mortality at 30 days was significantly greater in those supported by MCS than without (7 vs 1%, P < 0.05), and a greater proportion of patients had neurological (23 vs 8%, P < 0.01) and major respiratory sequelae (20 vs 4%, P < 0.001). There was no significant increase in the need for post-transplant mechanical support (10 vs 6%, P = 0.3) in those supported prior to transplant. The number of transplants performed increased from 67 in 1998-2005 to 100 in the most recent era (2005-2012), and an increased proportion of these patients have been supported mechanically prior to transplantation (51 vs 27%, P < 0.01). CONCLUSION: Along with strategies to increase donor utilization, MCS has allowed an increase in cardiac transplant activity at the expense of a higher early mortality and morbidity.

Modified technique for the implantation of berlin heart excor ventricular assist device in children.

The Berlin Heart Excor (BHE) assist device has become our standard mechanical support device for long-term support in children with heart failure. We report two useful surgical modifications for the implantation of the BHE in the pediatric population, improving ease of implantation as well as subsequent surgical procedures at the time of explantation or transplantation. The first modification entails the use of a polytetrafluoroethylene graft for cannulation via the innominate artery for arterial perfusion during cardiopulmonary bypass. This graft is preserved and reused for institution of bypass at the time of transplantation or explantation of the device after recovery. The second modification consists of an extension of the BHE arterial cannula using a length of knitted polyester graft. This allows for improved positioning of the arterial cannula in the ascending aorta or main pulmonary artery, facilitates vascular anastomosis, and improves hemostasis.