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As an essential component of the sarcomere, actin thin filament stems from the Z-disk extend toward the middle of the sarcomere and overlaps with myosin thick filaments. Elongation of the cardiac thin filament is essential for normal sarcomere maturation and heart function. This process is regulated by the actin-binding proteins Leiomodins (LMODs), among which LMOD2 has recently been identified as a key regulator of thin filament elongation to reach a mature length. Few reports have implicated homozygous loss of function variants of LMOD2 in neonatal dilated cardiomyopathy (DCM) associated with thin filament shortening. We present the fifth case of DCM due to biallelic variants in the LMOD2 gene and the second case with the c.1193G>A (p.W398*) nonsense variant identified by whole-exome sequencing. The proband is a 4-month male infant of Hispanic descent with advanced heart failure. Consistent with previous reports, a myocardial biopsy exhibited remarkably short thin filaments. However, compared to other cases of identical or similar biallelic variants, the patient presented here has an unusually late onset of cardiomyopathy during infancy. Herein, we present the phenotypic and histological features of this variant, confirm the pathogenic impact on protein expression and sarcomere structure, and discuss the current knowledge of LMOD2-related cardiomyopathy.

Background Common arterial trunk (CAT) is a rare anomaly with a spectrum of pathology. We sought to identify current trends and factors associated with postnatal outcomes. Methods This was a single-centre review including 153 live births with planned surgery. Patients were analyzed as 2 cohorts based on era of CAT diagnosis (1990 to 1999 vs 2000 to 2014) and complexity of disease (simple vs complex). “Complex” required the association with significant aortic arch obstruction, truncal valve (TV) stenosis/regurgitation, and/or branch pulmonary artery (PA) hypoplasia, respectively. Results Sixteen (10%) died preoperatively, and this outcome was associated with significant TV stenosis (odds ratio [OR] 4.55; P = 0.01) and regurgitation (OR 3.17; P = 0.04); 130 (95%) of 137 operated infants underwent primary complete repair. Their survival rates to 1 year improved from 54% to 85% after 2000, although this outcome remained substantially lower for cases with a complex vs simple CAT repair (76% vs 95%; OR 6.46; P = 0.006). Other risk factors associated with decreased 1-year survival included diagnosis before 2000 (OR 4.48; P = 0.038) and a lower birth weight (OR 8.0 per kg weight; P = 0.001). Finally, of 93 survivors beyond year 1 of life, 76 (82%) had undergone a total of 224 reinterventions. Only 15 (16%) were alive without any surgical or catheter-based reintervention at study end. Conclusions Despite recent surgical improvements, postnatal mortality continues to be substantial if CAT is complicated by significant pathology of the TV, aortic arch, or branch PAs. Reoperations and catheter interventions are eventualities for most patients during childhood.

BACKGROUND Hyperglycemia is common following cardiopulmonary bypass (CPB) surgery and is associated with poor outcomes, often attributed to hyperinsulinemia and an acquired state of insulin resistance. This study examined the underpinnings of hyperglycemia and the effects of nutrition on the association with inflammation and clinical outcomes. METHODS This prospective, observational cohort study enrolled consecutive children (

We hypothesized that preoperative patient characteristics and branch pulmonary artery (PA) size might influence the rate of postoperative branch PA reintervention in patients with transposition of the great arteries who undergo the arterial switch operation (ASO).The retrospective single-center study included 262 consecutive (2008-2017) newborns who underwent the ASO. Demographic characteristics, echocardiography, and clinical outcomes were reviewed. Competing risk analysis modeled incidence of branch PA reintervention and cause-specific hazard regression for predictors analyses.Median age and weight were 7 (range, 5-11) days and 3.4 (range, 3.1-3.8) kg, respectively. Various types of early branch PA reinterventions (concomitant revision or reintervention during the intensive care unit stay) were required in 28 (10.7%) patients. These patients had prolonged ventilation (P .001), intensive care unit duration (P .001), worse right ventricular function (P = .043), and high in-hospital mortality (P = .010). Branch PA dimensions significantly decreased immediately after ASO compared with baseline measurements. The median follow-up duration was 20.8 (range, 0.9-44.7) months. Branch PA reintervention was common among survivors without early reinterventions (9.4%), and even more frequent among those with early reinterventions (25%). Subsequent reintervention (all catheter-based) was necessary for more than one-third of patients after initial branch PA reintervention. The multivariable analysis showed preoperative dimension of the left PA (hazard ratio, 0.527 [95% CI, 0.337-0.823]; P = .005), and right PA (hazard ratio, 0.503 [95% CI, 0.318-0.796]; P = .003) were independently associated with late branch PA reinterventions.Branch PA reintervention was common and often required surgical or catheter-based reinterventions after ASO. PA branch diameters became significantly smaller after ASO. Smaller preoperative branch PA predicted late branch PA reintervention, indicating a smaller margin of geometrical tolerance to this effect.

Objectives To determine impact of enteral nutrition delivery on the relationship among inflammation, insulin resistance, and outcomes following pediatric cardiopulmonary bypass surgery. Design Pilot, randomized study analyzed according to intention-to-treat analysis. Setting Pediatric cardiac ICU. Patients Infants (≤ 6 mo) undergoing cardiopulmonary bypass. Interventions Patients randomly assigned to receive rapid escalation to enteral nutrition reaching goal feeds by 27 hours or standard feeding practice reaching goal feeds by 63 hours. Feeds were initiated on the first postoperative day. Measurements and main results Fifty patients were randomized equally to study arms. Patients were a median (interquartile range) of 16 days old (7-110 d old), undergoing biventricular surgery (88%) with a median cardiopulmonary bypass time of 125 minutes (105-159 min). Serial blood samples were drawn before and after cardiopulmonary bypass, cardiac ICU admission, and every 12 hours (up to 96 hr) for glucose, insulin, and cytokines (interleukin-1α, interleukin-6, interleukin-8, interleukin-10, and tumor necrosis factor-α) levels. Glucose-insulin ratio was calculated to quantify insulin resistance. Patient characteristics, time to enteral nutrition initiation, enteral nutrition interruptions, and insulin administration were similar across intervention arms. FF reached goal feeds at similar intervals as standard feeding (39 hr [30-60 hr] vs 60 hr [21-78 hr]; p = 0.75). No difference in cytokine, insulin, or glucose-insulin ratio was noted between groups. Higher inflammation was associated with increased glucose-insulin ratio and higher risk of adverse events. In multivariable models of interleukin-8, FF was associated with increased glucose-insulin ratio (estimate of effect [95% CI], 0.152 [0.033-0.272]; p = 0.013). Although higher interleukin-8 was associated with an elevated risk of adverse event, this relationship was possibly mitigated by FF (odds ratio [95% CI], 0.086 [0.002-1.638]; p = 0.13). Conclusions A FF strategy was not associated with changes to early enteral nutrition delivery. Inflammation, insulin resistance, and morbidity were similar, but FF may modify the relationship between inflammation and adverse event. Multicenter nutrition studies are possible and necessary in this vulnerable population.

We sought to evaluate the incidence of Fontan failure or complication and its relation to death in patients having contemporary Fontan strategies over 2 decades.Five hundred patients who underwent Fontan completion (extracardiac, n = 326; lateral tunnel, n = 174) from 1985 to 2012 were reviewed. Patient characteristics, modes of Fontan failure/complication and death, and predictors for Fontan failure/complication and death were analyzed.There were 23 early deaths (4.6%) and 17 late deaths (3.4%), with no early death since 2000. Survival has improved over time (p0.001). Twenty-three of 40 patients who died were identified as Fontan failure before death, including ventricular dysfunction (n = 14), pulmonary vascular dysfunction (n = 4), thromboembolism (n = 2), and arrhythmia (n = 4). Mode of death was circulatory failure (n = 18), multiorgan failure (n = 6), pulmonary failure (n = 3), cerebral/renal (n = 5), and sudden death (n = 4). Modes of failure/complication were directly (65%) or conceivably (10%) related to death in 30 of 40 patients (75%). Forty-eight percent of survivors had late Fontan complication(s). Five-year freedom from late Fontan complication was lower among patients who died compared with patients who survived (29.4% versus 53.3%, p0.001). Ventricular dysfunction (p = 0.001) and higher pulmonary artery pressures (p0.001) after Fontan were predictors for death. Longer cardiopulmonary bypass time (p = 0.032) and reinterventions (p0.001) were predictors for late Fontan complication.Early death in the early era has been overcome. Yet the incidence and causes of late death remain unchanged. There was a strong causative relationship between the mode of Fontan failure/complication and death, indicating the importance of early recognition and treatment of Fontan failure/complication.

Background: In infants requiring 3-stage single-ventricle palliation for hypoplastic left heart syndrome, attrition after the Norwood procedure remains significant. The effect of the timing of stage 2 palliation (S2P), a physician-modifiable factor, on long-term survival is not well understood. We hypothesized that an optimal interval between the Norwood and S2P that both minimizes pre-S2P attrition and maximizes post-S2P survival exists and is associated with individual patient characteristics. Methods: The National Institutes of Health/National Heart, Lung, and Blood Institute Pediatric Heart Network Single Ventricle Reconstruction Trial public data set was used. Transplant-free survival (TFS) was modeled from (1) Norwood to S2P and (2) S2P to 3 years by using parametric hazard analysis. Factors associated with death or heart transplantation were determined for each interval. To account for staged procedures, risk-adjusted, 3-year, post-Norwood TFS (the probability of TFS at 3 years given survival to S2P) was calculated using parametric conditional survival analysis. TFS from the Norwood to S2P was first predicted. TFS after S2P to 3 years was then predicted and adjusted for attrition before S2P by multiplying by the estimate of TFS to S2P. The optimal timing of S2P was determined by generating nomograms of risk-adjusted, 3-year, post-Norwood, TFS versus the interval from the Norwood to S2P. Results: Of 547 included patients, 399 survived to S2P (73%). Of the survivors to S2P, 349 (87%) survived to 3-year follow-up. The median interval from the Norwood to S2P was 5.1 (interquartile range, 4.1–6.0) months. The risk-adjusted, 3-year, TFS was 68±7%. A Norwood-S2P interval of 3 to 6 months was associated with greatest 3-year TFS overall and in patients with few risk factors. In patients with multiple risk factors, TFS was severely compromised, regardless of the timing of S2P and most severely when S2P was performed early. No difference in the optimal timing of S2P existed when stratified by shunt type. Conclusions: In infants with few risk factors, progressing to S2P at 3 to 6 months after the Norwood procedure was associated with maximal TFS. Early S2P did not rescue patients with greater risk factor burdens. Instead, referral for heart transplantation may offer their best chance at long-term survival. Clinical Trial Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT00115934.

Objective Patient-based congenital heart surgery (CHS) training is opportunity-based and difficult. Three-dimensional (3D) print models of the heart were used for hands-on surgical training (HOST) at the 2015 AATS and subsequently in 2 local institutions. We aim to introduce the process of 3D printing for surgical simulation and to present the attendee's responses. Methods Using CT or MR angiograms, the models of congenital heart disease were created and printed with flexible rubberlike material. Altogether, 81 established surgeons or trainees performed simulated surgical procedures with the expert surgeons' guidance and supervision. At the completion of the session, 50 of 81 attendees participated in the questionnaire assessment of the program. Results All responders found the course helpful in improving their surgical skills. All would consider including HOST sessions in the training programs. All found that the models showed the necessary pathologic findings. Most found that the consistency and elasticity of the model material were different from those of the human myocardium. However, the responders thought that the quality of the models was acceptable (88%) or manageable (12%) for surgical practice. The major weaknesses listed were related to the print material and poor representation of the cardiac valves. Conclusions HOST using 3D print heart models is achievable and allows surgical practice on pathological hearts without patients' risk. HOST is a highly applicable surgical simulation format for CHS. Incorporation of HOST in training programs could change the traditional opportunity-based education to the requirement-based standardized education.

Objective Poor survival following surgical palliation for hypoplastic left heart syndrome (HLHS) raises the question of the need for a paradigm shift. This is the first human study to investigate the possibility of primary "in-series" palliation in neonates and infants with HLHS in an acute setting with the aid of 2 types of mechanical assist: superior vena cava (SVC)–to–pulmonary artery (PA) pump assist and SVC–to–right atrium (RA) oxygenation assist. Methods By rearranging the cannula sites and flow rates for modified ultrafiltration, 2 types of mechanically assisted bidirectional cavopulmonary shunt (BCPS) circulation were simulated for 20 minutes. Three neonates undergoing a stage I Norwood procedure were assigned to SVC-PA pump assist, and 3 infants undergoing stage II BCPS were assigned to SVC-RA oxygenation assist. Hemodynamic parameters, blood gas values, and arterial (SaO 2 ) and regional cerebral tissue (rCTO 2 ) saturations were analyzed. Results All 6 patients completed the study without hemodynamic compromise. In the SVC-PA pump assist group, a mean arterial pressure >40 mm Hg was maintained. SVC pressure was lower ( P = .01) and cerebral perfusion pressure (CPP) was higher ( P = .03) during the last 10 minutes of assist compared with Norwood physiology. SaO 2 >80%, rCTO 2 >60%, and mixed venous saturation ≥59% were maintained, comparable to values with Norwood physiology. In the SVC-RA oxygenation assist group, with full or 50% support, mean blood pressure >50 mm Hg, SVC pressure 50%, and CPP >40 mm Hg were maintained, which were comparable to BCPS physiology. Conclusions Two types of mechanical assist to support primary in-series palliation are feasible in the acute setting. Both modes of mechanical assist maintained oxygenation, as well as systemic and cerebral perfusion.

Background It is unknown whether continuous cardiac resynchronization therapy (CRT) can lead to sustained improvement in hemodynamics after surgery for congenital heart disease (CHD). Objective We investigated whether CRT improves cardiac index (CI) and blood pressure in infants after biventricular repair of CHD. Methods We randomized infants younger than 4 months after biventricular CHD surgery to standard care or standard care plus CRT for 48 hours or until extubation if sooner. Change in the primary outcome of CI and blood pressure over time was compared between groups. For subgroup analysis, QRS duration was considered prolonged if greater than the 98th percentile. Results Forty-two patients were randomized: 21 controls and 21 patients receiving CRT (median weight 4 kg). There were no identified adverse events from pacing. The change in CI over time was not different between patients receiving CRT and controls, but trended toward improvement in patients with wide QRS who received CRT (n = 9) vs controls with wide QRS (n = 8) (+1.65 (0.86) L/(min·m2); P = .06). Controls with wide QRS experienced the smallest increase in CI (0.33 L/(min·m2)). Blood pressure was significantly higher in infants with wide QRS who received CRT than in controls (+7.14 (3.08) mm Hg; P = .02). Serum lactate level, catecholamine use, ventilation time, and length of intensive care unit stay were similar between the 2 groups. Conclusion CRT improved blood pressure and a trend toward higher CI in infants after repair of biventricular CHD with prolonged QRS duration. These findings warrant further study of CRT to improve postoperative recovery in infants with electrical dyssynchrony.

Articles, see p 581 and p 589 > Nothing is as powerful as an idea whose time has come. > > —Victor Hugo, Histoire d’un Crime (1852) On November 29, 1944, Dr Alfred Blalock performed the first anastomosis of the subclavian to the pulmonary artery in a child with cyanosis at the Johns Hopkins Hospital in Baltimore. This novel operation was supported by the laboratory work of Vivien Thomas and the encouragement of pediatric cardiologist Helen Taussig. The operation, thereafter called the Blalock-Taussig shunt (BTS), increased pulmonary blood flow (PBF) and relieved symptomatic cyanosis.1 This singular procedure ushered in the modern era of management of the child with complex congenital heart disease and became the foundation for all management strategies that followed. Although the procedure or 1 of its modifications (MBTS) has remained a stalwart for securing a source of pulmonary blood flow for >70 years, an appreciable mortality and morbidity remains.2 As such, alternative management strategies have been suggested and tested in lieu of primary repair.3 In 1991, Coe and Olley4 described a novel transcatheter approach to increase PBF by stenting the arterial duct. Although attractive, initial clinical applications were disappointing.5 However, with improved delivery and stent platforms paralleling an experience with ductal stenting in infants with dual-source PBF, the last decade has seen a number of clinical units apply the technique in single-source …

Background: Brain injury, impaired brain growth, and long-term neurodevelopmental problems are common in children with transposition of the great arteries. We sought to identify clinical risk factors for brain injury and poor brain growth in infants with transposition of the great arteries undergoing the arterial switch operation, and to examine their relationship with neurodevelopmental outcome. Methods: The brains of 45 infants with transposition of the great arteries undergoing surgical repair were imaged pre- and postoperatively using magnetic resonance imaging. Brain weight z scores were calculated based on brain volume and autopsy reference data. Brain injury scores were determined as previously described. Neurodevelopment was assessed at 18 months using the Bayley-III scores of infant development. The relationships between clinical variables, brain injury, perioperative brain growth, and 18-month Bayley-III scores were analyzed. Results: On preoperative imaging, moderate or severe white matter injury was present in 10 of 45 patients, whereas stroke was seen in 4 of 45. A similar prevalence of injury was seen on postoperative imaging, and we were unable to identify any clinical risk factors for brain injury. Brain weight z scores decreased perioperatively in 35 of 45 patients. The presence of a ventricular septal defect ( P =0.009) and older age at surgery ( P =0.007) were associated with impaired perioperative brain growth. When patients were divided into those undergoing surgery during the first 2 weeks of life (32/45) versus those being repaired later (13/45), infants repaired later had significantly worse perioperative brain growth (late repair postoperative brain weight z = –1.0±0.90 versus early repair z = –0.33±0.64; P =0.008). Bayley-III testing scores fell within the normal range for all patients, although age at repair ( P =0.03) and days of open chest ( P =0.03) were associated with a lower composite language score, and length of stay was associated with a lower composite cognitive score ( P =0.02). Conclusions: Surgery beyond 2 weeks of age is associated with impaired brain growth and slower language development in infants with transposition of the great arteries cared for at our center. Although the mechanisms underlying this association are still unclear, extended periods of cyanosis and pulmonary overcirculation may adversely impact brain growth and subsequent neurodevelopment.

We sought to identify modifiable factors to improve survival of neonatal biventricular repair by analyzing the cause of death and predictors of mortality and reintervention in the last 2 decades.Between 1995 and 2016, 991 consecutive neonates were included. The cohort was divided by era: era I was from 1995 to 1999, era II 2000 to 2007, and era III 2008 to 2016. The Kaplan-Meier method was used to estimate freedom from death and reintervention. Univariable and multivariable Cox regression was applied to assess predictors for mortality or reintervention in the contemporary cohorts (2000-2016).Median age was 8 days (range, 5-13), and median body weight at operation was 3.3 kg (range, 2.9-3.6). The most common diagnosis was transposition with intact ventricular septum (32%), followed by transposition with ventricular septal defect (14.5%), and simple left-to-right shunt lesion (10.9%). There was significant improvement in survival from era I to eras II and III but no difference between eras II and III (1 year: 82.1% vs 89.4% vs 89.6%, respectively; P.001). The most common cause of death was sudden death in eras I and III and cardiac in era II. Multivariable analysis revealed preoperative (P = .005)/postoperative (P.001) extracorporeal membrane oxygenation and postoperative renal replacement (P.001) as independent predictors for mortality. The reintervention rates were comparable between eras II and III (P = .53). Atrioventricular septal defects and common atrial trunk were identified as predictors for reintervention.Survival after neonatal biventricular repair remained unchanged. Preventing sudden death, myocardial protection, and minimizing residual lesions are potential targets to improve outcomes.

Background In this study we sought to determine whether preoperative superior vena cava (SVC) blood flow measured using cardiac magnetic resonance (CMR) predicts physiology and clinical outcome after bidirectional cavopulmonary shunt (BCPS). Methods The retrospective single-center study included 65 (2012-2017) patients who underwent BCPS. Preoperative CMR imaging, echocardiography, catheterization, and clinical outcomes were reviewed. SVC flow was measured using phase contrast CMR. The Kaplan-Meier method and Cox regression was used for BCPS takedown-free survival and predictor analyses. Results The absolute and indexed SVC flow was 0.5 (interquartile range [IQR], 0.4-0.7) L/min and 1.7 (IQR, 1.4-2.0) L/min/mm2 respectively, which was comparable with the SVC blood flow volume previously measured. The median age and body weight at BCPS was 6.5 (IQR, 5.5-8.5) months and 6.9 (IQR, 6.0-7.7) kg. After follow-up, at a median of 17.1 (IQR, 7.9-41.3) months, 14 patients (21.5%) underwent the Fontan completion and 40 (61.5%) with BCPS physiology were waiting for the Fontan completion. The 11 remaining patients (16.9%), included those who underwent takedown (n = 7; 10.8%) or died with a BCPS (n = 4; 6.2%). Severe hypoxia was the leading cause of mortality, directly accounting for two-thirds of deaths (66.6%; 6/9). The BCPS takedown-free survival was 96.8% at 6 months, and 79.9% at 3 years. Preoperative SVC blood flow was significantly positively correlated with early post-BCPS arterial saturation (P = .00). The multivariable analysis showed SVC flow was the only factor associated with BCPS failure (hazard ratio, 0.186; P = .04) among the predictors related to the pre-BCPS anatomy and physiology. Conclusions SVC blood flow might be as critically important as pulmonary artery anatomic and physiologic parameters in the evaluation of BCPS candidacy in the single-ventricle population.

Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare anomalies and data on outcomes after a fetal or neonatal EA/TVD diagnosis are conflicting.To examine the outcome and identify markers predictive of mortality, we reviewed our single-centre experience from 2000-2014. Variables were analyzed separately for cases diagnosed in utero without pregnancy termination and for all live-born patients.Of 47 fetal cases, 8 (17%) died in utero and 10 (21%) as neonates. Independent predictors associated with fetal demise included severe tricuspid regurgitation with a Doppler gradient40 mm Hg (odds ratio, 1.22 per mm Hg deduction; P = 0.003) and pulmonary regurgitation (odds ratio, 11.4; P = 0.03) at the baseline examination. A novel prognostic score (range, 0-10) combining the severity of 5 echocardiographic findings was independently associated with overall mortality (hazard ratio [HR], 1.39 per point increase; P = 0.01). Survival rates of 66 live births at 1 month, 1 year, and 5 years were 86%, 82%, and 80% respectively, and 75%, 60%, and 55% remained free from surgery at the same points in time. Factors associated with postnatal death in multivariate analysis included a younger gestational age at birth (HR per week, 1.59; P0.001), tricuspid annulus diameter (HR per z-score increase, 1.76; P = 0.004), and no pulmonary forward flow (HR, 4.63; P = 0.03).Our experience with fetal and neonatal EA/TVD shows better survival rates than previously reported. Mortality after a fetal diagnosis was significantly associated with hemodynamic changes indicative of a circular shunt, including pulmonary and tricuspid regurgitation severe enough to cause diastolic umbilical arterial flow reversal.

Background Aortic arch reconstruction is a challenging technical step in the Norwood operation or the comprehensive stage II operation. This study sought to analyze differences in aortic arch geometry and dimensions in patients undergoing Norwood or hybrid palliation. Methods Retrospective data collection included all patients who underwent Norwood or hybrid palliation at the Hospital for Sick Children in Toronto between 2007 and 2014; 139 patients were analyzed. Lateral angiograms obtained before stage II or comprehensive stage II and Fontan completion were measured at the level of the ascending aorta, transverse arch, isthmus, and descending aorta. Reintervention rate and type were assessed. Results Before stage II or comprehensive stage II hybrid procedures, patients had significantly larger descending aorta z-scores. Patients undergoing Norwood operations showed a significant increase in descending aorta z-scores before Fontan completion. Comparable dimensions (absolute and z-scores) were found at all points of measurements before Fontan completion. Geometry was similar in both groups but significantly different compared with normal dimensions. Reduction in aortic arch diameter happened almost solely in the segment between the transverse arch and the isthmus. Stent inclusion in patients undergoing hybrid procedures led to similar dimensions. Reintervention rates were very low (Norwood 9.6% vs hybrid 7.6%). Reintervention for excessive dimensions was as common as for recoarctation. Conclusions Aortic arch geometry and growth is not altered by palliation type. The increase in descending aorta diameter seen in patients undergoing Norwood operations is in accord with physiologic changes and may reflect catch-up growth. Reintervention rates are low and are not related to recoarctation alone.

Potential surgical strategies for hypoplastic branch pulmonary arteries (BPAs) during tetralogy of Fallot repair include (1) extensive patch augmentation to the hilum (PATCH), (2) limited extension arterioplasty to the proximal pulmonary artery (EXTENSION), or (3) leaving the native vessels unaugmented (NATIVE). We explored the effect of these strategies on reintervention and BPA growth.From 2000 to 2012, 434 children underwent complete tetralogy of Fallot repair. Risk-adjusted parametric models were used to analyze the risk of BPA reintervention for (1) all children, (2) children with BPAs of 4 mm or smaller, and (3) children with BPAs of 3 mm or smaller. Repeated-measures analysis of more than 2,000 echocardiograms was used to characterize postoperative BPA growth and right ventricular pressure by using nonlinear mixed models.Overall survival (99% [3 deaths]) was excellent. The 10-year freedom from BPA reintervention was 84%. In risk-adjusted models (including baseline BPA z-score), PATCH had a decreased freedom from reintervention (73%; p0.01) vs EXTENSION (87%) or NATIVE (91%). For children with BPAs of 4 mm or smaller (28 PATCH, 60 EXTENSION, 75 NATIVE), baseline characteristics were similar. The risk-adjusted 5-year freedom from reintervention was 68% for PATCH, 76% for EXTENSION, and 85% for NATIVE. PATCH trended toward an increased risk of reintervention (p = 0.07). For children with BPAs of 4 mm or smaller left in their NATIVE state, only ∼15% required reintervention. After adjustment for baseline BPA z-score, the time-related BPA growth was decreased (p0.014) and right ventricular pressure was increased (p = 0.03) for the PATCH group.Aggressive PATCH augmentation of hypoplastic BPAs improves the short-term geometry but may lead to late stenosis and higher rates of reintervention. Hypoplastic BPAs in tetralogy of Fallot tend (∼85%) to grow well without instrumentation.

Surgical management of patients with borderline left ventricular dimensions requires adequate patient selection. Therapeutic concepts have emerged to promote left ventricular growth and achieve adequate dimensions for staged biventricular repair. The hybrid approach thereby uses bilateral pulmonary artery banding and ductal stenting for initial palliation. A restrictive atrial septum is usually left unaddressed to ensure antegrade left ventricular filling. Final repair focuses on relief of obstructive left-sided lesions such as mitral valve stenosis, endocardial fibroelastosis, aortic valve stenosis, and aortic arch obstruction. The initial hybrid procedure is less traumatic than primary relief of left-sided obstructions. It remains to be determined if early or delayed relief of obstructive lesions improves the chances to grow left-sided structures.

BackgroundA wide variety of surgical strategies are used in tetralogy of Fallot repair. We sought to describe the international contemporary practice patterns for surgical management of tetralogy of Fallot.MethodsSurgeons from 18 international paediatric cardiac surgery centres (representing over 1800 tetralogy of Fallot cases/year) completed a Research Electronic Data Capture-based survey. Participating countries include: China (4), India (2), Nepal (1), Korea (1), Indonesia (1), Saudi Arabia (3), Japan (1), Turkey (1), Australia (1), United States of America (2), and Canada (1). Summary measures were reported as means and counts (percentages). Responses were weighted based on case volume/centre.ResultsPrimary repair is the prevalent strategy (83%) with variation in age at elective repair (range). Approximately 47% of sites use patient age as a factor in determining the strategy, with age 90% of all trans-annular repairs.ConclusionsIn this cohort representing 11 countries, there is variation in tetralogy of Fallot surgical management with no consensus on standard of practice. A large international prospective cohort study would allow analysis of impact of underlying anatomy and repair strategy on early and late outcomes.

Hearts with double outlet right ventricle are a heterogeneous group of malformations in which a comprehensive diagnostic approach is required for tailored surgical management. This pictorial essay revisits essential modifiers of clinical and surgical importance in management of the patients with double outlet right ventricle using 3-dimensional volume-rendered endocardial surface images and 3-dimensional print models. Special emphasis is paid to the infundibular morphology, including the size and orientation of the outlet septum, relative to the margin of the ventricular septal defect, and the extent of the muscular infundibulum as an additional modifier of the distance between the ventricular septal defect margin and the arterial valve or valves.

Background We hypothesized that reconstructed pulmonary artery (PA) size and postrepair PA pressure are associated with airway compression (AC) after complete unifocalization for pulmonary atresia, ventricular septal defects, and major aortopulmonary collateral arteries. Methods Complete unifocalization was performed in 48 consecutive patients between 2000 and 2016. Clinical course and outcome were reviewed, predictors for AC were identified by logistic regression, and the freedom from death was analyzed using Kaplan-Meier method. Results Postoperative respiratory distress occurred in 23 patients (48%), and AC occurred in 14 (29%). The median duration of follow-up was 3.7 years. AC was caused by central PA and aorta in 7, conduit in 3, and branch PA in 4. Surgical treatment was required in 5 patients (conduit downsizing, suspension of branch PA, conduit + aorta, branch PA + aorta, and aorta + trachea in 1 patient each). Three patients (21%) subsequently required airway stenting. Most (85.7%) of the AC occurred in patients with high right ventricular systolic pressure/left ventricular systolic pressure (>65%), large Nakata index (>200 mm2/m2), and large conduit index (>35 mm/m2). Patients with AC had significantly worse 3-year survival (no AC, 91.2%; AC, 64.2%; p = 0.01). Multivariate analysis identified higher right ventricular systolic pressure/left ventricular systolic pressure (p = 0.04), larger conduit index (p = 0.03), and Nakata index (p = 0.004) as predictors for AC. Conclusions AC is a common cause of postoperative respiratory distress and tends to be associated with higher postrepair PA pressure, more frequent right ventricular dysfunction, and worse medium-term survival. The study underscores the importance of incorporating all available lung segments to achieve a low PA pressure, potentially preventing pathologic dilatation of the reconstructed PA. Management of patients with poor major aortopulmonary collateral arteries anatomy and physiology remains a challenge.

We sought to describe the clinical course for patients with hypoplastic left heart syndrome and persistent ventricular dysfunction and identify risk factors for death or transplantation before stage II palliation. 138 children undergoing stage I palliation from 2004 to 2011 were reviewed. Twenty-two (16 %) patients (seven Hybrid, 15 Norwood) with two consecutive echocardiograms reporting at least moderate dysfunction were included and compared to case-matched controls. Eleven of the 22 patients with dysfunction (50 %) underwent stage II, seven (32 %) were transplanted, and four (18 %) died prior to stage II. Of the patients who survived to hospital discharge (n = 17) following stage 1, 14 (82 %) required readmission for heart failure (HF) compared to only two (10 %) for controls (p < 0.001). Among patients with ventricular dysfunction, there was an increased use of ACE inhibitors or beta-blockers (82 vs. 25 %; p = 0.001), inotropes (71 vs. 15 %; p = 0.001), ventilation (58 vs. 10 %; p = 0.001), and ECMO (29 vs. 0 %; p = 0.014) for HF management post-discharge when compared to controls. There was a lower heart transplant-free survival at 7 months in patients with dysfunction compared to controls (50.6 vs. 90.9 %; p = 0.040). ECMO support (p = 0.001) and duration of inotropic support (p = 0.04) were significantly associated with death or transplantation before stage II palliation. Patients with ventricular dysfunction received more HF management and related admissions. Longer inotropic support should prompt discussion regarding alternative treatment strategies given its association with death or transplant.

Background We hypothesized that primary sutureless (SL) repair of total anomalous pulmonary venous drainage (TAPVD) may have a lower incidence of postrepair pulmonary vein obstruction (PVO) and different modes of PVO compared with standard repair (SR). Methods One hundred ninety-five patients who underwent TAPVD repair (1990 to 2012) with the exception of congenital pulmonary vein stenosis, isomerism, and single-ventricle anomalies were included. Survival, reintervention, incidence, degree of PVO were compared between groups. The mode of PVO was expressed as central or peripheral. The Mann-Whitney test, Kaplan-Meier analysis, and Cox regression were used. Results The SL group had more infracardiac or mixed TAPVD ( p = 0.02) and preoperative PVO ( p = 0.07). There were no differences between SR and SL groups in survival (5-year survival, 83.1% versus 82.5%, respectively; p = 0.73) and composite outcome (death, intervention, PVO, 5-year survival, 76.4% versus 80.7%, respectively; p = 0.225). The SL group had a lower incidence of PVO of moderate or greater degree (SR, 11.3% versus SL, 2.9%; p = 0.05) than the SR group, especially in the infracardiac and mixed TAPVD cohort ( p = 0.011), with a lower pulmonary vein score (SR, 8 versus SL, 4; p = 0.01). The SL group had peripheral PVO exclusively (100%), whereas the SR group predominantly had central PVO (76.4%; p = 0.005). There was a trend toward less reoperation in the SL group (SR, 10.4% versus SL, 2.9%; p = 0.08). Survival after reoperation was comparable to primary TAPVD repair types as well as reoperation repair types. Conclusions Primary SL appeared to be associated with a lower incidence and severity of PVO. The primary SL repair eliminated the risk of developing central PVO, although a relatively benign type of peripheral PVO could occur.

Objectives To evaluate the durability of aortic valve (AoV) repair relative to other strategies for children with significant aortic insufficiency (AI). Methods From 2001 to 2012, 90 children with greater than or equal to moderate AI underwent surgery. Resulting procedures were classified according to final operative outcome: AoV repair (repair; n = 46, 51%), Ross procedure (Ross; n = 21, 23%) or replacement with mechanical or tissue prosthesis [aortic valve replacement (AVR); n = 23, 26%]. Repeated measures (n = 1081 echocardiograms) mixed-model analysis and parametric multiphase risk-adjusted hazard analysis were used to evaluate haemodynamic parameters and durability of operations. Results Mean age at operation was similar for repair and Ross groups, but slightly higher for the AVR group (10.6, 11 and 13.2, respectively; P = 0.04). Baseline annular dimensions were similar among groups. Of 46 repairs, 85% involved pericardial leaflet extensions (commonly with leaflet shaving and/or commisuroplasty). The remaining repairs were commissuroplasties. On multivariable analysis, repair was associated with increased early (∼1-2 years) AI and increased outflow tract peak pressure gradients relative to Ross and AVR procedures. On univariate analysis, repairs tended to have a larger annulus size compared with Ross or AVR; however, this was not significant on multivariable analysis. There were 25 reinterventions (surgical reoperation = 16; transcatheter intervention = 9) for 22 children. Freedom from surgical reoperation was 64, 100 and 51% at 6 years for repairs, Ross and AVR, respectively (P = 0.05); however, three of five reoperations after AVR were for failed bioprosthetic devices. The freedom from reintervention was not significantly influenced by the type of AoV operation (P = 0.43). Conclusions Durability of aortic valve repair for children is limited by recurrence of AI and/or stenosis, often within the first few years. After repair, reoperation should be anticipated within ∼7 years.

Background The purpose of this study was to evaluate the clinical utility of intraoperative aortic blood flow measurements on clinical outcome in patients undergoing pulmonary artery banding (PAB). Methods We reviewed 12 patients who underwent a PAB between September 2008 and March 2013 who also had intraoperative aortic blood flow measurements. Diagnosis included biventricular physiology in 6, single-ventricle physiology in 4, and inadequate systemic ventricle in 2 patients. Aortic blood flow was measured at the time of surgery by Transonic flow probe (Transonic Systems Inc, Ithaca, NY). Aortic flow, intraoperative hemodynamics, and clinical outcomes were analyzed to determine the potential predictive utility of intraoperative variables on postoperative outcomes. Results The aortic flow increased after PAB from 1.56 ± 0.73 to 2.20 ± 1.10 L · min −1 · m −2 (41.0% increase; p = 0.001). The efficacy of the PAB procedure was found to be directly related to the percentage increase in aortic blood flow measured intraoperatively. Three patients with less than 20% increase in aortic blood flow died, required re-PAB, or developed ventricular dysfunction, while patients with successful PAB had more than 40% increase in aortic blood flow. The percentage increase in aortic blood flow was not predictable based on pre-band or post-band absolute aortic blood flow measurements. The percentage increase in aortic blood flow was inversely correlated to the tightness of the PAB as defined relative to that predicted by the Trusler formula (r = 0.67; p = 0.01). Conclusions This study identifies the change in the aortic blood flow as a new, physiologically based parameter to help predict PAB outcome.

The number of patients with the failing Fontan physiology is increasing. We tested a novel in situ microaxial pump (Impella) to support the failing atrio-pulmonary Fontan circulation in an acute pig model. A Fontan model was established in eight juvenile pigs by connecting the right atrium to the main pulmonary artery after tricuspid valve destruction. The Impella pump was inserted retrograde from the distal main pulmonary artery into the right atrium. Hemodynamics, blood gas, and echocardiographic data were compared among baseline, pure Fontan physiology (10 minutes), and mechanically assisted Fontan physiology (up to 12 hours). The Impella system generated a blood flow of 75-85 ml/kg/minute in six animals, and 55-65 ml/kg/minute in two animals. The mechanically assisted Fontan attained a significantly higher mean blood pressure (39.6 ± 7 vs. 24.7 ± 3.3 mm Hg, p < 0.01), lower central venous pressure (5 ± 2.4 vs. 12.8 ± 1.7 mm Hg, p < 0.01), and higher mixed venous saturation (60.4 ± 10.8 vs. 23.4 ± 8.4 mm Hg, p < 0.01) compared with pure Fontan physiology. Cardiac output and stroke volume were similar during baseline and mechanically assisted Fontan (p = not significant). This acute pig study demonstrated the feasibility of mechanical circulatory support in the failing Fontan physiology. The in situ microaxial pump maintained cardiac output while increasing blood pressure and reducing venous pressure.

Infants listed for heart transplantation experience high waitlist and early post-transplant mortality, and thus, optimal allocation of scarce donor organs is required. Unfortunately, the creation and validation of multivariable regression models to identify risk factors and generate individual-level predictions are challenging. We sought to explore the use of data mining methods to generate a prediction model. CART analysis was used to create a model which, at the time of listing, would predict which infants listed for heart transplantation would survive at least 3 months post-transplantation. A total of 48 infants were included; 13 died while waiting, and six died within 3 months of heart transplant. CART analysis identified RRT, blood urea nitrogen, and hematocrit as terminal nodes with alanine transaminase as an intermediate node predicting death. No patients listed on RRT (n = 10) survived and only three of 12 (25%) patients listed on ECLS survived >3 months post-transplant. CART analysis overall accuracy was 83%, with sensitivity of 95% and specificity 76%. This study shows that CART analysis can be used to generate accurate prediction models in small patient populations. Model validation will be necessary before incorporation into decision-making algorithms used to determine transplant candidacy.

Objectives Paediatric coronary artery bypass grafting is indicated in cases of clinically significant and symptomatic coronary stenosis, with frequent complications occurring in the perioperative period. To reduce complications and improve outcomes of these procedures, surgical microscopes have been used at our centre with the anastomosis performed by a microvascular surgeon. The purpose of this article is to report our institutional experience in all patients who have undergone paediatric coronary artery bypass grafting procedures with and without microvascular techniques. Methods Twenty-four patients who underwent coronary artery bypass grafting from January 2000 to May 2017 were retrospectively reviewed. Results Eighteen patients underwent bypass without microvascular involvement and 6 patients required the use of microsurgical techniques. Median age at the time of operation was 9.79 and 2.02 years for the 2 groups, respectively. The median weight at the time of operation was 41.2 and 10.75 kg for the 2 groups, respectively. Procedures were performed emergently in 4 patients. Three major anastomotic complications occurred requiring reoperation, although none occurred in the microvascular group. The median follow-up was 3.40 years and 5.25 years for the 2 groups, respectively. Three patients were symptomatic at last follow-up and 2 deaths occurred, all in the non-microvascular group. All grafts were patent in both groups. There were no statistically significant differences between preoperative and postoperative ejection fraction between the groups. Conclusions Our results highlight the potential positive impact of collaboration between cardiac and microvascular surgeons during paediatric coronary artery bypass grafting procedures and the subsequent reduction in complications that may be expected.

Annulus-sparing repair of tetralogy of Fallot (TOF) carries a potential increased risk of reoperation for restenosis and unproven benefits on right ventricular (RV) geometry.Primary TOF repairs (n = 434) between 2000 and 2012 were studied using risk-adjusted parametric techniques. Progression of cardiac dimensions was analyzed using repeated measures regression using reports of all 2,103 echocardiograms undertaken throughout the study period, to a maximum follow-up of 13.7 years.Repair was at a mean age of 180 days: AS approach in 296 (68%) patients; and transannular patch in 138 (32%). Intraoperative revisions (for residual stenosis) were required in 135 patients (29%). There have been 4 deaths (survival 99%). Surgical reoperation for recurrent right ventricular outflow tract stenosis was occasionally required in both groups at comparable rates (transannular patch, 5 of 136 [4%]; annulus-sparing repair, 14 of 296 [5%]; p = 0.83). Larger increases in RV end-diastolic dimensions were evident in transannular patch patients versus annulus-sparing repair patients (p0.0001). Other risks for RV dilation included worse grade of postoperative pulmonary regurgitation, larger right ventricular end-diastolic dimension at the time of diagnosis, and higher operative weight (all p0.0001). Factors associated with successful annulus-sparing repair included (1) pulmonary annulus greater than 7 mm, right ventricular end-diastolic dimension greater than 1.2 cm, and tricuspid annulus greater than 1.4 cm (all preoperatively); and (2) right ventricular outflow tract diameter greater than 10 mm and right ventricular systolic pressure less than 50 mm Hg (both intraoperatively after repair).Pursuit of annulus-sparing repair strategies can lower the use of transannular patch to approximately 30% with low risk of reoperation for the patient. Annulus-sparing repair is associated with significantly reduced long-term RV dilation. Pulmonary valve enlargement to approximately 10 mm and right ventricular systolic pressure less than 50 mm Hg during annulus-sparing repair are associated with low risk of recurrent stenosis.

Objectives Mortality associated with the modified Blalock–Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity. Design A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox's proportional hazard regression. Results Median age was 8 (0–126) days, weight 3.1(1.7–5.4) kg. Seventy-three percent were neonates, 58% duct dependent and 73% had single ventricle physiology. Ninety-seven percent had a sternotomy approach for shunt placement with 70% receiving a 3.5 mm graft. Mean graft index (shunt cross sectional area [mm2]/BSA [m2]) was 44.39 ± 8.04 and shunt size (mm) to body weight (kg) ratio 1.1 ± 0.2. Hospital mortality was 12%, with an interval mortality of 6%. Shunt thrombosis/stenosis occurred in 23% and pulmonary over circulation in 30%, while shunt reoperation was required in 12% and catheter intervention in 8% of the cohort. At 1-year, survival was 82.0% (95% CI [72.7%, 88.4%]), and survival free of major morbidity 61.4% (95% CI [50.7%, 70.5%]). Duct dependency predisposed to mortality (P = .01, HR 6.74 [1.54, 29.53]) and composite outcome (mortality and major morbidity) (P = .04, HR 2.15, CI [1.036, 4.466]) and higher graft index to mortality (P = .005, HR 1.07 [1.02, 1.12]). Conclusions The commonest indication for a MBTS in the current era was single ventricle palliation. Morbidity and mortality was considerable, partly explained by the higher at risk population. Alternative methods to maintain pulmonary blood flow in place of a MBTS requires further investigation.

We hypothesized that chylothorax could be a sign of intolerance to the Fontan physiology, and thus patients who develop chylothorax or pleural effusion have worse medium-term to long-term survival.A total of 324 patients who underwent the Fontan operation between 2000 and 2013 were included. Chylothorax was defined as ≥5 mL/kg/day of chylomicron-positive chest drainage fluid no earlier than postoperative day 5 or drainage with80% lymphocytes. Outcomes were compared between the chylothorax and non-chylothorax groups by the Kaplan-Meier method and log-rank test. Independent predictors of chylothorax and number of days of any chest drainage were analyzed with multivariable logistic regression and multivariable generalized negative binomial regression for count data, respectively.Chylothorax occurred in 78 patients (24%). Compared with the non-chylothorax group, the chylothorax group had a longer duration of chest tube requirement (median, 18 days vs 9 days; P .000) and a longer length of hospital stay (median, 19 days vs 10 days; P .000). Eight patients (10.3%) required thoracic duct ligation. The chylothorax group had lower freedom from death (P = .013) and from composite adverse events (P = .021). No predictor was found for chylothorax. Pulmonary atresia (P = .031) and pre-Fontan pulmonary artery pressure (P = .01) were predictive of prolonged pleural effusion (14 days).Occurrence of chylothorax following the Fontan operation can be a marker of poorer medium-term clinical outcomes. It is difficult to predict occurrence of chylothorax owing to its multifactorial nature and involvement of lymphatic compensatory capacity that is unmasked only after the Fontan operation.

Objective The management of patients with tetralogy of Fallot (ToF) and pulmonary atresia (PA) with major aortopulmonary collateral arteries (MAPCAs) is challenging. Frequently it is difficult to predict whether complete repair with closure of the ventricular septal defect (VSD) will be tolerated. The aim of this study was to investigate whether measurements of preoperative pulmonary blood flow are associated with early postoperative outcome after VSD closure. Methods In this retrospective study, the data from 10 patients who had undergone a cardiac magnetic resonance imaging study with flow measurements before attempted surgical complete repair were collected. Systemic blood flow (Qs) was calculated as the sum of descending aortic blood flow distal to the MAPCA origins and superior vena cava flow. Pulmonary blood flow (Qp) was measured either from the sum of the pulmonary flow (n = 7) or calculated as the difference between ascending aortic flow and Qs. Results Preoperative Qp/Qs averaged 1.71 ± 0.68 and correlated inversely with right ventricular systolic pressure (relative to systolic blood pressure, r = −0.75, P = .03) and positively with the total neopulmonary artery index (TNPAI, r = 0.66, P = .04), as measured by fluoroscopic angiography. Two children did not tolerate VSD closure. This was likely related to bronchial compression in 1 patient while the other had the lowest TNPAI and the lowest Qp/Qs of all patients. Conclusions CMR provides not only anatomic but also functional information for surgical decision making in patients with ToF and PA with MAPCAs. Preoperative Qp/Qs is associated with postoperative right ventricular pressure and may be a marker of readiness for VSD closure.

Background Reconstruction of anomalous left pulmonary veins (ALPV) requires an anastomosis at a nonanatomic position, posing the potential risk of pulmonary vein obstruction (PVO). Methods The 514 patients who were diagnosed with a pulmonary vein abnormality from 1990 to 2010 were reviewed. Thirty-eight patients (7.4%; median age, 1.4 years; interquartile range, 0.1 to 5.7 years) were identified. ALPV was diagnosed as an isolated anomaly in 23 (61%) or as part of mixed total anomalous pulmonary venous drainage in 15 (39%). Patients were divided into 3 groups (group 1: partial ALPV, treated; group 2: total ALPV, treated; or group 3: partial ALPV, untreated). Freedom from poor clinical (death/reoperation) and functional (any PVO, mean pressure gradient > 3 mm Hg) outcomes were analyzed. Results Repair in 30 ALPV patients (79%) was performed with direct anastomosis to the left atrium (n = 26 [68%]) or by sutureless repair (n = 4 [11%]). Two deaths occurred in group 2 (heart failure and PVO). The 4 reoperations in group 2 were prompted by PVO and occurred within 6 months of the initial repair. There was a nonsignificant trend of lower freedom from poor outcomes in group 2 (74.6%) vs group 1 (100%) at 10 years ( p = 0.105). There was no difference in the incidence of any left PVO among the groups ( p = 0.381). Severe left PVO did not develop in group 3 (n = 8 [21%]). Conclusions Total ALPV carries a high risk of early PVO. Thus, the optimal surgical approach remains elusive. Untreated partial ALPV remained unobstructed during midterm follow-up. Therefore, surgical treatment may not be necessary in patients with partial ALPV.

Background Hypoplastic left heart syndrome with a highly restrictive or intact atrial septum (HLHS-RAS) has a very high mortality. Fetal left atrial (LA) hypertension results in abnormal lung development with lymphangiectasia and pulmonary vein muscularization. We report our initial experience with percutaneous ultrasound-guided stenting of the fetal atrial septum to decompress the LA. Methods Retrospective review of fetuses with HLHS-RAS or a variant that underwent active perinatal management from 2000 to 2012. Results Ten fetuses were identified. Two died in utero (33, 29weeks). Four required the urgent creation of an atrial communication immediately after birth but died subsequently (5–54days). Four fetuses (28–36weeks) underwent percutaneous stenting of the atrial septum, with ultrasound guidance and intravenous maternal sedation. Elevated LA pressure, pulmonary vein dilation and MRI estimated pulmonary perfusion all improved after stenting. Three of four stented fetuses were delivered vaginally. Atrial septectomy was performed within 48h of delivery to ensure complete LA decompression, rather than for hypoxemia. Intraoperative lung biopsy demonstrated muscularized pulmonary veins and lymphangiectasia in all four. Two fetuses developed stent stenosis in utero and died after birth, from pulmonary hypertension and sepsis respectively. Two are alive, representing an improved outcome over our previous experience (p=0.03). Conclusion Fetal atrial septal stenting is feasible without maternal complications and allows vaginal delivery of a more stable neonate. Fetal LA decompression ameliorates rather than reverses lung injury, and is one component of an approach that may improve survival in HLHS-RAS.

Background For neonates with atrioventricular septal defect and aortic arch obstruction including coarctation of the aorta, we sought to determine whether a difference in outcomes exists after a primary neonatal versus staged surgical repair (neonatal arch repair with delayed intracardiac repair). Methods This retrospective cohort study included consecutive neonates with atrioventricular septal defect and aortic arch obstruction who underwent cardiac surgery before 28 days of age at six centers from 1990 to 2009. Characteristics and outcomes between patients undergoing neonatal versus staged repair were compared. Results Of 66 study patients, 31 (47%) underwent primary neonatal repair and 35 (53%) underwent staged repair. At baseline echocardiogram, a greater percentage of neonatal repair patients had relative unbalanced ventricular size (56% versus 35%, p = 0.02). There were no other differences in demographic characteristics, cardiac anatomical or functional details, or surgical technique. Those undergoing neonatal repair tended to be more likely to have at least moderate left atrioventricular valve regurgitation early after repair (42% versus 19%, p = 0.05) and to have at least one major in-hospital complication (42% versus 20%, p = 0.06). After the initial cardiac operation, compared with the neonatal repair group, patients undergoing staged repair had greater survival (87% versus 57% at 6 years, log-rank p = 0.02) and freedom from the first unplanned cardiac reoperation (69% versus 45% at 6 years, log-rank p = 0.005). Conclusions For neonates with atrioventricular septal defect and aortic arch obstruction, when compared with neonatal repair, a staged approach was associated with improved survival and lower morbidity.

Objective The interdigitating technique in aortic arch reconstruction in hypoplastic left heart syndrome and variants (HLHS) reduces the recoarctation rate. Little is known on aortic arch growth characteristics and resulting clinical impact. Methods A total of 139 patients with HLHS underwent staged palliation between 2007 and 2014; 73 patients underwent arch reconstruction. Dimensions of ascending aorta, transverse arch, interdigitating anastomosis, and descending aorta in pre-stage II and pre-Fontan angiograms were measured. Aortic arch dimensions were analyzed. Ventricular and atrioventricular valve function were assessed. Results Diameters increased in all segments between pre-stage II and pre-Fontan ( P z scores remained unchanged in all segments but the descending aorta that was significantly larger pre-Fontan ( P = .039). Dimensions and z scores between pre-stage II and pre-Fontan correlated in proximal segments, but not at and distal to the interdigitating anastomosis. Pronounced tapering occurred between the transverse arch and the interdigitating anastomosis. Arch intervention of any type was performed in 7 (9.6%), and intervention for recoarctation in 3 (4.1%) patients. Conclusions The aortic arch after reconstruction with the interdigitating technique differs from normal. Growth was proportional with no further geometrical distortion. Recoarctation and reintervention rate is low. Further improvement may be achieved by optimizing patch configuration and material.

Background— Tetralogy of Fallot with cyanosis requiring surgical repair in early infancy reflects poor anatomy and is associated with more clinical instability and longer hospitalization than those who can be electively repaired later. We bridged symptomatic infants with risk factors for early primary repair by right ventricular outflow tract stenting (stent). Methods and Results— Four groups of tetralogy of Fallot with confluent central pulmonary arteries were studied: stent group (n=42), primary repair (aged 3mo group; n=45). Stent patients had the smallest pulmonary arteries with a median (95% credible intervals) Nakata index (mm 2 /m 2 ) of 79 (66–85) compared with the early-PA 139 (129–154), early-PS 136 (121–153), and surg>3mo 167 (153–200) groups. Only stent infants required unifocalization of aortopulmonary collaterals (17%). Stent and early-PA infants had younger age and lower weight than early-PS infants. Stent infants had the most multiple comorbidities. Stenting allowed deferral of complete surgical repair to an age (6 months), weight (6.3 [5.8–7.0] kg), and Nakata index (147 [132–165]) similar to the low-risk surg>3mo group. The 3 early treatment groups had similar intensive care unit/hospital stays and high reintervention rates in the first 12 months after repair, compared with the surg>3mo group. Conclusions— Right ventricular outflow tract stenting of symptomatic tetralogy of Fallot with poor anatomy (small pulmonary arteries) and adverse factors (multiple comorbidities, low weight) relieves cyanosis and defers surgical repair. This allowed pulmonary arterial and somatic growth with clinical results comparable to early surgical repair in more favorable patients.