Increased utilization of the hybrid procedure is not associated with improved early survival for newborns with hypoplastic left heart syndrome: a single-centre experience.

OBJECTIVES: The primary objectives were to examine utilization of the Hybrid versus the Norwood procedure for patients with hypoplastic left heart syndrome or variants and the impact on hospital mortality. The Hybrid procedure was 1st used at our institution in 2004. METHODS: Review of all subjects undergoing the Norwood or Hybrid procedure between 1 January 1984 and 31 December 2022. The study period was divided into 8 eras: era 1, 1984-1988; era 2, 1989-1993; era 3, 1994-1998; era 4, 1999-2003; era 5, 2004-2008; era 6, 2009-2014; era 7, 2015-2018 and era 8, 2019-2022. The primary outcome was in-hospital mortality. Mortality rates were computed using standard binomial proportions with 95% confidence intervals. Rates across eras were compared using an ordered logistic regression model with and adjusted using the Tukey-Kramer post-hoc procedure for multiple comparisons. In the risk-modelling phase, logistic regression models were specified and tested. RESULTS: The Norwood procedure was performed in 1899 subjects, and the Hybrid procedure in 82 subjects. Use of the Hybrid procedure increased in each subsequent era, reaching 30% of subjects in era 8. After adjustment for multiple risk factors, use of the Hybrid procedure was significantly and positively associated with hospital mortality. CONCLUSIONS: Despite the increasing use of the Hybrid procedure, overall mortality for the entire cohort has plateaued. After adjustment for risk factors, use of the Hybrid procedure was significantly and positively associated with mortality compared to the Norwood procedure.

From Surgical to Total Transcatheter Stage I Palliation: Exploring Evidence and Perspectives.

Neonates with single ventricle physiology and ductal-dependent systemic circulation, such as those with hypoplastic left heart syndrome, undergo palliation in the first days of life. Over the past decades, variations on the traditional Stage 1 palliation, also known as Norwood operation, have emerged. These include the hybrid palliation and the total transcatheter approach. Here, we review the current evidence and data on different Stage 1 approaches, with a focus on their advantages, challenges, and future perspectives. Overall, although controversy remains regarding the superiority or inferiority of one approach to another, outcomes after the Norwood and the hybrid palliation have improved over time. However, both procedures still represent high-risk approaches that entail exposure to sternotomy, surgery, and potential cardiopulmonary bypass. The total transcatheter Stage 1 palliation spares patients the surgical and cardiopulmonary bypass insults and has proven to be an effective strategy to bridge even high-risk infants to a later palliative surgery, complete repair, or transplant. As the most recently proposed approach, data are still limited but promising. Future studies will be needed to better define the advantages, challenges, outcomes, and overall potential of this novel approach.

Mortality and Heart Transplantation After Hybrid Palliation of Hypoplastic Left Heart Syndrome: A Systematic Review and Meta-Analysis.

BACKGROUND: Newborns with hypoplastic left heart syndrome (HLHS) who are considered at increased risk for death following Norwood/Sano surgery often undergo hybrid palliation (HP) as initial surgery. We aimed to compile the HP experience in HLHS and its variants and assess the rates of, and risk factors for, death and heart transplantation. METHODS: CINAHL, CINAHL PLUS, PubMed/MEDLINE, and SCOPUS were systematically searched for HP outcome studies of death or heart transplantation in HLHS between 1998 and 2022. Pooled incidence was estimated, and potential risk factors were identified using random-effects meta-analysis and reconstructed time-to-event data from Kaplan-Meier curves. RESULTS: Thirty-three publications were included in our review. Overall, of 1,162 patients 417 died and 57 underwent heart transplantation, resulting in a combined outcome of 40.7%, (474/1,162). There was a trend toward decreasing mortality risk across the stages of palliation. Pooled mortality between HP and comprehensive stage 2 palliation was 25%, after stage 2 up to Fontan palliation was 16%, and 6% post-Fontan. The incidence of death or heart transplantation was higher in high-risk patients-43% died and 10% received heart transplantation. CONCLUSION: Our systematic review and meta-analysis found high rates of death or heart transplantation in HP of HLHS patients between HP and Fontan surgeries. All patients should be closely followed during the initial interstage period, which is associated with the highest hazard. Prospective studies on appropriate patient selection, indications, and / or alternatives, as well as refining HP strategies for managing newborns with HLHS are needed to improve outcomes.

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Norwood procedure.

OBJECTIVES: To evaluate longitudinal systemic ventricular function and atrioventricular valve regurgitation in patients after the neonatal Norwood procedure. METHODS: Serial postoperative echocardiographic images before Fontan completion were assessed in neonates who underwent the Norwood procedure between 2001 and 2020. Ventricular function and atrioventricular valve regurgitation were compared between patients with modified Blalock-Taussig shunt and right ventricle to pulmonary artery conduit. RESULTS: A total of 335 patients were identified including 273 hypoplastic left heart syndrome and 62 of its variants. Median age at Norwood was 8 (7-12) days. Modified Blalock-Taussig shunt was performed in 171 patients and the right ventricle to pulmonary artery conduit in 164 patients. Longitudinal ventricular function and atrioventricular valve regurgitation were evaluated using a total of 4352 echocardiograms. After the Norwood procedure, ventricular function was initially worse (1-30 days) but thereafter better (30 days to stage II) in the right ventricle to pulmonary artery conduit group (P < 0.001). After stage II, the ventricular function was inferior in the right ventricle to the pulmonary artery conduit group (P < 0.001). Atrioventricular valve regurgitation between the Norwood procedure and stage II was more frequent in the modified Blalock-Taussig shunt group (P < 0.001). After stage II, there was no significant difference in atrioventricular valve regurgitation between the groups (P = 0.171). CONCLUSIONS: The effect of shunt type on haemodynamics after the Norwood procedure seems to vary according to the stage of palliation. After the Norwood, the modified Blalock-Taussig shunt is associated with poorer ventricular function and worse atrioventricular valve regurgitation compared to right ventricle to pulmonary artery conduit. Whereas, after stage II, modified Blalock-Taussig shunt is associated with better ventricular function and comparable atrioventricular valve regurgitation, compared to the right ventricle to pulmonary artery conduit.

Valved Sano conduit improves immediate outcomes following Norwood operation compared with nonvalved Sano conduit.

OBJECTIVE: Use of a valved Sano during the Norwood procedure has been reported previously, but its impact on clinical outcomes needs to be further elucidated. We assessed the impact of the valved Sano compared with the nonvalved Sano after the Norwood procedure in patients with hypoplastic left heart syndrome. METHODS: We retrospectively reviewed 25 consecutive neonates with hypoplastic left heart syndrome who underwent a Norwood procedure with a valved Sano conduit using a femoral venous homograft and 25 consecutive neonates with hypoplastic left heart syndrome who underwent a Norwood procedure with a nonvalved Sano conduit between 2013 and 2022. Primary outcomes were end-organ function postoperatively and ventricular function over time. Secondary outcomes were cardiac events, all-cause mortality, and Sano and pulmonary artery reinterventions at discharge, interstage, and pre-Glenn time points. RESULTS: Postoperatively, the valved Sano group had significantly lower peak and postoperative day 1 lactate levels (P = .033 and P = .025, respectively), shorter time to diuresis (P = .043), and shorter time to enteral feeds (P = .038). The valved Sano group had significantly fewer pulmonary artery reinterventions until the Glenn operation (n = 1 vs 8; P = .044). The valved Sano group showed significant improvement in ventricular function from the immediate postoperative period to discharge (P < .001). From preoperative to pre-Glenn time points, analysis of ventricular function showed sustained ventricular function within the valved Sano group, but a significant reduction of ventricular function in the nonvalved Sano group (P = .003). Pre-Glenn echocardiograms showed competent conduit valves in two-thirds of the valved Sano group (n = 16; 67%). CONCLUSIONS: The valved Sano is associated with improved multi-organ recovery postoperatively, better ventricular function recovery, and fewer pulmonary artery reinterventions until the Glenn procedure.

Higher Cumulative Dose of Opioids and Other Sedatives are Associated with Extubation Failure in Norwood Patients.

BACKGROUND: The primary purpose of this study is to evaluate the relationship between sedation usage and extubation failure, and to control for the effects of hemodynamic, oximetric indices, clinical characteristics, ventilatory settings pre- and post-extubation, and echocardiographic (echo) findings in neonates with hypoplastic left heart syndrome (HLHS) post-Norwood procedure. METHODS: Single-center, retrospective analysis of Norwood patients during their first extubation post-surgery from January 2015 to July 2021. Extubation failure was defined as reintubation within 48 h of extubation. Demographics, clinical characteristics, ventilatory settings, echo findings (right ventricular function, tricuspid regurgitation), and cumulative dose of sedation medications before extubation were compared between patients with successful or failed extubation. RESULTS: The analysis included 130 patients who underwent the Norwood procedure with 121 (93%) successful and 9 (7%) failed extubations. Univariate analyses showed that vocal cord anomaly (p = 0.05), lower end-tidal CO2 (p < 0.01), lower pulse-to-respiratory quotient (p = 0.02), and ketamine administration (p = 0.04) were associated with extubation failure. The use of opioids, benzodiazepines, dexmedetomidine, and ketamine are mutually correlated in this cohort. On multivariable analysis, the vocal cord anomaly (OR = 7.31, 95% CI 1.25-42.78, p = 0.027), pre-extubation end-tidal CO2 (OR = 0.80, 95% CI 0.65-0.97, p = 0.025), and higher cumulative dose of opioids (OR = 10.16, 95% CI 1.25-82.43, p = 0.030) were independently associated with extubation failure while also controlling for post-extubation respiratory support (CPAP/BiPAP/HFNC vs NC), intubation length, and echo results. CONCLUSION: Higher cumulative opioid doses were associated with a greater incidence of extubation failure in infants post-Norwood procedure. Therefore, patients with higher cumulative doses of opioids should be more closely evaluated for extubation readiness in this population. Low end-tidal CO2 and low pulse-to-respiratory quotient were also associated with failed extubation. Consideration of the pulse-to-respiratory quotient in the extubation readiness assessment can be beneficial in the Norwood population.

Inpatient Resource Utilization for Hypoplastic Left Heart Syndrome from Birth Through Fontan.

Completing 3-stage palliation for hypoplastic left heart syndrome requires significant resources. An analysis of recent data has not been performed. We aimed to determine total charges necessary to complete all 3 stages of single-ventricle palliation, including interstage encounters. We also aimed to determine overall resource utilization, including hospital days, interstage admissions, and interstage procedures. We performed a retrospective cohort study using data from the Pediatric Health Information System database between 2016 and 2021, including all patients who completed 3-stage palliation for hypoplastic left heart syndrome. We identified 199 patients who underwent 3-stage palliation of hypoplastic left heart syndrome between 2016 and 2021. Median total adjusted charges (interquartile range, IQR) over the course of 3-stage palliation were $1,475,800 ($1,028,900-2,191,700). Median adjusted charges (IQR) for stage 1, 2, and 3 hospitalizations were $604,300 ($419,000-891,400), $234,000 ($164,300-370,800), and $256,260 ($178,300-345,900), respectively. Median hospital length of stay (IQR) for stages 1, 2, and 3 was 36 (26,53), 9 (6,17), and 10 (7,14) days, respectively. Pulmonary artery stenosis was the most common admitting diagnosis for interstage hospitalizations (3.4% of hospitalizations). Cardiac catheterization (24.1% of procedures) and feeding tube placement (10.0% of procedures) were the most common principal procedures during interstage hospitalizations. Total inpatient charges incurred throughout 3-stage palliation of hypoplastic left heart syndrome are substantial and have risen since prior studies. Gastrointestinal comorbidities and feeding optimization contribute considerably to this resource utilization.

Safety and feasibility of adjunct autologous cord blood stem cell therapy during the Norwood heart operation.

BACKGROUND: We conducted this phase I, open-label safety and feasibility trial of autologous cord blood (CB) stem cell (CBSC) therapy via a novel blood cardioplegia-based intracoronary infusion technique during the Norwood procedure in neonates with an antenatal diagnosis of hypoplastic left heart syndrome (HLHS). CBSC therapy may support early cardiac remodeling with enhancement of right ventricle (RV) function during the critical interstage period. METHODS: Clinical grade CB mononucleated cells (CBMNCs) were processed to NetCord-FACT International Standards. To maximize yield, CBSCs were not isolated from CBMNCs. CBMNCs were stored at 4 °C (no cryopreservation) for use within 3 days and delivered after each cardioplegia dose (4 × 15 mL). RESULTS: Of 16 patients with antenatal diagnosis, 13 were recruited; of these 13 patients, 3 were not treated due to placental abruption (n = 1) or conditions delaying the Norwood for >4 days (n = 2) and 10 received 644.9 ± 134 × 106 CBMNCs, representing 1.5 ± 1.1 × 106 (CD34+) CBSCs. Interstage mortality was 30% (n = 3; on days 7, 25, and 62). None of the 36 serious adverse events (53% linked to 3 deaths) were related to CBMNC therapy. Cardiac magnetic resonance imaging before stage 2 (n = 5) found an RV mass index comparable to that in an exact-matched historical cohort (n = 22), with a mean RV ejection fraction of 66.2 ± 4.5% and mean indexed stroke volume of 47.4 ± 6.2 mL/m2 versus 53.5 ± 11.6% and 37.2 ± 10.3 mL/m2, respectively. All 7 survivors completed stage 2 and are alive with normal RV function (6 with ≤mild and 1 with moderate tricuspid regurgitation). CONCLUSIONS: This trial demonstrated that autologous CBMNCs delivered in large numbers without prior cryopreservation via a novel intracoronary infusion technique at cardioplegic arrest during Norwood palliation on days 2 to 3 of life is feasible and safe.

Utility of the NEONATE Score at an Institution that Routinely Performs the Hybrid Procedure for Hypoplastic Left Heart Syndrome.

NEONATE score > 17 has been proposed as a risk factor for interstage mortality/cardiac transplant (IM/T) for patients with single ventricle physiology. Hybrid procedure is assigned 6 points, the highest possible score for that surgical variable. Most centers reserve the hybrid procedure for high-risk patients. Goal of this study was to evaluate the NEONATE score at a center that routinely performs the hybrid procedure. Retrospective chart review of patients undergoing the hybrid procedure was performed (2008-2021). Demographics and variables used for the NEONATE score were collected. Maximization of Youden's J Statistic used to determine cohort-specific optimal threshold for patients undergoing comprehensive Stage II procedure (H-CSII) versus those with IM/T (H-IM/T). Total of 120 patients met inclusion criteria (H-CSII = 105, H-IM/T = 15). Gestational age was median 39 weeks (IQR 38, 39) and birth weight was 3.18 kg (2.91, 3.57). No patient was discharged with opiates or required post-operative extracorporeal circulatory support. Optimal threshold, as selected by maximizing Youden's J Statistic, was 22. Score > 22 had a positive predictive value of 0.33 (95% CI 0.12-0.62), negative predictive values of 0.90 (95% CI 0.83-0.95), and accuracy of 0.83 (95% CI 0.75-0.90) for IM/T. At a center that routinely performs the hybrid procedure, value of > 22 had the highest accuracy. This suggests that the hybrid procedure is not necessarily intrinsically a risk-factor for IM/T, but rather patient selection for the hybrid procedure may play a larger role at centers that do not routinely perform this procedure.

Cardiac Magnetic Resonance Imaging to Determine Single Ventricle Function in a Pediatric Population is Feasible in a Large Trial Setting: Experience from the Single Ventricle Reconstruction Trial Longitudinal Follow up.

The Single Ventricle Reconstruction (SVR) Trial was a randomized prospective trial designed to determine survival advantage of the modified Blalock-Taussig-Thomas shunt (BTTS) vs the right ventricle to pulmonary artery conduit (RVPAS) for patients with hypoplastic left heart syndrome. The primary aim of the long-term follow-up (SVRIII) was to determine the impact of shunt type on RV function. In this work, we describe the use of CMR in a large cohort follow up from the SVR Trial as a focused study of single ventricle function. The SVRIII protocol included short axis steady-state free precession imaging to assess single ventricle systolic function and flow quantification. There were 313 eligible SVRIII participants and 237 enrolled, ages ranging from 10 to 12.5 years. 177/237 (75%) participants underwent CMR. The most common reasons for not undergoing CMR exam were requirement for anesthesia (n = 14) or ICD/pacemaker (n = 11). A total of 168/177 (94%) CMR studies were diagnostic for RVEF. Median exam time was 54 [IQR 40-74] minutes, cine function exam time 20 [IQR 14-27] minutes, and flow quantification time 18 [IQR 12-25] minutes. There were 69/177 (39%) studies noted to have intra-thoracic artifacts, most common being susceptibility artifact from intra-thoracic metal. Not all artifacts resulted in non-diagnostic exams. These data describe the use and limitations of CMR for the assessment of cardiac function in a prospective trial setting in a grade-school-aged pediatric population with congenital heart disease. Many of the limitations are expected to decrease with the continued advancement of CMR technology.

Associations of Home Monitoring Data to Interventional Catheterization for Infants with Recurrent Coarctation of the Aorta and Hypoplastic Left Heart Syndrome.

The post-Norwood interstage period for infants with hypoplastic left heart syndrome is a high-risk time with 10-20% of infants having a complication of recurrent coarctation of the aorta (RCoA). Many interstage programs utilize mobile applications allowing caregivers to submit home physiologic data and videos to the clinical team. This study aimed to investigate if caregiver-entered data resulted in earlier identification of patients requiring interventional catheterization for RCoA. Retrospective home monitoring data were extracted from five high-volume Children's High Acuity Monitoring Program®-affiliated centers (defined as contributing > 20 patients to the registry) between 2014 and 2021 after IRB approval. Demographics and caregiver-recorded data evaluated include weight, heart rate (HR), oxygen saturation (SpO2), video recordings, and 'red flag' concerns prior to interstage readmissions. 27% (44/161) of infants required interventional catheterization for RCoA. In the 7 days prior to readmission, associations with higher odds of RCoA included (mean bootstrap coefficient, [90% CI]) increased number of total recorded videos (1.65, [1.07-2.62]) and days of recorded video (1.62, [1.03-2.59]); increased number of total recorded weights (1.66, [1.09-2.70]) and days of weights (1.56, [1.02-2.44]); increasing mean SpO2 (1.55, [1.02-2.44]); and increased variation and range of HR (1.59, [1.04-2.51]) and (1.71, [1.10-2.80]), respectively. Interstage patients with RCoA had increased caregiver-entered home monitoring data including weight and video recordings, as well as changes in HR and SpO2trends. Identifying these items by home monitoring teams may be beneficial in clinical decision-making for evaluation of RCoA in this high-risk population.

Attributable mortality benefit of digoxin treatment in hypoplastic left heart syndrome after the Norwood operation: An instrumental variable-based analysis using data from the Pediatric Health Information Systems Database.

BACKGROUND: Observational studies have demonstrated an association between the use of digoxin and reduced interstage mortality after Norwood operation for hypoplastic left heart syndrome (HLHS). Digoxin use has increased significantly but remains variable between different hospitals, independent of case-mix. Instrumental variable analyses have the potential to overcome unmeasured confounding, the major limitation of previous observational studies and to generate an estimate of the attributable benefit of treatment with digoxin. METHODS: A cohort of neonates with HLHS born from January 1, 2007 to December 31, 2021 who underwent Norwood operation at Pediatric Health Information Systems Database hospitals and survived >14 days after operation were studied. Using hospital-specific, 6-month likelihood of administering digoxin as an instrumental variable, analyses adjusting for both unmeasured confounding (using the instrumental variable) and measured confounders with multivariable logistic regression were performed. RESULTS: The study population included 5,148 subjects treated at 47 hospitals of which 63% were male and 46% non-Hispanic white. Of these, 44% (n = 2,184) were prescribed digoxin. Treatment with digoxin was associated with superior 1-year transplant-free survival in unadjusted analyses (85% vs 82%, P = .02). This survival benefit persisted in an instrumental-variable analysis (OR: 0.71, 95% CI: 0.54-0.94, P = .01), which can be converted to an absolute risk reduction of 5% (number needed to treat of 20). CONCLUSIONS: In this observational study of patients with HLHS after Norwood using instrumental variable techniques, a significant benefit in 1-year transplant-free survival attributable to digoxin was demonstrated. In the absence of clinical trial data, this should encourage the use of digoxin in this vulnerable population.

Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome: 2021 vs 2011.

We sought to examine current practices and changes in practice regarding initial counseling for families of patients with hypoplastic left heart syndrome (HLHS) given the evolution of options and outcomes over time. Counseling (Norwood with Blalock-Taussig-Thomas shunt (NW-BTT), NW with right ventricle to pulmonary artery conduit (NW-RVPA), hybrid palliation, heart transplantation, or non-intervention/hospice (NI)) for patients with HLHS were queried via questionnaire of pediatric care professionals in 2021 and compared to identical questionnaire from 2011. Of 322 respondents in 2021 (39% female), 299 respondents were cardiologists (92.9%), 17cardiothoracic surgeons (5.3%), and 6 were nurse practitioners (1.9%). Respondents were largely from North America (96.9%). In 2021, NW-RVPA procedure was the preferred palliation for standard risk HLHS patient (61%) and was preferred across all US regions (p < 0.001). NI was offered as an option by 71.4% of respondents for standard risk patients and was the predominant strategy for patients with end-organ dysfunction, chromosomal abnormality, and prematurity (52%, 44%, and 45%, respectively). The hybrid procedure was preferred for low birth-weight infants (51%). In comparison to the identical 2011 questionnaire (n = 200), the NW-RVPA was endorsed more in 2021 (61% vs 52%, p = 0.04). For low birth-weight infants, hybrid procedure was more recommended than in 2011 (51% vs 21%, p < 0.001). The NW-RVPA operation is the most recommended strategy throughout the US for infants with HLHS. The hybrid procedure for low birth-weight infants is increasingly recommended. NI continues to be offered even in standard risk patients with HLHS.

The effect of clinical and haemodynamic variables on post-operative length of stay immediately upon admission after biventricular repair with Yasui operation following an earlier Norwood operation.

BACKGROUND: There are a variety of approaches to biventricular repair in neonates and infants with adequately sized ventricles and left-sided obstruction in the presence of a ventricular septal defect. Those who undergo this in a staged manner initially undergo a Norwood procedure followed by a ventricular septal defect closure such that the neo-aorta is entirely committed to the left ventricle and placement of a right ventricular to pulmonary artery conduit (Yasui operation). This study aimed to determine clinical and haemodynamic factors upon paediatric cardiac ICU admission immediately after the two-stage Yasui operation that was associated with post-operative length of stay. METHODS: This was a retrospective review of patients who underwent the Yasui procedure after the initial Norwood operation between 1 January 2011 and 31 December 2020. Patients with complete data on admission were identified and analysed using Bayesian regression analysis. RESULTS: A total of 15 patients were included. The median age was 9.0 months and post-operative length of stay was 6days. Bayesian regression analysis demonstrated that age, weight, heart rate, mean arterial blood pressure, central venous pressure, pulse oximetry, cerebral near infrared spectroscopy, renal near infrared spectroscopy, pH, pCO2, ionised calcium, and serum lactate were all associated with post-operative length of stay. CONCLUSION: Discrete clinical and haemodynamic factors upon paediatric cardiac ICU admission after staged Yasui completion are associated with post-operative length of stay. Clinical target ranges can be developed and seem consistent with the notion that greater systemic oxygen delivery is associated with lower post-operative length of stay.

Sustained Total All-Region Perfusion During the Norwood Operation and Postoperative Recovery.

We developed a technique for the Norwood operation utilizing continuous perfusion of the head, heart, and lower body at mild hypothermia named Sustained Total All-Region (STAR) perfusion. We hypothesized that STAR perfusion would be associated with shorter operative times, decreased coagulopathy, and expedited post-operative recovery compared to standard perfusion techniques. Between 2012 and 2020, 80 infants underwent primary Norwood reconstruction at our institution. Outcomes for patients who received successful STAR perfusion (STAR, n = 37) were compared to those who received standard Norwood reconstruction utilizing regional cerebral perfusion only (SNR, n = 33), as well as to Norwood patients reported in the PC4 national database during the same timeframe (n = 1238). STAR perfusion was performed with cannulation of the innominate artery, descending aorta, and aortic root at 32-34°C. STAR patients had shorter median CPB time compared to SNR (171 vs 245 minutes, P < 0.0001), shorter operative time (331 vs 502 minutes, P < 0.0001), and decreased intraoperative pRBC transfusion (100 vs 270 mL, P < 0.0001). STAR patients had decreased vasoactive-inotropic score on ICU admission (6 vs 10.8, P = 0.0007) and decreased time to chest closure (2 vs 4.5 days, P = 0.0004). STAR patients had lower peak lactate (8.1 vs 9.9 mmol/L, P = 0.03) and more rapid lactate normalization (18.3 vs 27.0 hours, P = 0.003). In-hospital mortality in STAR patients was 2.7% vs 15.1% with SNR (P = 0.06) and 10.3% in the PC4 aggregate (P = 0.14). STAR perfusion is a novel approach to Norwood reconstruction associated with excellent survival, decreased transfusions, shorter operative time, and improved convalescence in the early post-operative period.

Mycotic Pseudoaneurysm: Rare Complication of a Norwood/Sano Graft Stump.

The Sano variant of the stage 1 Norwood procedure is commonly performed as initial palliation for hypoplastic left heart syndrome. We present a case of a 2-year-old who developed a mycotic pseudoaneurysm of the Sano graft and discuss the importance of imaging and operative management of this rare presentation. Localization and full description of the lesion require compulsive, often multimodality imaging.

Ductal arch decoded: the use of its spatial fingerprint to design a Norwood type of patch.

Reconstruction of the aortic arch for the Norwood procedure remains a focus of attention in terms of the management of the distal anastomosis [1,2], patch design and material [3,4], and fashioning the Damus-Kaye-Stansel itself [5]. The reconstructed aorta supplies the coronaries and the head and neck vessels and directs flow to the descending aorta. As the fetus develops, the right ventricle shunts to the aorta through the ductal arch, supporting a great percentage of the systemic and the placental circulation. We have developed a method of designing a Norwood patch by decoding the 3-dimensional geometry of the arterial duct and its arch.

HLHS: Complex Anatomic Surgical Considerations During Treatment Pathway.

The Norwood operation as first-stage palliation for hypoplastic left heart syndrome has been standardized in most centers. However, in the presence of anomalies of aortic arch anatomy or position, or other anatomical variants, important modifications of the procedure may become necessary. This report summarizes surgical techniques to address these challenges.

The pediatric heart network's study on long-term outcomes of children with HLHS and the impact of Norwood Shunt type in the single ventricle reconstruction trial cohort (SVRIII): Design and adaptations.

BACKGROUND: The Single Ventricle Reconstruction (SVR) Trial was the first randomized clinical trial of a surgical approach for treatment of congenital heart disease. Infants with hypoplastic left heart syndrome (HLHS) and other single right ventricle (RV) anomalies were randomized to a modified Blalock Taussig Thomas shunt (mBTTS) or a right-ventricular-to-pulmonary-artery shunt (RVPAS) at the time of the Norwood procedure. The aim of the Long-term Outcomes of Children with HLHS and the Impact of Norwood Shunt Type (SVR III) study is to compare early adolescent outcomes including measures of cardiac function, transplant-free survival, and neurodevelopment, between those who received a mBTTS and those who received an RVPAS. METHODS: Transplant-free survivors of the SVR cohort were enrolled at 10 to 15 years of age for multifaceted in-person evaluation of cardiac function (cardiac magnetic resonance [CMR], echocardiogram and exercise test) and neurodevelopmental evaluation. Right ventricular ejection fraction measured by CMR served as the primary outcome. Development of arrhythmias, protein losing enteropathy, and other comorbidities were assessed through annual medical history interview. Through the course of SVR III, protocol modifications to engage SVR trial participants were designed to enhance recruitment and retention. CONCLUSIONS: Evaluation of long-term outcomes will provide important data to inform decisions about the shunt type placed at the Norwood operation and will improve the understanding of cardiovascular and neurodevelopmental outcomes for early adolescents with HLHS.