Persistent acute kidney injury and fluid accumulation with outcomes after the Norwood procedure: report from NEPHRON.

BACKGROUND: Cardiac surgery-associated acute kidney injury (CS-AKI) is common, but its impact on clinical outcomes is variable. Parsing AKI into sub-phenotype(s) and integrating pathologic positive cumulative fluid balance (CFB) may better inform prognosis. We sought to determine whether durational sub-phenotyping of CS-AKI with CFB strengthens association with outcomes among neonates undergoing the Norwood procedure. METHODS: Multicenter, retrospective cohort study from the Neonatal and Pediatric Heart and Renal Outcomes Network. Transient CS-AKI: present only on post-operative day (POD) 1 and/or 2; persistent CS-AKI: continued after POD 2. CFB was evaluated per day and peak CFB during the first 7 postoperative days. Primary and secondary outcomes were mortality, respiratory support-free and hospital-free days (at 28, 60 days, respectively). The primary predictor was persistent CS-AKI, defined by modified neonatal Kidney Disease: Improving Global Outcomes criteria. RESULTS: CS-AKI occurred in 59% (205/347) neonates: 36.6% (127/347) transient and 22.5% (78/347) persistent; CFB > 10% occurred in 18.7% (65/347). Patients with either persistent CS-AKI or peak CFB > 10% had higher mortality. Combined persistent CS-AKI with peak CFB > 10% (n = 21) associated with increased mortality (aOR: 7.8, 95% CI: 1.4, 45.5; p = 0.02), decreased respiratory support-free (predicted mean 12 vs. 19; p < 0.001) and hospital-free days (17 vs. 29; p = 0.048) compared to those with neither. CONCLUSIONS: The combination of persistent CS-AKI and peak CFB > 10% after the Norwood procedure is associated with mortality and hospital resource utilization. Prospective studies targeting intra- and postoperative CS-AKI risk factors and reducing CFB have the potential to improve outcomes.

Sonographic comparison of subcutaneous fat layer thickness in the scalp area in patients with androgenetic alopecia compared to healthy individuals: Cross-sectional.

INTRODUCTION: Androgenetic alopecia (AGA) is one of the most common alopecia among men and women worldwide. It is a nonscarring alopecia that has a characterized pattern. In female pattern AGA, the hairline is stable but general thinning occurs most notably in the frontal region. In male-pattern AGA, the hairline is receding and the thinning is most notable in the frontotemporal region. AGA has a complex pathogenesis and relation of subcutaneous fat in the scalp region and the miniaturization of terminal hair follicles is vague. In this study, subcutaneous fat in the frontal scalp an important region for AGA is compared to the occipital scalp that is spared in AGA. METHOD: Our study is a cross-sectional study that has four groups. Male patient, female patient, male control, female control. Every group has 15 individuals. All of the people in the study are those referred to Rasoul Akram's dermatology clinic. The severity of alopecia is classified by Norwood scaling for male pattern AGA and Ludwig scaling for female pattern AGA. Subcutaneous tissue in the frontal and occipital regions is measured by ultrasonography. For evaluating the effect of aging on subcutaneous fat thickness, we subdivided any group into more than 40 years old and between 20 and 40 years old and compared these two subgroups. RESULTS: The mean age of the three groups of male patient, female patient, and female control is 40 y/o and the mean age of male control is 41 y/o. The mean subcutaneous fat layer thickness in frontal region in male patients group is 6.0 mm (more than 40 y/o = 6.6 mm, between 20 and 40 y/o = 5.5 mm), in female patients group 5.1 mm (more than 40 y/o = 5.7 mm, between 20 and 40 y/o = 4.6 mm), in the male control group is 4.4 mm (more than 40 y/o = 4.7 mm, between 20 and 40 y/o = 4 mm) and in the female control group is 4.1 mm (more than 40 y/o = 4.5 mm, between 20 and 40 y/o = 3.6 mm). The mean subcutaneous fat layer thickness in the occipital region in the male patient's group is 6.4 mm (more than 40 y/o = 6.7 mm, between 20 and 40 y/o = 6 mm), in the female patient's group 6.1 mm (more than 40 y/o = 6.5 mm, between 20 and 40 y/o = 5.7 mm), in the male control group is 6.3 mm (more than 40 y/o = 6.8 mm, between 20 and 40 y/o = 5.7 mm) and in the female control group is 6.2 mm (more than 40 y/o = 6.6 mm, between 20 and 40 y/o = 5.8 mm). CONCLUSION: This study demonstrates that the subcutaneous fat layer in the frontal region in both males and females is thicker in AGA patients than healthy group and the more severe the AGA, the thicker is subcutaneous layer in the frontal region. In the male patients group, the subcutaneous fat layer in the frontal region is thicker than in the female patients group but in the male and female control groups is not so different. The subcutaneous fat layer in the occipital region is thicker in older individuals in both patients and control groups but is not different when compared to AGA patients and control individuals.

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.

Short and Mid-term Outcomes of Ductal Graft with Bilateral Pulmonary Banding in Hypoplastic Left Heart Syndrome and Variants.

This study evaluates a novel surgical technique inspired by the hybrid Norwood procedure, which involves ductal graft placement and bilateral pulmonary artery banding. This technique is designed to avoid the high-risk classical Norwood stage 1 operation and to avoid the need for first stage invasive catheter interventions and interstage catheter interventions when ductal stent placement is not feasible in developing countries with limited resources. Between June 2015 and December 2022, 14 patients with hypoplastic left heart syndrome and variants were treated using this procedure at our center. Polytetrafluoroethylene graft was used for ductal graft procedure and polytetrafluoroethylene bands were used for bilateral pulmonary banding. Atrial septectomy was done selectively. Patients with adequate left heart growth underwent biventricular repair and pulmonary debanding. Patients without left heart growth underwent cavopulmonary anastomosis or necessary intermediate surgical revisions. Seven patients out of 14 reached the second stage. Two received biventricular repair, three underwent bidirectional cavopulmonary anastomosis, and two required repeat palliations other than bidirectional cavopulmonary anastomosis. Modifications of the Norwood procedure is an ongoing process due to the complex nature of hypoplastic left heart syndrome and variants. As experience grows, the mortality associated with ductal graft and bilateral pulmonary banding procedure may decrease, making this technique an alternative for centers where classical Norwood operation or hybrid palliation is not possible, especially in developing countries with limited resources. We might predict promising outcomes, especially for patients with hypoplastic left heart complex where biventricular repair is expected.

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.

Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome.

Controlling pulmonary blood flow in patients who have undergone Norwood palliation, especially early postoperatively, is challenging due to a change in the balance of systemic and pulmonary vascular resistance. We applied a combination therapy of clipping and balloon angioplasty for right ventricle-pulmonary artery (RV-PA) shunt to control pulmonary blood flow, but the influence of the combination therapy on the PA condition is uncertain. Retrospectively analysis was conducted of all infants with hypoplastic left heart syndrome who had undergone Norwood palliation with RV-PA shunt at Okayama University Hospital from January 2008 to September 2022. A total of 50 consecutive patients underwent Norwood palliation with RV-PA shunt in this study period. Of them, 29 patients underwent RV-PA shunt flow clipping, and the remaining 21 had unclipped RV-PA shunt. Twenty-three patients underwent balloon angioplasty for RV-PA shunt with clips. After balloon angioplasty, oxygen saturation significantly increased from 69 (59-76)% to 80 (72-86)% (p < 0.001), and the narrowest portion of the clipped conduit significantly improved from 2.8 (1.8-3.4) to 3.8 (2.9-4.6) mm (p < 0.001). In cardiac catheterizations prior to Bidirectional cavo-pulmonary shunt (BCPS), there were no significant differences in pulmonary-to-systemic flow ratio (Qp/Qs), ventricular end-diastolic pressure, Nakata index, arterial saturation, mean pulmonary artery pressure and pulmonary vascular resistance index. On the other hand, in Cardiac catheterizations prior to Fontan, Nakata index was larger in the clipped group (p = 0.02). There was no statistically significant difference in the 5-year survival between the two groups (clipped group 96%, unclipped group 74%, log-rank test: p = 0.13). At least, our combination therapy of clipping and balloon angioplasty for RV-PA shunt did not negatively impact PA growth. Although there is a trend toward better but not statistically significant difference in outcomes in the clipped group compared to the non-clipped group, this treatment strategy may play an important role in improving outcomes in hypoplastic left heart syndrome.

Factors that mediate change in creatinine and acute kidney injury after the Norwood operation: insights from high-fidelity haemodynamic monitoring data.

BACKGROUND: Acute kidney injury is a common postoperative complication of paediatric cardiac surgery associated with increased morbidity and mortality. The purpose of this study is to characterise associations between haemodynamic parameters, clinical parameters, and medical interventions, on acute kidney injury. METHODS: Nine patients with univentricular physiology undergoing the Norwood procedure from a single-centre tertiary care paediatric cardiac ICU were included (September 2022 to March 2023). Patients were monitored with the T3 software. Data were analysed using a Fisher exact test, Mann-Whitney-U test, LASSO-based machine learning techniques, and receiver operator curve analyses. RESULTS: Over 27,000 datapoints were included. Acute kidney injury occurred in 2 patients (22%) during this period. Net fluid balance and renal oxygen extraction were independently associated with acute kidney injury, while commonly used metrics of pressure (systolic, diastolic, or mean arterial blood pressure) were not. The resulting acute kidney injury risk score was (4.1 × fluid balance) + (1.9 × renal oxygen extraction). The risk score was significantly higher in acute kidney injury with a score of 32.9 compared to 7.9 (p < 0.01). Optimal cut-offs for fluid balance (7 mL/hr) and renal oxygen extraction (29%) were identified. Higher serum creatinine:baseline creatinine ratio was associated with a higher mean airway pressure, higher renal oxygen extraction, higher mean arterial blood pressure, higher vasoactive inotropic score, and fluid balance. CONCLUSION: Among patients with univentricular physiology undergoing the Norwood procedure, renal oxygen extraction and a higher net fluid balance are independently associated with increased risk of acute kidney injury. Renal perfusion pressure is not significantly associated with acute kidney injury.

Ventriculocoronary connection with mitral stenosis/aortic stenosis hypoplastic left heart: a case report.

Ventriculocoronary connections develop most commonly in children with mitral stenosis/aortic atresia hypoplastic left heart. These connections can lead to myocardial ischaemia and dysfunction. We report a newborn with mitral stenosis/aortic stenosis hypoplastic left heart who endured systemic ventricular injury post-Norwood, secondary to a large ventriculocoronary fistula. He was treated medically and had favourable outcomes following bidirectional Glenn procedure.

Factors associated with renal oxygen extraction in mechanically ventilated children after the Norwood operation: insights from high fidelity haemodynamic data.

BACKGROUND: Maintaining the adequacy of systemic oxygen delivery is of utmost importance, particularly in critically ill children. Renal oxygen extraction can be utilised as metric of the balance between systemic oxygen delivery and oxygen consumption. The primary aim of this study was to determine what clinical factors are associated with renal oxygen extraction in children after Norwood procedure. METHODS: Mechanically ventilated children who underwent Norwood procedure from 1 September, 2022 to 1 March, 2023 were identified as these patients had data collected and stored with high fidelity by the T3 software. Data regarding haemodynamic values, fluid balance, and airway pressure were collected and analysed using Bayesian regression to determine the association of the individual metrics with renal oxygen extraction. RESULTS: A total of 27,270 datapoints were included in the final analyses. The resulting top two models explained had nearly 80% probability of being true and explained over 90% of the variance in renal oxygen extraction. The coefficients for each variable retained in the best were -1.70 for milrinone, -19.05 for epinephrine, 0.129 for mean airway pressure, -0.063 for mean arterial pressure, 0.111 for central venous pressure, 0.093 for arterial saturation, 0.006 for heart rate, -0.025 for respiratory rate, 0.366 for systemic vascular resistance, and -0.032 for systemic blood flow. CONCLUSION: Increased milrinone, epinephrine, mean arterial pressure, and systemic blood flow were associated with decreased (improved) renal oxygen extraction, while increased mean airway pressure, central venous pressure, arterial saturation, and systemic vascular resistance were associated with increased (worsened) renal oxygen extraction.

Treatment of hypoplastic left heart syndrome: a systematic review and meta-analysis of randomised controlled trials.

BACKGROUND: This meta-analysis aimed to consolidate existing data from randomised controlled trials on hypoplastic left heart syndrome. METHODS: Hypoplastic left heart syndrome specific randomised controlled trials published between January 2005 and September 2021 in MEDLINE, EMBASE, and Cochrane databases were included. Regardless of clinical outcomes, we included all randomised controlled trials about hypoplastic left heart syndrome and categorised them according to their results. Two reviewers independently assessed for eligibility, relevance, and data extraction. The primary outcome was mortality after Norwood surgery. Study quality and heterogeneity were assessed. A random-effects model was used for analysis. RESULTS: Of the 33 included randomised controlled trials, 21 compared right ventricle-to-pulmonary artery shunt and modified Blalock-Taussig-Thomas shunt during the Norwood procedure, and 12 regarded medication, surgical strategy, cardiopulmonary bypass tactics, and ICU management. Survival rates up to 1 year were superior in the right ventricle-to-pulmonary artery shunt group; this difference began to disappear at 3 years and remained unchanged until 6 years. The right ventricle-to-pulmonary artery shunt group had a significantly higher reintervention rate from the interstage to the 6-year follow-up period. Right ventricular function was better in the modified Blalock-Taussig-Thomas shunt group 1-3 years after the Norwood procedure, but its superiority diminished in the 6-year follow-up. Randomised controlled trials regarding medical treatment, surgical strategy during cardiopulmonary bypass, and ICU management yielded insignificant results. CONCLUSIONS: Although right ventricle-to-pulmonary artery shunt appeared to be superior in the early period, the two shunts applied during the Norwood procedure demonstrated comparable long-term prognosis despite high reintervention rates in right ventricle-to-pulmonary artery shunt due to pulmonary artery stenosis. For medical/perioperative management of hypoplastic left heart syndrome, further randomised controlled trials are needed to deliver specific evidence-based recommendations.

Impact of calorie intake and weight gain after Norwood procedure on the outcome of stage II palliation.

BACKGROUND: This study aimed to assess the impact of caloric intake and weight-for-age-Z-score after the Norwood procedure on the outcome of bidirectional cavopulmonary shunt. METHODS: A total of 153 neonates who underwent the Norwood procedure between 2012 and 2020 were surveyed. Postoperative daily caloric intake and weight-for-age-Z-score up to five months were calculated, and their impact on outcome after bidirectional cavopulmonary shunt was analysed. RESULTS: Median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Modified Blalock-Taussig shunt was used in 95 patients and right ventricle to pulmonary artery conduit in 58. Postoperatively, total caloric intake gradually increased, whereas weight-for-age-Z-score constantly decreased. Early and inter-stage mortality before stage II correlated with low caloric intake. Older age (p = 0.023) at Norwood, lower weight (p < 0.001) at Norwood, and longer intubation (p = 0.004) were correlated with low weight-for-age-Z-score (< -3.0) at 2 months of age. Patients with weight-for-age-Z-score < -3.0 at 2 months of age had lower survival after stage II compared to those with weight-for-age-Z-score of -3.0 or more (85.3 versus 92.9% at 3 years after stage II, p = 0.017). There was no difference between inter-stage weight gain and survival after bidirectional cavopulmonary shunt between the shunt types. CONCLUSION: Weight-for-age-Z-score decreased continuously throughout the first 5 months after the Norwood procedure. Age and weight at Norwood and intubation time were associated with weight gain. Inter-stage low weight gain (Z-score < -3) was a risk for survival after stage II.

Preterm Birth Is Associated With Adverse Cardiac Remodeling and Worse Outcomes in Patients With a Functional Single Right Ventricle.

OBJECTIVE: To assess the effects of preterm birth on cardiac structure and function and transplant-free survival in patients with hypoplastic left heart syndrome and associated anomalies throughout the staged palliation process. STUDY DESIGN: Data from the Single Ventricle Reconstruction trial were used to assess the impact of prematurity on echocardiographic measures at birth, Norwood, Stage II, and 14 months in 549 patients with a single functional right ventricle. Medical history was recorded once a year using medical records or telephone interviews. Cox regression models were applied to analyze transplant-free survival to age 6 years. Causal mediation analysis was performed to estimate the mediating effect of birth weight within this relationship. RESULTS: Of the 549 participants, 64 (11.7%) were born preterm. Preterm-born participants had lower indexed right ventricle end-diastolic volumes at birth but higher volumes than term-born participants by age 14 months. Preterm-born participants had an increased risk of death or heart transplantation from birth to age 6 years, with an almost linear increase in the observed risk as gestational age decreased below 37 weeks. Of the total effect of preterm birth on transplant-free survival, 27.3% (95% CI 2.5-59.0%) was mediated through birth weight. CONCLUSIONS: Preterm birth is associated with adverse right ventricle remodeling and worse transplant-free survival throughout the palliation process, in part independently of low birth weight. Further investigation into this vulnerable group may allow development of strategies that mitigate the impact of prematurity on outcomes in patients with hypoplastic left heart syndrome.

A word of caution: Early failure of Magmaris® bioresorbable stent after pulmonary artery stenting.

Bioresorbable scaffolds (BRS) have been advocated as the fourth revolution in interventional cardiology medical devices with promising technology to improve the treatment of coronary artery disease with an event-free future. We describe the first reported use and early collapse of the Magmaris® Resorbable Magnesium Scaffold (RMS) stent (BIOTRONIK AG, Switzerland) to relieve left pulmonary artery severe stenosis in a newborn after the Norwood procedure. The stent collapse was detected 2 weeks after implantation and urgently treated with a balloon-expandable stent. This complication raises the alarm about the need to keep implanted RMS under scrutiny. The possibility of faster scaffold resorption in small babies or lack of sufficient radial force of RMS to resist acute vessel recoil has led to ineffective relief of branch pulmonary artery stenosis and failure to enable a safe short-term bridge to Stage II palliation.

Investigating the hemodynamics of Berlin Heart EXCOR support in Norwood patients across diverse clinical scenarios with computational modeling.

BACKGROUND: Infants with single-ventricle (SV) physiology undergo the 3-stage Fontan surgery. Norwood patients, who have completed the first stage, face the highest interstage mortality. The Berlin Heart EXCOR (BH), a pediatric pulsatile ventricular assist device, has shown promise in supporting these patients. However, clinical questions regarding device configurations prevent optimal support. METHODS: We developed a combined idealized mechanics-lumped parameter model of a Norwood patient and simulated two additional patient-specific cases: pulmonary hypertension (PH) and post-operative treatment with milrinone. We quantified the effects of BH support across different device volumes, rates, and inflow connections on patient hemodynamics and BH performance. RESULTS: Increasing device volume and rate increased cardiac output, but with unsubstantial changes in specific arterial oxygen content. We identified distinct SV-BH interactions that may impact patient myocardial health and contribute to poor clinical outcomes. Our results suggested BH settings for patients with PH and for patients treated post-operatively with milrinone. CONCLUSIONS: We present a computational model to characterize and quantify patient hemodynamics and BH support for infants with Norwood physiology. Our results emphasized that oxygen delivery does not increase with BH rate or volume, which may not meet patient needs and contribute to suboptimal clinical outcomes. Our findings demonstrated that an atrial BH may provide optimal cardiac loading for patients with diastolic dysfunction. Meanwhile, a ventricular BH decreased active stress in the myocardium and countered the effects of milrinone. Patients with PH showed greater sensitivity to device volume. In this work, we demonstrate the adaptability of our model to analyze BH support across varied clinical situations.

Markov model for detection of ECG instability prior to cardiac arrest in single-ventricle patients.

OBJECTIVES: Assess the degree of instability in the electrocardiogram (ECG) waveform in patients with single-ventricle physiology before a cardiac arrest and compare them with similar patients who did not experience a cardiac arrest. METHODS: Retrospective control study in patients with single-ventricle physiology who underwent Norwood, Blalock-Taussig shunt, pulmonary artery band, and aortic arch repair from 2013 to 2018. Electronic medical records were obtained for all included patients. For each subject, 6 h of ECG data were analyzed. In the arrest group, the end of the sixth hour coincides with the cardiac arrest. In the control group, the 6-h windows were randomly selected. We used a Markov chain framework and the likelihood ratio test to measure the degree of ECG instability and to classify the arrest and control groups. RESULTS: The study dataset consists of 38 cardiac arrest events and 67 control events. Our Markov model was able to classify the arrest and control groups based on the ECG instability with an ROC AUC of 82% at the hour preceding the cardiac arrests. CONCLUSION: We designed a method using the Markov chain framework to measure the level of instability in the beat-to-beat ECG morphology. Furthermore, we were able to show that the Markov model performed well to distinguish patients in the arrest group compared to the control group.

Medium-term Outcome of Prenatally Diagnosed Hypoplastic Left-Heart Syndrome and Impact of a Restrictive Atrial Septum Diagnosed in-utero.

OBJECTIVE: Surgical outcome data differs from overall outcomes of prenatally diagnosed fetuses with hypoplastic left heart syndrome (HLHS). Our aim was to describe outcome of prenatally diagnosed fetuses with this anomaly. METHODS: Retrospective review of prenatally diagnosed classical HLHS at a tertiary hospital over a 13-year period, estimated due dates 01/08/2006 to 31/12/2019. HLHS-variants and ventricular disproportion were excluded. RESULTS: 203 fetuses were identified with outcome information available for 201. There were extra-cardiac abnormalities in 8% (16/203), with genetic variants in 14% of those tested (17/122). There were 55 (27%) terminations of pregnancy, 5 (2%) intrauterine deaths and 10 (5%) babies had prenatally planned compassionate care. There was intention to treat (ITT) in the remaining 131/201(65%). Of these, there were 8 neonatal deaths before intervention, two patients had surgery in other centers. Of the other 121 patients, Norwood procedure performed in 113 (93%), initial hybrid in 7 (6%), and 1 had palliative coarctation stenting. Survival for the ITT group from birth at 6-months, 1-year and 5-years was 70%, 65%, 62% respectively. Altogether of the initial 201 prenatally diagnosed fetuses, 80 patients (40%) are currently alive. A restrictive atrial septum (RAS) is an important sub-category associated with death, HR 2.61, 95%CI 1.34-5.05, p = 0.005, with only 5/29 patients still alive. CONCLUSION: Medium-term outcomes of prenatally diagnosed HLHS have improved however it should be noted that almost 40% do not get to surgical palliation, which is vital to those doing fetal counselling. There remains significant mortality particularly in fetuses with in-utero diagnosed RAS.

Descriptors of Failed Extubation in Norwood Patients Using Physiologic Data Streaming.

The objective of this study is to evaluate the utility of high-frequency physiologic data during the extubation process and other clinical variables for describing the physiologic profile of extubation failure in neonates with hypoplastic left heart syndrome (HLHS) post-Norwood procedure. This is a single-center, retrospective analysis. Extubation events were collected from January 2016 until July 2021. Extubation failure was defined as the need for re-intubation within 48 h of extubation. The data included streaming heart rate, respiratory rate, blood pressure, arterial oxygen saturation, and cerebral/renal near-infrared spectroscopy (NIRS). The most recent blood laboratory results before extubation were also included. These markers, demographics, clinical characteristics, and ventilatory settings were compared between successful and failed extubations. The analysis included 311 extubations. The extubation failure rate was 10%. According to univariable analyses, failed extubations were preceded by higher respiratory rates (p = 0.029), lower end-tidal CO2 (p = 0.009), lower pH (p = 0.043), lower serum bicarbonate (p = 0.030), and lower partial pressure of O2 (p = 0.022). In the first 10 min after extubation, the failed events were characterized by lower arterial (p = 0.028) and cerebral NIRS (p = 0.018) saturations. Failed events were associated with persistently lower values for cerebral NIRS 2 h post-extubation (p = 0.027). In multivariable analysis, vocal cord anomaly, cerebral NIRS at 10 min post-extubation, renal NIRS at pre-extubation and post-extubation, and end-tidal CO2 at pre-extubation remained as significant co-variables. Oximetric indices before, in the 10 min immediately after, and 2 h after extubation and vocal cords paralysis are associated with failed extubation events in patients with parallel circulation.

Fluid Overload and AKI After the Norwood Operation: The Correlation and Characterization of Routine Clinical Markers.

The purpose of this study was to determine the correlation of different methods of assessing fluid overload and determine which metrics are associated with development of acute kidney injury (AKI) in the period immediately following Norwood palliation. This was a retrospective single-center study of Norwood patients from January 2011 through January 2021. AKI was defined using the Kidney Disease Improving Global Outcomes (KDIGO). Patients were separated into two groups: those with AKI and those without. A logistic regression analysis was conducted with AKI at any point in the study period as the dependent variable and clinical and laboratory data as independent variables. Analysis was conducted as a stepwise regression. The coefficients from the logistic regression were then used to develop a cumulative AKI risk score. Spearman correlations were conducted to analyze the correlation of fluid markers. 116 patients were included, and 49 (42.4%) developed AKI. The duration of open chest, duration of mechanical ventilation, need for dialysis, need for extracorporeal membrane oxygenation, and inpatient mortality were associated with AKI (p ≤ 0.05). Stepwise logistic regression demonstrated the following significant independent associations AKI: age at Norwood in days (p < 0.01), blood urea nitrogen (p < 0.01), central venous pressure (p = 0.04), and renal oxygen extraction ratio (p < 0.01). The area under the receiver operating characteristic curve for the logistic regression was 0.74. The fluid markers had weak R-value. Urea, central venous pressure, and renal oxygen extraction ratio are associated with AKI after the Norwood operation. Common clinical metrics used to assess fluid overload are poorly correlated with each other for postoperative Norwood patients.

Norwood Operation with Right Ventricular-Pulmonary Artery Shunt Versus Comprehensive Stage II After Bilateral Pulmonary Artery Banding Palliation.

As a strategy for the primary Norwood operation, the right ventricular-pulmonary artery shunt is associated with satisfactory early outcome. However, use of this shunt after bilateral pulmonary artery banding remains controversial. This study compared the operative outcomes and late hemodynamics in patients who underwent the Norwood operation, preceded by bilateral pulmonary artery banding, with a right ventricular-pulmonary artery shunt or with bidirectional Glenn anastomosis (comprehensive stage II strategy). We retrospectively reviewed 38 patients who underwent the Norwood operation preceded by bilateral pulmonary artery banding between 2004 and 2017. Of these, 17 underwent the Norwood operation with a right ventricular-pulmonary artery shunt (Group S), whereas 21 underwent the comprehensive stage II strategy (Group G). 5 years after the Norwood operation, 10 (60%) and 17 (81%) patients in Group S and Group G, respectively, underwent the Fontan procedure. Group S showed significantly lower pressure in the superior vena cava after bidirectional Glenn anastomosis than Group G (13 ± 2 mmHg vs. 18 ± 3 mmHg; p < 0.01), but pressures were similar after the Fontan procedure. The right ventricular end-diastolic volume at 1 year post-Fontan procedure was significantly higher in Group S than in Group G (142 ± 41% vs. 91 ± 28%; p < 0.01). In terms of early outcomes, the Norwood operation with a right ventricular-pulmonary artery shunt enabled low pressure in the superior vena cava, but in the long term, this shunt adversely influenced the right ventricular volume.

Prevalence and associated factors of post-traumatic stress disorder in parents whose infants have single ventricle heart disease.

INTRODUCTION: Post-traumatic stress disorder occurs in parents of infants with CHD, contributing to psychological distress with detrimental effects on family functioning and well-being. We sought to determine the prevalence and factors associated with post-traumatic stress disorder symptoms in parents whose infants underwent staged palliation for single ventricle heart disease. MATERIALS AND METHODS: A large longitudinal multi-centre cohort study evaluated 215 mothers and fathers for symptoms of post-traumatic stress disorder at three timepoints, including post-Norwood, post-Stage II, and a final study timepoint when the child reached approximately 16 months of age, using the self-report questionnaire Impact of Event Scale - Revised. RESULTS: The prevalence of probable post-traumatic stress disorder post-Norwood surgery was 50% of mothers and 39% of fathers, decreasing to 27% of mothers and 24% of fathers by final follow-up. Intrusive symptoms such as flashbacks and nightmares and hyperarousal symptoms such as poor concentration, irritability, and sudden physical symptoms of racing heart and difficulty breathing were particularly elevated in parents. Higher levels of anxiety, reduced coping, and decreased satisfaction with parenting were significantly associated with symptoms of post-traumatic stress disorder in parents. Demographic and clinical variables such as parent education, pre-natal diagnosis, medical complications, and length of hospital stay(s) were not significantly associated with symptoms of post-traumatic stress disorder. DISCUSSION: Parents whose infants underwent staged palliation for single ventricle heart disease often reported symptoms of post-traumatic stress disorder. Symptoms persisted over time and routine screening might help identify parents at-risk and prompt referral to appropriate supports.