Reconstruction of Fibrous Skeleton of the Heart for Double-Valve Replacement in A Pediatric Patient - Modified Commando Procedure.

The so-called Commando procedure, initially described by David and colleagues, consists in the reconstruction of the mitro-aortic fibrous lamina by a patch that enlarges both annuli. Its use has been described to upsize the aortic and mitral annulus for double valve replacement in adolescents. We describe a modified technique of this reconstruction of the fibrous skeleton of the heart, combined with Konno procedure to further enlarge the aortic annulus. In modified Commando procedure, following the reconstruction of aortomitral continuity with a bovine pericardium CardioCel patch (Admedus Regen Pty Ltd, Perth, WA, Australia), an aortic valved conduit that was made on the bench in order to have bottom skirt that enabled the suturing of the composite conduit far inside the left ventricle outflow tract. Coronary buttons were implanted at the supra-commissural level. The advantages of this modified Commando procedure are (1) the creation of a new aortic annulus when the integrity of this annulus has been compromised, (2) the upsizing of both annuli to any possible size of aortic and mitral prostheses, and (3) the relief of any residual left ventricular outflow tract obstruction.

Pediatric Tele-Critical Care: Initial Experience with a Continuous Surveillance Model Aiming to Prevent Cardiac Arrest in Children with Critical Heart Disease.

Introduction: Despite advances in treatment of children with critical heart disease, cardiac arrest (CA) remains a common occurrence. We provided virtual support to bedside teams (BTs) from a tele-critical care (TCC) unit in a pediatric cardiac intensive care unit (CICU) and focused on early detection of concerning trends (CT) and avoidance of CA. Virtual surveillance workflows included a review of remote monitoring, video feed from patient room cameras, medical records, and artificial intelligence tools. We present our initial experience with a focus on critical communications (CCs) to BTs. Methods: A retrospective, descriptive review of TCC activities was conducted from January 2019 to December 2022, involving electronic databases and electronic medical records of patients in the CICU, including related CCs to BTs, responses from BTs, and related CA. Results: We conducted 18,171 TCC activities, including 2,678 non-CCs and 248 CCs. Over time, there was a significant increase in the proportion of CCs related with CT (p = 0.002), respiratory concerns (<0.001), and abnormalities in cardiac rhythm (p = 0.04). Among a sample of 244 CCs, subsequent interventions by BTs resulted in adjustment of medical treatment (127), respiratory support (68), surgery or intervention (19), cardiac rhythm control (17), imaging study (14), early resuscitation (9), and others (10). Conclusions: CCs from a TCC unit in a pediatric CICU changed over time with an increased focus on CT and resulted in early interventions, potentially contributing to avoiding CA. This model of care in pediatric cardiac critical care has the potential to improve patient safety.

Survival benefits of the wait-and-grow approach in small babies (≤2000 g) requiring heart surgery.

Objective: The best approach to minimize the observed higher mortality of newborn infants with low birth weight who require congenital heart surgery is unclear. This retrospective study was designed to review outcomes of newborn infants weighing <2000 g who have undergone cardiovascular surgery to identify patient parameters and clinical strategies for care associated with higher survival. Methods: A retrospective chart review of 103 patients who underwent cardiovascular surgery from 2010 to 2021 who were identified as having low birth weight (≤2000 g). Patients who underwent only patent ductus arteriosus ligation or weighing >3500 g at surgery were excluded. Results: Median age was 24 days and weight at the time of surgery was 1920 g. Twenty-six (25%) operative mortalities were recorded. Median follow-up period was 2.7 years. The 1- and 3-year overall Kaplan-Meier survival estimate was 72.4% ± 4.5% and 69.1% ± 4.6%. The 1-year survival of patients who had a weight increase >300 g from birth to surgery was far superior to the survival of those who did not achieve such a weight gain (81.4% ± 5.6% vs 64.0% ± 6.7%; log-rank P = .04). By multivariable Cox-hazard regression analysis, the independent predictor of 1-year mortality was genetic syndrome (hazard ratio, 3.54; 95% CI, 1.67-7.82; P < .001), whereas following a strategy of increasing weight from birth to surgery resulted in lower mortality (hazard ratio, 0.49; 95% CI, 0.24-0.90; P = .02). Conclusions: A strategy of wait and grow for newborn infants with very low birth weight requiring heart surgery results in better survival than immediate surgery provided that the patient's condition allows for this waiting period.

Repair of the complete atrioventricular septal defect-impact of postoperative moderate or more regurgitation.

OBJECTIVES: To study the risk factors for mortality, moderate or more left atrioventricular valve regurgitation (LAVVR) and reoperation after the surgical repair of complete atrioventricular septal defect (cAVSD) in a single centre. METHODS: The current study is a retrospective review of patients who underwent surgical repair of cAVSD between 2000 and 2021. Patients with unbalanced ventricles not amenable to biventricular repair, double outlet right ventricle and malpositioned great arteries were excluded. The clinical predictors of outcome for end points were analysed with univariate and multivariable Cox regression analysis or Fine-Gray modelling for competing risks. Time-dependent end points were estimated using the Kaplan-Meier curve analysis and cumulative incidence curves. RESULTS: The median follow-up time was 2.3 years. Among 220 consecutive patients were 10 (4.6%) operative and 21 late mortalities (9.6%). A total of 26 patients were identified to have immediate postoperative moderate or more regurgitation and 10 of them ultimately died. By multivariable analysis prematurity and having more than moderate regurgitation immediately after the operation were identified as predictors of overall mortality (P = 0.003, P = 0.012). Five- and ten-year survival rates were lower for patients with immediate postoperative moderate or more LAVVR {51.9% [confidence interval (CI): 27.5-71.7%]} when compared to patients without moderate or more regurgitation [93.2% (CI: 87.1-96.4%) and 91.3% (CI: 83.6-95.5%)]. CONCLUSIONS: The patients who undergo cAVSD repair remain subjected to a heavy burden of disease related to postoperative residual LAVVR. Immediate postoperative moderate or more LAVVR contributes significantly to overall mortality. Whether a second run of bypass can decrease this observed mortality should be investigated.

Alarming rate of liver cirrhosis after the small conduit extracardiac Fontan: A comparative analysis with the lateral tunnel.

BACKGROUND: The association between the prevalence of cirrhosis and the types of Fontan operations remains unclear. METHODS: We conducted a retrospective chart review of 332 patients who underwent the Fontan procedure at our institution. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis. Patients who had intracardiac-extracardiac conduit (126) between 1989 and 2021 were pooled with those having extracardiac conduit (ECC) (134). The 260 patients who underwent the ECC and the 68 patients who had the lateral tunnel (LT) Fontan constitute the core of the study. RESULTS: Median age at the Fontan procedure was 23.7 months (interquartile range [IQR], 20.8-32.6) in the LT group, compared with 28.8 months (IQR, 24.6-39.5) in the ECC group (P < .01). The median follow-up was 14.8 years (IQR, 12.5-16.5) in the LT group and 7 years (IQR, 2.8-10.4) in the extracardiac conduit group. During the follow-up period, 3 patients (4.4%) with LT and 17 patients (6.5%) with ECC (11 patients with 16 mm or less conduit size) were diagnosed with cirrhosis. The prevalence of cirrhosis at 1, 5, 10, and 15 years was 0%, 0%, 0%, and 4.4% in the LT group, respectively, and 0%, 0.9%, 7.7%, and 29.8% in the ECC group (P < .01) Rates of mortality, Fontan revision, Fontan takedown, transplant, and complications were comparable between the 2 groups. CONCLUSIONS: The extracardiac conduit Fontan seems to be associated with faster development of cirrhosis.

Young infants with symptomatic tetralogy of Fallot: Shunt or primary repair?

Objectives: The optimal treatment strategy for symptomatic young infants with tetralogy of Fallot (TOF) is unclear. We sought to compare the outcomes of staged repair (SR) (shunt palliation followed by second-stage complete repair) versus primary repair (PR) at 2 institutions that have exclusively adopted each strategy. Methods: We performed propensity score-matched comparison of 143 infants under 4 months of age who underwent shunt palliation at one institution between 1993 and 2021 with 122 infants who underwent PR between 2004 and 2018 at another institution. The primary outcome was mortality. Secondary outcomes were postoperative complications, durations of perioperative support and hospital stays, and reinterventions. Median follow-up was 8.3 years (interquartile range, 8.1-13.4 years). Results: After the initial procedure, hospital mortality (shunt, 2.8% vs PR, 2.5%; P = .86) and 10-year survival (shunt, 95%; 95% confidence interval [CI], 90%-98% vs PR, 90%; 95% CI, 81%-95%; P = .65) were similar. The SR group had a greater risk of early reinterventions but similar rates of late reinterventions. Propensity score matching yielded 57 well-balanced pairs. In the matched cohort, the SR group had similar freedom from reintervention (55%; 95% CI, 39%-68% vs 59%; 95% CI, 43%-71%; P = .85) and greater survival (98%; 95% CI, 88%-99.8% vs 85%; 95% CI, 69%-93%; P = .02) at 10 years, as the result of more noncardiac-related mortalities in the PR group. Conclusions: In symptomatic young infants with TOF operated at 2 institutions with exclusive treatment protocols, the SR strategy was associated with similar cardiac-related mortality and reinterventions as the PR strategy at medium-term follow-up.

Excellent Survival With a Focus on Pulmonary Artery Reinterventions After the Arterial Switch Operation.

Background: We sought to evaluate the outcomes in patients who underwent the arterial switch operation (ASO) over a 20-year period at a single institution. Methods: The current study is a retrospective review of 180 consecutive patients who underwent the ASO for biventricular surgical correction of dextro-transposition of the great arteries (d-TGA) between 2002 and 2022. Results: Among 180 patients, 121 had TGA-intact ventricular septum, 47 had TGA-ventricular septal defect and 12 had Taussig-Bing Anomaly (TBA). The median follow-up time was 6.7 years (interquartile range: 3.9-8.7 years). There were five early (2.8%) and one late (0.6%) mortality. Survival was 96.6% at one year and beyond. Reoperations were performed in 31 patients (17%). Taussig Bing Anomaly was found to increase the risk of reoperation by 17 times (P < .0001). A total of 37 (21%) patients underwent 53 reinterventions (14 surgical procedures, 39 catheter interventions) specifically addressing pulmonary artery (PA) stenosis. Freedom from PA reintervention was 97%, 87%, 70%, and 55% at 1, 5, 10, and 15 years, respectively. By bivariable analysis, TBA (P = .003, odds ratio [OR]: 6.4, 95% confidence interval [CI]: 1.9-21.7), mild PA stenosis at discharge (P ≤ .001, OR: 6.1, 95% CI: 2.7-13.6), and moderate or severe PA stenosis at discharge (P ≤ .001, OR: 12.7, 95% CI: 5-32.2) were identified as predictors of reintervention on PA. In the last follow-up of 174 survivors, 24 patients (14%) had moderate or greater PA stenosis, two (1%) had moderate neoaortic valve regurgitation, and 168 were New York Heart Association status I. Conclusions: Our results demonstrated excellent survival and functional status following the ASO for d-TGA; however, patients remain subject to frequent reinterventions especially on the pulmonary arteries.

Left Atrioventricular Valve Regurgitation After Atrioventricular Septal Defect Repair.

BACKGROUND: Whether patients with moderate left atrioventricular valve regurgitation (LAVVR) after surgical repair of complete atrioventricular septal defect (CAVSD) should be observed or undergo reoperation remains unclear. METHODS: Moderate LAVVR was diagnosed in 87 of 220 patients who underwent CAVSD repair: 47 during the initial hospital stay and 40 after a median of 7 months (interquartile range, 2-18 months) after the initial operation. RESULTS: Of these 87 patients who had moderate LAVVR, 15 died, for an overall mortality of 17%. The regurgitation became severe in 39 patients (45%) within a median of 2 months (interquartile range, 1-7 months) leading to 33 reoperations and 10 deaths. In 23 of 87 patients (26%), regurgitation remained at a moderate level over a median follow-up period of 8 months (interquartile range, 1-48 months). In 25 of 87 patients (29%), the regurgitation decreased to mild after a median of 9 months (interquartile range, 5-19 months). The only independent risk factor for increased severity of regurgitation and reoperation was the echocardiographic appearance of the jet centered around the cleft rather than central at the time of diagnosis of moderate regurgitation (odds ratio, 3.5; 95% CI, 1.5-9.0; P = .007). CONCLUSIONS: Moderate LAVVR after CAVSD repair is often linked to death and reoperation, but regurgitation remains stable in one-quarter of patients and improves in one-third. The deterioration usually occurs within the first year after surgery. The initial observation of patients with residual or new moderate regurgitation for up to 1 year or until further deterioration seems reasonable, as long as the regurgitation is centrally located.

The Use of the CardioCel 3D 60° Patch for Aortic Arch Reconstruction in Infancy-A Word of Caution.

BACKGROUND: There are limited data on outcomes after implantation of the CardioCel 3D 60° patch in great vessel repair. After anecdotally witnessing an increase in negative outcomes, we reviewed our experience using this patch in our neonate and infant patients undergoing aortic arch repair. METHODS: Aortic arch repair with implantation of the CardioCel 3D 60° patch was performed in 24 patients between July 2018 and July 2021. Dominant cardiac morphologies were hypoplastic left heart syndrome (66%), atrioventricular canal defects (13%), and other (21%). Median age at implantation was 44 days (interquartile range [IQR], 6-112 days). Recurrent obstruction was defined as the need for reoperation or catheter intervention or recurrent peak pressure gradient of descending aorta ≥25 mm Hg on echocardiography. RESULTS: Five deaths occurred after a median of 217 days (IQR, 69-239 days). Twelve patients (50%) had recurrent obstruction. Three patients (13%) required redo aortic arch operation after a median of 148 days (IQR, 128-193 day), with extensive fibrous coating of the patch interior causing obstruction. Eleven patients (46%) required at least 1 balloon angioplasty on their aorta after a median of 102 days (IQR, 83-130 days) after repair, and 3 needed >1 catheter intervention. The estimated probability of having recurrent obstruction was 85% at 6 months and 71% at the 1-year follow (P = .06). CONCLUSIONS: Recurrent aortic obstruction occurred in half of our patients shortly after repair. The use of the CardioCel 3D 60° patch for aortic arch reconstruction in neonates and infants should be reevaluated.

Brain injury and neurodevelopmental outcomes in children undergoing surgery for congenital heart disease.

Objectives: Brain injury is commonly seen on magnetic resonance imaging in infants with complex congenital heart disease. The impact of perioperative brain injury on neurodevelopmental outcomes is not well understood. We evaluate the association of brain injury and other markers on neurodevelopmental outcomes in patients undergoing surgery for congenital heart surgery during infancy. Methods: Term newborns with infant cardiac surgery performed between 2008 and 2019 at a single tertiary center, and both preoperative and postoperative brain magnetic resonance imaging were included. Those with underlying genetic conditions were excluded. Brain injury was characterized using an magnetic resonance imaging scoring system. Neurodevelopmental outcomes were assigned using the Pediatric Stroke Outcome Measure and Glasgow Outcome Scale Extended. Independent risk factors for poor neurodevelopmental outcomes were determined by multivariable Cox regression. Results: A total of 122 patients were included. New or progressive postoperative brain injury was noted in 69 patients (57%). A total of 101 patients (83%) had at least 1 neurodevelopmental assessment (median age 36 months) with an early assessment (5-24 months) performed in 95 children. Multivariable Cox regression analysis of early neurodevelopmental outcomes identified new stroke on postoperative magnetic resonance imaging to be an independent predictor of poor neurodevelopmental outcome. Postoperative peak lactate was an independent predictor of poor outcome assessed by the Pediatric Stroke Outcome Measure and Glasgow Outcome Scale Extended. Conclusions: Our study reveals that evidence of new stroke on magnetic resonance imaging after infant congenital heart surgery is a predictor of poor neurodevelopmental outcomes in early childhood. Postoperative lactic acidosis is associated with poor neurodevelopmental outcome and may be a surrogate biomarker for ischemic brain injury.

Connecting Transcriptomics with Computational Modeling to Reveal Developmental Adaptations in the Human Pediatric Myocardium.

Background: Nearly 1% or 1.3 million babies are born with congenital heart disease (CHD) globally each year - many of whom will require palliative or corrective heart surgery within the first few years of life. A detailed understanding of cardiac maturation can help to expand our knowledge on cardiac diseases that develop during gestation, identify age-appropriate cardiovascular drug therapies, and inform clinical care decisions related to surgical repair, myocardial preservation, or postoperative management. Yet, to date, our knowledge of the temporal changes that cardiomyocytes undergo during postnatal development is largely limited to animal models. Methods: Right atrial tissue samples were collected from n=117 neonatal, infant, and pediatric patients undergoing correct surgery due to (acyanotic) CHD. Patients were stratified into five age groups: neonate (0-30 days), infant (31-364 days), toddler to preschool (1-5 years), school age (6-11 years), and adolescent to young adults (12-32 years). We measured age-dependent adaptations in cardiac gene expression, and used computational modeling to simulate action potential and calcium transients. Results: Enrichment of differentially expressed genes (DEG) was explored, revealing age-dependent changes in several key biological processes (cell cycle, cell division, mitosis), cardiac ion channels, and calcium handling genes. Gene-associated changes in ionic currents exhibited both linear trends and sudden shifts across developmental stages, with changes in calcium handling ( I NCX ) and repolarization ( I K1 ) most strongly associated with an age-dependent decrease in the action potential plateau potential and increase in triangulation, respectively. We also note a shift in repolarization reserve, with lower I Kr expression in younger patients, a finding likely tied to the increased amplitude of I Ks triggered by elevated sympathetic activation in pediatric patients. Conclusion: This study provides valuable insights into age-dependent changes in human cardiac gene expression and electrophysiology among patients with CHD, shedding light on molecular mechanisms underlying cardiac development and function across different developmental stages.