Multiphysiologic State Computational Fluid Dynamics Modeling for Planning Fontan With Interrupted Inferior Vena Cava.

Background: Single ventricle (SV) patients with interrupted inferior vena cava (iIVC) and azygos continuation are at high risk for unbalanced hepatic venous flow (HVF) distribution to the lungs after Fontan completion and subsequent pulmonary arteriovenous malformations (AVMs) formation. Objectives: The aim of the study was to utilize computational fluid dynamics (CFD) analysis to avoid maldistribution of HVF to the lungs after Fontan surgery. Methods: Four SV subjects with iIVC were prospectively studied with a 3-dimensional (3D) modeling workflow with digital 3D models created from segmented magnetic resonance images or computer tomography scans, virtual surgery, and CFD analysis over multiple physiologic states for the evaluation of operative plans to achieve balanced HVF to both lungs. Three of the patients were Fontan revision candidates with existing AVMs. All patients underwent Fontan completion or revision surgery. Results: CFD predicted that existing or proposed Fontan completion in all patients would result in 100% of HVF to one lung. Improved HVF balance was achieved with CFD analysis of alternative surgical approaches resulting in the average distribution of HVF to the right/left pulmonary arteries of 37%/63% ± 10.4%. A hepatoazygos shunt was required in all patients and additional creation of an innominate vein in one. CFD analysis was validated by the comparison of pre-operative predicted and postoperative MRI-measured total right/left pulmonary flow (51%/49% ± 5.4% vs 49%/51% ± 8.5%). Conclusions: A 3D modeling workflow with CFD simulation for SV patients with iIVC may avoid HVF maldistribution and development of AVMs after Fontan completion.

Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndrome.

Objective: Heterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome. Methods: This is a single-center retrospective review of patients with heterotaxy syndrome undergoing single-ventricle palliation or primary or staged biventricular repair from 1998 to 2018. Patients were stratified by single ventricle versus biventricular physiology, and the severity of atrioventricular valve regurgitation. Demographics, anatomic characteristics, and early and late outcomes, including the length of stay, mortality, and surgical or catheter reinterventions, were analyzed. Results: Among 250 patients, 150 (60%) underwent biventricular repair. In-hospital mortality was 7.6% (n = 19). Median follow-up was 5.2 (range, 0-16) years. Among survivors to discharge, mortality was 19% (n = 44) and reintervention was 52% (n = 120). Patients with moderate/severe atrioventricular valve regurgitation were older (32 vs 16 months, P = .02), were more likely to experience adverse events during their index surgical admission (72% vs 46%, P < .001), and had longer in-hospital length of stay (20 vs 12 days, P = .009). Among patients with moderate to severe atrioventricular valve regurgitation, single-ventricle palliation is associated with a greater risk of unplanned reintervention compared with patients undergoing biventricular repair (hazard ratio, 2.13; CI, 1.10-4.12; P = .025). Conclusions: There was no significant difference in early or late outcomes in single-ventricle versus biventricular repair strategies in heterotaxy. In the subgroup of patients with moderate/severe atrioventricular valve regurgitation, patients who underwent single-ventricle palliation were 2.5 times more likely to need a late reintervention compared with those undergoing biventricular repair.

Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success.

Erector spinae plane blocks for opioid-sparing multimodal pain management after pediatric cardiac surgery.

OBJECTIVE: Peripheral regional anesthesia is proposed to enhance recovery. We sought to evaluate the efficacy of bilateral continuous erector spinae plane blocks (B-ESpB) for postoperative analgesia and the impact on recovery in children undergoing cardiac surgery. METHODS: Patients aged 2 through 17 years undergoing cardiac surgery in the enhanced recovery after cardiac surgery program were prospectively enrolled to receive B-ESpB at the end of the procedure, with continuous infusions via catheters postoperatively. Participants wore an activity monitor until discharge. B-ESpB patients were retrospectively matched with control patients in the enhanced recovery after cardiac surgery program. Outcomes of the matched clusters were compared using exact conditional logistic regression and generalized linear modeling. RESULTS: Forty patients receiving B-ESpB were matched to 78 controls. There were no major complications from the B-ESpB or infusions, and operating room time was longer by a median of 31 minutes. While blocks were infusing, patients with B-ESpB received fewer opioids in oral morphine equivalents than controls at 24 hours (0.60 ± 0.06 vs 0.78 ± 0.04 mg/kg; P = .02) and 48 hours (1.13 ± 0.08 vs 1.35 ± 0.06 mg/kg; P = .04), respectively. Both groups had low median pain scores per 12-hour period. There was no difference in early mobilization, length of stay, or complications. CONCLUSIONS: B-ESpBs are safe in children undergoing cardiac surgery. When performed as part of a multimodal pain strategy in an enhanced recovery after cardiac surgery program, pediatric patients with B-ESpB experience good pain control and require fewer opioids in the first 48 hours.

Association of tetralogy of Fallot and complete atrioventricular canal: a single-centre 40-year experience.

OBJECTIVES: Outcome data in tetralogy of Fallot (ToF) and complete atrioventricular canal (CAVC) are limited. We report our experience for over 40 years in this patient population. METHODS: Single-centre, retrospective analysis of patients who underwent surgical repair with the diagnosis of ToF-CAVC from 1979 to 2022, divided into 2 different periods and compared. RESULTS: A total of 116 patients were included: 1979-2007 (n = 61) and 2008-2021 (n = 55). Balanced CAVC (80%) and Rastelli type C CAVC (81%) were most common. Patients in the later era were younger (4 vs 14 months, P < 0.001), fewer had trisomy 21 (60% vs 80%, P = 0.019) and fewer had prior palliative prior procedures (31% vs 43%, P < 0.001). In the earlier era, single-patch technique was more common (62% vs 16%, P < 0.001), and in recent era, double-patch technique was more common (84% vs 33%, P < 0.001). In the earlier era, right ventricular outflow tract was most commonly reconstructed with transannular patch (51%), while in more recent era, valve-sparing repairs were more common (69%) (P < 0.001). In-hospital mortality was 4.3%. The median follow-up was 217 and 74 months for the first and second eras. Survival for earlier and later eras at 2-, 5- and 10-year follow-up was (85.1%, 81.5%, 79.6% vs 94.2%, 94.2%, 94.2% respectively, log-rank test P = 0.03). CONCLUSIONS: The surgical approach to ToF-CAVC has evolved over time. More recently, patients tended to receive primary repair at younger ages and had fewer palliative procedures. Improved surgical techniques allowing for earlier and complete repair have shown a decrease in mortality, more valve-sparing procedures without an increase in total reoperations. Presented at the 37th EACTS Annual Meeting, Vienna, Austria.

Mitochondrial transplantation preserves myocardial function and viability in pediatric and neonatal pig hearts donated after circulatory death.

OBJECTIVE: Mitochondrial transplantation has been shown to preserve myocardial function and viability in adult porcine hearts donated after circulatory death (DCD) . Herein, we investigate the efficacy of mitochondrial transplantation for the preservation of myocardial function and viability in neonatal and pediatric porcine DCD heart donation. METHODS: Circulatory death was induced in neonatal and pediatric Yorkshire pigs by cessation of mechanical ventilation. Hearts underwent 20 or 36 minutes of warm ischemia time (WIT), 10 minutes of cold cardioplegic arrest, and then were harvested for ex situ heart perfusion (ESHP). Following 15 minutes of ESHP, hearts received either vehicle (VEH) or vehicle containing isolated autologous mitochondria (MITO). A sham nonischemic group (SHAM) did not undergo WIT, mimicking donation after brain death heart procurement. Hearts underwent 2 hours each of unloaded and loaded ESHP perfusion. RESULTS: Following 4 hours of ESHP perfusion, left ventricle developed pressure, dP/dt max, and fractional shortening were significantly decreased (P < .001) in DCD hearts receiving VEH compared with SHAM hearts. In contrast, DCD hearts receiving MITO exhibited significantly preserved left ventricle developed pressure, dP/dt max, and fractional shortening (P < .001 each vs VEH, not significant vs SHAM). Infarct size was significantly decreased in DCD hearts receiving MITO as compared with VEH (P < .001). Pediatric DCD hearts subjected to extended WIT demonstrated significantly preserved fractional shortening and significantly decreased infarct size with MITO (P < .01 each vs VEH). CONCLUSIONS: Mitochondrial transplantation in neonatal and pediatric pig DCD heart donation significantly enhances the preservation of myocardial function and viability and mitigates against damage secondary to extended WIT.

Transcatheter Palliation With Pulmonary Artery Flow Restrictors in Neonates With Congenital Heart Disease: Feasibility, Outcomes, and Comparison With a Historical Hybrid Stage 1 Cohort.

BACKGROUND: Neonates with complex congenital heart disease and pulmonary overcirculation have been historically treated surgically. However, subcohorts may benefit from less invasive procedures. Data on transcatheter palliation are limited. METHODS: We present our experience with pulmonary flow restrictors (PFRs) for palliation of neonates with congenital heart disease, including procedural feasibility, technical details, and outcomes. We then compared our subcohort of high-risk single ventricle neonates palliated with PFRs with a similar historical cohort who underwent a hybrid Stage 1. Cox regression was used to evaluate the association between palliation strategy and 6-month mortality. RESULTS: From 2021 to 2023, 17 patients (median age, 4 days; interquartile range [IQR], 2-8; median weight, 2.5 kilograms [IQR, 2.1-3.3]) underwent a PFR procedure; 15 (88%) had single ventricle physiology; 15 (88%) were high-risk surgical candidates. All procedures were technically successful. At a median follow-up of 6.2 months (IQR, 4.0-10.8), 13 patients (76%) were successfully bridged to surgery (median time since PFR procedure, 2.6 months [IQR, 1.1-4.4]; median weight, 4.9 kilograms [IQR, 3.4-5.8]). Pulmonary arteries grew adequately for age, and devices were easily removed without complications. The all-cause mortality rate before target surgery was 24% (n=4). Compared with the historical hybrid stage 1 cohort (n=23), after adjustment for main confounding (age, weight, intact/severely restrictive atrial septum or left ventricle to coronary fistulae), the PFR procedure was associated with a significantly lower all-cause 6-month mortality risk (adjusted hazard ratio, 0.26 [95% CI, 0.08-0.82]). CONCLUSIONS: Transcatheter palliation with PFR is feasible, safe, and represents an effective strategy for bridging high-risk neonates with congenital heart disease to surgical palliation, complete repair, or transplant while allowing for clinical stabilization and somatic growth.