Diaphragmatic Defects in Infants: Acute Management and Repair.

Congenital diaphragmatic hernia (CDH) is a complex and highly variable disease process that should be treated at institutions with multidisciplinary teams designed for their care. Treatment in the neonatal period focuses on pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction. Extracorporeal membrane oxygenation (ECMO) can be considered in patients refractory to medical management. Repair of CDH early during the ECMO course seems to improve mortality compared with other times for surgical intervention. The choice of surgical approach to CDH repair should consider the patient's physiologic status and the surgeon's familiarity with the operative approaches available, recognizing the pros/cons of each technique.

Cognitive Biases in High-Stakes Decision-Making: Implications for Joint Pediatric Cardiology and Cardiothoracic Surgery Conference.

Extensive research has consistently demonstrated that humans frequently diverge from rational decision-making processes due to the pervasive influence of cognitive biases. This paper conducts an examination of the impact of cognitive biases on high-stakes decision-making within the context of the joint pediatric cardiology and cardiothoracic surgery conference, offering practical recommendations for mitigating their effects. Recognized biases such as confirmation bias, availability bias, outcome bias, overconfidence bias, sunk cost fallacy, loss aversion, planning fallacy, authority bias, and illusion of agreement are analyzed concerning their specific implications within this conference setting. To counteract these biases and enhance the quality of decision-making, practical strategies are proposed, including the implementation of a no-interruption policy until all data is reviewed, leaders refraining from immediate input, requiring participants to formulate independent judgments prior to sharing recommendations, explicit probability estimations grounded in base rates, seeking external opinions, and promoting an environment that encourages dissenting perspectives.

Left Ventricular Dysfunction Following Repair of Ventricular Septal Defects in Infants.

Left ventricular systolic dysfunction (LVSD) is frequently observed following repair of ventricular septal defects (VSD), although little is known about its incidence, time course, or risk factors. Among infants undergoing VSD repair, for postoperative LVSD, we sought to determine (1) incidence, (2) predictors, and (3) time to resolution. We queried our institution's surgical database for infants who underwent repair of isolated VSDs from November 2001 through January 2019. The primary outcome was postoperative LVSD, which was defined as a shortening fraction (SF) of <26% by M-mode. Postoperative echocardiograms were reviewed, and measurements were made using standard methods. Receiver operating characteristic analysis was generated to determine the preoperative left ventricular internal dimension (LVIDd) z-score most predictive of LVSD. Multivariable analysis was conducted to determine associations with LVSD; covariates in the model were weight percentile, genetic syndrome, preoperative diuretic, VSD type, and preoperative LVIDd z-score. Of the 164 patients who met inclusion criteria, 62 (38%) had postoperative LVSD. Fifty-eight (94%) of patients had resolution of LVSD within 9 months of surgery. Preoperative LVIDd z-score of >3.1 was associated with both an increased incidence of postoperative LVSD and prolonged time to resolution. Multivariable logistic regression analysis showed only preoperative LVIDd z-score was independently associated with postoperative LVSD. LVSD following VSD closure is common, but nearly all cases resolve by 9 months postoperatively. Elevated LVIDd prior to surgery is associated with postoperative LVSD. These data suggest VSD closure should be considered prior to the development of significant left ventricular dilation.

Aberrant right subclavian artery: a novel approach and an overview of operative techniques.

An aberrant right subclavian artery (ARSA) is a rare variation of normal anatomy occurring in 0.5% to 1.8% of the population. No current guidelines are available regarding ARSA management, and surgical intervention should be evaluated carefully. Moreover, symptomatic patients with a dominant left arch and aberrant ARSA require a surgical approach from the right side of the chest for ligation and division of the aberrant artery at its origin on the aorta. The ARSA can then be reimplanted onto the right common carotid artery via a supraclavicular incision. The extensive mobilization in the chest allows for easy reimplantation in the supraclavicular region and eliminates reliance on the collateral circulation. Postoperative monitoring is reliable and easy with radial pulse examinations.

Optimal Fat-Modified Diet Duration for the Treatment of Postoperative Chylothorax in Children.

BACKGROUND: Dietary modification is the mainstay of treatment for postoperative chylothorax in children. However, optimal fat-modified diet (FMD) duration to prevent recurrence is unknown. Our aim was to determine the association between FMD duration and chylothorax recurrence. METHODS: Retrospective cohort study conducted across 6 pediatric cardiac intensive care units within the United States. Patients aged <18 years who developed chylothorax within 30 days after cardiac surgery between January 2020 and April 2022 were included. Patients with a Fontan palliation, who died, or were lost to follow-up or within 30 days of resuming a regular diet were excluded. FMD duration was defined as the first day of a FMD when chest tube output was <10 mL/kg/d without increasing until the resumption of a regular diet. Patients were classified into 3 groups (<3 weeks, 3-5 weeks, >5 weeks) based on FMD duration. RESULTS: A total of 105 patients were included: <3 weeks (n = 61) 3-5 weeks (n = 18), and >5 weeks (n = 26). Demographic, surgical, and hospitalization characteristics were not different across groups. In the >5 weeks group, chest tube duration was longer compared with the <3 weeks and 3-5 weeks groups (median, 17.5 days [interquartile range, 9-31] vs 10 and 10.5 days; P = .04). There was no recurrence of chylothorax within 30 days once chylothorax was resolving regardless of FMD duration. CONCLUSIONS: FMD duration was not associated with recurrence of chylothorax, suggesting that FMD duration can safely be shortened to at least <3 weeks from time of resolving chylothorax.

The MITRIS RESILIA mitral valve is a safe and effective option for mitral valve replacement in young patients requiring mitral valve replacement.

We report the first successful implantation in the United States of a novel mitral valve (MITRIS RESILIA by Edwards Lifesciences) in a patient with history of mitral valve replacement at a young age. This new bioprosthetic valve offers a unique profile and innovative option for mitral valve replacement in patients who are at risk of left ventricular outflow tract obstruction.

Development of consensus recommendations for the management of post-operative chylothorax in paediatric CHD.

OBJECTIVE: A standardised multi-site approach to manage paediatric post-operative chylothorax does not exist and leads to unnecessary practice variation. The Chylothorax Work Group utilised the Pediatric Critical Care Consortium infrastructure to address this gap. METHODS: Over 60 multi-disciplinary providers representing 22 centres convened virtually as a quality initiative to develop an algorithm to manage paediatric post-operative chylothorax. Agreement was objectively quantified for each recommendation in the algorithm by utilising an anonymous survey. "Consensus" was defined as ≥ 80% of responses as "agree" or "strongly agree" to a recommendation. In order to determine if the algorithm recommendations would be correctly interpreted in the clinical environment, we developed ex vivo simulations and surveyed patients who developed the algorithm and patients who did not. RESULTS: The algorithm is intended for all children (<18 years of age) within 30 days of cardiac surgery. It contains rationale for 11 central chylothorax management recommendations; diagnostic criteria and evaluation, trial of fat-modified diet, stratification by volume of daily output, timing of first-line medical therapy for "low" and "high" volume patients, and timing and duration of fat-modified diet. All recommendations achieved "consensus" (agreement >80%) by the workgroup (range 81-100%). Ex vivo simulations demonstrated good understanding by developers (range 94-100%) and non-developers (73%-100%). CONCLUSIONS: The quality improvement effort represents the first multi-site algorithm for the management of paediatric post-operative chylothorax. The algorithm includes transparent and objective measures of agreement and understanding. Agreement to the algorithm recommendations was >80%, and overall understanding was 94%.

Comparison of Postoperative, In-Hospital Outcomes After Complete Repair of Tetralogy of Fallot Between 22q11.2 Deletion Syndrome and Trisomy 21.

22q11.2 deletion syndrome (22q11) and trisomy 21 (T21) are frequently associated with tetralogy of Fallot (TOF). We hypothesized that there are differences in postoperative length of stay (LOS) and occurrence of postoperative interventions after complete repair of TOF when comparing children with 22q11 to those with T21. Using the Pediatric Health Information System, we performed a retrospective cohort study of patients who underwent complete repair of TOF from 2004 to 2019. Three groups were identified: 22q11, T21, and controls (those without a coded genetic syndrome). Outcomes were postoperative LOS and composite occurrence (yes/no) of at least one postoperative intervention. Bivariate and multivariate comparisons were made among groups; odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using the control group as the comparator. There were 6924 subjects (n = 493 22q11, n = 455 T21, n = 5976 controls). In bivariate analysis, 22q11 was associated with a longer LOS compared to T21 (OR 2.37 [2.16, 2.60] vs. 1.25 [1.12, 1.39], p < 0.001), and 22q11 more often underwent postoperative intervention (OR 3.42 [CI 2.56, 4.57] vs. 1.38 [CI 0.91, 2.11]; p < 0.001). In multivariate analysis, 22q11 was also associated with longer LOS (adjusted OR 1.35 [1.26, 1.44] vs. 1.12 [1.04, 1.20]; p < 0.001), but there was no difference in the adjusted odds of postoperative intervention. Children with 22q11 are more likely to experience adverse outcomes after repair of TOF compared to those with T21; the differences are most pronounced for LOS.

Sirtuin 6 Protects Against Oxidative Stress and Vascular Dysfunction in Mice.

Objective: Sirtuin deacetylases are major regulators of organismal aging, and while depletion of sirtuin 6 (SIRT6) in mice results in a profound progeroid phenotype, the role of SIRT6 in the regulation of vasomotor function is unknown. Thus, our objective was to test the hypothesis that reductions in SIRT6 elicit endothelial dysfunction in young, genetically altered mice. Results and Approach: We used young (3 month old), littermate-matched, SIRT6 wild-type (WT), and SIRT6 heterozygous (HET) mice. SIRT6 expression (qRT-PCR) was reduced by 50% in HET mice. Carotid vessel responses to acetylcholine, sodium nitroprusside, U46619, and serotonin were examined in isolated organ chamber baths. Relaxation in response to acetylcholine (ACH) was impaired in HET mice compared to littermate-matched WT controls (67 ± 3% versus 76 ± 3%, respectively; p < 0.05), while responses to sodium nitroprusside were unchanged. Short-term incubation of carotid rings with the NAD(P)H oxidase inhibitor, apocynin, significantly improved in vessels from HET mice but not their WT littermates. Peak tension generated in response to either U46619 or serotonin was significantly blunted in HET mice compared to their WT littermates. Conclusion: These data suggest that SIRT6 is a key regulator of vasomotor function in conduit vessels. More specifically, we propose that SIRT6 serves as a tonic suppressor of NAD(P)H oxidase expression and activation, as inhibition of NAD(P)H oxidase improved endothelial function in SIRT6 haploinsufficient mice. Collectively, SIRT6 activation and/or histone acetyltransferase inhibition may be useful therapeutic approaches to reduce endothelial dysfunction and combat age-associated cardiovascular disease.

Coronary Sinus Ostial Obstruction in Single-Ventricle Congenital Heart Disease: Two Patients With Different Outcomes.

Coronary sinus ostial obstruction is an exceedingly rare anomaly that is particularly important to diagnose in patients with single-ventricle heart disease before surgical palliation. We present 2 cases, an infant and an adult, diagnosed with coronary sinus ostial obstruction, with different clinical outcomes due to timing of diagnosis. (Level of Difficulty: Intermediate.).

Circumaortic double left innominate vein: a rare echocardiographic diagnosis confirmed during congenital heart surgery.

We report a case of a newborn infant with coarctation of the aorta and hypoplastic transverse aortic arch who was found to have a circumaortic double left innominate vein on echocardiography. This exceedingly rare finding was important for surgical planning and was confirmed during congenital heart surgery.

Management of Coronary Artery Aneurysms at the Time of Surgical Revascularization.

BACKGROUND: Coronary artery aneurysms (CAAs) represent a rare pathology occurring in 1.5%-5% of routine coronary angiograms. Limited data exist on the management of CAA at the time of cardiac surgery. MATERIALS AND METHODS: A single-institution retrospective review was performed on 53 patients who underwent cardiac surgery in the setting of atherosclerotic CAA between 1993 and 2015. Patients were stratified based on treatment strategy: exclusion and distal bypass (n = 26) versus revascularization alone (n = 27). Comparisons were made with respect to mortality, need for further/concomitant interventions, and long-term cardiac function including myocardial infarctions and congestive heart failure. RESULTS: A total of 53 patients underwent cardiac surgery in the setting of CAA disease. Management strategies included ligation and bypass in 26 patients and distal bypass only in 27 patients (with four of the patients in this group undergoing coronary stenting across the aneurysm). There were no significant differences in patient demographics between the two groups. No significant difference was found in either 30-d (P = 0.74) or long-term mortality when exclusion of the CAA was performed compared with revascularization alone (P = 0.20). More exclusion procedures were performed earlier in the experience (median surgical date 2000), whereas revascularization alone predominated later in the experience (median surgical date 2007; P ≤ 0.001). CONCLUSIONS: The practice of CAA exclusion, while still performed in selected cases, has largely been supplanted in patients undergoing revascularization. Exclusion does not appear to offer any advantage over isolated revascularization, supporting the current trends in managing this rare condition.

Repeat Coronary Bypass Surgery or Percutaneous Coronary Intervention After Previous Surgical Revascularization.

OBJECTIVE: To assess long-term survival with repeat coronary artery bypass grafting (RCABG) or percutaneous coronary intervention (PCI) in patients with previous CABG. METHODS: From January 1, 2000, through December 31, 2013, 1612 Mayo Clinic patients underwent RCABG (n=215) or PCI (n=1397) after previous CABG. The RCABG cohort was grouped by use of saphenous vein grafts only (n=75), or with additional arterial grafts (n=140); the PCI cohort by, bare metal stents (BMS; n=628), or drug-eluting stents (DES; n=769), and by the treated target into native coronary artery (n=943), bypass grafts only (n=338), or both (n=116). Multivariable regression and propensity score analysis (n=280 matched patients) were used. RESULTS: In multivariable analysis, the 30-day mortality was increased in RCABG versus PCI patients (hazard ratio [HR], 5.32; 95%CI, 2.34-12.08; P<.001), but overall survival after 30 days improved with RCABG (HR, 0.72; 95% CI, 0.55-0.94; P=.01). Internal mammary arteries were used in 61% (129 of 215) of previous CABG patients and improved survival (HR, 0.82; 95% CI, 0.69-0.98; P=.03). Patients treated with drug-eluting stent had better 10-year survival (HR, 0.74; 95% CI, 0.59-0.91; P=.001) than those with bare metal stent alone. In matched patients, RCABG had improved late survival over PCI: 48% vs 33% (HR, 0.57; 95% CI, 0.35-0.91; P=.02). Compared with RCABG, patients with PCI involving bypass grafts (n=60) had increased late mortality (HR, 1.62; 95% CI, 1.10-2.37; P=.01), whereas those having PCI of native coronary arteries (n=80) did not (HR, 1.09; 95% CI, 0.75-1.59; P=.65). CONCLUSION: RCABG is associated with improved long-term survival after previous CABG, especially compared with PCI involving bypass grafts.

Management dilemma of cholecysto-colonic fistula: Case report.

INTRODUCTION: Cholecystocolonic fistula is a rare condition and is found in roughly 1 in every 10,000. It represents 6.3% to 26.5% of all cholecystenteric fistulas (Chowbey et al., 2006; Angrisani et al., 2001; Yamashita et al., 1997). Cholecystocolonic fistula is the second most common intestinal fistula after cholecystoduodenal fistula (Costi et al., 2009). Rarity of this condition, atypical presentation, diagnostic and management challenges, makes it a unique surgical entity. CASE PRESENTATION: A 77-year old male presented with progressive abdominal distension and diarrhea. After initial evaluation, a cholecystocolonic fistula was suspected. Further diagnostic studies including Hepatobiliary Imino-Diacetic Acid (HIDA) scan and Endoscopic Retrograde Cholangiography (ERC) revealed complete occlusion of the cystic duct that could not be relieved. Shortly after, the patient developed septic shock likely of biliary origin and required an urgent open partial cholecystectomy and segmental resection of the involved colonic segment. DISCUSSION: In this particular case, the acute presentation together with the inflammatory features around the gallbladder pointed toward an acute inflammatory process and therefore we have tried to delay any operative intervention to allow the inflammation to subside and avoid operating in an inflamed field. Furthermore, our aim was to relieve any sort of biliary obstruction to allow the fistula -if present- to heal by minimizing bile flow through the fistula. Relieving biliary obstruction was not successful in our patient. CONCLUSION: Based on our experience with this particular case, we could safely conclude that an operation for cholecystocolonic fistula presence in the setting of biliary obstruction that failed decompressive attempts should be performed in an urgent fashion to avoid biliary sepsis development.

Endovascular Repair of a Thoracoabdominal Aortic Aneurysm With a Patient-Specific Fenestrated-Branched Stent-Graft.

PURPOSE: To describe the technical aspects of a thoracoabdominal aortic aneurysm (TAAA) repair using a patient-specific fenestrated-branched stent-graft. TECHNIQUE: The technique is demonstrated in a 69-year-old man with a 6.2-cm asymptomatic type III TAAA. A patient-specific fenestrated-branched stent-graft was designed with 2 down-going directional branches for the celiac and superior mesenteric arteries and 2 reinforced fenestrations for the renal arteries. The procedure was performed under general anesthesia and included sequential stenting of the celiac, superior mesenteric, and bilateral renal arteries. The patient was discharged from the hospital on postoperative day 5 with no complications. Follow-up computed tomography angiography demonstrated exclusion of the aneurysm and patent target vessels at 12-month follow-up. CONCLUSION: This article and illustrated video highlight the steps for procedure planning and implantation of fenestrated and branched endografts. As these techniques continue to evolve, outcomes are expected to be equivalent or improved as compared to those of long-established open repair.