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.

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.

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.

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.

The natural history and outcomes for thoracic and abdominal penetrating aortic ulcers.

OBJECTIVE: The objective of this report was to define the natural history of penetrating aortic ulcers (PAUs) in the descending thoracic and abdominal aorta. METHODS: Data from consecutive patients with PAU from January 1, 1998 to December 31, 2012 were retrospectively reviewed. Computed tomography (CT) scans were analyzed for anatomic changes. End points analyzed were changes in size, development of symptoms or signs of rupture, morbidity, and mortality. RESULTS: Ninety-three patients were identified; 57 were followed up with two or more CT studies 3 months apart (group 1), and 20 had immediate repair (group 2). Sixteen had one CT scan and no intervention or follow-up and were excluded from analysis. In group 1, mean age was 75 years (29 men, 28 women), with 28 descending thoracic aorta and 29 abdominal aorta PAUs. Fifty patients were asymptomatic, whereas five had pain and two had emboli. Mean follow-up was 38 months (range, 3-108 months). Ulcer growth rate was as follows: length, 2.0 mm/y; depth, 1.2 mm/y; and aortic diameter, 2.2 mm/y. Thirteen (23%) went on to repair at a mean of 37 months after diagnosis because of size (54%; 7/13), rapid growth (31%; 4/13), and high-risk morphology (15%; 2/13). During surveillance, 11 patients died, 10 of unrelated causes, and 1 of rupture after refusing repair. All repairs in group 1 were endovascular. The 30-day surgical mortality was 0%. One patient had an access site complication requiring bypass after descending thoracic aorta PAU repair. At a mean follow-up of 32 months, all ulcers were excluded on CT; one (8%) had a type II endoleak. Group 2 included 13 men and seven women with a mean age of 70 years, with 12 descending thoracic and eight abdominal aorta PAUs. Repair indications were rupture (n = 3), symptoms (n = 10), or size (n = 7) and included one open and 19 endovascular repairs with 0% 30-day mortality. Major complications (3/20; 15%) included myocardial infarction, access site disruption, and hematoma; four of 20 patients had type II endoleaks. CONCLUSIONS: PAU growth rate and risk of rupture are low. Endovascular repair of symptomatic, ruptured, and large PAUs is safe and effective with excellent long-term results. For asymptomatic PAUs, serial CT surveillance is associated with a low rate of rupture or complications.

Effectiveness of rivaroxaban for thromboprophylaxis of prosthetic heart valves in a porcine heterotopic valve model.

OBJECTIVES: Warfarin is used to reduce the risk of stroke and thromboembolic complications in patients with mechanical heart valves. Yet, despite frequent blood testing, its poor pharmacokinetic and pharmacodynamic profiles often result in variable therapeutic levels. Rivaroxaban is a direct competitive factor Xa inhibitor that is taken orally. It inhibits the active site of factor Xa without the need for the cofactor antithrombin, and thus, its mechanism of action is differentiated from that of the fractionated heparins and indirect factor Xa inhibitors. No in vivo data exist regarding the effectiveness of rivaroxaban in preventing thromboembolic complications of mechanical heart valves. We tested the hypothesis that rivaroxaban is as effective as enoxaparin for thromboprophylaxis of mechanical valves that use a previously described heterotopic aortic valve porcine model. METHODS: A modified bileaflet mechanical valved conduit that bypassed the native, ligated descending thoracic aorta was implanted into 30 swine. Postoperatively, the animals were randomly assigned to groups receiving no anticoagulation (n = 10), enoxaparin at 2 mg/kg subcutaneously twice daily (n = 10) or rivaroxaban at 2 mg/kg orally twice daily (n = 10). The amount of valve thrombus was measured on post-implantation day 30 as the primary end point. Quantitative evaluation of radiolabelled platelet deposition on the valve prostheses was done and embolic and haemorrhagic events were measured as secondary end points. RESULTS: Animals with no anticoagulation had a thrombus mean of 759.9 mg compared with 716.8 mg with enoxaparin treatment and 209.6 mg with rivaroxaban treatment (P = 0.05 for enoxaparin vs rivaroxaban). Similarly, the mean number of platelets deposited on the valve prosthesis was lower in the rivaroxaban group (6.13 × 10(9)) than in the enoxaparin group (3.03 × 10(10)) (P = 0.03). CONCLUSIONS: In this study, rivaroxaban was more effective than enoxaparin for short-term thromboprophylaxis of mechanical valve prosthetics in the heterotopic aortic position. It reduced valve thrombus and platelet deposition on day 30 following implantation without increased adverse events. Future studies would provide additional support for clinical trials evaluating rivaroxaban as an alternative to warfarin for appropriately selected patients with prosthetic aortic valves.

Should there be sex-specific criteria for the diagnosis and treatment of heart failure?

All-cause mortality from cardiovascular disease is declining in the USA. However, there remains a significant difference in risk factors for disease and in mortality between men and women. For example, prevalence and outcomes for heart failure with preserved ejection fraction differ between men and women. The reasons for these differences are multifactorial, but reflect, in part, an incomplete understanding of sex differences in the etiology of cardiovascular diseases and a failure to account for sex differences in pre-clinical studies including those designed to develop new diagnostic and treatment modalities. This review focuses on the underlying physiology of these sex differences and provides evidence that inclusion of female animals in pre-clinical studies of heart failure and in development of imaging modalities to assess cardiac function might provide new information from which one could develop sex-specific diagnostic criteria and approaches to treatment.