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Abstract Objectives Identify pediatric heart transplant (HT) recipients with heterotaxy and other complex arrangements of cardiac situs (heterotaxy/situs anomaly) and compare mortality, morbidities, length of stay (LOS), and costs to recipients with congenital heart disease without heterotaxy/situs anomaly. Methods Using linked registry data (2001-2016), we identified 186 HT recipients with heterotaxy/situs anomaly and 1254 with congenital heart disease without heterotaxy/situs anomaly. We compared post-HT outcomes in univariable and multivariable time-to-event analyses. LOS and cost from HT to discharge were compared using Wilcoxon rank-sum tests. Sensitivity analyses were performed using stricter heterotaxy/situs anomaly group inclusion criteria and through propensity matching. Results HT recipients with heterotaxy/situs anomaly were older (median age, 5.1 vs 1.6 years; P <.001) and more often black, Asian, Hispanic, or "other" nonwhite (54% vs 32%; P <.001). Heterotaxy/situs anomaly was independently associated with increased mortality (hazard ratio, 1.58; 95% confidence interval, 1.19-2.09; P =.002), even among 6-month survivors (hazard ratio, 1.86; 95% confidence interval, 1.09-3.16; P =.021). Heterotaxy/situs anomaly recipients more commonly required dialysis (odds ratio, 2.58; 95% confidence interval, 1.51-4.42; P =.001) and cardiac reoperation (odds ratio, 1.91; 95% confidence interval, 1.17-3.11; P =.010) before discharge. They had longer ischemic times (19.2 additional minutes [range, 10.9-27.5 minutes]; P <.001), post-HT intensive care unit LOS (16 vs 13 days; P =.012), and hospital LOS (26 vs 23 days; P =.005). Post-HT hospitalization costs were also greater ($447,604 vs $379,357; P =.001). Conclusions Heterotaxy and other complex arrangements of cardiac situs are associated with increased mortality, postoperative complications, LOS, and costs after HT. Although increased surgical complexity can account for many of these differences, inferior late survival is not well explained and deserves further study. [ABSTRACT FROM AUTHOR]

Objective Our institution uses a valved polytetrafluoroethylene conduit as an alternative to homografts. The objective of this study was to investigate the performance of bicuspid valved polytetrafluoroethylene conduits used for right ventricular outflow tract reconstruction in children aged less than 2 years and to evaluate risk factors for earlier conduit explant. Methods We performed an Institutional Review Board–approved retrospective chart review of all patients aged less than 2 years who underwent surgical right ventricular outflow tract reconstruction with a bicuspid valved polytetrafluoroethylene conduit or homograft conduit from July 2004 to December 2014. The end points of the study were defined as conduit explant, conduit explant or reintervention, conduit stenosis, and conduit insufficiency. Results Fifty-four patients underwent 65 right ventricular outflow tract reconstructions with a bicuspid valved polytetrafluoroethylene conduit (n = 39) or a homograft conduit (n = 26, 23 pulmonary, 3 aortic). The majority of diagnoses were truncus arteriosus (n = 28) and tetralogy of Fallot with pulmonary atresia (n = 19). Median age of patients at surgery was 134 (8-323) days and 128 (7-384) days in the PTFE and homograft groups, respectively. There was no difference in demographic data between the 2 groups. Time-to-event analysis demonstrated no difference in time to explant ( P = .474) or time to explant or reintervention ( P = .206) between the 2 conduit types. Younger age at surgery was the only independent risk factor for conduit explant (subdistribution hazard ratio 1.104 per 30 days younger, P < .001). There was no significant influence of conduit type on the development of moderate conduit stenosis ( P = .931) or severe conduit insufficiency ( P = .880). Larger conduit z score was protective for the development of moderate conduit stenosis (subdistribution hazard ratio, 0.46; P = .001). Conclusions Bicuspid valved polytetrafluoroethylene conduits are a satisfactory choice for right ventricular outflow tract reconstruction in patients aged less than 2 years. Their availability, low cost, and lack of potential sensitization make them an appealing alternative to homograft conduits. [ABSTRACT FROM AUTHOR]

Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal leaflet and abnormal rotation of the valve towards the right ventricular outflow tract. In severe forms, it results in significant tricuspid regurgitation and requires surgical repair. There is an increased interest in understanding the anatomy of the tricuspid valve in this lesion as the surgical repair has evolved with the invention and wide adoption of the cone operation. Multimodality imaging plays an important role in diagnosis, follow-up, surgical planning and post-operative care. This review provides anatomical tips for the cardiac imagers caring for patients with Ebstein anomaly and will help provide image-based personalized medicine. [ABSTRACT FROM AUTHOR]

Objective: We report our initial 3 years 4 months’ single institution experience in 31 consecutive patients with pectus excavatum treated with minimally invasive endoscopic pectus excavatum repair utilizing a modification of the ‘Nuss’ technique. Methods: Under general anesthesia, a curved steel bar is individually shaped for each patient to match the ideal chest wall shape and is placed through an endoscopically created retrosternal tunnel between two bilateral midaxillary line 2-cm incisions. The tunnels initially go along the outside of the rib cage, under the pectoral muscles. At the level of the sternum, these tunnels go retrosternal and communicate with each other. The steel bar is passed with the convexity facing posteriorly, within a protective flat silastic drain. Under endoscopic guidance, the curved steel bar is passed through one tunnel, under the sternum, and out the other tunnel. Once positioned, the bar is turned over, thereby correcting the deformity. An epidural catheter provides perioperative pain relief. Results: Minimally invasive endoscopic pectus excavatum repair has been performed on 31 patients (age: range 4.4–31.0 years, median 15.0 years, mean 14.5 years). Median hospital length of stay is 4 days (range 3–10 days, mean 4.6 days). Pneumothorax occurred in five patients requiring tube thoracostomy in three. One patient developed delayed bilateral pleural effusions requiring drainage. Two patients developed evidence of sterile seroma formation at the skin incision several months after minimally invasive repair of pectus excavatum. These seromas resolved with non-interventional conservative medical treatment. No other complications occurred. Conclusion: The minimally invasive endoscopic pectus repair is safe and effective and currently our procedure of choice for primary pectus excavatum in all ages. Endoscopic visualization facilitates the safe creation of the retrosternal tunnel. Short-term results have been excellent. Further follow-up will be necessary to determine long-term results. [Copyright &y& Elsevier]

Patients with Tetralogy of Fallot with pulmonary stenosis (ToF/PS), the most common form of cyanotic congenital heart disease (CHD), develop adverse right ventricular (RV) remodeling, leading to late heart failure and arrhythmia. We recently demonstrated that overactive β-adrenergic receptor signaling inhibits cardiomyocyte division in ToF/PS infants, providing a conceptual basis for the hypothesis that treatment with the β-adrenergic receptor blocker, propranolol, early in life would increase cardiomyocyte division. No data are available in ToF/PS infants on the efficacy of propranolol as a possible novel therapeutic option to increase cardiomyocyte division and potentially reduce adverse RV remodeling. Using a randomized, double-blind, placebo-controlled trial, we will evaluate the effect of propranolol administration on reactivating cardiomyocyte proliferation to prevent adverse RV remodeling in 40 infants with ToF/PS. Propranolol administration (1 mg/kg po QID) will begin at 1 month of age and last until surgical repair. The primary endpoint is cardiomyocyte division, quantified after 15N-thymidine administration with Multi-isotope Imaging Mass Spectrometry (MIMS) analysis of resected myocardial specimens. The secondary endpoints are changes in RV myocardial and cardiomyocyte hypertrophy. This trial will be the first study in humans to assess whether cardiomyocyte proliferation can be pharmacologically increased. If successful, the results could introduce a paradigm shift in the management of patients with ToF/PS from a purely surgical approach, to synergistic medical and surgical management. It will provide the basis for future multi-center randomized controlled trials of propranolol administration in infants with ToF/PS and other types of CHD with RV hypertension. Clinical trial registration: The trial protocol was registered at clinicaltrials.gov (NCT04713657). • Design of clinical trial to induce heart muscle cell (cardiomyocyte) proliferation in infants with tetralogy of Fallot. • New approach using 15N-thymidine administration to label generated cardiomyocytes. • Multi-isotope Imaging Mass Spectrometry (MIMS) as readout on resected pieces of myocardium. • Positive result could introduce a new concept of increasing cardiomyocyte division with β-blocker administration [ABSTRACT FROM AUTHOR]