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We present a case of a 10-year-old female with a cervical aortic arch and distal transverse arch tortuosity causing coarctation and aneurysmal formation and describe the surgical repair of this rare congenital anomaly., (© 2018 Wiley Periodicals, Inc.)

Background: Bovine jugular vein (BJV) grafts (Medtronic, Inc, Minneapolis, Minn) are used to restore right ventricle-to-pulmonary artery continuity. Recent studies have associated these grafts with the development of infective endocarditis. The purpose of this study was to report the incidence of endocarditis in BJV grafts., Methods: All BJV grafts placed in the right ventricle-to-pulmonary artery position between 2001 and 2017 at our institution were included. Freedom from endocarditis was analyzed using the Kaplan-Meier method and parametric survival regression models., Results: Overall, 228 patients underwent placement of 253 BJV grafts. The median duration of conduit follow-up was 6 years (5 months to 14 years). Twenty-five conduits developed endocarditis, yielding an incidence of 10% at a median of 7.5 years after surgery. Median duration of symptoms before the diagnosis of endocarditis was 21 days (3-180 days). The most common infectious agents were viridans streptococci (n = 13; 52%). Freedom from endocarditis at 5 and 10 years was 97% and 77%, respectively. After controlling for confounders, BJV grafts had a higher incidence of endocarditis compared with homografts (P < .001). Twenty-three (92%) of the conduits that developed endocarditis were managed surgically, with no mortality., Conclusions: The incidence of late endocarditis affecting BJV is high. Increased surveillance and a high index of suspicion for endocarditis are warranted in patients who have undergone implantation of BJV grafts, especially if the graft has been in place for more than 7 years. When infective endocarditis has been diagnosed in these grafts, surgical replacement is recommended, with excellent outcomes., (Copyright © 2018. Published by Elsevier Inc.)

Background: The purpose of this study was to determine the incidence and risk factors for the development and rate of progression of scoliosis and moderate/severe scoliosis in patients undergoing cardiothoracic operations (CTOs)., Methods: Included were patients aged younger than 12 years who underwent CTOs in 1995 to 2006 with a preoperative chest roentgenogram (CRG) and a CRG at least 8 years after CTOs. Scoliosis and moderate/severe scoliosis were defined as a Cobb angle of 10 degrees or more and an angle of 25 degrees or more or the need for surgical intervention, respectively. Risk factors were analyzed using nonparametric and parametric survival analyses. For patients that developed scoliosis, progression rate was analyzed using linear regression models for repeated measures using CRG at 6-month intervals., Results: The study included 871 patients (380 girls [44%]). Median CRG follow-up was 11 years (interquartile range, 9 to 13 years). Overall 10-year incidence of scoliosis and moderate/severe scoliosis was 12% and 3%, respectively. Independent predictors for scoliosis included female sex (hazard ratio [HR], 1.7; 95% confidence interval [CI], 1.2 to 2.5), syndrome (HR, 1.9; 95% CI, 1.3 to 2.8), and isolated developmental delay (HR, 2.4; 95% CI, 1.4 to 4.2). For development of moderate/severe scoliosis, independent risk factors included female sex (HR, 2.8; 95% CI, 1.4 to 5.8), syndrome (HR, 3; 95% CI, 1.5 to 6.1), isolated developmental delay (HR, 3.1; 95% CI, 1 to 9.2]), and prematurity for neonates/infants (HR, 2.3; 95% CI, 1 to 5.2). Rate of angle progression was 0.17 times the current angle per year. Age, syndrome, and developmental delay were risk factors for angle progression., Conclusions: Patients that undergo pediatric CTO, regardless of the operative approach, are at increased risk for development of scoliosis and moderate/severe scoliosis. Long-term follow-up of these patients is warranted, in particular for girls and patients with genetic syndromes or developmental delay., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Circumflex aorta and double aortic arch are two forms of rare vascular rings. We present a case of an infant who was diagnosed with circumflex aorta and double aortic arch, and describe the surgical management of this rare anomaly., (© 2018 Wiley Periodicals, Inc.)

Graft rejection is the most common factor that limits graft survival after transplantation. During infancy, the humoral immune system is partially suppressed and humoral rejection of a cardiac allograft has not been reported in the absence of risk factors such as prior transplantation, blood transfusions, ventricular assist devices, and elevation of panel reactive antibodies. We present a case of an infant with dilated cardiomyopathy who developed multiple episodes of acute humoral rejection after heart transplantation in the absence of risk factors., (© 2018 Wiley Periodicals, Inc.)

Objective: The purpose of this study was to prospectively analyze the outcomes of patients with anomalous aortic origin of a coronary artery undergoing surgical intervention according to a standardized management algorithm., Methods: All patients aged 2 to 18 years undergoing surgical intervention for anomalous aortic origin of a coronary artery between December 2012 and April 2017 were prospectively included. Patients underwent stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and retrospectively electrocardiogram-gated computed tomography angiography preoperatively. Patients were cleared for exercise at 3 months postoperatively if asymptomatic and repeat stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and computed tomography angiography showed normal results., Results: A total of 44 patients, with a median age of 14 years (8-18 years), underwent surgical intervention: 9 (20%) for the anomalous left coronary artery and 35 (80%) for the anomalous right coronary artery. Surgical procedures included unroofing in 35 patients (80%), translocation in 7 patients (16%), ostioplasty in 1 patient (2%), and side-side-anastomosis in 1 patient (2%). One patient who presented with aborted sudden cardiac death from an anomalous left coronary and underwent unroofing presented 1 year later with a recurrent episode and was found to have an unrecognized myocardial bridge and persistent compression of the coronary requiring reintervention. At last follow-up, 40 patients (91%) are asymptomatic and 4 patients have nonspecific chest pain; 42 patients (95%) have returned to full activity, and 2 patients are awaiting clearance., Conclusions: Surgical treatment for anomalous aortic origin of a coronary artery is safe and should aim to associate the coronary ostium with the correct sinus, away from the intercoronary pillar. After surgery, the majority of patients are cleared for exercise and remain asymptomatic. Longer follow-up is needed to assess the true efficacy of surgery in the prevention of sudden cardiac death., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: The goal of this study was to assess the effect of associated gastrointestinal malformations (GI) on the outcomes of patients undergoing congenital heart operations., Methods: Neonates and infants with thoracic (esophageal atresia, tracheoesophageal fistula) and abdominal (duodenal stenosis/atresia, imperforate anus, Hirschsprung disease) GI malformations undergoing congenital heart operations between 1995 and 2015 were included. Two control groups were created, one for each group. Patients were matched by diagnosis, procedure, history of prematurity, presence of genetic syndrome, and a propensity score including weight and year of operation., Results: The cohort included 383 patients: 52 (14%) with thoracic GI malformations and 98 (25%) thoracic GI controls, 80 (21%) with abdominal GI malformations and 153 (40%) abdominal GI controls. Median follow-up was 6 years (range, 16 days to 20 years). Patients with thoracic GI malformations had longer length of stay (p < 0.001), longer intubation times (p = 0.002), and higher perioperative death (p = 0.015) than controls. There was a tendency for worse overall survival than controls, mainly explained by the higher risk of early death (p = 0.06). No difference was found in outcomes between patients with abdominal GI malformations and controls., Conclusions: Patients with thoracic GI malformations have worse perioperative outcomes than controls, but their long-term survival does not seem to be significantly different. Abdominal GI malformations do not have a significant effect on outcomes. The presence of GI malformations should likely not preclude patients from undergoing congenital heart operations, but careful family counseling is necessary, especially for thoracic GI malformations., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We describe a patient with anomalous left coronary artery with a short intramural course and a previously unrecognized myocardial bridge who presented with a recurrent episode of aborted sudden cardiac death. Intravascular ultrasound and fractional flow reserve showed significant compression at the left coronary artery ostium by the intercoronary pillar and at the myocardial bridge. Intravascular ultrasound and fractional flow reserve were normal after coronary translocation and unroofing of the myocardial bridge. All potential anatomic culprits should be addressed when operating on patients with anomalous coronaries., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: The purpose of this systematic review is to provide a comprehensive overview on the clinical course, perinatal outcome, and effectiveness of prenatal management options for pericardial teratoma., Methods: A comprehensive search including Ovid MEDLINE, Ovid EMBASE, and Scopus was conducted from inception to September 2016. All studies that reported the prenatal course of pericardial teratoma in singleton or twin gestations were considered eligible. Standardized forms were used for data abstraction by two independent reviewers., Results: Out of 217 screened abstracts, 59 studies reporting 67 fetuses with pericardial teratoma were included. Twenty-three singleton fetuses and 3 fetuses in twin gestations underwent prenatal treatment, and 20 (76.9%) of them were hydropic at the time of intervention. Of those, 15/20 (75%) had a favorable outcome. In the non-intervention group (n = 41), 26 (63.4%) developed hydrops, and out of those, 8 (30.8%) had a favorable outcome., Conclusion: Prenatal fluid drainage and other prenatal techniques have been utilized in the treatment of intrapericardial teratoma. While most fetuses tolerated pericardiocentesis, the neonatal benefit of this procedure is still uncertain, and outcomes of other interventions had variable success. Prenatal intervention for pericardial teratoma may be an option in specialized units but, given the maternal and fetal risks, needs careful consideration. © 2017 John Wiley & Sons, Ltd., (© 2017 John Wiley & Sons, Ltd.)

Background: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes in the USA. Long-term outcome data for these patients are lacking to date. There is insufficient knowledge on the best approach to these patients and they are managed in a nonuniform manner., Methods: An online survey of 15 questions regarding management of AAOCA was sent out to 198 cardiac healthcare providers. The goal was to define gaps in knowledge to justify a dedicated scientific forum for discussion of AAOCA. Descriptive statistics were performed., Results: A total of 91 providers (46%) completed the survey including pediatric cardiology subspecialists (40%), general pediatric cardiologists (24%), cardiovascular (CV) surgeons (22%), adult cardiologists (10%), nurse practitioners (8%), cardiology fellows (3%) and CV anesthesiologist (1%). Forty-eight percent had been practicing for over 15 years and 28% were in their first 5 years of practice. Fifty-two percent of the providers cared for adults and 93% cared for children/adolescents. Eighty-eight percent were affiliated with an academic institution. All but one provider practiced in the USA, 62% practiced in Texas. Half of participants (50%) were very comfortable managing AAOCA patients and 36% were somewhat comfortable. Providers utilized various imaging tests to confirm the anatomy including computed tomography angiography 88%, cardiac magnetic resonance imaging 70%, cardiac catheterization 60%, echocardiogram 12%, IVUS 2% and myocardial perfusion scan 1%. The majority felt comfortable in counseling the families and felt that depending on the type of lesion these patients should get surgical referral (85%) vs clinical follow up (67%) with exercise restriction (65%)., Conclusion: There is heterogeneity in the way AAOCA patients are currently evaluated and managed. A knowledge gap exists even with participants from academic institutions. Long-term data with a defined approach to management of these patients may help to improve outcomes and prevent unnecessary exercise restriction or surgery., (© 2017 Wiley Periodicals, Inc.)

Background: The Infant Jarvik 2015 is an implantable axial-flow ventricular assist device (VAD) that has undergone the major evolutionary design modifications to improve hemocompatibility. This study was conducted in anticipation of data submission to the US Food and Drug Administration to obtain Investigational Device Exemption approval., Methods: The VAD was implanted via a left thoracotomy in Barbado sheep (n = 10, 26 (19-34] kg). Anticoagulation was maintained with coumadin, with a target international normalized ratio of greater than the individual sheep's baseline values. The VAD was managed at the highest possible speed as clinically tolerable. Complete necropsy was performed at the end of the study., Results: There were 2 early mortalities: tension pneumothorax (n = 1) and shower emboli of the fragmented myocardium (n = 1). The remaining 8 sheep (2 with 30-day and 6 with 60-day protocols) completed the anticipated study duration in excellent condition, with the 6 completing 60-day sheep showing appropriate weight gain during support. There were no signs of clinically significant hemolysis, with the final plasma-free hemoglobin of 2 (1-17) mg/dL. Necropsy showed old renal infarction in 7 sheep. Although thromboembolism can be the potential etiology, given the mild anticoagulation regimen, other sources of emboli were identified in 2 sheep (graft coating material and fragmented myocardium). Flow study demonstrated favorable increase in flow (up to 3.0 L/min) in proportion to change in pump speed., Conclusions: This study has demonstrated that the Infant Jarvik 2015 VAD is capable of maintaining its functionality for an extended period of time with minimal hemolysis., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

The world lost a medical trailblazer and inspirational humanitarian with the passing of Denton A. Cooley on November 18, 2016. The 28th president of The Society of Thoracic Surgeons, Cooley was an icon of the astounding progress of cardiovascular medicine in the 20th century and a visionary pioneer who shaped modern surgery., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Optimal management of patients with congenitally corrected transposition of the great arteries (ccTGA) is unclear. The goal of this study was to compare the outcomes in patients with ccTGA undergoing different management strategies., Study Design: Patients with ccTGA believed suitable for biventricular circulation, treated between 1995 and 2016, were included. The cohort was divided into 4 groups: systemic right ventricle (RV) (patients without surgical intervention or with a classic repair), anatomic repair, Fontan palliation, and patients receiving only a pulmonary artery band (PAB) or a shunt. Transplant-free survival from presentation was calculated for each group., Results: The cohort included 97 patients: 45 (46%) systemic RV, 26 (27%) anatomic repair, 9 (9%) Fontan, and 17 (18%) PAB/shunt. Median age at presentation was 2 months (range 0 days to 69 years) and median follow-up was 10 years (1 month to 28 years). At initial presentation, 10 (11%) patients had any RV dysfunction (8 mild, 2 severe), and 16 (18%) patients had moderate or severe tricuspid regurgitation (TR). During the study, 10 (10%) patients died, and 3 (3%) patients underwent transplantation. At last follow-up, 11 (11%) patients were in New York Heart Association class III/IV, 5 (5%) had moderate or severe systemic ventricle dysfunction, and 16 (16%) had moderate or severe systemic atrioventricular valve regurgitation. Transplant-free survivals at 10 years were 93%, 86%, 100%, and 79% for systemic RV, anatomic repair, Fontan palliation, and PAB/shunt, respectively (p = 0.33). On multivariate analysis, only systemic RV dysfunction was associated with a higher risk for death or transplant (p = 0.001)., Conclusions: Transplant-free survival in ccTGA appears to be similar between patients with a systemic RV, anatomic repair, and Fontan procedure. Systemic RV dysfunction is a risk factor for death and transplant., (Copyright © 2017 American College of Surgeons. Published by Elsevier Inc. All rights reserved.)

The Berlin Heart EXCOR Pediatric Ventricular Assist Device (BH) was approved for use in the United States in December 2011, based on a prospective investigational device exemption (IDE) trial. Strict exclusion criteria for the trial selected a low-risk "ideal" cohort. We sought to determine whether postapproval usage of the BH in a "real world" cohort of recipients would result in similar outcomes. Preimplant diagnostic information was collected for all patients. Efficacy was evaluated by comparison of all children (efficacy group, n = 247) implanted between FDA approval and April 2015 to those in the IDE trial (IDE, n = 48), with regard to achievement of one of four end-states: transplanted, successful weaning, death/unsuccessful weaning, or still-on-device. Safety outcomes were compared between IDE patients and a subset of postapproval patients (safety group, n = 39) for whom adjudicated adverse events were tracked in a regulator-mandated dataset. Diagnostic categories were similar between groups: IDE (congenital 19%, dilated cardiomyopathy/myocarditis/other 81%) versus Efficacy Group (congenital 24%, dilated cardiomyopathy/myocarditis/other 75%). Patients in the IDE cohort were larger (median 14.8 kg, range 3.6-58.1 kg vs. 10.7 kg, 2.9-112.0 kg, p = 0.02). More IDE patients were successfully supported than in the efficacy group cohort (90% vs. 77%, p = 0.05). Proportions with bleeding and stroke were similar between the IDE and safety group cohorts (46% vs. 41%, p = 0.65; 29% vs. 33%, p = 0.68, respectively). With usage of the BH in a less-ideal population, rates of bridge to transplant and weaning have declined slightly, but remain encouragingly high. Bleeding and neurologic event rates have not increased.

The evaluation of the vast majority of children with anomalous aortic origin of a coronary artery (AAOCA) and/or myocardial bridges is performed with non-invasive testing. However, a subset of these patients may benefit from invasive testing for risk stratification. All patients included in the Coronary Anomalies Program (CAP) at Texas Children's Hospital who underwent cardiac catheterization were included. Techniques included selective coronary angiograms (SCA), intravascular ultrasound (IVUS), and fractional flow reserve (FFR) measurements with provocative testing using adenosine and/or dobutamine infusions. Out of the 131 patients followed by the CAP between 12/12-4/16, 8 (6%) patients underwent 9 cath investigations at median age 13.1 (2.6-18.7) years and median weight 49.5 (11.4-142.7) kg. Six patients presented with cardiac signs/symptoms. Four patients had myocardial bridges of the left anterior descending (LAD) coronary artery, 2 patients had isolated AAOCA, and 2 patients had an anomalous left coronary artery (LCA) with an intramyocardial course of the LAD. SCA was performed in all patients. FFR was positive in 4/6 patients: IVUS showed >70% intraluminal narrowing in 3/5 patients. One patient had hemodynamic instability that reversed with catheter removal from the coronary ostium. Based on the catheterization data obtained, findings were reassuring in three patients, surgery was performed in three patients, and two patients are being medically managed/restricted from competitive sports. In our small cohort of patients, we demonstrated that IVUS and FFR can safely be performed in children and may help to risk stratify some patients with AAOCA and myocardial bridges.

Objective: Routine preoperative laboratory testing (RLT) is common practice in pediatric cardiothoracic surgery and is associated with significant cost burden to patients and families. We sought to examine the value of RLT in patients undergoing elective pediatric cardiothoracic surgery., Methods: We conducted a retrospective study of all scheduled elective pediatric cardiothoracic surgery at our institution from 2012 to 2014. Inpatients were excluded. Patient charts were reviewed to obtain preoperative laboratory values and determine relationship to case cancellation. RLT includes complete blood count, prothrombin time, partial thromboplastin time, urinalysis, 7 chemistry metabolic panel, electrocardiogram, and 2-view chest radiograph., Results: RLT was completed for 1106 scheduled elective cases. Six (0.5%) cancellations were related to abnormal preoperative laboratory test results: 5 complete blood counts and 1 urinalysis. Hospital charge for RLT averaged $2064 per patient. Based on this incidence, we estimated that 184 routine preoperative laboratory tests, which generated a total hospital charge of $379,776, were required to capture 1 abnormal test significant enough to cancel surgery. An estimated charge of $2,169,552 was generated on prothrombin time, partial thromboplastin time, 7 chemistry metabolic panel, electrocardiogram, and 2-view chest radiograph, and none of these tests resulted in a cancellation., Conclusions: RLT does not significantly impact decision-making in elective pediatric cardiothoracic surgery. The decision to order a specific screening test should be clinically driven. Selective preoperative laboratory testing may have a positive impact on healthcare costs without affecting outcomes., (Copyright © 2016. Published by Elsevier Inc.)

Background: Predictors for single ventricle palliation (SVP) or successful biventricular repair (BVR) in patients with borderline left-side heart structures are not well defined. The goal was to evaluate the role of echocardiography and intracardiac exploration in determining feasibility of BVR., Methods: All neonates surgically treated from 1995 to 2015 with mitral valve (MV), aortic valve, or left ventricle end-diastolic dimension z score of -2 or less for whom management was controversial were included. Data were analyzed using Fisher's exact test, Kruskal-Wallis test, and Kaplan-Meier analysis., Results: The cohort consisted of 42 patients: 7 SVP (17%) and 35 BVR (83%). Median follow-up was 7 years (range, 6 months to 18 years). Intracardiac exploration was performed in 29 patients (69%). There was poor correlation between echocardiographic and intraoperative MV measurements (intraclass correlation coefficient 0.14). Preoperative echocardiography significantly underestimated MV size in 14 patients (54%). Two BVR patients were converted to SVP, and 4 (including 1 converted patient) had cardiac-related deaths. All patients with MV greater than 8 mm on preoperative echocardiography had successful BVR. An intraoperative MV less than 8 mm and an abnormal subvalvar apparatus was present in 5 of 6 SVP (83%) and 3 of 3 (100%) failed BVR patients who had intracardiac exploration, and in only 1 of 20 successful BVR patients (5%) who had an intracardiac exploration., Conclusions: The decision to proceed to BVR in patients with borderline left-side heart structures should not rely strictly on echocardiographic measurements. Intracardiac exploration of the MV and subvalvar apparatus is useful before committing a patient to SVP. Patients with low MV z scores, especially those with a normal subvalvar apparatus, may undergo BVR with good outcomes., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

In a rat model of right free wall replacement, the transplantation of an engineered multilayered myocardial patch fabricated from a polycaprolactone membrane supporting a chitosan/heart matrix hydrogel induces significant muscular and vascular remodeling and results in a significantly higher right ventricular ejection fraction compared to use of a commercially available pericardium patch., (© 2017 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.)

The neonatal arterial switch operation has become the standard of care for transposition of the great arteries, including transposition with intact ventricular septum, transposition with ventricular septal defect with or without aortic arch hypoplasia, and double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly). While technically demanding, the operation is enormously gratifying and exciting for the congenital heart surgeon. In the current era, outcomes and expectations for the neonatal arterial switch operation are extremely high with many centers, including our own, reporting 30-day or hospital survivorship approaching 100%. Long-term results are also outstanding, although these patients do require lifelong follow-up and have the potential for need of remedial surgical intervention., (Copyright © 2017 Elsevier Inc. All rights reserved.)

The last decade has witnessed significant advancement in the field of ventricular assist device (VAD) support. Although device options for pediatric patients were previously severely limited because of body size constraints, this frustrating situation has gradually been changing, owing to ongoing device miniaturization. Recognition of the superiority of VAD support compared with conventional extracorporeal membrane oxygenation support has spurred enthusiasm for VAD support in children. In this article, we discuss the current status of pediatric VAD support; where do we stand now and where will we be heading? Because this field is rapidly changing, it is anticipated that this article will provide a general overview of what is currently occurring in the field of pediatric VAD support., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Previous studies have demonstrated that surgical patches comprised of small intestinal submucosa-derived extracellular matrix (ECM) have biological remodeling potential. This pilot study investigated histological, mechanical, and bioelectrical properties of an ECM patch implanted in the ovine right-ventricular outflow tract (RVOT)., Materials and Methods: ECM patches (2 × 2 cm 2 ) were implanted in four Western Range sheep (wether males, 37-49 kg, age <1 year) and explanted at 5 months (n = 2) and 8 months (n = 2). In vivo analysis included epicardial echocardiography and contact electrical mapping. Optical mapping was used to map electrical activity of two hearts on a Langendorff preparation. Mechanical testing quantified stiffness. Histological stains characterized structure, neovascularization, and calcification; immunohistochemistry (IHC) assessed cell phenotype., Results: In vivo analysis showed that ECM patch tissue was contractile by M-mode and two-dimensional echocardiographic evaluation. In vivo electrical mapping, and optical mapping confirmed that ECM conducted an organized electrical signal. Mechanical testing of native and ECM patched RVOT tissue showed an elastic modulus of the implanted patch comparable to native tissue stiffness., Conclusions: At 5 and 8 months, the ECM had undergone extracellular matrix remodeling and neovascularization without calcification. The ECM was populated with locally aligned muscle cells positive for sarcomeric alpha-actinin, CD45, and troponin I and T. In sheep, the ECM patch appears to have the potential of remodeling to resemble native, functional ventricular tissue as evidenced by histological, mechanical, and electrical properties. © 2015 Wiley Periodicals, Inc. J Biomed Mater Res Part B: Appl Biomater, 104B: 1713-1720, 2016., (© 2015 Wiley Periodicals, Inc.)

Objectives: The goal of this study was to use decision analysis to evaluate the impact of varying uncertainties on the outcomes of patients with anomalous aortic origin of a coronary artery., Methods: Two separate decision analysis models were created: one for anomalous left coronary artery (ALCA) and one for anomalous right coronary artery (ARCA). Three strategies were compared: observation, exercise restriction, and surgery. Probabilities and health utilities were estimated on the basis of existing literature. Deterministic and probabilistic sensitivity analyses were performed., Results: Surgery was the optimal management strategy for patients <30 years of age with ALCA. As age increased, observation became an equivalent strategy and eventually surpassed surgery as the treatment of choice. The advantage on life expectancy for surgery over observation ranged from 2.6 ± 1.7 years for a 10-year-old patient to -0.03 ± 0.1 for a 65-year old patient. In patients with ARCA, observation was the optimal strategy for most patients with a life expectancy advantage over surgery of 0.1 ± 0.1 years to 0.2 ± 0.4 years, depending on age. Surgery was the preferred strategy only for patients <25 years of age when the perceived risk of sudden cardiac death was high and the perioperative mortality was low. Exercise restriction was a suboptimal strategy for both ALCA and ARCA in all scenarios., Conclusions: The optimal management in anomalous aortic origin of a coronary artery depends on multiple factors, including individual patient characteristics. Decision analysis provides a tool to understand how these characteristics affect the outcomes with each management strategy and thus may aid in the decision making process for a particular patient., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: A descending thoracic aorta that traverses the midline is an uncommon cause of airway compression affecting the distal trachea and proximal main bronchi. Posterior aortopexy has had inconsistent results., Methods: A retrospective review determined that, since 2004, 5 children have undergone descending aortic translocation at Texas Children's Hospital. The average age at the time of surgical treatment was 4.2 years, and all patients presented with recurring respiratory illness requiring hospitalization. All patients had preoperative imaging (4 patients with computed tomography scans and 1 with magnetic resonance imaging) confirming a compromised airway caused by a midline aorta, and 4 of the 5 patients had perioperative bronchoscopy. Three patients had a right-dominant double aortic arch. Descending aortic translocation was performed through a midline sternotomy with cardiopulmonary bypass and deep hypothermia. The proximal descending aorta was transected distal to the subclavian artery, brought up through the transverse sinus caudad to the tracheal carina and pulmonary artery, and anastomosed in an end-to-side fashion to the ascending aorta., Results: Mean cardiopulmonary bypass was 144.8 ± 32.6 minutes, with an aortic cross-clamp time of 59 ± 40.9. Absence of perfusion to the descending thoracic aorta averaged 44.4 ± 13.7 minutes. Concomitant procedures were performed in 4 of the 5 patients. At a median follow-up of 26 months (range, 3 to 101 months), all patients had resolution of symptoms., Conclusions: A midline descending aorta can cause compression of the tracheal carina and proximal bronchi, with debilitating symptoms. Translocation of the descending aorta is a reliable procedure that relieves the compression and results in long-term resolution of symptoms., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: Malnutrition is common among children with complex heart disease (CHD). Feeding gastrostomies are often used to improve the nutritional status of such patients. Our purpose was to evaluate a cohort of children with CHD following open Stamm gastrostomy without fundoplication., Methods: We reviewed all CHD patients who underwent feeding gastrostomy placement from 1/1/2004 to 4/7/2015. Demographic data, cardiac diagnoses, operative details, post-operative complications, and the need for GJ feeding and fundoplication were examined., Results: Open Stamm gastrostomy was performed in 111 patients. Median age at surgery was 37 weeks (3 weeks-13.7 years); average weight was 5.3 ± 4.9 kg. Thirty-four patients (30 %) experienced a total of 37 minor complications, including tube dislodgement after stoma maturation (20), superficial surgical site infection (13), mechanical failure (3), and bleeding (1). Three patients experienced a major complication (need for return to the OR or peri-operative death <30 days). Three patients required a subsequent fundoplication. Fifty-six surviving patients (62 %) continue gastrostomy feeds, of which 7 (13 %) patients require GJ feeds., Conclusion: Children with CHD tolerate an open Stamm gastrostomy well with minimal major complications. These results support very selective use of fundoplication in infants and children with CHD who require a feeding gastrostomy.

Objective: To determine the incidence and risk factors for endocarditis and reintervention in patients undergoing placement of right ventricle-to-pulmonary artery valve conduits., Methods: All right ventricle-to-pulmonary artery valved conduits placed between 1995 and 2014 were included. Freedom from endocarditis, reintervention, and replacement were analyzed using the Kaplan-Meier method and parametric survival regression models., Results: A total of 586 patients underwent placement of a total of 792 valved conduits, including 289 (36%) pulmonary homografts, 121 (15%) aortic homografts, 245 (31%) bovine jugular grafts, and 137 (17%) porcine heterografts. There were 474 (60%) primary placements and 318 (40%) replacements. The median duration of conduit follow-up was 7 years; 23 conduits developed endocarditis at a median of 5 years after surgery. The use of bovine jugular grafts was the sole significant risk factor associated with endocarditis (hazard ratio, 9.05; 95% confidence interval, 2.6-31.8 compared with homografts). The hazard was greater for bovine jugular grafts compared with the other conduit types and increased with time; however, bovine jugular grafts were associated with a lower risk for reintervention (P < .0001) and replacement (P = .0002). Factors associated with greater risk of both reintervention and replacement were younger age and smaller conduit size. In addition, a diagnosis of truncus arteriosus was associated with a greater risk for replacement (P = .03)., Conclusions: Bovine jugular grafts are associated with a significantly greater risk of late endocarditis but with lower reintervention rates compared with other valved conduits. The risk of endocarditis and durability must be balanced during conduit selection. Antibiotic prophylaxis and a high index of suspicion for endocarditis are warranted in patients with bovine jugular grafts., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Efforts to correlate outcomes of children undergoing heart surgery with center volume and characteristics are not novel. In the current era, outcomes are defined as, and in many cases limited to, mortality rates. Over the past two decades, several investigators have explored various aspects of the volume-mortality relationship. The association between center volume and mortality, although not uniform, is highly implicated by the current literature. Notwithstanding, varied population densities in the United States makes regionalization of specialized services, such as pediatric cardiac surgery, undeniably challenging. There may be an unfortunate reality that larger centers have some advantage in achieving, at the very least, timely measures. However, as pediatric cardiac surgery progresses as a specialty, the definition of 'outcomes' must be expanded beyond simplified, dichotomous parameters. While mortality has been our historical primary focus, as it should be, it is reasonable to propose that our focus should be increasingly refined towards patient- and family-centric measures, including morbidity, cost/value ratio, and overall hospital experience., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Heart surgery at Texas Children's Hospital can trace its origins to the beginning of pediatric and congenital heart surgery. Pioneers in the field--Dr. Denton Cooley and Dr. Dan McNamara--started the program in 1954 at a new pediatric hospital in Houston. Over the past 60 years, what is now Texas Children's Heart Center has grown become one of the leading pediatric heart institutions., (Copyright © 2015. Published by Elsevier Inc.)

We sought to determine whether ventricular assist device (VAD) support is an effective therapy in children with cardiac graft dysfunction. We conducted a retrospective review of VAD usage in this scenario at our institution. Although short-term VAD support was highly successful (89% [eight out of nine] were bridged to recovery), only 29% (2 out of 7) with long-term VAD survived to retransplant. Of note, three out of five mortalities with long-term VAD were related to sepsis (two fungal and one Gram-negative bacterial). Infectious risk imposed by ongoing immunosuppressive therapy limits the role of long-term VAD in this population.

Background: Although surgical results for repair of coarctation of the aorta (CoA) have steadily improved, management of this condition remains controversial. The purposes of this study were to analyze the long-term outcomes of patients undergoing CoA repair through left thoracotomy and to define risk factors for reintervention., Methods: All patients who were less than 18 years old and who underwent initial repair of CoA through left thoracotomy from 1995 to 2013 at Texas Children's Hospital (Houston, TX) were included. Patients were classified into 3 groups: 143 (42%) neonates (0 to 30 days old), 122 (36%) infants (31 days to 1 year old), and 78 (23%) older children (1 to 18 years old). Univariate and multivariate analyses were performed., Results: A total of 343 patients (129 [38%] girls) with median age of 53 days (interquartile range [IQR],12 days to 9 months) and weight of 4.1 kg (IQR, 3.1 to 8.0) underwent repair with extended end-to-end anastomosis (291 patients [85%]), end-to-end anastomosis (44 patients [13%]), interposition graft (2 patients [0.6%]), or subclavian flap (6 patients [2%]). Concomitant diagnoses included genetic abnormalities (48 patients [14%]), isolated ventricular septal defects (58 patients [17%]), small left-sided structures (53 patients,16%), or other complex congenital heart disease (18 patients [5%]). Perioperative mortality was 1% (n = 4, all neonates). At a median follow-up of 6 years (7 days to 19 years), only 14 (4%) patients required reintervention (10 catheter-based procedures, 6 surgical repairs). A postoperative peak velocity of 2.5 m/s or greater was an independent risk factor for reintervention (odds ratio [OR], 4.0; 95% confidence interval [CI], 1.4 to 11.6). Within the cohort, 95 (33%) patients were hypertensive or remained on cardiac medications a median of 12 years (6 months to 19 years) after the surgical procedure. Development of perioperative hypertension was associated with higher risk of chronic hypertension or cardiac medication dependency (OR, 1.9; 95% CI, 1.1 to 3.3)., Conclusions: CoA repair through left thoracotomy is associated with low rates of morbidity, mortality, and reintervention. Aortic arch obstruction should be completely relieved at the time of surgical intervention to minimize the risk of long-term recoarctation., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Management of systemic semilunar valve disease in growing, young patients is challenging. When replacement is necessary, use of a pulmonary autograft is sometimes not possible for anatomic, pathologic, or technical reasons or due to parental or patient preference. We employed a stentless, porcine, full-root bioprosthesis in this setting and report our outcomes., Methods: Over 9 years (2005 to 2013), 24 patients of mean age 13.1 years (range, 3 months to 20.3 years) underwent operation for mixed stenosis and insufficiency in 16 of 24 (67%), pure insufficiency in 7 of 24 (29%), and pure stenosis in 1 of 24 (4%). Twenty patients had previous interventions of repair or replacement, valvuloplasty, or multiple operations. Survival, follow-up echocardiographic findings, and outcomes were documented. All patients were maintained on daily aspirin., Results: There were no hospital deaths and no early or late deaths over a mean follow-up for 23 patients of 46.1 months (range, 14 months to 9.2 years). One patient moved abroad and was lost to follow-up. Echocardiographic follow-up (mean 34.0 months) demonstrated that no patient developed more than mild insufficiency or moderate stenosis. In total, 20 of 24 (83%) showed no insufficiency and 11 of 24 patients (46%) showed no stenosis. Near or complete normalization of left ventricular mass and dimension was demonstrated. There were no explants and no thromboembolic or bleeding events., Conclusions: When use of a pulmonary autograft is not an option, the porcine full-root bioprosthesis appears favorable for systemic semilunar valve replacement in the pediatric and young adult population. Of note, when prosthetic degeneration does occur, stenosis predominates rather than insufficiency. Longer term studies are warranted., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Anomalous left coronary artery from pulmonary artery (ALCAPA) is associated with high mortality if left uncorrected. We describe long-term outcomes after surgical repair and ventricular function changes on long-term follow-up., Methods: A retrospective review of patients who had ALCAPA repair from January 1996 to December 2011 was completed. Ventricular function was assessed by shortening fraction and ejection fraction, left ventricular end-diastolic dimension, and severity of mitral regurgitation. Speckle tracking echocardiography served as a marker of early myocardial dysfunction., Results: In total, 34 patients underwent ALCAPA repair at median age of 5 months (range, 3 days to 39 years). Surgical interventions included coronary translocation in 31 patients (91%), Takeuchi repair in 2 (6%), and coronary ligation in 1 patient (3%). Concomitant mitral valve repair was performed in 5 patients (15%). No patient required mechanical circulatory support postoperatively. There was no early mortality. At median follow-up of 6 years (range, 1 month to 14 years), there were 5 reoperations: 2 heart transplants (6%), 1 mitral valve replacement (3%), 1 coronary artery bypass graft (3%), and 1 ventricular septal defect closure (3%); there was 1 death 2 years after surgery. Ejection fraction improved from 21% ± 6% to 60% ± 7% (p = 0.008), mean shortening fraction from 25% ± 14% to 38% ± 5% (p = 0.01), mean left ventricular end-diastolic dimension Z-score from 6.0 ± 3.8 to 0.9 ± 0.7 (p < 0.001), and number of patients with moderate-severe mitral regurgitation from 44% (15 of 34) to 5% (1 of 21). All patients had normal ejection fraction and shortening fraction at last follow-up. Speckle tracking echocardiography showed decreased global longitudinal (p = 0.01) and circumferential strain (p = 0.03) for 11 of 14 patients (79%)., Conclusions: Excellent outcomes are achieved with repair of ALCAPA without mechanical circulatory support and with low reintervention rates. Normal ejection fraction and shortening fraction do not accurately convey myocardial dysfunction in these patients., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: We sought to evaluate the impact of the evolution of a pediatric mechanical circulatory support (MCS) program on outcomes of children listed for heart transplantation at our institution., Methods: All patients listed for isolated heart transplantation from 1995 to 2013 were included. The use of MCS while on the wait-list was recorded. Wait-list and posttransplant outcomes were compared before and after 2005, which was when we became capable of providing long-term MCS without size limitation., Results: In total, 259 patients were listed for transplant and 201 (78%) reached transplant. The use of MCS was significantly increased between the eras (13% and 37%, p = 0.0001). Wait-list mortality was significantly decreased (25% and 11%, p = 0.0006). Among transplant recipients, the proportion of patients who underwent MCS was significantly increased (13% and 37%, p = 0.0002). Of these MCS patients, the use of long-term devices was significantly increased (50% and 98%, p = 0.0004). Median duration of MCS was significantly increased (12 and 78 days, p = 0.004). Kaplan-Meier estimates showed a trend (p = 0.08) toward improved survival after bridge-to-transplant both at 1 year (70% in the early era and 88% in the late era) and at 5 years (60% and 78%, respectively)., Conclusions: Outcomes of pediatric heart transplantation have significantly improved over the last 2 decades. We believe such improvement is, at least in part, attributable to maturation of MCS strategy, characterized by avoiding the use of temporary devices such as extracorporeal membrane oxygenation as a bridge-to-transplant and a more aggressive use of long-term devices., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Ductal origin of a pulmonary artery (DOPA) is commonly misdiagnosed as agenesis of a pulmonary artery (PA), which may result in inadequate treatment. The objective is to describe the results of resuscitation of unilateral DOPA., Methods: This study is a retrospective review of all patients with unilateral DOPA who underwent PA resuscitation at Texas Children's Hospital from 1993 to 2012. Patients with other cardiac or contralateral lung anomalies were excluded., Results: Ten patients, median age 2 years (range, 3 days to 9 years), with unilateral DOPA were included. Symptoms were present in 6 patients. Cardiac catheterization was performed in all and showed a patent duct or a ductal stump in most patients and a small PA on wedge angiography of the pulmonary veins. Two patients underwent single-stage centralization. The other 8 underwent ductal stenting (n=2) or a systemic-to-PA shunt (n=6) as the first stage before centralization. The 2 patients with ductal stenting developed pulmonary edema. The 2 patients with a cryopreserved vein shunt developed early thrombosis requiring reintervention. Nine patients have undergone centralization. Six patients have required further interventional procedures. There have been no deaths. Symptoms and lung hypoplasia have improved in all patients. Median relative lung perfusion at follow-up was 26% (range, 12%-46%) with significant improvement in the size of the affected PA., Conclusions: PA resuscitation is effective at restoring flow to the affected lung resulting in improved diameter of the PA, lung growth, and resolution of symptoms. PA resuscitation should be considered in all children with DOPA, including those beyond infancy., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)