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We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair. Median age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P =.01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P =.0006; right, P =.0002); z -scores significantly increased for right (P =.004) but not left (P =.11). Successful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation. [Display omitted] [ABSTRACT FROM AUTHOR]

We studied pulmonary artery size, reinterventions, and panel reactive antibodies in patients with single-ventricle physiology who underwent a pulmonary arterioplasty with decellularized (DAPAP) and non-decellularized allogeneic pulmonary artery patches (non-DAPAP). Retrospective review identified 59 patients with single-ventricle physiology who underwent pulmonary arterioplasty from 2008 to 2017: 28 patients underwent arterioplasty with DAPAP and 31 patients with non-DAPAP. Demographic and operative variables were similar between groups. Among patients who underwent a Norwood procedure, a right ventricle to pulmonary artery shunt was more commonly used in the DAPAP group (12/20, 60%) and a modified Blalock-Taussig shunt was more commonly used in the non-DAPAP group (17/22, 77%). On multivariable analysis, the use of DAPAP was associated with higher pre-Fontan angiography Z-scores in right (estimate = 0.17, standard error = 0.04, P = 0.0005) and left pulmonary arteries (estimate = 0.12, standard error = 0.05, P = 0.01). No areas of calcification, discrete coarctation, or pulmonary dilation were noted in any of the pulmonary arteries. On multivariable analysis, the use of DAPAP was associated with higher freedom from pulmonary artery reinterventions (Hazard ratio = 0.36, 95% confidence interval = 0.13-0.9, P = 0.04). The median value for Class I panel reactive antibodies was 0% (IQR 0, 4) in the DAPAP and 23% (IQR 14, 36) in the non-DAPAP group. The median value for Class II panel reactive antibodies was 15% (IQR 0, 17) in the DAPAP and 21% (IQR 10, 22) in the non-DAPAP group. Pulmonary arterioplasty with DAPAP was associated with higher pre-Fontan pulmonary artery Z-scores and higher freedom from pulmonary artery reinterventions. [Display omitted] [ABSTRACT FROM AUTHOR]

We studied conduit-related risk factors for mortality, conduit reintervention, conduit replacement, and pulmonary artery (PA) reinterventions after truncus repair. Patients who underwent truncus repair at our institution between 1995 and 2019 were studied. Cox proportional hazards modeling evaluated variables for association with mortality, time to conduit reintervention, time to conduit replacement, and time to PA reintervention. Truncus was repaired in 107 patients at median age of 17 days (IQR 9-45). Median follow-up time was 7 years. Aortic homografts were implanted in 57 (53%) patients, pulmonary homograft in 40 (37%), and bovine jugular conduit in 10 (9%). Median conduit size was 11 mm (IQR 10-12) and median conduit Z-score was 1.71 (IQR 1.08-2.34). At 5 years, there was 87% survival, 21% freedom from conduit reinterventions, 37% freedom from conduit replacements, and 55% freedom from PA reinterventions. Conduit size (HR 0.7, 95%CI 0.4-1.4, p=.41) and type (aortic homograft reference; bovine jugular vein graft HR 0.6, 95% CI 0.08-5.2, p=.69; pulmonary homograft HR 0.7, 95% CI 0.2-2.3, p=.58) were not associated with mortality. On multivariate analysis, the hazard for conduit reintervention, conduit replacement, and PA reintervention decreased with increasing conduit Z-score values of 1 to 2.5 (non-linear relationship, p<.01), with little additional reduction in hazard beyond this range. Implantation of a larger conduit within Z-score values of 1 and 2.5 is associated with a decreased hazard for conduit reintervention, conduit replacement, and PA reintervention after truncus repair. The type and size of the conduits did not impact mortality. [ABSTRACT FROM AUTHOR]

Truncus arteriosus is associated with coronary anomalies. We identified coronary artery lesions in patients undergoing repair of truncus arteriosus, defined the impact of lesions on mortality, and studied the effect of surgical intervention of coronary lesions. A retrospective review identified 107 patients with truncus repair (1995-2019). Coronary lesions were categorized as ostial stenosis, intramural, juxtacommissural origin, and single coronary. Survival analysis characterized survival after truncus repair and studied the association of coronary lesions and mortality. Among 107 patients with truncus repair 34 patients had at least 1 coronary lesion. Median follow-up time was 7 years, with 85% 5-year survival. Coronary lesions including ostial stenosis, intramurality, and juxtacommissural origin were associated with increased mortality, whereas single coronaries did not impact survival. Eleven patients had 1 coronary lesion and 6 patients with 2 coronary lesions had similar (80% and 83%, respectively) 5-year survival. Eight patients with 3 coronary lesions had 24% 5-year survival (P =.0003). Among patients with 1 or 2 lesions, surgical intervention on the coronary lesions tended to be associated with longer 5-year survival (100% vs 62%, respectively; P =.06). All patients with 3 lesions underwent coronary artery intervention, with 24% 5-year survival. Impact of coronary lesions on mortality after truncus repair increases with the number of lesions. Coronary artery intervention may be associated with improved time-related survival among patients with 1 or 2 lesions. Patients with the most complex anomalies (3 lesions) have poor survival and warrant ongoing study of repair techniques. [Display omitted] [ABSTRACT FROM AUTHOR]

Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We compared outcomes data of patients who underwent transection and reimplantation (TAR) and patients who underwent an unroofing. Patients who presented to the Coronary Artery Anomalies Program were evaluated and managed following a standardized approach. Anatomy was determined using computed tomography angiography, myocardial perfusion using advanced stress imaging, and surgical intervention according to anatomic features. Sixty-one patients underwent surgical repair of AAOCA between 2012 and 2019: 16 (26%) patients underwent TAR of the anomalous coronary without an aortic button and 45 (74%) patients underwent coronary unroofing. Compared with patients who underwent an unroofing, patients who underwent TAR had similar intramural length (5 mm with interquartile range of 4-7.7 vs 6 mm with interquartile range of 5-7; P =.6). One patient with an anomalous right coronary underwent coronary artery bypass grafting after TAR because of persistent postoperative ischemic changes. One patient with unroofing of an anomalous left coronary artery presented with recurrent aborted sudden cardiac death and underwent subsequent TAR, without further events. At last follow-up, 15 of 16 patients (94%) who underwent TAR and 42 of 45 (93%) patients who underwent an unroofing were released to unrestricted exercise activities. Coronary artery TAR is a useful surgical alternative for AAOCA when there is a course below the commissure, when unroofing does not relocate the ostium to the appropriate sinus, or when unroofing results in compression by the intercoronary pillar. TAR is a useful surgical alternative for AAOCA when there is a course below the commissure, when unroofing does not relocate the ostium to the appropriate sinus, or when unroofing results in compression by the intercoronary pillar. AAOCA , Anomalous aortic origin of the coronary artery; TAR , transection and reimplantation; CTA , computed tomography angiography; CABG , coronary artery bypass graft. [Display omitted] [ABSTRACT FROM AUTHOR]

We evaluated the range of prosthetic size-to-weight ratio to optimize valve survival in small children. A single-institution retrospective review of mechanical mitral valve replacements from 1995 to 2019 was performed. Prosthetic valve size-to-weight ratio was calculated as the prosthetic valve diameter divided by the patient's operative weight in children less than or equal to 35 kg. Patient death or reoperation on the valve was analyzed by size-to-weight ratio. Identifying a U-shaped distribution of events, patients were stratified as being in the nadir of the distribution or on the edges. Mechanical mitral valve replacements were performed in 56 (75%) children weighing less than or equal to 35 kg. Median follow-up time was 3.7 (interquartile range, 0.46-12) years. Median size-to-weight ratio was 1.5 (interquartile range, 1.0-2.0). A second replacement was required in 15 (27%) patients. Death occurred in 6 (11%) patients, including 3 after reoperation. The nadir of U-shaped distribution of events by size-to-weight ratio was bounded by a ratio from 1 to 2, which included 29 (52%) patients. A size-to-weight ratio from 1 to 2 provided optimal outcomes regardless of patient age. Reoperation-free survival at 5 years was 96% for patients with a ratio from 1 to 2 and 46% for patients with a ratio less than 1 or greater than 2. Patients with size-to-weight ratio 1 to 2 had longer reoperation-free survival than patients with a ratio less than 1 or greater than 2 (P <.001). Regardless of patient age, in patients less than or equal to 35 kg, optimal reoperation-free survival after prosthetic mitral valve replacement can be obtained by placing a prosthetic valve whose diameter is between 1 and 2 times the patient's weight in kilograms. [ABSTRACT FROM AUTHOR]

Birth weight, preterm delivery, and size for gestational age are surrogate markers for development that are commonly used in congenital heart surgery. Understanding the associations of these variables with patient outcomes is of great importance. This study included all patients with hypoplastic left heart syndrome who underwent a Norwood procedure at a single institution from 1995 to 2018. Low birth weight was defined as weight less than 2.5 kg, and preterm delivery occurred at less than 37 weeks' gestation. Overall and conditional analyses were performed to evaluate for association with outcomes after the Norwood procedure. Secondary analyses evaluated the association of development measures with postoperative length of stay and ventilator duration. In total, 303 neonates (60% male) underwent the Norwood procedure and were followed for a median of 3.9 years (interquartile range, 0.5 to 10.4 years). Median birth weight was 3.1 kg (interquartile range, 2.8 to 3.4 kg). Patients with low birth weight had decreased transplant-free survival compared with patients with a normal birth weight (hazard ratio, 1.7; 95% confidence interval, 1.03 to 2.82; P =.039). When conditioning on survival to second-stage palliation, patients born small for gestational age had decreased transplant-free survival compared with patients born at appropriate size for gestational age (hazard ratio, 2.8; 95% confidence interval, 1.31 to 6.09; P =.008). Patients delivered preterm had a longer hospital length of stay (median, 55 days vs 31 days; P =.02) and more ventilator days compared with patients delivered at term (median, 7 days vs 4 days; P =.004). Various developmental markers have differing prognostic importance for patients undergoing the Norwood procedure. Understanding these differences can help guide preoperative decision making and patient selection. [ABSTRACT FROM AUTHOR]

Multiple techniques are available for repair of supracardiac partial anomalous pulmonary venous return (PAPVR); however, most series fail to compare the techniques in contemporary cohorts. This study aimed to describe outcomes of the Warden procedure with a single-patch repair cohort to serve as a control. A retrospective cohort analysis of all patients at a single institution (Texas Children's Hospital, Houston, TX) included patients undergoing either the Warden procedure or single-patch repair from 1996 to 2019 for PAPVR. Reintervention was defined as any catheter or surgical procedure on the superior vena cava (SVC) or pulmonary veins. Subgroup analysis was performed within the Warden cohort to evaluate for association between an SVC patch and reintervention-free survival. In total, 158 patients (122 in the Warden group and 36 in the single-patch group) were identified. The median age at operation was younger for patients in the Warden cohort (5.4 years; interquartile range, 3.3 to 10.2 years) compared with patients in the single-patch cohort (13.3 years; interquartile range, 6.5 to 18.7 years; P <.001). One patient in each cohort died. One patient required reoperation after the Warden procedure for dehiscence of the intracardiac patch. Ten patients required transcatheter reinterventions. Reintervention-free survival was not different between patients in the Warden cohort and patients in the single-patch cohort (P =.54) or within the Warden cohort in patients with an SVC patch (P =.27). When controlling for repair type, older age at repair was associated with longer reintervention-free survival (hazard ratio, 0.81; 95% confidence interval, 0.71 to 0.93; P =.002). The Warden procedure is a viable option for younger patients requiring supracardiac PAPVR repair, although these younger patients are likely at greatest risk for reintervention regardless of surgical technique. [ABSTRACT FROM AUTHOR]

Multiple techniques exist for the repair of supravalvular aortic stenosis (SVAS), but given the lesion's rarity, analyses comparing the efficacy of each repair have been limited. A retrospective review of all children at a single institution who underwent repair of SVAS from June 1995 to May 2019 was performed. Anatomic and physiologic measurements across time points were compared between 2 predominant surgical techniques. Time-to-event outcomes were compared using the log-rank test. SVAS was repaired in 89 patients, by using a single-patch in 31 (35%) and the Doty repair in 58 (65%). Median age at operation was 2.5 years (interquartile range [IQR], 1.0 to 6.8 years), with median follow-up of 5.8 years (IQR, 1.8 to 10.7 years). Reoperation was required in 8 (9%) patients at a median of 1.5 years postoperatively (IQR, 0.3 to 4.8 years). There was 1 death after multiple reinterventions. The change from the preoperative to the postoperative sinotubular junction z-score was greater for patients after Doty repair (median change +2.5; IQR, 1.5, 4.1) than for patients after single-patch repair (median change +0.8; IQR, −0.1, 2.1; P =.001). Freedom from reoperation was longer for patients after Doty repair than after the single-patch technique (P =.008). The Doty repair provides longer freedom from reoperation after supravalvular aortic stenosis repair compared with a single-patch technique, likely through a greater increase in the sinotubular junction at the time of initial operation. [ABSTRACT FROM AUTHOR]

Anomalous aortic origin of a coronary artery with an intraseptal course of the left coronary artery is a rare anomaly that can cause myocardial ischemia. Surgical technique known to improve outcome is lacking because of the challenging complexity of the anatomy. We herein describe a novel surgical approach in a young athlete with myocardial infarction and ongoing ischemia. Supraarterial myotomy of the intraseptal segment through a right ventriculotomy and direct reimplantation of the left coronary artery were performed successfully. After postoperative resolution of inducible myocardial hypoperfusion, the patient was allowed to return to competitive activities with no further symptoms. [ABSTRACT FROM AUTHOR]

This single-institution study assessed the midterm outcomes of patients undergoing complete vascular ring (CVR) repair and the need for reintervention. The study included all patients who underwent surgical repair of an isolated CVR from 1996 to 2018 at our institution. Patients who underwent concomitant intracardiac repair were excluded. Data analysis included demographics, type of anomaly, other congenital heart disease, clinical symptomatology, operative technique, perioperative outcomes, reoperation rates, and mortality. CVR repair through open thoracotomy was performed in 148 patients (80 boys [54%]), median age, 1.04 years (interquartile range, 0.4-5.2 years), and median weight, 12.8 kg (interquartile range, 7.5-26.5 kg). The cohort included 72 patients with double aortic arch (DAA), 69 with right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum arteriosum (LLA), 5 with RAA with left descending aorta and LLA, and 2 with RAA with mirror-image branching and LLA. There was 1 outpatient perioperative death (0.7%) 15 days postoperatively. Perioperative complications occurred in 20 patients (14%): 18 (12%) with chylothorax (3 required reintervention), 1 pneumothorax, and 1 vocal cord paresis. Two of 36 patients (5.5%) without primary diverticulum resection required reoperation and subclavian reimplantation at 3 and 4 years, and 1 patient required aortic translocation 9 years later for persistent symptoms. Freedom from reoperation after CVR repair was 93% at 5 years and 86% at 10 years. A small proportion of patients who do not undergo diverticulum resection and aberrant left subclavian artery reimplantation at the time of CVR repair will require reintervention in the future. [ABSTRACT FROM AUTHOR]

Patients with Marfan syndrome (MFS) often require surgical intervention on the mitral valve (MV), aortic root or valve (AV), or thoracic aorta (TA) during childhood and adolescence. We aim to utilize a national database to evaluate outcomes in pediatric and young adult patients with MFS undergoing MV, AV, and aortic surgical procedures, and describe factors associated with increased mortality. The Pediatric Hospital Information System (PHIS) database, a multi-institutional administrative database of 48 pediatric hospitals, was queried for patients less than 25 years of age with a diagnosis of MFS (ICD-9 759.82) who underwent MV, AV, or thoracic aortic surgery between January 2004 and October 2015. We assessed comorbidities and complications, and performed univariate analysis to evaluate factors associated with inpatient mortality. Included were 321 hospital encounters in 294 patients. Fifty-one patients underwent 54 MV surgeries, 213 patients underwent 224 aortic/AV surgeries, and 43 patients underwent both MV and aortic/AV surgery in the same encounter. Postoperative complications were common for all surgeries (46.3% for MV procedures and 45.5% for aortic/AV procedures). Overall in-hospital mortality was 2.2% (3.7% for MV procedures, 1.8% for AV/aortic procedures, and 2.3% in the combined MV and aortic/AV procedure group). Aortic dissection or rupture was reported in 3.4%, with no in-hospital mortalities. Death after MV as well as after aortic/AV surgery was associated with younger age. Postoperative complications are common in pediatric and young adult patients with MFS after intervention on the MV, AV, and TA, although mortality is relatively low. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The optimal treatment of patients with complex coarctation of the aorta and hypoplastic aortic arch is controversial. Children undergoing repair of obstructive arch lesions are at lifelong risk of recurrence. Multiple techniques have been described to address complex primary and recurrent coarctation. Our approach at Texas Children’s Hospital (Houston, TX) has been to address these lesions in an anatomic fashion via median sternotomy, under cardiopulmonary bypass using the techniques of aortic arch advancement and ascending sliding arch aortoplasty. Anatomic repair of complex primary or recurrent aortic obstruction is safe and produces a favorable repair with a low rate of recurrence. Both operations restore normal arch contour using native aortic tissue. The decision of which particular surgical strategy to use depends on the patient’s age, anatomy, and the elasticity of the tissue. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background The optimal treatment for infants with aortic coarctation and hypoplastic aortic arch is controversial. The goal of this study was to report the short-term and mid-term outcomes of aortic arch advancement (AAA) in infants with hypoplastic aortic arch. Methods All infants who underwent AAA at our institution from 1995 to 2012 were included. AAA consisted of coarctectomy and end-to-side anastomosis of the descending aorta to the distal ascending aorta/proximal arch through a median sternotomy. The cohort was divided into four groups: (1) isolated AAA (n = 29, 11%), (2) AAA with closure of ventricular septal defect (n = 56, 20%), (3) AAA with other biventricular repairs (n = 115, 42%), and (4) AAA as part of single-ventricle palliation (n = 75, 27%). Results The cohort included 275 patients: 125 (45%) were female, and the median age was 14 days (interquartile range, 7-34 days). Genetic abnormalities were present in 48 patients (17%). Neurologic adverse events occurred in 3 patients (1%), all in group 4. Left bronchial compression was seen in 2 patients (0.7%); only one required intervention. Vocal cord dysfunction was noted in 36 of 95 patients (38%) on routine laryngoscopy. Only 1 patient had clinical residual dysfunction at the last follow-up visit. Perioperative mortality was 3% (n = 8). At a median follow-up time of 6 years, 8 patients (3%) had reinterventions at a median time of 5 months (3-17 months) after repair. Conclusions AAA is a safe, effective, and durable operation with low rates of adverse events and mid-term reintervention. The advantages include native tissue-to-tissue reconstruction and preserved potential for growth. As such, it is the ideal technique for the management of hypoplastic aortic arch in neonates and infants. [ABSTRACT FROM AUTHOR]

Background. There is a paucity of data on the current outcomes of surgical intervention for recurrent aortic arch obstruction (RAAO) after initial aortic arch repair in children. The goal of this study is to report the long-term results in these patients. Methods. All patients undergoing surgical intervention for RAAO at Texas Children's Hospital from 1995 to 2012 were included. The cohort was divided into four groups based on initial procedure: (1) simple coarctation repair, (2) Norwood procedure, (3) complex congenital heart disease, and (4) interrupted aortic arch. Results. A total of 48 patients age 9 months (range, 22 days to 36 years) underwent 49 procedures for RAAO. All patients had an anatomic repair consisting of either patch aortoplasty (n = 27, 55%), aortic arch advancement (n = 8, 16%), sliding arch aortoplasty (n = 6, 12%), placement of an interposition graft (n = 2, 17%), reconstruction with donor allograft (n = 4, 8%), extended end-to-end anastomosis (n = 1, 2%), or redo Norwood-type reconstruction (n = 1, 2%). Most procedures (n = 46, 94%) were performed through a median sternotomy using cardiopulmonary bypass. At a median follow-up of 6.1 years (range, 9 days to 17 years), only 2 patients required surgical or catheter-based intervention for RAAO. Hypertension was present in 10% of patients at last follow-up. There were no neurologic or renal complications. There was 1 perioperative death after an aortic arch advancement in group 1. Four other patients have died during follow-up, none of the deaths related to RAAO. Conclusions. Anatomic repair of RAAO is a safe procedure associated with low morbidity and mortality, and low long-term reintervention rates. [ABSTRACT FROM AUTHOR]

Anomalous aortic origin of a coronary artery (AAOCA) is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. It is the second most common cause of sudden cardiac death in young athletes. Its exact prevalence, the pathophysiological mechanisms that cause sudden cardiac death, the actual risk of death for the different types of AAOCA, the optimal way to evaluate these patients, and whether any treatment strategies decrease the risk of sudden cardiac death in patients diagnosed with AAOCA are unknown. This article analyzes what is currently known and unknown about this disease. It also describes the creation of a dedicated multidisciplinary coronary anomalies program and the development of a framework in an initial attempt to standardize the evaluation and management of these patients. [ABSTRACT FROM AUTHOR]

Surgical treatment of the Tetralogy of Fallot (ToF) is one of the great successes of medicine and also a topic of controversy. Different strategies have been proposed, including age-based (neonatal) management strategies as well as anatomic-based management strategies. Regardless of the management strategy entailed, the surgical management of ToF has considerably evolved over the years. As a result, patients can now expect excellent early results with survival approaching 100% for those without genetic syndromes. The goals of current surgical therapy should be to mitigate the late right ventricular (RV) dysfunction that may occur by minimizing the extent of surgical injury during the intial repair. As the surgical techniques continue to advance, the outcomes will continue to improve. [Copyright &y& Elsevier]

Background: In this study we report magnetic resonance imaging (MRI) brain injury and 12-month neurodevelopmental outcomes when regional cerebral perfusion (RCP) is used for neonatal aortic arch reconstruction. Methods: Fifty-seven neonates receiving RCP during aortic arch reconstruction were enrolled in a prospective outcome study. RCP flows were determined by near-infrared spectroscopy and transcranial Doppler monitoring. Brain MRI was performed preoperatively and 7 days postoperatively. Bayley Scales of Infant Development III was performed at 12 months. Results: Mean RCP time was 71 ± 28 minutes (range, 5 to 121 minutes) and mean flow was 56.6 ± 10.6 mL/kg/min. New postoperative MRI brain injury was seen in 40% of patients. For 35 RCP patients at age 12 months, mean Bayley Scales III Composite standard scores were: Cognitive, 100.1 ± 14.6 (range, 75 to 125); Language, 87.2 ± 15.0 (range, 62 to 132); and Motor, 87.9 ± 16.8 (range, 58 to 121). Increasing duration of RCP was not associated with adverse neurodevelopmental outcomes. Conclusions: Neonatal aortic arch repair with RCP using a neuromonitoring strategy results in 12-month cognitive outcomes that are at reference population norms. Language and motor outcomes are lower than the reference population norms by 0.8 to 0.9 standard deviations. The neurodevelopmental outcomes in this RCP cohort demonstrate that this technique is effective and safe in supporting the brain during neonatal aortic arch reconstruction. [ABSTRACT FROM AUTHOR]

Background: Newborns with single-ventricle anatomy, transposition of the great arteries, and systemic outflow obstruction are challenging patients most often managed with a Norwood or Damus-Kaye-Stansel (DKS) procedure. The palliative arterial switch operation (pASO) offers the theoretical advantage of avoiding a systemic-to-pulmonary artery shunt physiology and posterior entrapment of the left pulmonary artery while aligning the single left ventricle with the posterior semilunar valve. Limited outcome data exist for the pASO. The purpose of this study was to examine the clinical course of patients after this operation. Methods: We conducted a retrospective review of all neonates undergoing a pASO at our institution from July 1995 to June 2011. Results: Fourteen patients underwent pASO at a median age of 7 days (2–16 days). Primary diagnoses included double-inlet left ventricle (6 patients [43%]), tricuspid atresia (TA) (4 patients [29%]), and other (4 patients [29%]). Concomitant procedures at initial operation included aortic arch reconstruction (13 patients [93%]), pulmonary artery banding (6 patients [43%]), and placement of a systemic-to-pulmonary artery shunt (1 patient [7%]). Median intensive care unit stay and hospital length of stay were 11 days (4–60 days) and 21 days (9–84), respectively. There were no deaths. All patients had advanced to a bidirectional cavopulmonary shunt (BCPS). Four patients required intervention between the pASO and BCPS procedures: systemic-to-pulmonary artery shunt (3 patients [21%]) and pulmonary artery banding revision (1 patient [7%]). Eleven patients had Fontan completion; the remaining 3 patients are candidates for this procedure. At the time of the Fontan operation, 7 (64%) patients required pulmonary artery augmentation. All patients were alive at last follow-up (median, 5 years [3 months–14 years]). At last echocardiographic follow-up (median, 5 years [3 months–10 years]), all patients had normal ventricular function, trivial to mild neoaortic insufficiency, and no left ventricular outflow obstruction. Conclusions: The pASO should be considered in neonates with single ventricle, transposition, and systemic outflow obstruction because it affords a favorable anatomic arrangement for long-term palliation, with excellent survival and preserved ventricular function. [Copyright &y& Elsevier]

Background: Expectations for outcomes after the neonatal arterial switch operation (ASO) continue to change. This cohort study describes neurodevelopmental outcomes at age 12 months after neonatal ASO, and analyzes both modifiable and nonmodifiable factors for association with adverse outcomes. Methods: Patients who underwent an ASO (n = 30) were enrolled in a prospective outcome study, with comprehensive clinical data collection during the first 12 months of life. Brain magnetic resonance imaging was done preoperatively and 7 days postoperatively, and the Bayley Scales of Infant Development III was performed at age 12 months. Results: Ten of 30 patients (33%) had preoperative magnetic resonance imaging injury; 13 of 30 patients (43%) had new postoperative magnetic resonance imaging injury. Twenty patients (67%) had Bayley Scales of Infant Development III: Cognitive Composite standard score mean was 104.8 ± 15.0, Language Composite standard score median was 90.0 (25th to 75th percentile, 83 to 94), and Motor Composite standard score mean was 92.3 ± 14.2. Best subsets multivariable analysis found associations between lower preoperative and intraoperative cerebral oxygen saturation, preoperative magnetic resonance imaging brain injury, total bypass time, and total midazolam dose and lower Bayley Scales of Infant Development III scores at age 12 months. Conclusions: At 12 months after ASO, neurodevelopmental outcome means were within normal population ranges. The new associations reported in this study between potentially modifiable perioperative factors and outcomes require investigations in larger patient cohorts. Beyond survival, which was 100% in this cohort, factors influencing quality of life including neurodevelopmental outcomes should be routinely investigated in studies of ASO patients. [ABSTRACT FROM AUTHOR]

Introduction: In lung transplantation (LTx), the arterial partial pressure of oxygen (PaO2) is traditionally regarded as critical information for assessment of donor lung function. Each center sets its own thresholds; by convention, a donor PaO2 of less than 300 mm Hg has been considered disqualifying. Limited literature exists to support such a practice. We analyzed all LTxs performed in the United States over a 9-year period to assess the effect of donor PaO2 on graft survival. Methods: The United Network for Organ Sharing (UNOS) database was queried for LTx (January 2000–November 2009). Of 12,545 LTx performed, 12,045 (96%) had donor PaO2 data on a fraction of inspired oxygen of 1.0, recorded at the time of procurement. Results: Mean donor PaO2 was 407 ± 140 mm Hg. The majority of LTxs had a donor PaO2 greater than 300 mm Hg (9593 (80%]) whereas PaO2 was 200 mm Hg or less in 1830 (15%) and 201 to 300 in 582 (5%) donors. Use of donors with a PaO2 of less than 200 increased over time from 5% (45) in 2000 to 21% (295) in 2009 (P = .002). Kaplan-Meier survival analysis showed no difference in graft survival with differing donor PaO2s, irrespective of whether patients had a single or double LTx. A Cox multivariable analysis of 21 donor characteristics demonstrated that donor PaO2 had no association with graft survival. Conclusions: Donor PaO2 levels did not affect graft survival. The use of donors with lower PaO2s could substantially increase the donor pool. We are not suggesting that donor PaO2 is not important when assessing potential lung donors but its level of importance in regard to other criteria appears less than previously believed. [ABSTRACT FROM AUTHOR]