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Open in new tab Download slide Open in new tab Download slide OBJECTIVES Acute thoracic aortic dissection and rupture (TADR) has an incidence of 5–7 per 100 000-person years. Today, most children with congenital heart disease (CHD) survive to become adults with congenital heart disease (ACHD). This study evaluates TADR in patients with ACHD in a large, hospitalized patient population over 11 years to evaluate the incidence, risk factors and outcomes associated with TADR. METHODS This was a retrospective review of the Texas Inpatient Discharge Data Set from 1 January 2009 to 31 December 2019. All non-trauma discharges of patients ≥18 years were included. ACHD discharges were identified by International Classification of Diseases, 9th edition (ICD-9)/10 diagnosis codes. TADR were identified using 2 definitions: TADR1 is an ICD-9/10 code for TADR, and TADR2 is TADR1 with an ICD-9/10 procedure code for aortic intervention. Descriptive, univariate and logistic regression statistics were used. RESULTS A total of 22 154 664 eligible discharges were identified, of which 12 584 (0.06%) were TADR1 and a subgroup of 5699 (0.03%) were TADR2. CHD was more prevalent in TADR1 (0.2% vs 0.05%; P  < 0.001) and TADR2 (0.3% vs 0.04%; P  < 0.001). Adjusting for known TADR risk factors, CHD had an odds ratio of 1.69 (95% confidence interval: 1.09–2.63; P  = 0.020) for TADR1 and an odds ratio of 1.69 (95% confidence interval: 0.99–2.88; P  = 0.056) for TADR2. No in-hospital deaths were found in patients with CHD with TADR. CONCLUSIONS ACHD discharges had a higher frequency of TADR versus the general population (0.9–1.2 vs 0.3–0.6 per 1000 discharges). There is an indication that CHD confers an increased adjusted odds of TADR. As the ACHD population continues to grow in number as well as age, it will be important to continue to assess the risk of TADR from CHD and how traditional risk factors impact this risk. [ABSTRACT FROM AUTHOR]

Objective: To perform a statewide characteristics and outcomes analysis of the Trisomy 18 (T18) population and explore the potential impact of associated congenital heart disease (CHD) and congenital heart surgery., Study Design: Retrospective review of the Texas Hospital Inpatient Discharge Public Use Data File between 2009 and 2019, analysing discharges of patients with T18 identified using ICD-9/10 codes. Discharges were linked to analyse patients. Demographic characteristics and available outcomes were evaluated. The population was divided into groups for comparison: patients with no documentation of CHD ( T18NoCHD ), patients with CHD without congenital heart surgery ( T18CHD ), and patients who underwent congenital heart surgery ( T18CHS )., Results: One thousand one hundred fifty-six eligible patients were identified: 443 (38%) T18NoCHD , 669 (58%) T18CHD , and 44 (4%) T18CHS . T18CHS had a lower proportion of Hispanic patients (n = 9 (20.45%)) compared to T18CHD (n = 315 (47.09%)), and T18NoCHD (n = 219 (49.44%)) (p < 0.001 for both). Patients with Medicare/Medicaid insurance had a 0.42 odds ratio (95%CI: 0.20-0.86, p = 0.020) of undergoing congenital heart surgery compared to private insurance. T18CHS had a higher median total days in-hospital (47.5 [IQR: 12.25-113.25] vs. 9 [IQR: 3-24] and 2 [IQR: 1-5], p < 0.001); and a higher median number of admissions (n = 2 [IQR: 1-4]) vs. 1 [IQR: 1-2] and 1 [IQR: 1-1], (p < 0.001 for both). However, the post-operative median number of admissions for T18CHS was 0 [IQR: 0-2]. After the first month of life, T18CHS had freedom from in-hospital mortality similar to T18NoCHD and superior to T18CHD ., Conclusions: Short-term outcomes for T18CHS patients are encouraging, suggesting a freedom from in-hospital mortality that resembles the T18NoCHD . The highlighted socio-economic differences between the groups warrant further investigation. Development of a prospective registry for T18 patients should be a priority for better understanding of longer-term outcomes.

Introduction: Hypoplastic Left Heart Syndrome accounts for a significant proportion of CHD morbidity and mortality, despite improvements in care and improved survival. This study evaluates number of, reasons for, and trends in discharges of patients with hypoplastic left heart syndrome over 11 years in Texas., Methods: The Texas Inpatient Discharge Dataset Public Use File captures almost all discharges in Texas and was reviewed from 2009 to 2019. Discharges of patients ≥5 years of age and diagnosis codes for Hypoplastic Left Heart Syndrome were included. The admitting and principle diagnoses were categorised and all discharges were evaluated for procedures performed. Descriptive and univariate statistical analyses were performed., Results: A total of 1024 discharges were identified with a 16.9% annual increase over the study period. Median length of stay was 4 [IQR: 2-8] and there were 17 (1.7%) in-hospital mortalities with no differences across age groups. Seven (17.1%) discharges of patients 25+ years were uninsured, higher than other age groups (p < 0.001). The most common admitting diagnosis was CHD and 224 (21.9%) of discharges included a procedure, including 23 heart transplants. Discharges occurred from 67 different hospitals with 4 (6.0%) representing 71.4% of all discharges., Conclusions: Discharges of Hypoplastic Left Heart Syndrome have increased rapidly, particularly in the older age groups and were spread over a large number of hospitals. Further work is needed to understand the interplay between Hypoplastic Left Heart Syndrome and other conditions and care experiences that occur within the general population, which will become more common as this population ages and grows.

We aimed to describe the short- and long-term outcomes of patients after an arterial switch operation (ASO) at a single institution during a 23-year period. A retrospective chart review of all patients <18 months of age who underwent an ASO between January 1995 and March 2018 at Texas Children's Hospital, Houston, TX was performed. Primary endpoints include mortality and reintervention. Perioperative mortality was defined as mortality occurring in-hospital and/or <30 days after surgery. Survival and freedom-from-reintervention were analyzed using Kaplan-Meier method, log-rank tests, and Cox regression models. The cohort included 394 patients. Diagnoses included 204 patients (52%) with intact ventricular septum, 137 (35%) with a ventricular septal defect, 17 (4%) with a ventricular septal defect and left ventricular outflow tract obstruction (LVOTO), and 36 (9%) with Taussig-Bing anomaly. Median age at surgery was 8 days (range: 1 day to 17 months) and median weight was 3.4 (range: 0.8-12.0) kg. Overall perioperative mortality was 1.3% (n = 5), 0.3% (n = 1) since 1999. Overall survival at 5, 10, and 15 years was 98.2%, 97.8%, and 97.8%, respectively. Perioperative morality was associated with prematurity (P = 0.012), <2.5 kg (P< 0.001), and longer circulatory arrest (P = 0.024) after univariate analysis. Reintervention was associated with a longer cross-clamp time (P < 0.001), <2.5 kg (P = 0.009), LVOTO resection (P = 0.047), and genetic syndrome (P= 0.011) after multivariable analysis. Current ASO expectations should include a perioperative mortality risk of <1% and good long-term survival. Reinterventions are more frequent in patients <2.5 kg, concomitant LVOTO resection, a genetic syndrome, and longer cross-clamp time., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: Instantaneous wave-free ratio (iFR) has been proven to correlate with coronary flow reserve better than fractional flow reserve (FFR) and is non-inferior to FFR in guiding coronary revascularization in ischemic heart disease. There has been no study validating the utility of iFR in children., Methods: We performed a retrospective review of clinically indicated cases in which both FFR and iFR were obtained at Texas Children's Hospital from July, 2016 to March, 2019. FFR and iFR were obtained at baseline. Adenosine FFR (FFRa) was used for assessment of coronary artery (CA) stenoses and diastolic dobutamine FFR (dFFRd) for myocardial bridges (MBs). FFRa or dFFRd ≤0.8 and iFR ≤0.89 indicated significant flow impairment., Results: A total of 22 coronary arteries (9 CA stenoses and 13 MBs) were assessed in 20 patients with median age of 13 years (range, 4-21 years) and median weight of 60 kg (range, 19-110 kg). iFR correlated with FFRa (Spearman's rho, 0.87; P<.01) in CA stenoses and with dFFRd (Spearman's rho, 0.74; P<.01) in MBs and agreed with FFR in 20/22 cases (90.9%). In 1 patient with CA stenosis and 1 MB with normal FFR, iFR was positive and both patients underwent coronary revascularization., Conclusions: iFR correlated with FFR in the assessment of CA stenoses in children. iFR does not require administration of pharmacological agents; thus, it may reduce procedural time, cost, and complications, and result in more widespread adoption of invasive assessment of CA lesions in young patients.

Background: Pulmonary atresia with intact ventricular septum (PAIVS) with right ventricle-dependent coronary circulation (RVDCC) carries suboptimal outcomes primarily due to cardiac ischemia. We hypothesize clinical outcomes are affected by the level of coronary obstruction, a surrogate for vulnerable myocardium., Methods: We conducted a single-institution retrospective analysis of all neonates with PAIVS with RVDCC from 1995 to 2017. RVDCC was defined as the presence of any coronary-cameral fistula with coronary obstruction proximal to the fistula and angiographic evidence of RV perfusion of the myocardium through the fistulous communication. Location of coronary obstruction was categorized as either proximal or distal segments, using the SYNTAX score criteria. Transplant-free survival was compared between patients with proximal and distal obstruction, then these groups were compared with patients without RVDCC., Results: Of 103 neonates with PAIVS, 28 (27%) had RVDCC: 18 proximal (64%), 10 distal (36%). Median age at last follow-up for patients with RVDCC was 1.8 years (interquartile range, 0.3-8.1 years). All deaths (10 of 28, 36%) occurred at 6 months old or earlier. Proximal coronary artery obstruction was associated with decreased transplant-free survival relative to distal obstruction (hazard ratio = 3.63; 95% confidence interval, 1.01-13.00; P = .048). Transplant-free survival at 1 year was 33% and 70% in the proximal and distal obstruction groups, respectively. Compared with patients without RVDCC, patients with proximal obstruction had significantly lower transplant-free survival (P < .001), whereas patients with distal obstruction did not (P = .217)., Conclusions: The location of coronary artery obstruction affects clinical outcome and may represent a potential branch point in the management for PAIVS with RVDCC., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Children with Down syndrome (DS) have lower mortality compared to nonsyndromic (NS) children after atrioventricular septal defect (AVSD) repair. Limited data exist regarding hospital mortality and utilization after other congenital heart disease (CHD) operations in DS. We compared hospital mortality and utilization after CHD operations in both populations and hypothesized that the survival benefit in children with DS is not consistent across CHD lesions. The Texas Inpatient Public Use Datafile was queried for all patients <18 years old undergoing operations for CHD between 1999 and 2016. Hospital mortality, length-of-stay and charges were compared between DS and NS groups, stratified by CHD operation using mixed-effects multivariable analyses and propensity score matching analyses adjusting for prematurity, low birth weight, age, and sex. Over the 18-year period, 2841 cases with DS underwent CHD operations compared to 25,063 NS cases. The most common types of interventions performed in DS were AVSD repair, isolated ventricular septal defect (VSD) repair and tetralogy of Fallot (TOF) repair. By multivariable analyses, DS was associated with lower mortality after isolated AVSD repair (RR 0.40 [IQR 0.20-0.79]), and higher hospital mortality after bidirectional Glenn anastomosis (BDG) (RR 5.17 [IQR 2.10-12.77]) and TOF/pulmonary atresia repair (RR 9.71 [IQR 2.16-43.68]) compared to NS children. Similar results were noted using propensity score matching. Children with DS had lower mortality after AVSD repair than NS children, but higher mortality after operations for BDG and TOF/pulmonary atresia. Further study is needed to determine if the presence of pulmonary hypertension in DS modifies the association between DS and mortality depending on cardiac lesion., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: There are limited studies analyzing pulsatile Glenn as a long-term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution., Design: A retrospective review was performed., Setting: Study performed at a single pediatric hospital., Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included., Outcome Measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk factors were assessed by Cox multivariable competing risk analyses., Results: Seventy-eight patients underwent pulsatile Glenn at age 9 months (interquartile range, 5-14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time-to-palliation of 4 years (interquartile range, 3-5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five- and 10-year transplant-free survival were 91% and 84%, respectively. At a median follow-up of 6 years (interquartile range, 2-8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3-11], oxygen saturation 83% ± 4%). Preoperative moderate-severe atrioventricular valve regurgitation (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80-33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08-6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for covariates. Reaching further palliation was less likely in patients with preoperative moderate-severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08-0.59; P =.002)., Conclusion: Pulsatile Glenn can be an effective tool to be used in challenging circumstances, these patients can have a favorable long-term prognosis without reducing their suitability for further palliation., (© 2018 Wiley Periodicals, Inc.)

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: 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., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)