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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.

Introduction: With advances in care, survival into adulthood of single-ventricle (SV) palliation is more frequent. Fertility and family planning in this setting is complex. In the general population, maternal mortality is 0.02%, post-partum hemorrhage (PPH) is 3.1% and pre-eclampsia is 7.5%. There are limited data on outcomes of pregnancies of women with SV physiology. This study reviews deliveries of SV mothers in Texas over the past 14 years. Methods: This is a retrospective review of the Texas Inpatient Discharge Dataset, an administrative dataset containing most hospital discharges. All discharges of females≥15 years old between 1/1/2006-12/31/2019 were reviewed. ICD-9/10 diagnosis codes consistent with Hypoplastic Left Heart Syndrome (HLHS), Double Inlet Ventricle (DIV)/Common Ventricle (CV), and Hypoplastic Right Heart Syndrome (HRHS)/Tricuspid Atresia (TA) were included. Pregnancy-related discharges were identified by ICD-9/10 codes. Descriptive and univariate statistics were utilized. Results: A total of 56 pregnancy-related discharges were identified, with 40 (71.4%) deliveries (Table). One (2.5%) was a twin gestation and 4(10%) had fetal demise. Number of annual deliveries increased from 0 in 2006 to 8 in 2019. Median gestational age was 37[IQR: 33.25-38] weeks, 7 (17.5%) had fetal anomalies and 7(17.5%) had poor fetal growth. Twelve (30%) underwent C-Section. Median length of stay was 3.5[2-5] days, 3(7.5%) mothers had PPH, 2(5%) required ventilator support, and there were no in-hospital mortalities. Conclusions: As the number of patients with SV surviving into adulthood increases, accurate counseling on risks of pregnancy is required. While these data cannot evaluate the outcomes for the delivered baby, they suggest favorable short-term outcomes for mothers with no in-hospital mortalities. Further research is needed to both understand outcomes for the delivered babies as well as potential longer-term sequelae for mothers.

Background: There are more than 1.4 million adult congenital heart disease (ACHD) patients estimated in the United States. As this population continues to grow, as will their utilization of the healthcare system. Objectives: This study aims to analyze trends in discharges of ACHD patients in the state of Texas from 2009 through 2018. Methods: This is a retrospective review of the Texas Inpatient Discharge Public Use File from 2009 through 2018. Discharges from acute care hospitals of patients ≥18 years of age with an International Classification of Diseases 9 or 10 diagnosis code consistent with a CHD diagnosis which could be categorized by the American Heart Association/American College of Cardiology lesion severity score (LSS). Isolated atrial septal defect and congenital aortic valve insufficiency diagnoses were excluded from the analysis. Results: A total of 10,515 ACHD discharges were identified. Annual discharges increased from 930 to 1,315, a 41% increase during the study period (p-trend

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 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.

In 2007, the Texas legislature appropriated money for a pilot study to evaluate cardiovascular screening of student athletes to identify those who might be at risk of sudden death using a questionnaire, physical examination, electrocardiography, and limited echocardiography. We sought to determine (1) the feasibility of a state-wide cardiovascular screening program, (2) the ability to reliably identify at-risk subjects, and (3) problems in implementing screening state wide. The data were analyzed using established pediatric electrocardiographic and echocardiographic criteria. Positive results were confirmed by a blinded reviewer. In 31 venues (2,506 students), the electrocardiographic findings met the criteria for cardiovascular disease in 57 (2.3%), with 33 changes suggestive of hypertrophic cardiomyopathy, 14 with long QT syndrome, 7 with Wolff–Parkinson–White syndrome, and 3 with potential ischemic findings related to a coronary anomaly. Of the 2,051 echocardiograms, 11 had findings concerning for disease (9 with hypertrophic cardiomyopathy and 1 with dilated cardiomyopathy). In patients with electrocardiographic findings consistent with hypertrophic cardiomyopathy, the limited echocardiograms were normal in 24 of 33. Of the 33 who remained at risk of sudden death on the electrocardiogram or echocardiogram, 25 (65.8%) pursued the recommended evaluation, which confirmed long QT syndrome in 4, Wolff–Parkinson–White syndrome in 7, and dilated cardiomyopathy in 1. The interobserver agreement was 100% for electrocardiography and 79% for echocardiography. The questionnaire identified 895 (35% of the total) potentially at-risk students, with disease confirmed in 11 (1.23%). In conclusion, in this large state-funded project, electrocardiographic and echocardiographic screening identified 11 of 2,506 patients potentially at risk of cardiovascular disease. The questionnaire was of limited value and had a large number of false-positive results. Interobserver variation was significant for echocardiography and might create problems with limited echocardiographic screening. Finally, many subjects with abnormal screening results declined a follow-up evaluation.