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.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.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.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.
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.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).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.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.
Background: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death (SCD) in children. Surgical intervention is recommended for AAOLCA with an interarterial course, with other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of AAOLCA in a prospective cohort following a standardized approach. Methods: All patients with AAOLCA <20 years old were prospectively enrolled. Anatomic details were obtained with coronary computerized tomography angiography (CTA). Myocardial functional studies were obtained under provocative stress, including exercise stress test, stress nuclear perfusion imaging, or dobutamine stress cardiac magnetic resonance. Patients were stratified as high-risk if: 1) episode of aborted SCD, 2) interarterial and/or intramural course, or 3) evidence of myocardial ischemia on functional studies. Results: A total of 47 patients (32 male, 68%) with AAOLCA were enrolled, median age 12 [IQR 6.0;15.0] years. Of these, 20 had exertional symptoms (42.5%) and 5 had aborted SCD (10.5%). Thirty-one patients (79%) were classified high-risk, 28% low-risk, and 3 (6%) work-up is ongoing. Of patients with completed work-up, 16/43 (37%) had inducible ischemia and, of these, 2/16 (13%) presented without symptoms and one with aborted SCD as the first symptom. Two patients in our cohort had AAOLCA arising close to the noncoronary sinus, just above the left and noncoronary commissure, resulting in ostial stenosis: one presented with aborted SCD and another with left ventricular dysfunction. To date, 17 patients have undergone repair, median age 10 years [IQR 6.6;15.7]. Median follow-up was 3.9 [IQR 1.3;6.3] years with no significant symptoms and all but 4 patients are cleared from exercise restrictions. Conclusion: AAOLCA may present with diverse anatomic configuration and risk stratification is essential to determine management. AAOLCA from the noncoronary sinus may also present with myocardial ischemia and, thus, be classified as high-risk. A multimodality imaging approach is necessary to adequately risk stratify this population. All patients are alive at last follow-up and the majority free from exercise restriction.
