Defining Expectations for Infants With Hypoplastic Left Heart Syndrome Who Survive Initial Surgical Palliation

Abstract

Background Overall one-year non-mortality outcomes for surgically palliated hypoplastic left heart syndrome (HLHS) patients remain understudied. Using the metric Days Alive and Outside of Hospital (DAOH), the present study sought to characterize expectations for surgically palliated patients’ first year of life. Methods The Pediatric Health Information System database was used to identify by ICD-10 code all HLHS patients who underwent surgical palliation (Norwood/hybrid and/or heart transplantation [HTx]) during their index neonatal admission and were successfully discharged alive (n = 2227) and for whom one-year DAOH could be calculated. DAOH quartiles were used to group patients for analysis. Results Median one-year DAOH was 304 (interquartile range [IQR] 250-327), including a median index admission length of stay of 43 days (IQR 28-77). Patients required a median 2 (IQR 1-3) readmissions, each spanning 9 days (IQR 4-20). One-year readmission mortality or hospice discharge occurred in 6% of patients. Patients with lower-quartile DAOH had a median DAOH of 187 (IQR 124-226), whereas upper-quartile DAOH patients had a median DAOH of 335 (IQR 331-340) ( P < .001). Readmission mortality/hospice-discharge rates were 14% and 1%, respectively ( P < .01). On multivariable analysis, factors independently associated with lower-quartile DAOH included interstage hospitalization (odds ratio [OR] 44.78 [95% confidence interval [CI] 25.1-80.2]), index-admission HTx (8.73 [4.66-16.3]), preterm birth (1.97 [1.34-2.90]), chromosomal abnormality (1.85 [1.26-2.73]), age >7 days at surgery (1.50 [1.14-1.99]), and non-white race/ethnicity (1.33 [1.01-1.75]). Conclusions In the current era, surgically palliated HLHS infants spend approximately 10 months alive and outside of the hospital, although outcomes are highly variable. Knowledge of the factors associated with lower DAOH can inform expectations and guide management decisions.

Days alive and out of hospital for children born with single-ventricle heart disease

“…While other studies have focused on characterising the number of days alive and out of hospital within 30 days of operation or in the first year of life for hypoplastic left heart syndrome patients, there are few studies that have quantified the number of days alive and out of hospital for multiple single-ventricle subgroups. [11][12][13] Overall median number of days alive and out of hospital in the first year of life for patients with a single-ventricle diagnosis was 278 days.…”

Textbook Outcome for Superior Cavopulmonary Connection: A Metric for Single Ventricle Heart Surgery

“…The authors performed multivariate analysis and found that preterm birth, presence of chromosomal abnormality, nonwhite race, and heart transplant as index operation were factors independently associated with lower-quartile DAOH. 21 Taken together, these data may be used to inform resource allocation, clinician index of suspicion for complications, and preoperative hemodynamic optimization. For instance, a child with a low probability of achieving the Glenn TO due to nonmodifiable risk factors such as an anatomic diagnosis of HLHS with moderate tricuspid regurgitation may be allocated more intensive care resources.…”