Efficiency at Heart: Navigating the Hybrid Single-Ventricle Pathway

Abstract

Background: Single-ventricle cardiac defects (SVCDs) are among of the most health care resource-intensive congenital diseases. Although SVCDs are traditionally palliated using the Norwood pathway, in the last 2 decades select programs have used the hybrid strategy, which redistributes the operative and interstage risks. This study sought to characterize resource use for a cohort of patients with hybrid-palliated SVCD.

Methods: All patients with SVCDs who underwent palliation with the hybrid strategy and who were followed up exclusively at our institution from January 2008 to December 2021 were included. End points were death, Norwood conversion, orthotopic heart transplantation, 6 months post-Fontan status, or 4 years of age in those patients who had not completed staged palliation. Primary end points included total days hospitalized, number of cardiology visits, echocardiograms, catheterizations, and advanced imaging performed.

Results: Of 135 patients with a diagnosis of SVCD, 72 survived for 6 months after the Fontan procedure. By patient-year for the entirety of the pathway, patients had a median hospital length of stay of 16 days (interquartile range [IQR], 12-25 days), 7 cardiology visits (IQR, 6-9), 8 echocardiograms (IQR, 7-10), and 0.7 catheterizations (IQR, 0.4-1.1). The interstage 1 period had the heaviest resource burden requiring intense cardiology follow-up and echocardiography surveillance. Cardiac catheterizations and advanced imaging were most prevalent during interstage 2 period, with a median of 2 (IQR, 1-2) catheterizations and 36 (40%) patients undergoing advanced imaging. The total median number of hospital days per patient was 63 days (IQR, 47-98.5 days).

Conclusions: Resource use for the care of patients with SVCDs is significant. The intensity of surveillance decreases as patients progress through the pathway. In comparison with published Norwood pathway data, resource intensity and use patterns in hybrid palliation are comparable.

Graphical abstract

Figure 1

Consolidated Standards of Reporting Trials (CONSORT) diagram of 221 patients with a diagnosis of single-ventricle cardiac defect (SVCD). (CS2, comprehensive stage 2; HS1, hybrid stage 1; OHT, orthotopic heart transplantation.)

Figure 2

Univariate analysis of select risk factors for increased resource use. Forest plots of select risk factors for increased resource use represent the results of univariable negative binomial regression models. The dots represent the incidence rate ratio (IRR) and the line of 95% CI. The dashed lines indicate the line of no effect (IRR of 1). An IRR less than 1 indicates that the exposure is associated with a lessened rate of event, whereas an IRR greater than 1 indicates that the exposure is associated with an increased rate of event. Era 2: April 2014 to May 2017. Era 3: June 2017 to the present. (HLHS, hypoplastic left heart syndrome; LOS, length of stay.)

Supplementary Figure