Timing of Transfer and Mortality in Neonates with Hypoplastic Left Heart Syndrome in California

Abstract

Maternal race/ethnicity is associated with mortality in neonates with hypoplastic left heart syndrome (HLHS). We investigated whether maternal race/ethnicity and other sociodemographic factors affect timing of transfer after birth and whether timing of transfer impacts mortality in infants with HLHS. We linked two statewide databases, the California Perinatal Quality Care Collaborative and records from the Office of Statewide Health Planning and Development, to identify cases of HLHS born between 1/1/06 and 12/31/11. Cases were divided into three groups: birth at destination hospital, transfer on day of life 0–1 (“early transfer”), or transfer on day of life ≥ 2 (“late transfer”). We used log-binomial regression models to estimate relative risks (RR) for timing of transfer and Cox proportional hazard models to estimate hazard ratios (HR) for mortality. We excluded infants who died within 60 days of life without intervention from the main analyses of timing of transfer, since intervention may not have been planned in these infants. Of 556 cases, 107 died without intervention (19%) and another 52 (9%) died within 28 days. Of the 449 included in analyses of timing of transfer, 28% were born at the destination hospital, 49% were transferred early, and 23% were transferred late. Late transfer was more likely for infants of low birthweight (RR 1.74) and infants born to US-born Hispanic (RR 1.69) and black (RR 2.45) mothers. Low birthweight (HR 1.50), low 5-min Apgar score (HR 4.69), and the presence of other major congenital anomalies (HR 3.41), but not timing of transfer, predicted neonatal mortality. Late transfer was more likely in neonates born to US-born Hispanic and black mothers but was not associated with higher mortality. Supplementary Information The online version of this article (10.1007/s00246-021-02561-w) contains supplementary material, which is available to authorized users.

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

“…19 Lastly, PHIS does not contain the level of granularity afforded by institutional data; anatomic and morphologic variations previously linked to poor outcomes (eg, aortic/mitral atresia, intact atrial septum), specific genetic analyses, precise details of surgical or interventional procedures, and the impacts of procedural complications on DAOH could not be evaluated. [14][15][16][17] Future studies should therefore seek to validate and expand upon the findings of the present analysis using institutional data.…”

Distance from home to birth hospital, transfer, and mortality in neonates with hypoplastic left heart syndrome in California

“…Two prior studies have linked birth far from a cardiac center with mortality in infants with critical congenital heart disease (Kaltman et al, 2020; Morris et al, 2014). However, many smaller studies, including one by our own team, have not found transfer after birth to be associated with a higher risk of mortality (Bennett et al, 2010; Fixler et al, 2012; Purkey et al, 2021). Future studies should investigate if other outcomes, including long‐term morbidities, are associated with birth location and transfer.…”

“…Our group recently published data on transfer patterns in neonates with HLHS in California from 2006 to 2011. We found that late transfer was more likely in low birthweight infants and infants born to U.S.‐born Hispanic and Black mothers (Purkey et al, 2021). However, this analysis did not address whether or not infants were born close to home or the distance between the birth and repair hospitals.…”

“…Despite advances in the field, HLHS is still associated with a high rate of neonatal mortality, between 21% and 30% in recent studies (Hirsch et al, 2011; Kane, Canar, Kalinowski, Johnson, & Hoehn, 2016; Morris et al, 2014). Our own group recently published a rate of 28% neonatal mortality and 47% 1‐year mortality for infants with HLHS in California from 2006 to 2011 (Purkey et al, 2021).…”

Management of Hypoplastic Left Heart Syndrome in Low-Resource Settings and the Ethics of Decision-Making

“…Some children in whom the management intent was not primarily comfort care still do not survive after the diagnosis is made. They are often high risk (low birthweight, low five-min Apgar score, or have other major congenital anomalies), 7 too ill to make it to the operating room or to a facility with treatment capabilities, inadequately resuscitated prior to transfer, or decompensate during transport or on arrival at the treatment facility prior to intervention. Pretransport and/or presurgical mortality occurs in low-resource settings where patients must travel long distances to reach care.…”