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Distinguishing duct dependent congenital heart disease (DDCHD) from other causes of hypoxaemia, notably persistent pulmonary hypertension (PPHN), represents a diagnostic and therapeutic dilemma for neonatal transport teams, specifically in relation to use of prostaglandin E1 (PGE1). In non-DDCHD, PGE1 is considered unnecessary and likely to produce unwanted side-effects including apnoea and hypotension.1
We report our observations of the effects of PGE1 infusion in a group of babies with evidence of PPHN but without DDCHD.
We reviewed the data on all infants ≤10 days and ≥34 weeks gestation with suspected DDCHD and/or PPHN transferred by the Newborn Emergency Transport Service (NETS), Victoria, from non-tertiary neonatal units to the regional paediatric cardiac centre at the Royal Children's Hospital Melbourne between 1 May 2007 and 31 May 2011. Cases were identified from NETS and cardiac databases. Final diagnosis was based on echocardiogram.
Of 142 eligible infants, 81 infants had DDCHD. Of 61 non-DDCHD infants, 51 had PPHN, and 10 infants had neither DDCHD nor PPHN. PGE1 was commenced in 71 (50%) of all infants; 52 (63%) of DDCHD group, 9 (18%) of the PPHN group and all others. Median (range) PGE1 dose was 20 (5–100) nanograms/kg/min.
Overall, PGE1 use was not associated with higher rates of ventilation or inotrope use (table 1). However, within the DDCHD subgroup, PGE1 use was associated with higher rates of ventilation (44% vs 14%, p=0.01) and inotrope use (29% vs 3%, p=0.01).
PGE1 use was associated with significantly shortened length of stay in PPHN (figure 1) (3 (1–14) vs 14 (13–27) days, p=0.004), but not in DDCHD patients (19 (21–37) vs 13 (13–32) days, p=012).
There was no significant difference in mortality between PGE1 and no-PGE1 groups in either PPHN or DDCHD subgroups (N (%): PPHN PGE1 vs PPHN noPGE1: 1 (11) vs 9 (21), p=0.66; DDCHD PGE1 versus DDCHD no PGE1: 6 (11) vs 3 (10), p=1.0).
Our data suggest that concerns regarding inadvertent PGE1 use in non-DDCHD may be misplaced and indeed 75% reduction in length of hospital stay for those with PPHN treated with PGE1 supports the beneficial effect of PGE1 in PPHN. This is consistent with previous observations of improved oxygenation in PPHN treated with PGE1 analogue.2 The mechanism of this effect could be the known pulmonary vasodilating effects of PGE1,3 and the benefits of maintaining ductal patency as a ‘blow-off’ valve to improve function in the pressure and volume-loaded right ventricle.4
As previous studies have also reported safe transport of infants receiving PGE1 without need for ventilation,5 ,6 we suggest that there may be no need to try to establish a formal diagnosis of DDCHD versus PPHN prior to transfer, but rather to treat with PGE1 regardless of the diagnosis since both stand to benefit.
Contributors All authors were responsible for planning and reporting. NG and NP conducted the data collection and analysis.
Competing interests None.
Ethics approval Royal Children's Hospital, Melbourne, Australia.
Provenance and peer review Not commissioned; externally peer reviewed.
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