Accuracy of clinical diagnosis and decision to commence intravenous prostaglandin E1 in neonates presenting with hypoxemia in a transport setting.

BACKGROUND: Setting goals for monitoring and initiating life-saving interventions such as prostaglandins (prostaglandin E(1) [PGE(1)]) during transport stabilization are dependent on establishing an accurate clinical diagnosis. OBJECTIVE: The aim of this study was to determine the accuracy of clinical diagnosis of suspected congenital heart disease (CHD) and the decision to initiate PGE(1) in neonates presenting with hypoxemia. METHODS: A retrospective cohort study (2002-2004) on hypoxemic neonates who were transported to an outborn neonatal intensive care unit (NICU) was conducted. Provisional diagnosis established by the transport team was categorized as suspected CHD (group 1), suspected persistent pulmonary hypertension of the newborn (group 2), and suspected CHD and/or persistent pulmonary hypertension of the newborn (group 3) based on history, physical examination, laboratory test, chest radiograph, and initial response to treatment. A definitive diagnosis was established on arrival to NICU by echocardiography. RESULTS: A total of 115 neonates were included in the study. The mean gestational age at birth, median age at admission to NICU, and the mean stabilization time were 38.2 (2.4) weeks, 1 (1-26) days, and 217 (108) hours, respectively. The interventions provided during transport stabilization included mechanical ventilation (n = 86, 75%), PGE(1) (n = 70, 61%), inotropes (n = 41, 36%), and fluid bolus (n = 50, 43%). The accuracy of a provisional diagnosis of CHD by transport team was 87.7% and the positive predictive value was 88.1%. Sixty neonates (88%) received PGE(1) appropriately. Eight neonates (12%) with duct-dependent CHD (n = 68) did not receive PGE(1) and were considered as missed opportunities. Ventilated neonates in groups 1 and 3 were identified as the groups that can potentially benefit from more liberal use of PGE(1) and without any adverse effects. CONCLUSION: Although the accuracy of a diagnosis of CHD and the decision to initiate PGE(1) was high, 12% of neonates with a duct-dependent CHD were transported without commencement of PGE(1). Lower thresholds for PGE(1) administration to hypoxemic neonates may potentially improve preoperative stabilization and minimize neonatal morbidity.

Review of interventional procedures in the very low birth-weight infant (<1.5 kg): complications, lessons learned and current practice.

BACKGROUND: Interventional radiology (IR) procedures in very low birth-weight (VLBW) infants (<1.5 kg) are challenging due to size, immaturity, comorbidities and lack of devices of suitable size. Infants are moved from the neonatal intensive care unit to the IR suite, further exposing them to risk. Our purpose was to review our experience of interventional procedures in VLBW infants, specifically complications and potential risks. MATERIALS AND METHODS: VLBW infants referred for image-guided therapy between 1998 and 2005 were identified and medical records reviewed. "Complications" were divided into: major or minor, periprocedural or postprocedural, and intervention-/device-related, patient-related or equipment-related. Transport risk index of physiological stability (TRIPS) scores were calculated. RESULTS: A total of 116 infants (68 male, 48 female) underwent 176 procedures (159 vascular access-related and 17 nonvascular). Of 158 complications identified, 116 were major and 42 were minor. Major complications included hypothermia (n=33), line manipulations/removals (n=25), bleeding (n=12), thrombosis (n=4), cardiac arrest (n=3), tamponade (n=2), and multiorgan failure (n=1). Of the complications, 119 were categorized as intervention-/device-related, 32 patient-related and 7 equipment-related. There were no significant differences between pre- and postprocedural TRIPS scores. CONCLUSION: Successful completion of IR procedures in the VLBW infant is possible, but complications still occur in these fragile infants.

Duodenal and biliary atresia associated with facial, thyroid and auditory apparatus abnormalities: a new mandibulofacial dysostosis syndrome?

We report a female child born at 36 weeks of gestation with multiple abnormalities including dysmorphic and coarse facial features with features of mandibulofacial dysostosis that include bilateral microtia with the absence of external auditory meati and Mondini dysplasia as well as, duodenal atresia, intestinal malrotation, anterior displacement of the anus, left hemiaplasia of the thyroid and biliary atresia in sibs. The associations of duodenal atresia with intrahepatic and extrahepatic biliary atresia in sibs have been reported, suggesting an autosomal recessive syndrome. However, the associated external, middle and internal ear anomalies and the thyroid malformation, however, have not been reported in this condition. To the best of our knowledge, this is a hitherto new syndrome with an unknown inheritance.

Milrinone improves oxygenation in neonates with severe persistent pulmonary hypertension of the newborn.

BACKGROUND: Many neonates with severe persistent pulmonary hypertension of the newborn (PPHN) are nonresponders to inhaled nitric oxide (iNO). Milrinone is a promising adjunctive therapy because of its pulmonary vasodilator properties and cardiotropic effects. DESIGN: Case series of neonates with severe PPHN (defined as oxygenation index [OI] >20, failure of iNO therapy, and echocardiographic confirmation of PPHN). SETTING: Tertiary neonatal intensive care unit. SUBJECTS: Full-term (> or =37 weeks) neonates with severe PPHN who received intravenous milrinone. MEASUREMENTS: The primary end point was the effect of intravenous milrinone on OI and hemodynamic stability over a 72-hour study period. Secondary end points examined included duration of iNO and degree of cardiorespiratory support. RESULTS: Nine neonates at a mean gestation of 39.25 +/- 2.76 weeks, birth weight of 3668 +/- 649.1 g, and baseline OI of 28.1 +/- 5.9 received milrinone treatment after a poor initial response to iNO treatment. Intravenous milrinone was commenced at a median age of 21 hours (range, 18-49 hours), and patients were treated for median of 70 hours (range, 23-136). Oxygenation index was significantly reduced after milrinone treatment, particularly in the immediate 24 hours of treatment (8.0 +/- 6.6, P < .001). There was a significant improvement in heart rate (179 +/- 15.2 vs 149.6 +/- 22.4, P < .001) over the same period. Infants who received milrinone did not develop systemic hypotension; in fact, there was a nonsignificant trend toward improved blood pressure. CONCLUSIONS: Intravenous milrinone produces early improvements in oxygenation without compromising systemic blood pressure.