Respiratory Severity Score as a Predictive Factor for the Mortality of Congenital Diaphragmatic Hernia

다국어 초록 (Multilingual Abstract)

Purpose: Congenital diaphragmatic hernia (CDH) is rare but potentially fatal. The overall outcome is highly variable. This study aimed to identify a simple and dynamic parameter that helps predict the mortality of CDH patients in real time, without invasive tests.
Methods: We conducted a retrospective chart review of 59 CDH cases. Maternal and fetal information included the gestational age at diagnosis, site of defect, presence of liver herniation, and lung-to-head ratio (LHR) at 20 to 29 weeks of gestational age. Information regarding postnatal treatment, including the number of days until surgery, the need for inhaled nitric oxide (iNO), the need for extracorporeal membrane oxygenation (ECMO), and survival, was collected. The highest respiratory severity score (RSS) within 24 hours after birth was also calculated.
Results: Statistical analysis showed that a younger gestational age at the initial diagnosis (P<0.001), a lower LHR (P=0.001), and the presence of liver herniation (P= 0.003) were prenatal risk factors for CDH mortality. The RSS and use of iNO and ECMO were significant factors affecting survival. In the multivariate analysis, the only remaining significant risk factor was the highest preoperative RSS within 24 hours after birth (P=0.002). The area under the receiver operating characteristic curve was 0.9375, with a sensitivity of 91.67% and specificity of 83.87% at the RSS cut-off value of 5.2. The positive and negative predictive values were 82.14% and 92.86%, respectively.
Conclusion: Using the RSS as a prognostic predictor with simple calculations will help clinicians plan CDH management.

참고문헌 (Reference)

1 Green C, "Umbilical arterial and venous catheters: placement, use, and complications" 17 : 23-28, 1998

2 Snoek KG, "Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: The CDH EURO Consortium Consensus 2015 Update" 110 : 66-74, 2016

3 McHoney M, "Role of ECMO in congenital diaphragmatic hernia" 103 : F178-F181, 2018

4 Akinkuotu AC, "Risk-stratification of severity for infants with CDH: prenatal versus postnatal predictors of outcome" 51 : 44-48, 2016

5 van den Hout L, "Risk factors for chronic lung disease and mortality in newborns with congenital diaphragmatic hernia" 98 : 370-380, 2010

6 Malkar MB, "Respiratory severity score on day of life 30 is predictive of mortality and the length of mechanical ventilation in premature infants with protracted ventilation" 50 : 363-369, 2015

7 Mhanna MJ, "Respiratory severity score and extubation readiness in very low birth weight infants" 58 : 523-528, 2017

8 Katz LA, "Repeat surfactant therapy for postsurfactant slump" 26 : 414-422, 2006

9 Kim SY, "Pulmonary arterial hypertension after ibuprofen treatment for patent ductus arteriosus in very low birth weight infants" 179 : 49-53, 2016

10 Kamata S, "Prolonged preoperative stabilization using high-frequency oscillatory ventilation does not improve the outcome in neonates with congenital diaphragmatic hernia" 13 : 542-546, 1998

11 Lusk LA, "Persistence of pulmonary hypertension by echocardiography predicts short-term outcomes in congenital diaphragmatic hernia" 166 : 251-256, 2015

12 Abman SH, "Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society" 132 : 2037-2099, 2015

13 Barriere F, "One-year outcome for congenital diaphragmatic hernia: results from the French National Register" 193 : 204-210, 2018

14 Iyer NP, "Non-invasively derived respiratory severity score and oxygenation index in ventilated newborn infants" 48 : 364-369, 2013

15 Del Vecchio A, "Neonatal transfusion practice: when do neonates need red blood cells or platelets?" 33 : 1079-1084, 2016

16 Baird R, "Mortality prediction in congenital diaphragmatic hernia" 43 : 783-787, 2008

17 Ijsselstijn H, "Long-term outcome of children treated with neonatal extracorporeal membrane oxygenation: increasing problems with increasing age" 38 : 114-121, 2014

18 Campbell BT, "Inhaled nitric oxide use in neonates with congenital diaphragmatic hernia" 134 : e420-e426, 2014

19 Ballard RA, "Inhaled nitric oxide in preterm infants undergoing mechanical ventilation" 355 : 343-353, 2006

20 The Neonatal Inhaled Nitric Oxide Study Group (NINOS), "Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia" 99 : 838-845, 1997

21 Inamura N, "Extracorporeal membrane oxygenation for congenital diaphragmatic hernia in Japan" 57 : 682-686, 2015

22 DiBlasi RM, "Evidence-based clinical practice guideline: inhaled nitric oxide for neonates with acute hypoxic respiratory failure" 55 : 1717-1745, 2010

23 Putnam LR, "Evaluation of variability in inhaled nitric oxide use and pulmonary hypertension in patients with congenital diaphragmatic hernia" 170 : 1188-1194, 2016

24 Grushka JR, "Effect of hospital case volume on outcome in congenital diaphragmatic hernia: the experience of the Canadian Pediatric Surgery Network" 44 : 873-876, 2009

25 Merrill JD, "Dysfunction of pulmonary surfactant in chronically ventilated premature infants" 56 : 918-926, 2004

26 Weems MF, "Congenital diaphragmatic hernia: maximizing survival" 17 : e705-e718, 2016

27 Keller RL, "Congenital diaphragmatic hernia: endothelin-1, pulmonary hypertension, and disease severity" 182 : 555-561, 2010

28 Schultz CM, "Congenital diaphragmatic hernia: a simplified postnatal predictor of outcome" 42 : 510-516, 2007

29 Glenski JA, "Calculation of mean airway pressure during mechanical ventilation in neonates" 12 : 642-644, 1984

30 Huning BM, "Blood sampling via umbilical vein catheters decreases cerebral oxygenation and blood volume in preterm infants" 96 : 1617-1621, 2007

31 Fletcher K, "An overview of medical ECMO for neonates" 42 : 68-79, 2018

32 Hollinger LE, "A risk-stratified analysis of delayed congenital diaphragmatic hernia repair: does timing of operation matter?" 156 : 475-482, 2014

33 Brindle ME, "A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns" 134 : e413-e419, 2014