Pulmonary support on Day 30 as a predictor of morbidity and mortality in congenital diaphragmatic hernia

Abstract

Purpose Congenital diaphragmatic hernia (CDH) is associated with significant in-hospital mortality, morbidity and length-of-stay (LOS). We hypothesized that the degree of pulmonary support on hospital day-30 may predict in-hospital mortality, LOS, and discharge oxygen needs and could be useful for risk prediction and counseling. Methods 862 patients in the CDH Study Group registry with a LOS≥30 days were analyzed (2007–2010). Pulmonary support was defined as (1) room-air (n=320) (2) noninvasive supplementation (n=244) (3) mechanical ventilation (n=279) and (4) extracorporeal membrane oxygenation (ECMO, n=19). Cox Proportional hazards and logistic regression models were used to determine the case-mix adjusted association of oxygen requirements on day-30 with mortality and oxygen requirements at discharge. Results On multivariate analysis, use of ventilator (HR 5.1, p=.003) or ECMO (HR 19.6, p<.001) were significant predictors of in-patient mortality. Need for non-invasive supplementation or ventilator on day-30 was associated with a respective 22-fold (p<.001) and 43-fold (p<.001) increased odds of oxygen use at discharge compared to those on room-air. Conclusions Pulmonary support on Day-30 is a strong predictor of length of stay, oxygen requirements at discharge and in-patient mortality and may be used as a simple prognostic indicator for family counseling, discharge planning, and identification of high-risk infants.

Pulmonary support on day of life 30 is a strong predictor of increased 1 and 5-year morbidity in survivors of congenital diaphragmatic hernia

“…(27) Our previous analysis of the CDHSG registry found that pulmonary support (PS) on DOL-30 is the greatest predictor of inpatient mortality, length-of-stay and short-term pulmonary morbidity. (10) In the present study we found that PS on DOL-30 is also a strong independent predictor of longer-term outcomes, including pulmonary and developmental morbidity at both 1 and 5 years of age. Similar to our previous analysis, we again noted that PS on DOL-30 is a good summary indicator for the severity of pulmonary disease and highly correlated with most other predictors of outcome.…”

“…Similar to our previous analysis, we again noted that PS on DOL-30 is a good summary indicator for the severity of pulmonary disease and highly correlated with most other predictors of outcome. (9, 10) We found that PS on DOL-30 was a stronger independent predictor of long-term morbidities than either defect size or presence of a V/Q mismatch. Taken together with the previous analyses of the CDHSG registry, we believe that the degree of pulmonary support on DOL-30 may be used as a simple cross-institutional tool to identify patients with a higher risk of long-term morbidity.…”

“…(9) A recent analysis of the CDHSG registry found that the degree of pulmonary support on DOL-30 (PS on DOL-30) is a measure that is both simple to use and the strongest independent predictor of both subsequent mortality and pulmonary morbidity prior to discharge. (10)…”

Vasopressin Improves Hemodynamic Status in Infants with Congenital Diaphragmatic Hernia

“…These agents are often ineffective making extracorporeal membrane oxygenation therapy (ECMO) the only therapeutic option to stabilize hemodynamics. Published studies from the CDH registry report a 27-35% (8,9) ECMO utilization rate, making alternate therapies that may be more effective in the setting of refractory hypotension essential. Two recent case reports describe the efficacy of terlipressin, an arginine vasopressin analogue, in the setting of hemodynamic instability in CDH (7,10).…”

Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline

“…Long-term follow-up of CDH in Canada, however, appears more variable and may be influenced by centre volume, with larger centres having interdisciplinary follow-up clinics and smaller centres providing decentralized surveillance through local pediatric surgeons and subspecialty pediatricians. 78 In addition to regular surveillance, we recommend intensified screening for patients identified as high risk, which includes those requiring pulmonary support at 30 days of age, 79 who need patch repair 80 and who need extracorporeal life support. 81 These CDH survivors should be offered screening (e.g., growth, hearing), including standardized neurodevelopmental testing.…”