Evaluating mortality and disease severity in congenital diaphragmatic hernia using the McGoon and pulmonary artery indices.
OBJECTIVE
Lung hypoplasia is associated with mortality in congenital diaphragmatic hernia (CDH). However, the association between lung hypoplasia and disease severity is unclear. Early prediction of disease severity would provide parents with more precise information about the anticipated course of treatment, minimize treatment disruption, and maximize the efficient management of patients with CDH. We aimed at identifying the relationship between McGoon index (MGI) and pulmonary artery index (PAI) scores and disease severity among infants with CDH.


METHODS
We retrospectively reviewed the medical records of 19 high-risk patients with CDH born between January 2006 and December 2007. McGoon index and PAI scores were determined on admission. We evaluated statistically the relationship between these scores and variables representing severity as follows: number of vasodilators, use of inhaled nitric oxide (iNO), closed method of diaphragm, duration of intubation, duration of hospitalization, and use of home oxygen therapy. Statistical significance was P < .05.


RESULTS
Overall median MGI and PAI scores were 1.40 and 108, respectively; scores for nonsurvivors were significantly (P < .05 and P < .01, respectively) lower than those for survivors. Among survivors, PAI scores were significantly (P < .05) lower in infants requiring iNO than in infants not requiring iNO and patch repair. The PAI scores were significantly correlated with the number of vasodilators (r = -0.789; P < .01) and duration of intubation (r = -0.610; P < .05).


CONCLUSIONS
McGoon index (cutoff value, 1.31) and PAI (cutoff value, 90) are reliable indices for predicting mortality in CDH. Pulmonary artery index appears to be more useful than MGI for predicting disease severity among survivors.
Takahashi
Shigehiro
S
Division of Neonatology, Department of Maternal and Perinatal Medicine, National Center for Child Health and Development, Tokyo 157-8535, Japan. takahashi-s@ncchd.go.jp
Oishi
Yoshihisa
Y
Ito
Naoki
N
Nanba
Yukiko
Y
Tsukamoto
Keiko
K
Nakamura
Tomoo
T
Ito
Yushi
Y
Hayashi
Satoshi
S
Sago
Haruhiko
H
Kuroda
Tatsuo
T
Honna
Toshiro
T
D000280:Administration, Inhalation; D001013:Aorta, Thoracic / Q000000981:diagnostic imaging / Q000473:pathology; D001830:Body Surface Area; D004452:Echocardiography; D006331:Heart Diseases / Q000151:congenital / Q000473:pathology; D006548:Hernia, Diaphragmatic / Q000401:mortality* / Q000601:surgery; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000473:pathology; D007226:Infant Mortality; D007231:Infant, Newborn; D007442:Intubation, Intratracheal / Q000706:statistics & numerical data; D008168:Lung / Q000002:abnormalities / Q000000981:diagnostic imaging; D009569:Nitric Oxide / Q000008:administration & dosage / Q000627:therapeutic use; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000473:pathology*; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D017741:Survivors / Q000706:statistics & numerical data; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use
D014665:Vasodilator Agents; D009569:Nitric Oxide