Le et al reported the use of the Congenital Diaphragmatic Hernia Composite Prognostic Index (CDH-CPI) for patients with a left-sided congenital diaphragmatic hernia. This can help to identify a patient who may require more aggressive management such as ECMO. The authors are from Cincinnati Children’s Hospital and Children’s Hospital Colorado.
Patient selection: left-sided diaphragmatic hernia
Parameters:
(1) karyotype
(2) signs of a hereditary syndrome
(3) congenital heart disease
(4) left ventricular to right ventricular ratio (LV/RV)
(5) modified McGoon index on MRI
(6) hernia sac on ultrasonography
(7) liver location on MRI
(8) lung-to-heart ratio in early gestation
(9) percentage of observed to predicted lung volume (PPLV) in late gestation
(10) total lung volume in late gestation
|
Parameter |
Finding |
Points |
|
karyotype |
normal |
1 |
|
|
abnormal |
0 |
|
signs of hereditary syndrome |
absent |
1 |
|
|
present |
0 |
|
congenital heart disease |
none |
1 |
|
|
VSD, ASD or coarctation |
0 |
|
|
double outlet heart disease |
-1 |
|
LV to RV ratio |
normal |
1 |
|
|
disproportionate |
0 |
|
McGoon index |
>= 1.2 |
1 |
|
|
< 1.2 |
0 |
|
hernia sac |
yes |
1 |
|
|
no |
0 |
|
liver |
down (in abdomen) |
1 |
|
|
up (in chest) |
0 |
|
lung to heart ratio |
>= 1 |
1 |
|
|
< 1 |
0 |
|
PPLV |
>= 15% |
1 |
|
|
< 15% |
0 |
|
total lung volume |
>= 18 mL |
1 |
|
|
< 18 mL |
0 |
total index =
= SUM(points for all 10 parameters)
Interpretation:
• minimum index: -1
• maximum index: 10
• The higher the index the better the outcome.
• An index < 8 was associated with worse survival.
• An index <= 5 was associated with extracorporeal membrane oxygenation (ECMO).
Specialty: Pulmonology, Pedatrics
