APSA paper
The congenital diaphragmatic hernia composite prognostic index correlates with survival in left-sided congenital diaphragmatic hernia

https://doi.org/10.1016/j.jpedsurg.2011.10.020Get rights and content

Abstract

Purpose

We developed the congenital diaphragmatic hernia congenital prognostic index (CDH-CPI) to incorporate all known prognostic variables into a single composite index to improve prognostic accuracy. The purpose of this study is to examine the ability of the CDH-CPI to predict survival in patients with left-sided congenital diaphragmatic hernia and to determine if the index has a stronger correlation with survival than each of the individual components.

Methods

A retrospective review of patients with left-sided congenital diaphragmatic hernia between 2004 and 2010 was conducted. Ten prenatal parameters of the CDH-CPI were collected, total score was tabulated, and patients stratified according to total score and survival.

Results

Sixty-four patients with a prenatal diagnosis of left-sided congenital diaphragmatic hernia were identified. Patients with a CDH-CPI score of 8 or higher had a significantly higher survival than patients with a CDH-CPI score of lower than 8. The CDH-CPI has the strongest correlation with survival compared with the individual parameters measured. The CDH-CPI correlates with extracorporeal membrane oxygenation use, and 75% of patients with a score of 5 or lower were placed on extracorporeal membrane oxygenation.

Conclusions

The CDH-CPI accurately stratifies survival in left-sided congenital diaphragmatic hernia. The amalgamation of 10 prenatal parameters of the CDH-CPI may be a better prenatal predictor than any single prognostic variable currently used.

Section snippets

Patient data

A retrospective review of medical records for all patients with left-sided CDH evaluated prenatally at the Fetal Care Center (FCC) of Cincinnati and treated postnatally at Cincinnati Children's Hospital Medical Center between 2004 and 2010 was conducted. This study was approved by the institutional review board (IRB no. 2008-0894). Patients generally had 2 visits to the FCC with imaging performed at both visits. A fetal magnetic resonance imaging (MRI), ultrasound, and echocardiogram are

Study population

Of a total of 73 cases of CDH evaluated prenatally at the FCC, we identified 64 patients with a prenatal diagnosis of left-sided CDH and postnatal treatment at our institution. The average gestational age of patients seen during the first visit is 24 5/6 weeks (range, 19 1/7-30 2/7 weeks), and the average gestational age of patients seen at the second visit is 34 1/7 weeks (range, 31-37 5/7 weeks). The overall survival of this population is 65.6% (42/64). Table 1 contains the data of the 10

Discussion

The results of this single institution retrospective study suggest that the CDH-CPI may provide useful information for the counseling of parents with a fetus that has been diagnosed with an isolated left-sided CDH. There is a positive correlation between CDH-CPI score and survival. Patients with a score of 8 or higher have excellent outcomes with survival approaching 90%, whereas patients with scores lower than 8 had significantly decreased survival. A primary end point of this study was to

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