Influence of pulmonary hypertension on outcome of Egyptian patients with congenital diaphragmatic hernia : an experience in low-resource settings

Abstract EN

Congenital Diaphragmatic Hernia (CDH) is a rare congenital anomaly that includes pulmonary hypoplasia and pulmonary hypertension and associated with high mortality and morbidity.

Pulmonary hypertension is considered the main cause of mortality in patients with CDH.

Recently, many new management modalities mainly directed to control pulmonary hypertension have been introduced which led to improved survival.

These modalities are not available in countries with low resource settings as in Africa and Asia.

This work detected the effect of pulmonary hypertension on the outcome of patients with CDH in absence of such new management modalities.

The study included retrospective analysis of 50 patients with CDH referred to the echocardiography unit, Cairo University Children‘s Hos-pital from the Neonatal Surgical Unit (September 2010 to September 2011).

60 % of patients were male with a median age 6.5 days.

(41) 82 % had CDH repair at a median of 4 days from admission, 26 (63.4 %) needed postoperative inotropic support.

The median hospital stay was 8 days.

There was 50 % mortality rate, 18 % of them without surgical intervention.

The mean pre-operative PAP was significantly higher than postoperatively (p value .000).

Non survival patients had significantly higher mean PAP both pre and post operatively, more inotropic support, more delayed surgery, and lower age at admission compared to survival.

Patients were classificed regarding severity of PH, those with severe PH had significantly younger age, more delayed surgery, needed more inotropic support postoperatively and with highest mortality compared to the mild / moderate PH ones.

There was a significant negative correlation between preoperative PAP and age at admission and positive correlation with both, day of surgery and postoperative PAP.

Post-operative PAP had positive correlation with day of surgery and hospital stay.

There was an associa-tion between both pre and postoperative PAP and use of inotropes.

Logistic re-gression analysis showed that postoperative PAP is a significant predictor for morta-lity P value .001, OR 1.164, 9.50 C.I.

for OR 1.068 : 1.270.