Significance of pulmonary artery size and blood flow as a predictor of outcome in congenital diaphragmatic hernia.

Aim: To determine if pulmonary artery size and blood flow have prognostic value in congenital diaphragmatic hernia (CDH).

Methods: Twenty-eight consecutive left-sided CDH patients treated according to a standard protocol with high frequency oscillatory ventilation (HFOV) + nitric oxide (NO) had right and left pulmonary artery (RPA, LPA) diameters, LPA/RPA diameter (L/R) ratios, and PA blood flows examined by echocardiography (EC) on days 0, 2, and 5 after birth and compared prospectively.

Results: Twenty-two patients (78.6%) survived. Of these, 15 required NO (NO-s), and seven did not (non-NO-s). All six patients that died required NO (NO-d). RPA in the NO-d group was significantly smaller than in the NO-s or non-NO-s groups on day 0 (2.90 +/- 0.41 vs. 3.40 +/- 0.49 or 4.01 +/- 0.43; P < 0.01, respectively). LPA in the NO-d group was significantly smaller than in the non-NO-s on day 0 (2.13 +/- 0.45 vs. 3.39 +/- 0.34; P < 0.01). L/R ratios in NO subjects were significantly smaller (NO-s 0.74 +/- 0.11; NO-d 0.73 +/- 0.11) than in non-NO-s subjects (0.84 +/- 0.03) on day 0 (P < 0.01). PA diameters and L/R ratios did not change significantly from day 0 to day 5 in all three groups. There was LPA flow on day 0 in all non-NO-s subjects, but none in all NO subjects. In the NO-s group, LPA flow was confirmed in 87% (13/15) on day 2 and in 100% on day 5, however, there was no LPA flow from day 0 to day 5 in any of the NO-d group.

Conclusion: Our data indicate that PA diameters on day 0 and LPA flow are strongly prognostic in left-sided CDH and L/R ratio would appear to be a simple highly reliable indicator of the necessity for NO therapy.