Haemodynamic features at presentation in persistent pulmonary hypertension of the newborn and outcome

Thirty four newborns presenting with persistent hypoxaemia in the first three days of life underwent detailed haemodynamic assessment using Doppler echocardiography, including measurements of pulmonary arterial pressure (PAP), left ventricular (LV) function, and left ventricular output (LVO). Results were compared with values from 51 healthy babies, and those of survivors were compared with non-survivors. Four of the 34 babies were excluded from this analysis because one was found to have transposed great arteries, one had a large left-to-right shunt with no evidence of persistent pulmonary hypertension, and two had diffuse skeletal myopathy. Tricuspid regurgitation was present in 70%, permitting systolic PAP estimation. The pulmonary:systemic arterial pressure ratio range was 0.7:1 to 1.83:1 (mean 1.02:1). A patent duct was present in 83%, and flow patterns indicated PAP approaching, or above, systemic pressure in all. Systolic time interval ratio TPV/RVET (time to peak velocity at the pulmonary valve/right ventricular ejection time) was mostly (65%) in the normal range, and did not correlate with other PAP measurements. LV function was below the 10th centile in only 11%, but values for LVO lay below the 10th centile in 41%, and for left ventricular stroke volume index (LSVI) in 66%. Results of 18 survivors were compared with 10 non-survivors (excluding two premature babies who died early with pulmonary interstitial emphysema). There were no significant differences for any parameter of PAP or LV function, but LVO and LSVI were significantly lower in non-survivors: LVO survivors (mean (SD)), 205 (57), non-survivors 138 (63) ml/kg/minute (P<0.01); LSVI survivors, 1.29 (0.51), non-survivors 0.86 (0.31) ml/kg (P<0.05). All four babies with LVO <100 ml/kg/minute died, and 6/7 babies with LSVI <1 ml/kg died. Detailed echocardiographic evaluation shows that the haemodynamic features of persistent pulmonary hypertension are diverse and that clinical diagnosis can be incorrect. Low LV output and stroke volume, usually with normal LV function, were the only Doppler echocardiographic parameters to predict subsequent death. This correlation with outcome requires further prospective evaluation.