Noninvasive Assessment of Pulmonary Artery Pressure in Patients with Extracardiac Conduit Total Cavopulmonary Connection

To assess quantitative measurement of mean pulmonary artery pressure (PAP) in extracardiac total cavopulmonary connection (TCPC) patients by noninvasive echocardiographic inferior vena cava collapsibility index (IVC-CI) and also correlation between the peripheral vein pressure and mean PAP. In 19 TCPC patients with at least 1-year follow-up after completion of TCPC, complete echocardiography including IVC-CI was recorded. All patients underwent cardiac catheterization for mean PAP pressure, peripheral vein pressure (PVP) and contrast study. Different cutoff points of mean PAP were analyzed, and based on the highest cutoff point, patients were categorized into two groups: mean PAP < 17 mmHg (acceptable) and > = 17 mmHg (high) for comparison analysis. Between 2006 and 2015, from 43 patients who had undergone TCPC, nineteen patients with a mean age 12.9 ± 6.6 years and mean follow-up time of 3.4 ± 1.9 years were included in this study. Mean PAP was 14.6 ± 3.97 (range 8–22 mmHg). Thirteen (68 %) patients had PAP < 17 mmHg. No significant statistical difference was detected with respect to age, sex, type of congenital heart disease and fenestration between these two groups. But, right ventricular dominancy was more prevalent in the high PAP group (50 % vs. 7.7 % P value 0.03). IVC-CI had a correlation with mean PAP (r 0.67, P < 0.001). IVC-CI < 21.8 % can predict PAP > = 17 mmHg with 83 % sensitivity and 100 % specificity. Regression analysis proposed an equation for PAP measurement: PAP (mmHg) = 20.2097−0.1796 × (IVC-CI), (r2 = 0.56). Peripheral vein pressure measurement also showed a good correlation with mean PAP and may be used to estimate PAP with the following equation: PAP (mmHg) = 0. 8675 × PVP, (r 0.90, P < 0.0001). In conclusion, IVC-CI as noninvasive and peripheral vein pressure measurement as a minimal invasive method may be useful for quantitative estimation of PAP in patients with extracardiac TCPC.