Quantitative assessment of pulmonary vascular resistance and reactivity in children with pulmonary hypertension due to congenital heart disease using a noninvasive method: new Doppler-derived indexes.

We assessed the usefulness of transthoracic Doppler-derived indexes obtained in the proximal pulmonary artery (PA) branch for estimating pulmonary vascular resistance (PVR) in 45 children with congenital heart disease (CHD) and 23 normal control subjects. The acceleration time, inflection time (InT), deceleration index, and peak velocity, which were measured from the systolic PA flow velocity curve obtained at the sites of the main PA, and right and left PA, were compared with the PVR in patients with CHD. In addition, changes in either Doppler-derived indexes or PVR during 100% oxygen administration were compared in 22 patients showing a baseline PVR >or=4.6 U/m(2) (high PVR). The heart-rate-corrected InT (InTc) values obtained in the left PA in the high PVR group were significantly lower than those in the main PA (4.7 +/- 1.5 vs. 7.5 +/- 3.0; p < 0.001). The InTc obtained from the left PA separated patients with high and low PVR (4.7 +/- 1.4 vs. 9.9 +/- 2.4; p < 0.001) and no significant differences in InTc were found between the low PVR and the control groups. An increase in InTc to >6 during 100% oxygen administration for the high PVR group indicated good PA reactivity with a sensitivity of 93%, specificity of 100%, and agreement of 95% (kappa = 0.83). Moreover, this InTc index correlated inversely with PVR (r = -0.80). In conclusion, our method can noninvasively separate high and low PVR and assess the PA reactivity for high PVR in children with CHD.

Transthoracic Doppler assessment of coronary flow velocity reserve in children with Kawasaki disease: comparison with coronary angiography and thallium-201 imaging.

OBJECTIVES: The purpose of this study was to determine the feasibility of coronary flow velocity reserve (CFVR) measurement by transthoracic Doppler echocardiography (TTDE) in children with Kawasaki disease (KD). BACKGROUND: Doppler-derived CFVR is a reliable marker predicting the presence of myocardial ischemia. METHODS: We studied 49 patients (median age 11 years) with KD. The CFVR was calculated as the ratio of hyperemic to basal peak (peak CFVR) and mean (mean CFVR) diastolic flow velocities in the posterior descending coronary artery (PD) and left anterior descending coronary artery (LAD). The CFVR measurements by TTDE were compared with the results of coronary angiography, thallium-201 (Tl-201) single-photon emission computed tomography (SPECT), and intracoronary Doppler study. RESULTS: The CFVR measurements by TTDE were obtained in 92 (94%) of 98 vessels of the PD and LAD in 49 study patients. Both peak and mean CFVRs for 21 stenotic vessels were significantly smaller than those for 35 normal vessels and for 20 vessels with aneurysmal lesions (p < 0.0001). Peak and mean CFVR <2.0 predicted significant coronary stenosis, as determined by coronary angiography, with sensitivities and specificities of 89% and 96% and 89% and 97%, respectively. Also, both peak and mean CFVRs were correlated with reversible perfusion defects on Tl-201 SPECT (agreement 80%; kappa 0.4). The correlation between peak and mean CFVRs determined by the TTDE and intracoronary Doppler studies in 36 vessels of 23 patients were 0.76 and 0.80, respectively. CONCLUSIONS: The CFVR measured by TTDE predicts the presence of significant coronary stenosis of either the right coronary artery or LAD, as well as myocardial ischemia of these territories in children with KD.