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We studied the change in pulmonary arterial diameter under prostaglandin E1 (PGE1) therapy in infants with ductus-dependent cyanotic heart disease (pulmonary atresia). Ten infants undergoing administration of PGE1 for more than 2 weeks were selected for this study. A classic Blalock–Taussig (BT) shunt was performed in seven patients and a modified BT shunt in three. The shunt was successful in all patients. The internal diameters of the right and left pulmonary arteries were measured before and after the start of PGE1 therapy using two-dimensional echocardiography. The pulmonary arterial index (PA index) was calculated to correct the diameters for body surface area. Both pulmonary arteries enlarged during the first week of PGE1 therapy in all 10 patients. Further increases in the two diameters were observed even after the first week of treatment in nine patients. Both the mean diameters at 2 weeks after the start of PGE1 were about 50% larger than the initial diameters (right; increased from 3.1 to 4.7 mm; left; increased from 3.0 to 4.4 mm). Of the eight patients given PGE1 for more than 3 weeks, four showed no changes in pulmonary arterial diameters after the first 2 weeks and the remaining four showed a slight increase. PA indexes also showed a rapid increase during the first 2 weeks and no significant change thereafter. We suggest that, in infants with pulmonary atresia and small pulmonary arteries, the optimal pulmonary artery size for BT shunt insertion is achieved after 2 weeks of PGE1 infusion, with no further significant increase in size being observed after this time. [ABSTRACT FROM AUTHOR]

The objective of this study was to test the hypothesis that accelerated endothelial dysfunction and the development of premature atherosclerosis are associated with age in subjects with coronary artery lesions after Kawasaki disease (KD). A case-control study was performed at a university hospital that included 35 post-KD subjects across a wide age range (range, 8-42 years) without traditional cardiovascular risk factors and 35 age- and sex-matched healthy control subjects (Cont). Flow-mediated dilatation (FMD) of the brachial artery-induced by reactive hyperemia, intima media thickness (IMT), and elastic modulus (Ep) of the common carotid artery were compared between KD and Cont subjects assessed against age. KD subjects had slightly higher levels of body mass index, lipid profile, and HbA1c than Cont subjects, but the differences were not significant. The mean IMT (p < 0.001), age-adjusted percentage normal IMT (%N IMT; p < 0.0001), and Ep (p < 0.001) were significantly higher in KD than Cont subjects, and the peak FMD% (p < 0.01) was significantly lower in KD than Cont subjects. There were significant correlations between FMD% and age (r = -0.51 p < 0.0001), IMT and age (r = 0.68, p < 0.001), and Ep and age (r = 0.58, p < 0.01) in KD but not Cont subjects. When the difference in FMD% between KD and matched Cont subjects (DeltaFMD%) was plotted against age, no significant relationship was found, although significant correlations between DeltaIMT and age (r = 0.52, p < 0.01) as well as between DeltaEp and age (r = 0.46, p < 0.05) were observed. When we defined values that were +2.0 SD over the mean control values (i.e., %N IMT >or= 120% and/or Ep >or= 50 kPa) as markers of subclinical atherosclerosis, 15 subjects met the criteria. Subjects over the age of 22 years were more likely to have (OR = 16.54, p = 0.0001) subclinical atherosclerosis in this cohort. Our results suggest that endothelial dysfunction and the development of premature atherosclerosis were accelerated in adult post-KD compared to Cont subjects.

The aim was to compare the right ventricular (RV) Tei index obtained by the tissue Doppler imaging (TDI) method with that obtained by the pulsed Doppler method in 29 fetuses aged 24-39 weeks (29.9 +/- 4.0 weeks). From pulsed Doppler recordings, the tricuspid closing-to-opening time (a) and RV ejection time (b) were measured. The Tei index determined by the pulsed Doppler method was calculated as (a - b)/b. From TDI recordings, the time interval during diastole (a') and the duration of the systole S-wave (b') were measured. The modified Tei index obtained by TDI was calculated as (a' - b')/b'. The time a' correlated strongly with a (r = 0.90, p < 0.0001). The mean difference between a' and a was 0.3 +/- 5.0 ms. There was also a strong correlation between b' and b (r = 0.94, p < 0.0001). The mean difference between b' and b was 0.5 +/- 3.3 ms. The TDI-Tei index correlated with the pulsed Doppler-Tei index (r = 0.83, p < 0.0001). The mean difference between the TDI-Tei index and the pulsed Doppler-Tei index was -0.003 +/- 0.04. This study demonstrated that the TDI-Tei index correlates well with the Tei index determined by pulsed Doppler in fetuses, suggesting that the TDI-Tei index is a feasible approach to assess global RV function in fetuses.

B-type natriuretic peptide (BNP) reflects volume overload on left ventricle and pulmonary hypertension (PH) in patients with ventricular septal defect (VSD). Pulmonary vascular resistance (PVR) has been reported to correlate positively with BNP in VSD patients with various degrees of PH. We aimed to investigate the relationship between PVR and BNP in VSD patients with severe PH. We examined 24 subjects with VSD concomitant severe PH aged from 2 months to 17 years (median: 4 months). The ratio of pulmonary to systemic pressure (Pp/Ps), the ratio of pulmonary to systemic flow (Qp/Qs), the ratio of pulmonary to systemic resistance (Rp/Rs), and PVR were determined by cardiac catheterization. PVR and Rp/Rs ranged from 1.6 to 15.5 (mean: 5.7 +/- 3.9) Wood unit . m(2) and 0.1 to 0.8 (mean: 0.4 +/- 0.2), respectively. BNP ranged from 5.5 to 69 (mean: 31 +/- 19) pg/ml. Negative correlations were observed between BNP and PVR (r = -0.56, p = 0.004) and BNP and Rp/Rs (r = -0.51, p = 0.01). BNP was significantly lower (<10 pg/ml) in VSD patients with Eisenmenger physiology as compared with the others (p = 0.003). We should draw attention to evaluate BNP values in VSD patients with severe PH.

Transthoracic Doppler echocardiography offers a noninvasive approach for imaging posterior descending coronary artery (PD) running in the posterior longitudinal sulcus along the middle cardiac vein (MCV). To evaluate whether the MCV flow velocity reserve can reflect the PD flow reserve, 22 children with various heart diseases were examined using transthoracic Doppler echocardiography. Introduction of a modified transthoracic two chamber view with the transducer rotated counterclockwise and angulated posteriorly allows visualization of the MCV and PD. Peak systolic flow velocity and average peak systolic flow velocity in the MCV and peak diastolic flow velocity and average peak diastolic flow velocity in the PD were measured at rest and hyperemic conditions (intravenous administration of adenosine of 0.16 mg/kg/min). Coronary flow reserve was defined as the ratio of peak hyperemic to basal average peak flow velocity. ATP infusion induced significant increases in the peak systolic flow velocity and average peak systolic flow velocity in the MCV. The mean MCV flow velocity reserve in the patients was 1.94 +/- 0.44. Significant increases in the peak diastolic flow velocity and the average peak diastolic flow velocity in the PD were also observed during ATP infusion, and the mean PD flow velocity reserve (2.19 +/- 0.62) was significantly higher than the GCV flow velocity reserve (p < 0.0001). There was a good correlation between the MCV flow velocity reserve and PD flow velocity reserve (r = 0.86, p < 0.0001). This study demonstrated that it was possible to measure the MCV flow velocity and MCV flow velocity reserve in pediatric patients by transthoracic Doppler echocardiography. The MCV flow reserve correlated highly with the PD flow reserve. However, the degree of the MCV flow during hyperemia was less than that of the PD flow. This underestimation should be considered when the reactive hyperemic response is evaluated from the MCV flow velocity.

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) are known to be associated with cardiomyopathy. Systolic and diastolic left ventricular functions were assessed by M-mode and Doppler echocardiography in four patients with MELAS and in 14 normal controls. The interventricular septal thickness and left ventricular posterior wall thickness were greater (11.0 +/- 1.6 mm vs. 5.8 +/- 0.7 mm and 11.0 +/- 2.2 mm vs. 5.9 +/- 0.8 mm) in patients with MELAS than in a control group. Parameters of systolic left ventricular functions (ejection fraction, shortening fraction, systolic time intervals, and mean Vcf) and left ventricular dimensions were not significantly different between the two groups. To assess the diastolic function, blood flow velocity across the mitral valve was measured by Doppler echocardiography and various indexes were obtained. In patients with MELAS, the impairment of diastolic left ventricular filling was demonstrated by decrease in the following indexes: peak flow velocity in the early passive filling period (E) (0.76 +/- 0.10 m/s vs. 0.94 +/- 0.09 m/s), integrated velocity for total E (10.2 +/- 1.3 vs. 13.0 +/- 0.9), the ratio of E and late atrial filling integrated velocities (1.72 +/- 0.06 vs. 2.49 +/- 0.29).

A 4-month-old male infant with acute febrile mucocutaneous lymph-node syndrome developed mitral regurgitation, pericardial effusion and multiple pulsating masses in the upper and lower extremities. Two-dimensional echocardiography demonstrated aneurysms in both coronary arteries. Arteriography of the right arm demonstrated multiple aneurysms. Seventeen months later, arteriography and two-dimensional echocardiography demonstrated regression of the aneurysms. This case provides evidence that regression of aneurysms can occur in mucocutaneous lymph node syndrome.