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A two-dimensional echocardiographic study was performed in patients (pts) with isolated ventricular septal defect (VSD). Group I included 48 pts with membranous VSD (aged from 3 months to 11 years) diagnosed by cardiac catheterization and angiocardiography. In 25 of 27 pts demonstrated a defect only by two-dimensional echocardiography (2DE), mean pulmonary artery pressure (MPAP) was higher than 25 mmHg and 26 of these 27 pts showed a large left to right shunt of more than 30 per cent, and the remaining one pt had Eisenmenger complex. In 15 of 16 pts demonstrated VSD with the pouch formation of the septal leaflet (PSL) by 2DE, MPAP was lower than 25 mmHg. These 16 pts showed various left to right shunt ratios (small to large). In all 4 pts demonstrated VSD with membranous septal aneurysm (MSA) and 1 pt demonstrated no findings by 2DE, MPAP was low (under 25 mmHg) and a left to right shunt was small. Group II included 400 pts with membranous VSD (aged 11 days to 15 years) diagnosed clinically. All 221 pts demonstrated VSD had PSL or MSA. PSL was demonstrated in 261 pts, MSA in 27 pts, both PSL and MSA in 33 pts and no findings in 79 pts by 2DE. Group III included 41 pts with spontaneous closed VSD (aged 3 months to 8 years) diagnosed clinically. PSL was demonstrated in 15 pts, MSA in 11 pts and no findings in 15 pts by 2DE. On the basis of these observation, PSL and MSA may play an important role in spontaneous diminution or closure of membranous VSD, and in most of VSD pts without PSL and MSA pulmonary hypertension is shown and spontaneous diminution of VSD size can not be anticipated. Visualization of PSL and MSA by 2DE may predict the prognosis and is clinically very useful in the management of pts with isolated membranous VSD.

The cross-sectional echocardiographic demonstration of the "goose-neck" deformity is described in four patients with endocardial cushion defect. The diagnosis was confirmed in each patient by left ventricular angiocardiogram. The subxiphoid approach of cross-sectional echocardiography in diastole allowed visualization of an elongated, narrowed, and somewhat horizontally inclined configuration of the left ventricular outflow tract, which appeared almost identical to that obtained by angiocardiography. In systole, the right border of the left ventricle was composed of the cleft anterior mitral leaflet, the left-sided line of which was convex toward the left ventricular cavity. The mitral valvular echoes were thickened, jagged and irregular, which seemed to correspond to the scalloped appearance of the right border of the left ventricular silhouette in a systolic phase of the left ventricular angiocardiogram. There were no obvious differences between the goose-neck configuration of complete-type endocardial cushion defect and that of ostium primum atrial septal defect.

Our previous study provided a new 2-dimensional echocardiographic technique to detect peripheral right coronary artery aneurysms in Kawasaki disease, with use of the subcostal approach. An additional study was performed to detect peripheral left coronary artery aneurysms. Because the left anterior descending artery runs along the anterior interventricular sulcus and the left circumflex artery along the mitral valve ring, these regions were searched for coronary aneurysms by use of the subcostal imaging approach. Among 143 patients with Kawasaki disease, 44 left coronary aneurysms were visualized in 22 patients. Three aneurysms at the origin of the obtuse marginal artery and 1 in the further peripheral site of the left circumflex artery were observed in 3 patients. Two aneurysms at the origin of the second diagonal branch of the peripheral left anterior descending artery were detected. These echocardiographic studies were done prospectively, and their features coincided well in size, shape, and anatomic location with confirmatory angiographic appearances.

The cross-sectional echocardiographic technique for detecting coronary artery aneurysms of mucocutaneous lymph node syndrome (MLNS) was examined. When cutting the aortic root in round slices by the echocardiographic scanner, each of the right and left coronary arteries was imaged as a linear echo-free structure arising from the aortic root. Of 22 normal subjects, the left main coronary artery was imaged in all, the anterior descending branch in 18, the circumflex branch in six, and the right coronary artery in 18. Of 41 patients with MLNS, coronary artery aneurysms were detected as large echo-free spaces in four patients who were proved later to have these lesions by coronary angiography or autopsy. In addition, in four patients, whose echocardiograms demonstrated the coronary arteries to be normal, the angiographic studies showed that the coronary arteries were intact. These results suggest that the cross-sectional echocardiographic technique is a useful noninvasive diagnostic method for imaging the coronary arteries and the aneurysms in MLNS.

Twenty-seven patients with ventricular septal defect (VSD), ages 2 months to 2 years, were evaluated by both M-mode and two-dimensional echocardiography (2-D echo) with the aid of a peripheral venous contrast technique. M-mode echocardiography was unreliable for imaging VSD. Using 2-D echo, defects in the interventricular septum were visualized in all patients examined; defects in the membranous portion (24 patients) were visualized by the subcostal frontal approach and defects in the supracristal portion (three patients) by the subcostal sagittal approach. The 2-D echo contrast study revealed negative contrast jets appearing through the defect in the right ventricle in 22 patients with a pulmonary-to-systemic blood flow ratio greater than 2.0, and positive contrast jets appearing through the defect in the left ventricle in 13 patients with a right-to-left ventricular peak pressure ratio greater than 0.71. We conclude that 2-D echo with peripheral contrast technique confirmed the anatomic location of VSD and indicated the approximate right-to-left ventricular peak pressure ratio, showing the contrast material shunting through the defect itself.

The clinical validity and some problems concerning pulsed Doppler echocardiography (PD) in non-invasive estimates of pressure difference (delta P) across a ventricular septal defect were studied. The maximum velocity (max V) of the left to right shunt flow in the right ventricle was converted to delta P using the simplified Bernoulli equation: delta P = 4V2. We also used the equation: delta P = 4(V2(2) - V1(2)) to estimate the delta P in cases who had left to right shunt flows of high velocity in the left ventricle. Simulatenous recordings of both left and right ventricular pressures and PD were obtained during cardiac catheterization of 11 cases. Accurate Doppler estimates of delta P only from the maximum velocity of the left to right shunt flow in the right ventricle were impossible in nine cases whose actual delta P's were large (more than 41 mmHg) and also in eight cases whose right ventricular systolic pressure was high (either equal to or higher than left ventricular systolic pressure). Besides these 17 cases, delta P estimated by PD using the simplified Bernoulli equation in 39 cases, with pansystolic left to right shunt flows in the right ventricle, correlated well with the actually measured delta P (Y = 0.99X + 2.77, r = 0.91, p less than 0.01). The difference in the maximal instantaneous pressure gradient and Doppler delta P was considered insignificant (between 0 and 7 mmHg, mean 4 mmHg). In nine cases, the left to right shunt flows of relatively high speed (0.63 approximately 2.00 m/sec, mean 1.31 m/sec) were observed also in the left ventricle, and calculated delta P using the simplified Bernoulli equation overestimated the actually measured delta P by 2 to 16 mmHg (Y = 1.52X + 4.88, r = 0.95, p less than 0.01). However, if the delta P is estimated by using the equation, delta P = 4(V2(2) - V1(2)), without ignoring the maximum speed in the left ventricle (V1), it correlates well with the actually measured delta P (Y = 1.07X + 0.76, r = 0.98, p less than 0.01). Thus, in cases with left to right shunt flows with high speeds in the left ventricle, the equation: delta P = 4(V2(2) - V1(2)) was more accurate in estimating the delta P by pulsed Doppler echocardiography.

Our previous study provided a new two-dimensional echocardiographic technique for detecting peripheral "right" coronary artery aneurysms in Kawasaki disease using a subcostal approach. In the present paper, we presented an additional study for detecting peripheral "left" coronary artery aneurysms. Because the left anterior descending artery runs along the anterior interventricular sulcus and the left circumflex artery around the mitral valve ring, both coronary regions are difficult to search by a conventional method. Then coronary aneurysms of these regions were searched using the subcostal approach in this study. First, the sector beam was directed toward the heart on a plane parallel to a line between the patient's shoulders which allowed simultaneous visualization of four chambers (P1). The area around the mitral valve ring in this plane was examined for coronary aneurysms of the peripheral left circumflex artery. Secondary, the beam section was positioned parallel to the line cutting both the long axes of the sternum and spinal column which allowed visualization of the right ventricular outflow tract, pulmonary valves, interventricular septum, mitral valves and left atrium (P2). Thirdly, the sector plane was angled to the left (P3), and still more to the left (P4) until the left atrium just disappeared. By scanning from P2 to P4, the area around the mitral valve ring was examined for coronary aneurysms of the peripheral left circumflex artery. By scanning from P4 to the cardiac apex (P5), the anterior interventricular sulcus was searched for coronary aneurysms of the peripheral left anterior descending artery. Of 143 patients with Kawasaki disease, aged two months to 8 years, 7 peripheral left coronary aneurysms were visualized in five patients. These were three aneurysms in the left circumflex artery at the origin of the obtuse marginal artery, two aneurysms in segment 13, and two aneurysms in the left anterior descending artery at the origin of the second diagonal branch. These echocardiographic features coincided well in size, shape and anatomic location with angiographic appearances. In one patient whose echocardiogram in sector P2 showed a small echo-free space (2 X 2 mm) at the area around the mitral valve ring, the angiographic study showed the intact left circumflex artery, suggesting that there was a limitation to distinguish a very small coronary aneurysm from a normal one. There was no false negative diagnosis. These results showed that this new echocardiographic technique is useful for detecting peripheral left coronary aneurysms in patients with Kawasaki disease.

A new two-dimensional echocardiographic technique was developed to detect peripheral right coronary aneurysms in Kawasaki disease. Because the main stem of the right coronary artery runs along the tricuspid valve ring and the posterior interventricular groove, these regions were examined for coronary aneurysms using the subcostal approach. Of 52 patients with Kawasaki disease, 14 right coronary aneurysms were visualized in eight patients. Most of the coronary aneurysms were elicited as circular or oval echo-free spaces on the right side of the right ventricle or at the area around the tricuspid valve ring. These echocardiographic features coincided well in size, shape and anatomic position with angiographic appearances. The subcostal approach could not image the normal coronary artery. In three patients whose echocardiograms showed no abnormal echo-free space, the angiographic studies proved that the right coronary arteries were intact. These results suggest that this echocardiographic technique is useful for detecting peripheral right coronary aneurysms in patients with Kawasaki disease.