Shinjiro Shimazaki

Percutaneous transluminal coronary angioplasty for Kawasaki disease: A case report and literature review

SummaryA 31-month-old boy developed right coronary artery stenosis after Kawasaki disease for which he underwent percutaneous transluminal coronary angioplasty (PTCA). The narrowing of the right coronary artery was successfully dilated by angioplasty without apparent complication. This case suggests that PTCA may have a potential advantage as a temporary method to postpone the aortocoronary bypass surgery in a child with coronary artery stenosis due to Kawasaki disease. However, strict patient selection is recommended for coronary angioplasty.

Clinical features of aortic arch anomaly with malalignment ventricular septal defect.

The clinical features and outcome after various surgical procedures on 9 patients with coarctation or interruption of the aortic arch and malalignment ventricular septal defect (group 1) were compared with those of 9 patients with the arch anomaly without malalignment ventricular septal defect (group 2). Cardiomegaly and metabolic acidosis were prominent in group 1. Five of the 9 patients in group 1 died in the immediate postoperative period (56% mortality), but no operative deaths occurred among 8 patients in group 2 (p less than 0.01). The ratio of left ventricular outflow tract to ascending aortic diameter was 0.59 +/- 0.09 in group 1 and 1.03 +/- 0.11 in group 2 (p less than 0.01). Three of 4 patients with a ratio of less than 0.6 died, but no operative deaths occurred among the 6 patients who had a palliative operation and in whom the ratio was more than 0.6. These data suggest that left ventricular outflow tract obstruction is critical when the ratio of left ventricular outflow tract to ascending aortic diameter is 0.6 or less. The presence of severe left ventricular outflow tract obstruction necessitates modification of the present surgical strategy.

Multicore myopathy associated with multiple pterygium syndrome and hypertrophic cardiomyopathy

A boy with multicore myopathy associated with multiple pterygium syndrome and hypertrophic cardiomyopathy is described. Light microscopy of biopsy samples from the skeletal muscle and myocardium revealed multiple cores in the muscle fibers in the former but their absence in the latter. These results suggest that the pathogenesis of the histologic changes might differ between skeletal muscle and myocardium, and that further electron microscopic examination be done on both types of specimen. The prognosis of multicore myopathy is not usually good when cardiac involvement is present.

Coronary artery calcification in Kawasaki disease.

To evaluate the angiographic features of coronary lesions in Kawasaki disease with coronary artery calcification, cinefluoroscopy and cineangiography were retrospectively reviewed in 116 patients who had undergone coronary angiography between 1982 and 1989. Angiographic abnormalities of coronary arteries were demonstrated in 55 of the 116 patients. In 5 (9.1%) of the 55 patients, 9 with calcification were identified by cinefluoroscopy and chest x-ray. Eight of the 9 calcified lesions showed a circular or ring-shape configuration. Coronary angiography revealed a total occlusion of the right coronary artery with collateral circulation from the distal left coronary artery in 2 patients and a severe stenosis of the right coronary artery in 2 patients, in whom anticoagulant therapy had not been continued during the follow-up periods. The remaining patient in whom anticoagulant therapy had been continued had bilateral aneurysms but no significant stenosis. These results indicate that a ringshape calcification on chest x-ray in a patient with a history or Kawasaki disease may suggest an involvement by coronary artery stenosis even when anticoagulant drugs had been given. Therefore, coronary angiography should be performed to evaluate the stenotic lesions if this type of calcification is found by routine radiographic examination.

Left aortic arch with right descending aorta and right ligamentum arteriosum associated with d-TGA and large VSD: Surgical treatment of a rare form of vascular ring

A 1-month-old boy with respiratory distress underwent pulmonary artery banding after diagnosis of d-loop transposition of the great arteries and ventricular septal defect. Respiratory distress recurred after surgery. Angiography, esophagography, and magnetic resonance imaging showed left aortic arch, retroesophageal aorta, and right descending aorta. Surgery at 9 months of age showed a right-sided ligamentum arteriosum. Division of the ligamentum relieved the constriction of the esophagus and trachea caused by this rare form of vascular ring.

Plasma endothelin concentration: Relation with vascular resistance and comparison before and after balloon dilatation procedures

Endothelin (ET) is a potent vasoconstrictor peptide with an as yet uncertain physiological role in cardiovascular disease. We measured blood plasma ET concentrations using a recently developed radioimmunoassay and analysed the relations between ET concentration, systemic arterial pressure and systemic vascular resistance. In addition, ET levels before and after percutaneous balloon valvuloplasty and angioplasty were measured. Fifty-one patients were studied: (1) 13 patients with small left-to-right shunting or Kawasaki heart disease (age ranged from 4 to 144 months); (2) 10 patients who had undergone ballon valvuloplasty or angioplasty (age ranged from 1 to 233 months) and (3) 28 healthy infants and children (age ranged from 3 to 152 months). Systemic vascular resistance was calculated by the formula (mean aortic pressure — mean right atrial pressure) X 80/cardiac output (dyne·sec·cm−5). Plasma ET concentrations in healthy children less than 2 years were significantly higher than those over 2 years (2.48±0.62 vs 1.31±0.53 pg/ml). In eight patients in groups 1 and 2, plasma ET concentration in the pulmonary artery (2.00±0.43 pg/ml) was significantly lower than that in the femoral vein (2.39±0.69 pg/ml) and aorta (2.23±0.59 pg/ml), suggesting ET secretion derived from endothelial cells in peripheral pulmonary vessels. There was a significant positive correlation between ET concentrations in the femoral vein and systemic vascular resistance (r=0.55,p<0.05). After balloon dilatation ET concentration rose from 2.15±0.82pg/ml to 2.61±1.38 pg/ml. These results suggest that ET may be a stress-induced hormone which is secreted by the transient hypotension following percutaneous balloon dilatation and which regulates peripheral vascular tonus.

Differences in Renal Tubular Toxicity of High- and Low-Osmolality Contrast Media

Kazunari Kaneko, Department of Pediatrics, Juntendo University School of Medicine, 2-1-1, Hongo, Bunkyo-KU, Tokyo 113 (Japan) Dear Sir, We read with great interest the article ‘Nephrotoxicity of Highand Low-Osmolality Contrast Media, [1]. In this article, Jevnikar et al. reported that no significant change in glomerular filtration rate was found in any type of contrast media (CM) so the significance of the enzymuria remained uncertain and, therefore, no evidence that the newer CM were less nephrotoxic than the high-osmolal-ity variety could be found. We have also investigated nephrotoxicity including tubular dysfunction of highland low-osmolality CM. Our results were almost similar to those of Jevnikar et al. In addition to urinary excretion of enzyme as an index of tubular injury, urinary excretion of low molecular weight protein, ß2-microglobulin (ß2-MG), was determined to evaluate tubular function in our study. Our subjects, methods and results were as follows (all results are expressed as mean ± SE). Thirty-five patients admitted to our hospital for the purpose of undergoing cardiac angiography were randomized into two groups receiving iopamidol (800 mosm/kg H20) or diatrizoate (2,070 mosm/kg H20). As shown in table 1, there were no significant differences in age and loaded dose of iodine between the two groups. On both the days before and after cardiac angiography, blood sampling and 24-hour urine collecting were performed. Values for creatinine and electrolytes in blood and urine samples were determined by routine method. Serum and urine concentration of ß2-MG were determined by radioimmunoassay, and urinary N-acetyl-ß-D-glucosaminidase (NAG) activity was determined by a spectrophotometric method [2]. In this study, there were no significant changes in both serum creatinine and creatinine clearance following the administration of any of the CM [data not shown, p > 0.05 Table I. CM description

Balloon occlusion aortography

We review the validity of balloon occlusion aortography (BOA) on the basis of our personal experience with 18 patients with congenital heart disease (mean weight 4.55 g, including 8 neonates). Four of the 18 patients underwent aortic arch angiography using balloon occlusion of the descending aorta. Pulmonary angiography was also performed in 9 patients via a patent ductus arteriosus and in 3 patients via a Blalock-Taussig shunt. The remaining 2 patients underwent coronary arteriography by balloon occlusion of the ascending aorta. The information obtained was satisfactory in 17 of the 18 patients. However, in one patient with a double-outlet right ventricle and pulmonary stenosis, the pulmonary arteries were not clearly visualized because of dominant antegrade flow from the right ventricle. BOA is a safe and useful procedure which can be used to image the aortic arch, pulmonary artery, and coronary arteries in infants with congenital heart diseases. In children over 3 years of age, however, the balloon may not be able to occlude the appropriate site of the aorta, so selective angiography is required to obtain precise information.