Kei Nishimoto

Application of Percutaneous Transluminal Coronary Angioplasty to Coronary Arterial Stenosis in Kawasaki Disease

BACKGROUND Percutaneous transluminal coronary angioplasty (PTCA) has rarely been performed on patients with coronary lesions that result from Kawasaki disease. In this study, we retrospectively evaluated the effectiveness of PTCA in five patients with coronary arterial stenosis that resulted from Kawasaki disease and reviewed previous reports for possible indicators of PTCA effectiveness. METHODS AND RESULTS Five patients, ranging in age from 2 to 16 years (median 8 years) underwent conventional PTCA for localized stenosis. The lesion targeted for PTCA was located in the middle right coronary artery of three patients and in the left anterior descending artery in two patients. In four of the five patients, PTCA was angiographically effective, with stenosis rates improving from 84 +/- 10% to 33 +/- 11% (P<.05). When the previously reported cases of six similar patients were taken into consideration, the only predictor of successful PTCA seemed to be the time elapsed between the onset of Kawasaki disease and performance of this procedure. CONCLUSIONS In cases in which patients show significant localized stenosis as a result of Kawasaki disease, PTCA should be attempted within 6 to 8 years of the onset of the disease. Additionally, intravascular ultrasound imaging was found to be a useful tool for evaluating internal morphology before and after PTCA. In older patients with coronary calcification, other alternatives to PTCA, such as the use of a rotablator or an atherectomy catheter, should be considered.

Apoptosis as a Possible Cause of Wall Thinning in End-Stage Hypertrophic Cardiomyopathy

A 15-year-old boy with hypertrophic cardiomyopathy died of congestive heart failure with progressive left ventricular wall thinning with poor systolic function. Microscopic examination revealed patchy fibrosis in the ventricular myocardium with wall thinning, and immunohistochemical evaluation of apoptosis showed apoptotic cells and bodies in the destroyed myocytes along the border between the fibrotic area and myofibril.

Dilatation Mechanism of Balloon Angioplasty in Children: Assessment by Angiography and Intravascular Ultrasound

AbstractPurpose: Little information is available about the dilatation mechanism in children. This prospective study aimed to (1) evaluate the dilatation mechanism of balloon angioplasty in children with arterial stenosis, and (2) compare the morphological changes seen by intravascular ultrasound (IVUS) and angiography. Methods: Twenty consecutive patients, who had undergone a total of 23 procedures, were examined before and immediately after balloon angioplasty with a 4.3 Fr, 30 MHz rotational tip IVUS system. The lesions for IVUS study had resulted from coarctation of the aorta in six patients, pulmonary arterial stenosis in five, Blalock-Taussig shunt stenosis in three, subclavian artery stenosis in two, renal artery stenosis in two, coronary artery stenosis in one and ductus arteriosus in one. Results: Four distinctive morphological types were identified: type I with arterial stretching, type IIa with superficial tearing, type IIb with deep intimal-medial tearing, type III with flap formation, and type IV with dissection. The diameter of the narrowest site before and after balloon angioplasty increased significantly from 2.1 ± 1.4 mm to 4.6 ± 3.4 mm (p < 0.001). Eighteen of the 23 angioplasty procedures (78%) were considered to be successful, with a dilatation ratio of more than 50%. In most patients with successful dilatation, non-stretch mechanisms such as tearing, flap formation, or dissection were found. The positive percent (70%) of non-stretch mechanisms seen by IVUS was significantly higher than the positive findings (39%) by angiography (Χ2= 6.47, p < 0.02). Conclusions: Non-stretch morphology of the arterial wall may be a common mechanism of dilatation after balloon angioplasty in children with arterial stenosis. IVUS is a useful modality for evaluating the effectiveness of balloon angioplasty and the mechanism of dilatation in individual cases.

Progressive vascular lesions in Williams-Beuren syndrome

SummaryWe report two patients with Williams-Beuren syndrome. The first patient showed no evidence of coarctation of the aorta at the first examination. Seven years later, she developed coarctation of the aorta. In the second patient, we found the progression of renal artery stenosis by serial angiography. We report that vascular lesions may be progressive in Williams-Beuren syndrome.

Late persistent expressions of ICAM-1 and VCAM-1 on myocardial tissue in children with lymphocytic myocarditis.

BACKGROUND Both intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) have been implicated in cardiac allograft rejection. However, there is little information about the relationship between the expression of these adhesion molecules and myocarditis in children. METHODS AND RESULTS Immunoreactivities of ICAM-1 and VCAM-1 were examined by enzyme immunoassay in 31 biopsy specimens obtained from 11 pediatric patients with biopsy-proven myocarditis or cardiomyopathy. Five of the 11 patients had clear evidence of acute myocarditis. The other 6 had ECG abnormalities identified by mass screening for heart disease, and subsequently had been histologically diagnosed as having non-specific cardiomyopathy. The period between onset of myocarditis or identification of ECG abnormality and immunohistochemical studies was 23 to 60 days and 8 months to 3 years, respectively. Expression of ICAM-1 and VCAM-1 was assessed by counting ICAM-1 and VCAM-1 positive vessels and dividing by the total number of vessels. ICAM-1 was significantly present on 81% (P < 0.01) of myocardial tissue samples in the 5 patients with healing-stage acute myocarditis, and on 45% (P < 0.05) in the remaining 6 patients with non-specific cardiomyopathy, compared with 24% in control specimens obtained from right ventricular muscle resected at surgery for tetralogy of Fallot. VCAM-1 was also present on 50% (P < 0.05) of the samples from the 5 patients with acute myocarditis, but was not present in those with non-specific cardiomyopathy. CONCLUSION This persistent expression of ICAM-1 suggests that myocardial cell damage may persist immunologically for a long period in myocarditis. In addition, immunostaining for these adhesion molecules may be diagnostic value in clinically silent lymphocytic myocarditis and chronic cardiomyopathy.

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.

Corticosteroid therapy for ventricular tachycardia in children with silent lymphocytic myocarditis

OBJECTIVE The objective of our study was to describe the efficacy of corticosteroids for ventricular tachycardia in four children with structurally normal hearts in whom endomyocardial biopsy revealed histologic changes of lymphocytic myocarditis. PATIENTS The four patients had unexplained ventricular tachycardia. Three dysrhythmias were sustained, and one was inducible by exercise. Patient ages ranged from 4 months to 12 years. Three of the four patients had no symptoms. In two of them, ventricular tachycardia was identified by mass screening for heart disease. Two patients received oral steroids and two received pulse steroid therapy. RESULTS In all four patients, significant underlying diseases were not found by noninvasive evaluation. Right ventricular endomyocardial biopsy revealed abnormal histologic findings of chronic lymphocytic myocarditis in all patients. Steroid therapy was effective in all four patients, two of whom received methylprednisolone pulse therapy. CONCLUSIONS We conclude that unexplained ventricular tachycardia may be the only manifestation of clinically silent myocarditis. Steroid therapy should therefore be considered if conventional antiarrhythmic medication is not effective and histologic findings confirm the presence of lymphocytic myocarditis.

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.

Mechanism of balloon angioplasty in children with arterial stenosis assessed by intravascular ultrasound and angiography

Fifteen patients were examined before and immediately after balloon angioplasty with a 4.3F, 30 MHz rotational tip intravascular ultrasound system. In 12 (80%) patients, 13 procedures could be analyzed because of sufficient image quality. The lesions for intravascular ultrasound (IVUS) study consisted of pulmonary arterial stenosis in 4 patients, Blalock-Taussig shunt stenosis in 3, coarctation of the aorta in 2, subclavian artery stenosis in 1, renal artery stenosis in 1, and ductus arteriosus in 1. Three distinctive morphologic types were identified: Group I had arterial stretching (3 patients); group IIa had superficial tearing (3); group IIb had deep intimal-medial tearing (5); group III had dissection (2). In the narrowest site, minimal and maximal luminal diameters, luminal area before and after balloon angioplasty were 3.5 +/- 1.8 mm vs 4.5 +/- 2.5 mm, 4.1 +/- 2.1 mm vs 5.4 +/- 3.5 mm, and 49.8 +/- 30.2 mm vs 88.3 +/- 45.2 mm2, respectively. The recoil value of group IIb with appropriate balloon angioplasty was approximately 0.3. IVUS may be an useful modality for evaluating the morphologic mechanism of dilatation after balloon angioplasty.

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.