Association of Severity of Coronary Artery Aneurysms in Patients With Kawasaki Disease and Risk of Later Coronary Events.

Importance: Few studies with sufficient statistical power have shown the association of the z score of the coronary arterial internal diameter with coronary events (CE) in patients with Kawasaki disease (KD) with coronary artery aneurysms (CAA). Objective: To clarify the association of the z score with time-dependent CE occurrence in patients with KD with CAA. Design, Setting, and Participants: This multicenter, collaborative retrospective cohort study of 44 participating institutions included 1006 patients with KD younger than 19 years who received a coronary angiography between 1992 and 2011. Main Outcomes and Measures: The time-dependent occurrence of CE, including thrombosis, stenosis, obstruction, acute ischemic events, and coronary interventions, was analyzed for small (z score, <5), medium (z score, ≥5 to <10; actual internal diameter, <8 mm), and large (z score, ≥10 or ≥8 mm) CAA by the Kaplan-Meier method. The Cox proportional hazard regression model was used to identify risk factors for CE after adjusting for age, sex, size, morphology, number of CAA, resistance to initial intravenous immunoglobulin (IVIG) therapy, and antithrombotic medications. Results: Of 1006 patients, 714 (71%) were male, 341 (34%) received a diagnosis before age 1 year, 501 (50%) received a diagnosis between age 1 and 5 years, and 157 (16%) received a diagnosis at age 5 years or older. The 10-year event-free survival rate for CE was 100%, 94%, and 52% in men (P < .001) and 100%, 100%, and 75% in women (P < .001) for small, medium, and large CAA, respectively. The CE-free rate was 100%, 96%, and 79% in patients who were not resistant to IVIG therapy (P < .001) and 100%, 96%, and 51% in patients who were resistant to IVIG therapy (P < .001), respectively. Cox regression analysis revealed that large CAA (hazard ratio, 8.9; 95% CI, 5.1-15.4), male sex (hazard ratio, 2.8; 95% CI, 1.7-4.8), and resistance to IVIG therapy (hazard ratio, 2.2; 95% CI, 1.4-3.6) were significantly associated with CE. Conclusions and Relevance: Classification using the internal diameter z score is useful for assessing the severity of CAA in relation to the time-dependent occurrence of CE and associated factors in patients with KD. Careful management of CE is necessary for all patients with KD with CAA, especially men and IVIG-resistant patients with a large CAA.

Coil occlusion of PDA in patients younger than 1 year: risk factors for adverse events.

BACKGROUND: Transcatheter occlusion of infantile patent ductus arteriosus (PDA) challenges the interventionist. PURPOSE: To analyze the risk factors for adverse events from this procedure in patients younger than 12 months. SUBJECTS: We retrospectively analyzed data on 32 patients younger than 12 months in whom transcatheter coil occlusion of a PDA was attempted. Ages ranged from 1 to 11 (median, 7) months and body weight from 1.2 to 10 (6.0) kg. The minimum ductal diameter ranged from 1.0 to 4.6 (3.3) mm and pulmonary to systemic flow ratio from 0.7 to 12.5 (2.2). Major adverse events were defined as those requiring surgery, while minor adverse events included transient hemolysis not needing treatment, coil migration with successful transcatheter retrieval, and mild left pulmonary artery (PA) stenosis. We determined whether any factors such as age, body weight, minimum PDA diameter, angiographic type, operator, and use of a 0.052-in. Gianturco coil related to the occurrence of adverse events. RESULTS: In two patients, coils could not be deployed in the appropriate position. They underwent surgery following transcatheter retrieval of coils. Coils were successfully deployed in the other 30 patients, however, one patient developed persistent hemolysis that required surgical retrieval of the coils and closure. PDA was completely closed in the other 29 patients (clinical success rate, 91%). Thus, there were three major adverse events, while minor adverse events occurred in five patients. Univariate analysis did not identify any single factor that contributed to either major, minor, or total adverse events. However, all major adverse events occurred in patients under 6 months and less than 6 kg body weight with a minimum duct diameter of more than 3.5 mm. CONCLUSION: Transcatheter coil occlusion of PDA is feasible in selected patients younger than 1 year. A minimum diameter more than 3.5 mm in patients under 6 kg may be a risk factor for major adverse events.

Development of a reexpandable covered stent for children.

The use of covered stents in children is limited by the need for a large long sheath for delivery and the impossibility of redilation once implanted. The authors developed a reexpandable covered stent implantable in children through a small sheath and evaluated its clinical feasibility in mini piglets. An original Palmaz stent was covered with a polyurethane membrane that could be stretched up to 700%. Under general anesthesia, the authors implanted the covered stents in six mini piglets using a long sheath with a diameter approximately 1 French larger than the recommended size required to deliver an uncovered Palmaz stent. The implantation technique was similar to conventional stent implantation. In six piglets, the stent could be redilated from 7.5 to 8.7 mm 28-70 days after implantation. Macroscopic and microscopic examination showed intimal coverage of the coating with minimal inflammatory reaction around the stent. Our newly designed reexpandable stent covered with a polyurethane membrane is promising for use in children.

Coil occlusion of patent ductus arteriosus.

BACKGROUND: Coils are the only devices available for transcatheter occlusion of patent ductus arteriosus (PDA) in Japan. Since April 1999, we have introduced a 0.052-inch Gianturco coil (0.052-inch coil) to close PDA >or=2.5 mm. METHODS AND RESULTS: A retrospective survey of the outcome of coil occlusions for PDA >or=2.5 mm before and after the 0.052-inch coil became available found that (1) the frequency of PDA >or=2.5 mm among all candidates for coil occlusion significantly increased after the availability of a 0.052-inch coil (p<0.01); (2) deployment complicated by migration (p<0.01), and prolonged procedure time (p<0.05) were significantly decreased after the introduction of the 0.052-inch coil. In a multivariate logistic regression model for uneventful deployment adjusted for age, pulmonary to systemic flow ratio, and use of a 0.052-inch coil, use of the 0.052-inch coil significantly decreased eventful deployment (p<0.05); and (3) successful deployment of a coil for PDA >or=4 mm significantly increased with the 0.052-inch coil (p<0.01). Complete occlusion was achieved once deployment was successful. CONCLUSION: Introduction of the 0.052-inch coil decreased complicated coil occlusion deployment for PDA >or=2.5 mm, and contributed to a better likelihood of coil occlusion for PDA >or=4 mm.

Coil occlusion for patent ductus arteriosus larger than 3 mm.

BACKGROUND: Coil occlusion of patent ductus arteriosus (PDA) is now widely accepted as the first-line treatment, but there are few reports of age-dependent differences in the complications associated with this technique. METHODS AND RESULTS: Sixteen patients (11 adults, 5 children) with a PDA larger than 3 mm, who underwent coil occlusion at Sapporo Medical University Hospital between September 1995 and August 2004, were enrolled. Immediate and intermediate outcomes and complications were analyzed. Procedural success rate was 72.7% (8/11) in the children and 100% (5/5) in the adults. Coil migration occurred in 4 children and 1 adult, and 3 adult patients had hemolysis. CONCLUSION: Hemolysis was more frequent in adults than in children even though the residual shunt was trivial.

Late distortion of the original Palmaz stent implanted in postoperative lesions associated with congenital heart disease.

The objective of this study was to report late distortion of a Palmaz stent. Late distortion of an original Palmaz stent, implanted in an extracardiac lesion, is rare. We completed a 1-year follow-up of 54 patients who had been implanted with 80 Palmaz stents in extracardiac lesions. Distortion of two stents was detected in two patients. For case 1, we implanted a P188 stent for supravalvar pulmonary stenosis complicating an arterial switch operation in a 14-year-old girl. Seven months later, we found compression of the stent. Although we implanted two P308 stents anterior to the distorted stent, distortion of both stents developed after 1 month. Two more P308 stents placed inside each stent were gradually recompressed. A CAT scan showed compression of the stent by a dilated sinus of valsalva. For case 2, we implanted a P308 stent for stenosis of the superior vena cava after Williams operation in an 11-year-old boy. A chest X-ray documented longitudinal compression of the stent 27 months after implantation and a CAT scan showed the ascending aorta was in contact with the stent. A Palmaz stent may be distorted when implanted in a lesion adjacent to a pulsating aorta.

The throat flora and its mitogenic activity in patients with Kawasaki disease.

The etiology of Kawasaki disease (KD) remains unknown, although some infectious organism has been suggested as the cause. Recent studies suggest that some bacterial toxins with superantigen activity are involved in its pathogenesis, but no specific bacterial toxin has yet been identified. Throat swabs for bacterial culture were obtained from 21 patients with KD and 20 with other febrile illnesses as controls. Mitogenic activity in culture supernatants obtained from individual bacterial strains was measured by lymphocyte proliferation assay. Sixty-one bacterial strains were isolated from KD patients, and 62 strains from control patients. There was no apparent difference in bacterial species in the throat flora between KD patients and febrile controls. Moreover, total and individual mitogenic activity of strains from KD patients was no greater than that of strains from febrile controls. The bacterial superantigen activity of throat flora may not play a major role in the pathogenesis of KD.

Pulse wave velocity and ankle brachial index in patients with Kawasaki disease.

BACKGROUND: Pulse wave velocity (PWV) and ankle brachial index (ABI) are a useful method to estimate atherosclerosis in adults, while a history of Kawasaki disease (KD) may be a risk factor for later atherosclerosis of systemic arteries. PWV and ABI in patients with a history of KD have not been reported. METHOD: In total, 90 patients ranging in age from 4 to 25 years who had a history of KD previously, any time from 1 month to 19 years ago, were studied. As a control group, 119 patients with other trivial cardiovascular diseases such as a small ventricular, an atrial septal defect or mild arrhythmias, were also evaluated. A high value of PWV was determined as >/=120% of normal predicted value of PWV, while a low value of ABI was determined as /= 120%, and ABI