Reassessment of carotid intima-media thickness by standard deviation score in children and adolescents after Kawasaki disease.

Previous studies that used carotid ultrasound have been largely conflicting in regards to whether or not patients after Kawasaki disease (KD) have a greater carotid intima-media thickness (CIMT) than controls. To test the hypothesis that there are significant differences between the values of CIMT expressed as absolute values and standard deviation scores (SDS) in children and adolescents after KD and controls, we reviewed 12 published articles regarding CIMT on KD patients and controls. The mean ± SD of absolute CIMT (mm) in the KD patients and controls obtained from each article was transformed to SDS (CIMT-SDS) using age-specific reference values established by Jourdan et al. (J: n = 247) and our own data (N: n = 175), and the results among these 12 articles were compared between the two groups and the references for comparison of racial disparities. There were no significant differences in mean absolute CIMT and mean CIMT-SDS for J between KD patients and controls (0.46 ± 0.06 mm vs. 0.44 ± 0.04 mm, p = 0.133, and 1.80 ± 0.84 vs. 1.25 ± 0.12, p = 0.159, respectively). However, there were significant differences in mean CIMT-SDS for N between KD patients and controls (0.60 ± 0.71 vs. 0.01 ± 0.65, p = 0.042). When we assessed the nine articles on Asian subjects, the difference of CIMT-SDS between the two groups was invariably significant only for N (p = 0.015). Compared with the reference values, CIMT-SDS of controls was within the normal range at a rate of 41.6 % for J and 91.6 % for N. These results indicate that age- and race-specific reference values for CIMT are mandatory for performing accurate assessment of the vascular status in healthy children and adolescents, particularly in those after KD considered at increased long-term cardiovascular risk.

[Cardiac scintigraphy-Pharmacological stress and appropriate management of pediatric radiopharmaceutical administration in patients after Kawasaki disease].

Cardiac scintigraphy accounted for 2.5 % of all 3,884 patients based on a survey ques- tionnaire of pediatric nuclear medicine examinations performed at 14 Japanese institutes in 2011. Myocardial perfusion imaging, classified as cardiac scintigraphy, is essential to detect myocardial ischemia in patients after Kawasaki disease (KD), although its less frequent performance is reported. Adenosine is widely noticed as a medication for pharma- cological stress testing in coronary arterial lesions after KD. We describe characteristics of adenosine including newly-devised administration protocol and pathway making of intravenous injection for stress testing. We comment on optimal radiopharmaceutical administered doses proposed in Japanese consensus guidelines for pediatric nuclear medi- cine. Their proposal doses approximate that calculated by Pediatric Dosage Card of Euro- pean Association of Nuclear Medicine. We hope myocardial perfusion imaging is performed under appropriate management of pediatric radiopharmaceutical administration.

Japanese consensus guidelines for pediatric nuclear medicine. Part 1: Pediatric radiopharmaceutical administered doses (JSNM pediatric dosage card). Part 2: Technical considerations for pediatric nuclear medicine imaging procedures.

The Japanese Society of Nuclear Medicine has recently published the consensus guidelines for pediatric nuclear medicine. This article is the English version of the guidelines. Part 1 proposes the dose optimization in pediatric nuclear medicine studies. Part 2 comprehensively discusses imaging techniques for the appropriate conduct of pediatric nuclear medicine procedures, considering the characteristics of imaging in children.

Long-term prognostic impact of dobutamine stress echocardiography in patients with Kawasaki disease and coronary artery lesions: a 15-year follow-up study.

OBJECTIVES: This study sought to determine the prognostic value of dobutamine stress echocardiography (DSE) over a 15-year follow-up for predicting cardiac events in adolescent Kawasaki disease (KD) patients with coronary artery lesions (CALs). BACKGROUND: Although DSE is an established technique for the detection of coronary artery disease, its prognostic value to predict cardiac events in adolescent KD patients with CALs is unknown. METHODS: Fifty-eight adolescent KD patients, including 36 patients with CALs documented by coronary angiography, and 22 patients with normal coronary arteries documented by echocardiography who underwent DSE were reviewed at initial testing (mean age: 13.6 years) and at 15 years' follow-up. Follow-up events were tabulated as major adverse cardiac events (MACEs) that included cardiac death, nonfatal myocardial infarction, and revascularization. RESULTS: During a mean follow-up of 14.7 years, there were 16 patients with MACEs (acute myocardial infarction: n = 1; old myocardial infarction: n = 7; coronary artery bypass grafting: n = 4; percutaneous coronary intervention: n = 4). Significant coronary artery disease (CAD) (>70% coronary stenosis) was detected in 31.0% of patients at initial testing and 42.1% at follow-up. However, there were no significant differences in wall motion score indices (WMSI) at peak DSE between initial testing and follow-up (p = 0.762). Five of 6 patients (85%) with false-positive DSE results (WMSI: ≥1.25) at initial testing, who had giant aneurysms without CAD, developed CAD with MACEs during follow-up. Cumulative event-free survival rate to 15 years was 25.0% in patients with WMSI ≥1.25 and 91.7% in patients with WMSI <1.25. Cox regression analysis showed the grade of peak WMSI at initial testing to be the only independent predictor of MACEs (relative risk: 3.28; 95% confidence interval: 1.73 to 6.20). CONCLUSIONS: DSE provided independent prognostic information up to 15 years in adolescent KD survivors.

[Survey questionnaire of pediatric nuclear medicine examinations in 14 Japanese institutes].

Under the auspices of the Japanese Society of Pediatric Nuclear Medicine, an annual aggregate from a 5-year period, 2007 to 2011, of a survey questionnaire of pediatric nuclear medicine examinations performed at 14 institutes in the Kanto region was conducted. The subjects were pediatric patients 15 years old or younger. The survey questions included the determination method for administered dose of radiopharmaceuticals, the items examined and number of examinations. Of 14 institutes, 11 determined administered doses using the formula: adult dose X (age +1) / (age+7), while the remaining 3 used the adult dose as the maximum dose and used a conversion formula based on age and physical condition. In 2011, in a total of 3,884 pediatric patients, renoscintigraphy accounted for 41.5%, brain 14.4%, pulmonary scintigraphy 12.9%, oncology 9.0%, hepatobiliary scintigraphy 6.3%, gastrointestinal scintigraphy 4.8%, musculoskeletal scintigraphy 4.3%, cardiology 2.5%, and other 4.9% of all nuclear medicine examinations. Pediatric nuclear medicine examinations in general hospitals accounted for only 3.4% of all examinations. A similar trend was observed in previous years. Since pediatric patients have a longer reproductive term and higher sensitivity to radiation exposure, pediatric nuclear medicine requires a strict selection of appropriate studies and administered dose. These results show the current practice and would warrant further consideration.

Characteristics of earlier atherosclerotic involvement in adolescent patients with Kawasaki disease and coronary artery lesions: significance of gray scale median on B-mode ultrasound.

OBJECTIVE: To test the hypothesis that textural changes in the carotid intima-media complex (IMC) detected by B-mode ultrasound are associated with the difference of remodeling process in earlier atherosclerotic involvement in patients with Kawasaki disease (KD) and coronary artery lesions (CALs). METHODS: Eighteen patients with KD and CALs (mean age 17.2 years), 17 patients with heterozygous familial hypercholesterolemia (FH) (mean age 16.9 years) and 15 age-matched healthy controls (Cont) were assessed and compared for carotid intima-media thickness (CIMT), elastic property (E(p)), and first- and second-order statistics. RESULTS: KD showed significantly higher gray scale median (GSM) than FH and Cont. KD and FH showed significantly higher CIMT, entropy and lower angular second moment than Cont, but no significant difference was found between KD and FH. CONCLUSION: Higher GSM in KD may indicate alteration of tissue components and heterogeneity of IMC, suggesting the development of arteriosclerotic vascular remodeling after vasculitis. This is distinct from that of atherosclerosis with lower GSM often observed in FH.

Easy fabrication of a new type of mouthguard incorporating a hard insert and space and offering improved shock absorption ability.

The positive effects of wearing a mouthguard have been indicated in various epidemiological surveys and experiments, and their usage appears to be increasing in many sports. However, many preventable sports-related dental injuries still occur even with the use of a conventional mouthguard. We have developed a mouthguard (the Hard & Space mouthguard) with sufficient injury prevention ability (more than 95% shock absorption ability against impact with a steel ball carrying 15.2 kg m(2) S(-2) potential energy) and ease of clinical application. This mouthguard consists of an outer and an inner EVA layer and a middle layer of acrylic resin (hard insert), with a space to prevent contact between the inner surface of the mouthguard and the buccal surfaces of the maxillary front teeth or teeth already weakened through prior damage or treatment. The purpose of this article is to describe the method by which the Hard & Space mouthguard may easily be fabricated. We believe that this new type of mouthguard has the potential to reduce sports-related dental injuries.

Changes in the textural characteristics of intima-media complex in young patients with familial hypercholesterolemia: implication for visual inspection on B-mode ultrasound.

BACKGROUND: To test the hypothesis that textural changes in the carotid intima-media complex (IMC) on visual inspection by B-mode ultrasound are associated with early atherosclerotic involvement in patients with heterozygous familial hypercholesterolemia (FH). METHODS: 55 patients (mean age 13.4 years) were categorized into three groups according to the degree of thickness in IMC (intima-media thickness [IMT]) (groups I-III) and 15 healthy controls within the same age range as the patients were assessed for first- and second-order statistics and visual scoring of textural changes in IMC (1, normal; 2, proximal interface disruption; 3, granulation). RESULTS: There was no significant difference in first-order statistics among the four groups. As for second-order statistics, groups II (moderately increased IMT) and III (markedly increased IMT) had significantly higher entropy and lower angular second moment than group I (normal IMT) and control. Likewise, groups II and III received significantly higher visual scoring than group I. Visual scoring correlated with entropy (r = 0.57) and angular second moment (r = -0.50). Multiple regression analysis identified entropy (beta = 0.52) and visual scoring (beta = 0.42) as significant determinants of IMT. CONCLUSIONS: These findings demonstrate that higher visual scoring may indicate dishomogeneity of IMC, suggesting early medial infiltration. This seems to be a simple visual marker to more effectively identify high-risk young patients with FH.

Electrophysiological characteristics of idiopathic ventricular tachycardia in children.

BACKGROUND: Idiopathic ventricular tachycardia (VT) has been reported to have a good prognosis, but there still might be the potential risk of sudden death. METHODS AND RESULTS: The 46 consecutive children (mean age 11.7 ± 3.4 years) with idiopathic VT were enrolled in this study. Monomorphic VT was detected in 39 patients and polymorphic VT in 7 patients. The VT originated from the right ventricle (RV) in 22 patients, and left ventricle (LV) in 17 patients. The VT was induced by exercise in 68% of the RVVT, 41% of the LVVT, and 100% of the polymorphic VT. The VT was induced by programmed ventricular stimulation in 41% of the RVVT, 35% of the LVVT, and none of the polymorphic VT. Adenosine tri-phosphate terminated the VT in 9 of 15 patients (60%). The mechanism of the VT was suspected to be triggered by activity in 36.4%, automaticity in 40.9%, and re-entry in 22.7% of the RVVT, whereas it was 52.9%, 5.9%, and 41.2% of the LVVT, respectively. CONCLUSIONS: The exercise inducibility was higher in polymorphic VT than the RVVT and LVVT, but no difference in the programmed stimulation. The sensitivity to adenosine tri-phosphate was not different between the RVVT and LVVT. In some patients with idiopathic VT, a non-verapamil sensitive re-entry was documented, which was more common in patients with ischemic heart disease or cardiomyopathy.

Quantitative evaluation of coronary artery wall echogenicity by integrated backscatter analysis in Kawasaki disease.

BACKGROUND: Coronary artery wall echogenicity increases on echocardiograms during the acute phase of Kawasaki disease (KD). According to this background, echogenicity of the coronary artery wall in patients with KD is quantified by using integrated backscatter (IB) analysis. METHODS: IB analysis is a quantitative method for evaluating echogenicity. We examined the value of IB in the wall of the left anterior descending coronary artery and compared it with that in adjacent intracardiac blood as a measure of background. The difference between these values is represented as corrected IB for the coronary artery wall. RESULTS: Corrected IB for the coronary artery wall was higher in patients with KD than in controls (KD with pre-immunoglobulin therapy vs. controls: 27.4 +/- 5.3 dB vs. 22.0 +/- 3.5 dB, P < .05) and in patients with coronary enlargement after intravenous immunoglobulin (with vs. without coronary enlargement, 29.2 +/- 5.2 dB vs. 24.1 +/- 5.5 dB, P < .05). CONCLUSION: The magnitude of IB from the coronary artery wall reflects the effectiveness of immunoglobulin therapy. Furthermore, this method and its value might be useful to predict the occurrence of coronary enlargement in patients with KD.

MDCT of the anomalous origin of the right coronary artery from the left sinus of Valsalva associated with bicuspid aortic valve.

Anomalous origin of the right coronary artery is a relatively rare coronary artery anomaly which is known to cause myocardial ischemia and sudden death. Association of anomalous origin of the right coronary artery with congenital bicuspid aortic valve is even rarer with only a few cases being reported previously. We describe, for the first time, MDCT findings of anomalous origin of the right coronary artery associated with congenital bicuspid aortic valve.

Dual myocardial scintigraphy mismatch in an infant with Bland-White-Garland syndrome.

Bland-White-Garland syndrome (BWG) is a rare disorder that includes abnormalities of the coronary arteries that cause severe myocardial ischemia or infarction in infancy. In this case report, we describe an infant with BWG evaluated by dual single photon emission computed tomography using thallium-201 and I-123 beta-methyl-p-iodophenyl-pentadecanoic acid before and after surgery.

Age-related acceleration of endothelial dysfunction and subclinical atherosclerosis in subjects with coronary artery lesions after Kawasaki disease.

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.

Association of sinus node dysfunction, atrioventricular node conduction abnormality and ventricular arrhythmia in patients with Kawasaki disease and coronary involvement.

BACKGROUND: This study was performed to investigate the incidence of arrhythmias in patients with Kawasaki disease (KD). METHODS AND RESULTS: Electrophysiologic studies (EPS) were performed in 40 patients (mean age: 10.3+/-5.1 years; 30 males, 10 females) with KD who had severe to moderate coronary artery disease. Clinical arrhythmias were documented in 4 patients (premature ventricular contractions, ventricular tachycardia, atrioventricular block, and ventricular fibrillation). Dual atrioventricular nodal pathways were demonstrated in 3 patients. Nonsustained atrial fibrillation was induced in 1 patient. The AH interval was prolonged in 2 patients. The Wenckebach rate was 164+/-37 beats/min, and 4 of the patients had a decreased Wenckebach rate. The maximum and corrected sinus node recovery times were 997+/-257 ms and 281+/-130 ms, respectively, and 7 patients were thought to be abnormal. The sino-atrial conduction time was 108+/-64 ms, and 2 patients had prolonged conduction times. CONCLUSIONS: Although there was no relationship between coronary stenosis or obstruction and the EPS parameters, the incidence of abnormal sinus node and atrioventricular node function is apparently higher in KD patients than in the normal population. These functional abnormalities may possibly be caused by myocarditis or an abnormal microcirculation in the sinus node and atrioventricular node artery. In some patients, myocardial ischemia may provoke malignant ventricular arrhythmia.

Multidetector-row computed tomographic depiction of congenital coronary artery aneurysms and ectasia.

Coronary artery aneurysm or ectasia is an uncommon clinical problem in the pediatric population. When found, it is usually associated with the sequelae of Kawasaki disease. We report a 12-year-old boy with multiple focal coronary artery aneurysms and ectasia without history of Kawasaki disease on multidetector-row computed tomography (MDCT).

Recommendations for weaning off cardiopulmonary support in children with fulminant myocarditis.

BACKGROUND: The purpose of this study was to determine the appropriate recommendations for weaning off cardiopulmonary support (CPS) in children with fulminant myocarditis. METHODS AND RESULTS: Four consecutive patients diagnosed with fulminant myocarditis who were treated with CPS were enrolled (mean age: 9 years). The relationships between timing of weaning from CPS and various factors, including bleeding episodes, platelet count, serum concentration of the MB isoform of creatine kinase (CK-MB), echocardiographic findings, and the mixed venous blood oxygen saturation (SvO2), were retrospectively analyzed. All patients had CPS performed safely for a mean duration of 53.1 h without exchange of the circuit. Three of the 4 patients had a bleeding episode before discontinuation. The minimum platelet count occurred during weaning in all 4 patients. The peak serum CK-MB concentration when initiating CPS was higher than the peak value on the day of weaning. Echocardiographic findings before stopping CPS were similar to those after weaning. The SvO2 was 62.5% when initiating CPS and 71.3% before weaning. CONCLUSIONS: In children with fulminant myocarditis CPS can be used without exchanging the circuit and can be discontinued before bleeding episodes become symptomatic, with improvement in the biomarkers and SvO2 on weaning off CPS.

Association of atrial arrhythmia and sinus node dysfunction in patients with catecholaminergic polymorphic ventricular tachycardia.

BACKGROUND: This study was performed to investigate the frequency and importance of supraventricular arrhythmia and sinus node (SN) dysfunction in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). METHODS AND RESULTS: Eight patients with CPVT (mean age: 16.8+/-8.1 years) underwent an electrophysiological study. SN recovery time (1,389+/-394 ms) was slightly prolonged, and 4 of 8 patients had abnormal values. Atrial flutter (AF) was induced by low-rate atrial pacing in 2 patients and by isoproterenol infusion in 1 patient. Atrial fibrillation (Af) was induced by isoproterenol infusion in 2 patients. One patient presented with Af during the follow-up period, and 2 of 4 patients with AF/Af presented with increased SN recovery time. CONCLUSIONS: Patients with CPVT frequently have associated with SN dysfunction, and inducible atrial tachyarrhythmias, which indicate that the pathogenesis of CPVT is limited not only to the ventricular myocardium, but also to broad regions of the heart, including the SN and atrial muscle.

[Advantages of multislice spiral computed tomography for evaluation of serious coronary complications after Kawasaki disease].

OBJECTIVES: Novel multislice spiral computed tomography (MSCT) findings were identified in patients after Kawasaki disease that could not be detected by coronary angiography (CAG). METHODS: Eighteen patients had suffered from serious coronary arterial lesions after Kawasaki disease (mean age 21.7 years, range 13-34 years). Seventeen patients had stenotic lesions, and all of them had coronary aneurysms. MSCT was performed using a Siemens SOMATOM Volume Zoom (4-detector row) or a Toshiba Aquillion 16 (16-detector row). Findings of coronary calcification, stenotic lesion, and intimal hypertrophy in all coronary arteries were compared to those of CAG. RESULTS: Eleven of the 18 patients (61%) had novel findings detected by MSCT. Coronary calcifications were found in 11 of the 18 patients (61%). Five patients had concentric calcified aneurysms, four had eccentric calcified aneurysms, and two had mixed calcified aneurysms. Coronary stenotic lesions were present in 6 of the 18 patients (33%) with calcified aneurysms. Two patients had intimal hypertrophy (11%). One patient had intimal hypertrophy along the left main trunk with a giant calcified aneurysm along the left anterior descending artery. Two patients had severe stenoses just distal to giant calcified aneurysms that were regarded as false positive findings, and were identified as mild stenoses by CAG. CONCLUSIONS: MSCT offers advantages over CAG in the evaluation of calcified aneurysms and intimal hypertrophy, and is a potential diagnostic modality for coronary intervention in patients after Kawasaki disease.

Usefulness of real-time 3-dimensional echocardiography for the evaluation of coronary artery morphology in patients with Kawasaki disease.

OBJECTIVES: This study was designed to evaluate the usefulness of real-time 3-dimensional echocardiography (RT-3DE) for evaluating coronary artery morphology in patients with Kawasaki disease. BACKGROUND: The diagnosis of coronary artery morphology in the acute phase of the disease is of prime importance for evaluating the likelihood of cardiovascular sequelae. Occasionally, visualization of the right coronary artery and bifurcated regions, including the circumflex artery, has proved challenging with traditional echocardiographic methods. METHODS: A total of 111 patients with Kawasaki disease were studied. Coronary aneurysms were detected in 8 patients (4 had giant aneurysms), and coronary dilation was found in 11 patients. Coronary artery visualization was evaluated and scored as 1 of 4 grades, 0 to 3 points, for both 2-dimensional echocardiography (2DE) and RT-3DE. Evaluation criteria for each coronary artery were defined according to the American Heart Association classification of coronary angiographic features. Total scores for each patient and for individual coronary branches were compared between 2DE and RT-3DE. RESULTS: The total scores for coronary artery visualization were, respectively, showing a significantly higher score for RT-3DE than for 2DE (P < .01). A mural thrombus could be clearly delineated in the giant coronary aneurysms by RT-3DE. CONCLUSIONS: RT-3DE is superior to 2DE for coronary artery visualization. This diagnostic system is expected to improve the screening of coronary artery abnormalities in Kawasaki disease.