Hidetoshi Hayakawa

Elevated Levels of High-Sensitivity C-Reactive Protein and Serum Amyloid-A Late After Kawasaki Disease: Association Between Inflammation and Late Coronary Sequelae in Kawasaki Disease

Background—Coronary sequelae that persist after Kawasaki disease (KD) have been associated with obstructive changes of the lesions and coronary vascular events in adolescents and young adults. However, little is known about the association between sequelae late after KD and inflammatory markers, which are potential mediators and markers for atherogenesis. Methods and Results—Cross-sectional study was performed to test the hypothesis that coronary sequelae are associated with elevated levels of inflammatory markers in patients late after KD (mean time interval after the onset, 10 years, 10 months). Levels of high-sensitivity C-reactive protein (CRP), serum amyloid-A (SAA), interleukin-6, and soluble intercellular adhesion molecule-1 were measured in the 4 groups (n=80): the referent group (n=15) and KD subgroups with normal coronary arteries from the onset (n=27); with regressed aneurysms (n=18); and with coronary artery lesions, such as persistent aneurysms, stenosis, and occlusion (n=20). CRP levels were significantly elevated in a KD subgroup with coronary artery lesions compared with the referent or other KD subgroups, as analyzed by ANOVA and ANCOVA after adjustment for a confounding factor body mass index. Levels of CRP, SAA, and interleukin-6 were positively correlated. Stepwise regression and logistic regression analyses support the association between the persistence of coronary artery lesions and the levels of CRP and SAA. Conclusions—Results demonstrate that the persistence of coronary lesions late after KD was independently associated with levels of CRP and SAA, suggesting that inflammation may be a novel functional aspect of coronary artery diseases late after KD.

In Vivo Plaque Composition and Morphology in Coronary Artery Lesions in Adolescents and Young Adults Long After Kawasaki Disease. A Virtual Histology–Intravascular Ultrasound Study

Background— Coronary artery lesions (CALs) late after Kawasaki disease were characterized by endothelial dysfunction and low-grade inflammation, surrogate markers for atherosclerosis. We tested the hypothesis that CALs in patients long after Kawasaki disease are accompanied by atheroma-like features, as assessed by virtual histology–intravascular ultrasound, a new method to assess coronary plaque composition and morphology in vivo. Methods and Results— Virtual histology–intravascular ultrasound was performed in 13 Japanese Kawasaki disease patients (median age, 18.3 years; interquartile range, 16.9 to 23.3 years) an interval after Kawasaki disease (median, 15.9 years; interquartile range, 14.3 to 21.9 years). We investigated 6 sites with localized stenosis, 15 sites with an aneurysm, 29 sites with a regressed aneurysm, and 50 sites with a normal coronary segment. Plaque components were categorized into 4 parts: fibrous, fibrofatty, necrotic core, and dense calcium areas. Qualitatively, the normal segment had no or trivial intravascular ultrasound–visible plaque area, whereas the CAL exhibited a heterogeneous plaque area with the 4 components in different amounts and proportions. Quantitatively, a combined group of CALs had a higher absolute value of fibrous, dense calcium, and necrotic core areas than the normal segment. In further analyses of 3 subtypes of CALs, localized stenosis, an advanced lesion, exhibited higher absolute and relative values of dense calcium and necrotic core areas and a lower relative value of the fibrous area than regressed and persistent aneurysms. Conclusion— The present limited but initial virtual histology–intravascular ultrasound findings give new insight into the potential role of atherogenesis in the evolution of CALs in adolescents and young adults long after Kawasaki disease and therefore warrant further investigation.

Strategy for pulmonary atresia and intact ventricular septum

Pulmonary atresia with an intact ventricular septum is characterized by varying degrees of right ventricular cavity hypoplasia. This factor is critical in determining the most appropriate surgical approach for each patient. We describe a patient who underwent definitive biventricular surgical repair in early infancy. We used an atrial septal defect patch with a one-way valve and performed a right ventricular overhaul after a balloon valvotomy.

Dynamic Right Ventricular Outflow Tract Obstruction Due to a Swinging Echolucent Cyst on Interventricular Septum in a 4-Year-Old Girl

A 4-year-old girl was referred to our hospital for evaluation of an incidentally found heart murmur. She had a history of perimembranous ventricular septal defect in infancy, which was reported to be spontaneously closed sonographically in a nearby hospital at 1 year of age. She had no history of traumatic injury. On examination, a grade 4/6 systolic ejection murmur was audible at the left upper sternal border. Chest radiograph and ECG were unremarkable. Echocardiography (Figure 1; Movies I and II in the online-only Data Supplement) revealed that an echolucent cyst (10 mm in diameter) originating from the membranous interventricular septum (IVS) was swinging like a pendulum and obstructing the right ventricular outflow tract, as represented by a peak flow velocity of 4.0 m/s. There was no visible shunt jet across the IVS or the cystic wall. Cardiac catheterization and angiography (Figure 2; Movies III …

Isolated atrioventricular discordance with solitus viscera, inverted atria, D-loop ventricles, and solitus normally related great arteries: report of a rare case with successful surgical management.

tricular and concordant ventriculoarterial alignments (isolated atrioventricular discordance) is rare.1-3 Only 29 cases of this defect have been reported.4 We report the case of an infant with isolated atrioventricular discordance and complete common atrioventricular canal. To our knowledge, this case is the first successful surgical repair without atrioventricular block for isolated atrioventricular discordance with complete common atrioventricular canal.1 Clinical summary. The patient was an 8-month-old girl, weighing 3.7 kg, with poor physical development. She was a 2798-gm baby of a full-term pregnancy complicated by fetal bradycardia. Immediately after birth, she required pacemaker implantation because of sick sinus syndrome. Four months later she was noted to have congestive heart failure and was readmitted for treatment. Radiographic examination showed a left-sided stomach bubble, indicating visceral situs solitus, dextrocardia with marked cardiac enlargement, and increased vascular markings. Echocardiography showed isolated atrioventricular discordance and complete atrioventricular canal (Rastelli type A), with severe mitral and tricuspid valve regurgitation. Cardiac catheterization and angiography revealed interrupted inferior vena cava. The left-sided right atrium opened into the left-sided left ventricle and to the aorta. The left atrium was connected with a right-sided right ventricle. The pulmonary valve was leftward, anterior, and superior to the aortic valve; however, the left ventricle was anterior and to the left, with the right ventricle posterior and to the right. Systolic pressures were equal in both ventricles and in the aorta. There was a partial anomalous pulmonary The Journal of Thoracic and Cardiovascular Surgery Volume 117, Number 2 Brief communications 393