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Coronary reimplantation after neoaortic reconstruction (CRANR) in the arterial switch operation (ASO) allows easy selection of accurate coronary transfer sites in the distended neoaorta. However, neoaortic valve injury may occur during coronary reimplantation. We determined whether the CRANR procedure increased the incidence of aortic valve regurgitation (AR) after ASO. Between March 1994 and August 2017, 227 patients underwent ASO. Since September 2000 CRANR has been performed on 155 patients and open coronary reimplantation (OCR) on 72. Patients who had undergone aortocoronary flaps procedures (n = 13), had early or late mortality (n = 27), or lacked data (n = 11) were excluded. We enrolled and retrospectively reviewed the medical records of 176 patients who were followed up for postoperative AR: 38 underwent OCR and 138 underwent CRANR. We compared the incidences of early and late postoperative AR in both groups. We defined mild or greater AR as "significant AR." The groups did not differ in body weight at operation, great artery relationship, and coronary artery anatomy. The incidences of significant AR at discharge were 21.1% (8/38) in the OCR group and 16.6% (23/138) in the CRANR group (p = 0.53). The freedom from significant AR at 5 years was 59.9% in the OCR group and 62.4% in the CRANR group with no difference between the two groups (p = 0.73). Moderate AR occurred in one patient in the CRANR group. No surgical intervention was required for the aortic valve in either group. ASO using the CRANR technique did not increase the incidence of postoperative early and late AR.

The presence of fragmented QRS is a known marker of heterogeneous ventricular activation around the myocardial scar area. We validated whether fragmented QRS shows any association with ventricular dysfunction, fibrosis, or ventricular arrhythmias in patients with Duchenne muscular dystrophy (DMD). Thirty-seven patients with DMD were evaluated using electrocardiography (ECG), echocardiography, cardiac magnetic resonance, and 24-h Holter monitoring. Associations between fragmented QRS and ventricular dysfunction, fibrosis, or ventricular arrhythmia were investigated. Fragmented QRS complexes were present in 31 of 37 (83.7%) patients, and they were associated with a significantly lower left ventricular ejection fraction along with an increased left ventricular Tei index as evaluated by echocardiography, and more frequent ventricular arrhythmia as indicated using 24-hour Holter monitoring compared with patients without fragmented QRS. The number of leads with fragmented QRS correlated negatively with left ventricular ejection fraction both using echocardiography (r = -0.616) and CMR (r = -0.516). Further, the number of leads with fragmented QRS showed a significant correlation with several other echocardiographic measurements (mitral Em and Sm, and left ventricular Tei index), and 2-dimensional speckle-tracking echocardiography derived global left ventricular longitudinal strain. The frequency of ventricular arrhythmia observed using Holter monitoring showed a significant positive correlation with the frequency of fragmented QRS on ECG (r = 0.674). There was a positive trend of correlation between fragmented QRS and the amount of myocardial fibrosis as assessed by late gadolinium enhancement using CMR, but the statistical significance of the relationship was low (r = 0.433, p = 0.056). Fragmentation of QRS complexes is associated with degrees of left ventricular dysfunction, fibrosis, and ventricular arrhythmias in patients with DMD.

The management of pulmonary atresia with a ventricular septal defect (PA/VSD) depends on the anatomy of the pulmonary artery or on the surgical strategy used at individual institutions. In our institution, we adopted a right ventricle-to-pulmonary artery (RV-PA) shunt in 2011 as a palliative procedure for PA/VSD to overcome the disadvantages of a Blalock-Taussig shunt. We evaluated the results of the RV-PA shunt as initial palliative surgery for PA/VSD. Thirteen patients with ductus-dependent PA/VSD from August 2011 to August 2015 were enrolled. The mean age at surgery was 17.9 ± 15.3 (range 5–60) days, and the mean body weight was 2.9 ± 0.6 (range 2.2–4.0) kg. A Gore-Tex tube graft was used in all patients. We retrospectively observed intra- and postoperative complications, early and late mortality, and palliation duration to definitive repair. Left pulmonary artery angioplasty was performed as a concomitant procedure in three patients. There were no early hospital mortalities, although two inter-stage deaths occurred 34 and 47 days postoperatively: one patient died of aspiration and the other of right ventricular outflow tract (RVOT) pseudoaneurysm rupture. Two patients (15.4%) required the extracorporeal membrane oxygenation support postoperatively: one because of failure to wean from a bypass caused by persistent hypoxemia and the other because of sudden massive bleeding from the RVOT suture line in the intensive care unit 2 days postoperatively. These two patients underwent second-stage definitive repair successfully. Five patients (41.7%) required catheter intervention, for juxtaductal left pulmonary artery stenosis in three patients, right pulmonary artery stenosis in one, and shunt inflow stenosis in one. Two patients (15.4%) required re-operation because of shunt inflow stenosis and RVOT pseudoaneurysm, respectively. All patients who survived the RV-PA shunt underwent total correction at a mean interval of 13.1 months. A RV-PA shunt is an option for the initial palliation of ductus-dependent PA/VSD. Major complications can occur, including RVOT pseudoaneurysm, shunt inflow stenosis, persistent hypoxemia during the immediate postoperative period, and dehiscence of the shunt anastomosis site. Caution should be taken when performing the RV-PA shunt for palliation of PA/VSD.

Kawasaki disease (KD) is the most common cause of acquired heart disease in children. Intravenous immunoglobulin (IVIG) is the standard therapy for KD, but more than 10% of KD patients do not respond to IVIG and are at high risk for the development of coronary artery lesions (CALs). To identify clinical and genetic risk factors associated with CAL development and IVIG nonresponsiveness, this study analyzed the clinical data for 478 Korean KD patients. Multivariate logistic regression analysis showed that incomplete KD, IVIG nonresponse, fever duration of 7 days or longer, and the CC/AC genotypes of the rs7604693 single nucleotide polymorphism (SNP) in the PELI1 gene were significantly associated with the development of CALs, with odds ratios (ORs) ranging from 2.06 to 3.04. The risk of CAL formation was synergistically increased by the addition of individual risk factors, particularly the genetic variant in the PELI1 gene. Multivariate analysis also showed that a serum albumin level of 3.6 g/dl or lower was significantly associated with nonresponsiveness to IVIG [OR, 2.76; 95% confidence interval (CI), 1.34-5.68; P = 0.006]. Conclusively, incomplete KD, IVIG nonresponsiveness, long febrile days, and the rs7604693 genetic variant in the PELI1 gene are major risk factors for the development of CALs, whereas low serum albumin concentration is an independent risk factor for IVIG nonresponsiveness.

Kawasaki disease (KD) is an acute self-limiting form of vasculitis that afflicts infants and children and manifests as fever and signs of mucocutaneous inflammation. Children with KD show various laboratory inflammatory abnormalities, such as elevations in their white blood cell (WBC) count, C-reactive protein (CRP) level, and erythrocyte sedimentation rate (ESR). We here performed a genome-wide association study (GWAS) of 178 KD patients to identify the genetic loci that influence 10 important KD laboratory markers: WBC count, neutrophil count, platelet count, CRP, ESR, hemoglobin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and total protein. A total of 165 loci passed our arbitrary stage 1 threshold for replication (p < 1 × 10(-5)). Of these, only 2 SNPs (rs12068753 and rs4786091) demonstrated a significant association with the CRP level in replication study of 473 KD patients (p < 0.05). The SNP located at the CRP locus (rs12068753) demonstrated the most significant association with CRP in KD patients (beta = 4.73 and p = 1.20 × 10(-6) according to the stage 1 GWAS; beta = 3.65 and p = 1.35 × 10(-8) according to the replication study; beta = 3.97 and p = 1.11 × 10(-13) according to combined analysis) and explained 8.1% of the phenotypic variation observed. However, this SNP did not demonstrate any significant association with CRP in the general population (beta = 0.37 and p = 0.1732) and only explained 0.1% of the phenotypic variation in this instance. Furthermore, rs12068753 did not affect the development of coronary artery lesions or intravenous immunoglobulin resistance in KD patients. These results indicate that common variants in the CRP promoter can play an important role in the CRP levels in KD.

High-dose intravenous immunoglobulin (IVIG) therapy is the highly effective and standard treatment for Kawasaki disease (KD). However, ~20 % of KD patients have persistent fever or recurrence of fever after the initial IVIG treatment, which increases the risk for coronary artery lesions (CALs). Furthermore, the mechanism of IVIG resistance in KD patients still is unknown. The number of CC chemokine ligand 3-like 1 (CCL3L1) gene copies is reported to be associated with KD and IVIG resistance in Japanese patients. In addition, the authors observed significant upregulation of the CCL3L1 gene expression after in vitro immunoglobulin treatment in B cell lines derived from KD patients. Therefore, this study of 459 KD patients and 496 healthy control subjects tested whether the number of CCL3L1 gene copies is associated with a risk of KD, CALs, and/or IVIG resistance in Korean KD patients. However, the number of CCL3L1 gene copies was not associated with KD (P = 0.18), CAL formation (P = 0.062), or the IVIG resistance (P = 0.90). Therefore, the results indicate that the number of CCL3L1 gene copies does not have a role in susceptibility to KD or CALs nor with IVIG resistance in Korean KD patients.