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Myocardial ischemia changes the electrophysiological properties of the myocardium, but it is difficult to detect the abnormalities of depolarization and repolarization noninvasively in patients with coronary artery disease. Depolarization and repolarization abnormalities were retrospectively investigated in 61 patients (48 males and 13 females) with coronary artery lesions (CALs) caused by Kawasaki disease (KD) from 2007 to 2014 using magnetocardiography (MCG). CAL had been diagnosed by selective coronary angiography. The integral value was computed for each channel, and isointegral maps were constructed during depolarization and repolarization. The MCG findings were compared between the stenotic lesions group (SL group) and the non-stenotic lesions group (non-SL group). The incidence of MCG abnormalities was significantly higher in the SL group than in the non-SL group (p

We describe the case of a 4-year-old boy whose clinical course after Kawasaki disease resulted in coronary artery bypass grafting (CABG) due to acute myocardial infarction (AMI) causing cardiogenic shock. He had developed an ischemic cardiomyopathy due to severe localized stenosis of the left main coronary artery (LCA) and went into cardiogenic shock due to AMI on the day before a scheduled operation. He underwent successful emergency CABG within 4 h of MI. Postoperatively his neurological status was intact. This is the first report of a successful emergency CABG in a small child with cardiogenic shock due to LCA occlusion. CABG should be undertaken in small patients when appropriate indications exist, if bodyweight is >10 kg.

We report the importance of evaluating the right coronary artery (RCA) by two-dimensional echocardiography (2DE) in the acute phase of Kawasaki disease (KD), based on our experience with three patients whose coronary artery lesions (CAL) involving the RCA were missed in the acute phase of KD. Two patients had asymptomatic occlusion of the RCA, and it was speculated that one patient had a cardiac arrest due to CAL of the RCA. An abnormal Q wave in lead III in all 3 patients had indicated stenotic lesions of the RCA. Because it is difficult to diagnose CAL by 2DE in the late period, it is essential to identify the proximal RCA using a right parasternal approach while the patient is lying in the right lateral position.

Methotrexate (MTX) is used for the treatment of polyarticular juvenile idiopathic arthritis (JIA), and an anti-interleukin-6 receptor monoclonal antibody (tocilizumab: TCZ) is also used and added for the treatment of intractable JIA. It has been reported that MTX might induce Epstein-Barr virus (EBV)-associated lymphoma, but the discussion about the effect of MTX and/or TCZ against reactivation of EBV in pediatric patients has been incomplete.The EBV loads in four polyarticular JIA and three systemic arthritis JIA patients treated with MTX and/or TCZ, and the percentage of EBV-specific killer T cells (EBV-CTLs) in some patients were prospectively monitored.No patients had EBV-associated symptoms during the observation period. EBV loads in all patients were not significantly increased, and the levels of EBV loads were the same as EBV-seropositive healthy children following the administration of MTX and/or TCZ. EBV-CTLs were detectable during the observation period, but some patients had slightly low levels of EBV-CTLs.Treatment with MTX and/or TCZ did not severely affect EBV load and prevent induction of EBV-CTLs in JIA patients.

Background. Hyponatremia (HN) is relatively common in adults with congenital heart disease and is a powerful predictor of mortality. However, the precise relationship of HN to the Fontan pathophysiology remains unknown. Purpose. Our study aimed to clarify the association of HN to the Fontan pathophysiology. Methods and Results. We measured the plasma sodium (Na) level in 169 consecutive Fontan patients (78 children) and HN (≤137 mEq/L) was observed in 50 patients (30% of the total patients, 31% of the children). The HN patients showed a lower peak oxygen uptake (VO2) with a greater New York Heart Association class (P < .0001). The plasma level of norepinephrine (NE), rennin activity (PRA), arginine vasopressin, central venous pressure (CVP) and medications were associated with the Na levels and the NE, PRA, and diuretic use were the independent determinants (P < .01−.0001). The plasma B-type natriuretic peptide was not correlated with the Na levels. In the children, diuretic use and the PRA independently determined the Na levels without any association to the CVP or peak VO2. During a median follow-up of 2.1 years, the HN in addition to the CVP and peak VO2 independently predicted the unscheduled hospitalizations in all patients, while the HN was the only independent predictor of the hospitalizations in the adult patients (hazard ratio: 3.1, 95% confidence interval 1.2–8.0, P= .021). Conclusions. Child and adult Fontan patients exhibited a high prevalence for HN that closely reflected some neurohumoral activation and predicted adverse clinical events, especially in adult Fontan patients.

Information about acute coronary syndrome caused by Kawasaki disease-related coronary artery lesions in adults is sketchy. We reviewed the clinical features of 50 adult patients who had an acute coronary syndrome caused by coronary artery lesions due to Kawasaki disease or probable Kawasaki disease from 1980 to 2008. Of the 50 patients, 43 (90%) were male and seven were female (10%). Their ages at the onset of acute coronary syndrome ranged from 18 to 69 years, with a median of 28 years. The culprit lesion in 43 patients was thrombotic occlusion of an aneurysm, and 40 patients had giant aneurysms. In the three patients in whom no aneurysms were seen in coronary angiograms performed at the time of acute myocardial infarction, either giant aneurysms or aneurysms had been visualised in childhood. The initial treatment of acute coronary syndrome was as follows: intracoronary thrombolysis, 11; primary percutaneous coronary intervention, 9; emergency coronary artery bypass grafting, 3; and medication, 26. Elective coronary artery bypass grafting was performed in 15 patients. Three patients (6%) died. Of the 27 patients with additional coronary risk factors, 20 were smokers. Giant aneurysms due to Kawasaki disease continued to cause acute coronary syndrome in adult life with onset at a younger age than typifies that due to atherosclerosis in the general population, especially in male population rather than female population. Even when giant aneurysms regressed after the acute phase, a few patients still developed acute coronary syndrome in adult life. Smoking appears to be the most prominent additional risk factor.

Background: Noninvasive recognition of abnormalities of depolarization (DE) and repolarization (RE) in patients with coronary artery disease is difficult. Purpose: Our aim was to identify abnormalities of DE and RE in patients with coronary artery lesions (CAL) due to Kawasaki disease (KD) using magnetocardiography (MCG). Methods: We evaluated sixty one pts (48 males and 13 females) with CAL due to KD, The age at MCG ranged from 11 months to 38 years (median 15 years). Eleven pts had had previous myocardial infarction (MI), and 22 pts had had at least an occlusion of a major coronary artery branch. MCG was performed at rest with a multichannel superconducting quantum interference device system. The integral value was computed for each channel and isointegral maps were constructed during DE and RE. We analyzed abnormalities of DE and RE depending on occlusion of coronary arteries (OC) or a history of MI. Univariate analysis was performed on respective factors for DE and RE. Results: In the MI group, the number of abnormal DE and RE, abnormal DE and abnormal RE were 3 , 1 and 3, respectively. In OC group, the number of abnormalities of DE and RE was 2, and abnormality of RE was 9. In the non-OC group, the number of abnormal DE and RE was 2, and abnormal RE was 5. The factor of MI was significantly related on abnormal DE and abnormal RE (p Conclusion: Although symptomatic coronary occlusion affects both DE and RE, occlusion of coronary artery only affects RE. MCG is useful to detect changes in DE and RE.

An intraatrial reentrant circuit was identified using an electroanatomical mapping system and evaluation of postpacing intervals in a patient with isomerism of the left atrial appendages and atrioventricular septal defect. Intraatrial reentrant tachycardia was eliminated on the basis of our interpretation of the reentry circuit being dependent on a new anatomical obstacle consisting of a right-sided atrioventricular annulus and atrial septation patch. We must consider the possibility of arrhythmogenic obstacles changing, as a patient grows, long after congenital heart disease surgery. (PACE 2012; 35:e299–e301)

We describe the case of a 4-year-old boy whose clinical course after Kawasaki disease resulted in coronary artery bypass grafting (CABG) due to acute myocardial infarction (AMI) causing cardiogenic shock. He had developed an ischemic cardiomyopathy due to severe localized stenosis of the left main coronary artery (LCA) and went into cardiogenic shock due to AMI on the day before a scheduled operation. He underwent successful emergency CABG within 4 h of MI. Postoperatively his neurological status was intact. This is the first report of a successful emergency CABG in a small child with cardiogenic shock due to LCA occlusion. CABG should be undertaken in small patients when appropriate indications exist, if bodyweight is10 kg.

Hyponatremia (HN) is relatively common in adults with congenital heart disease and is a powerful predictor of mortality. However, the precise relationship of HN to the Fontan pathophysiology remains unknown.Our study aimed to clarify the association of HN to the Fontan pathophysiology.We measured the plasma sodium (Na) level in 169 consecutive Fontan patients (78 children) and HN (≤137 mEq/L) was observed in 50 patients (30% of the total patients, 31% of the children). The HN patients showed a lower peak oxygen uptake (VO(2) ) with a greater New York Heart Association class (P.0001). The plasma level of norepinephrine (NE), rennin activity (PRA), arginine vasopressin, central venous pressure (CVP) and medications were associated with the Na levels and the NE, PRA, and diuretic use were the independent determinants (P.01-.0001). The plasma B-type natriuretic peptide was not correlated with the Na levels. In the children, diuretic use and the PRA independently determined the Na levels without any association to the CVP or peak VO(2) . During a median follow-up of 2.1 years, the HN in addition to the CVP and peak VO(2) independently predicted the unscheduled hospitalizations in all patients, while the HN was the only independent predictor of the hospitalizations in the adult patients (hazard ratio: 3.1, 95% confidence interval 1.2-8.0, P = .021).Child and adult Fontan patients exhibited a high prevalence for HN that closely reflected some neurohumoral activation and predicted adverse clinical events, especially in adult Fontan patients.