Shintaro Kishimoto

Increased plasma type B natriuretic peptide in the acute phase of Kawasaki disease

Background:  The aim of this study was to identify possible factors associated with type‐B natriuretic peptide (BNP) production in the acute phase of Kawasaki disease (KD).

Microembolic signals measured by transcranial Doppler during transcatheter closure of atrial septal defect using the Amplatzer septal occluder.

PURPOSE To determine the frequency and factors associated with increase in microembolic signals during transcatheter closure of atrial septal defect using the Amplatzer septal occluder. METHODS During the procedure in 16 patients, we measured microembolic signals using transcranial Doppler. Procedure time was divided into five periods: right cardiac catheterisation; left cardiac catheterisation; left cardiac angiocardiography; sizing and long sheath placement; device placement and release. We compared numbers of microembolic signals among the five periods and identified factors associated with them. RESULTS Mean size of septal occluder was 16 millimetres in diameter. Total number of microembolic signals was a median of 31.5, ranging from 3 to 113. Microembolic signals in three periods, left cardiac catheterisation; sizing, and long sheath placement; and device placement and release, were not significantly different from one another, but were significantly higher than those in the remaining two periods, right cardiac catheterisation and left cardiac angiocardiography (median was 9 in left cardiac catheterisation; 6 in sizing and long sheath placement; 6.5 in device placement and release, versus 0 in right cardiac catheterisation and 1 in left cardiac angiocardiography, p less than 0.05, respectively). Importantly, the time for device manipulation positively correlated with total number of microembolic signals (r equals 0.77, p less than 0.001), although fluoroscopic time, age, or size of septal occluder did not. CONCLUSIONS Transcatheter closure of atrial septal defect using the Amplatzer septal occluder produces microemboli, especially during device placement. To minimise the risk of systemic embolism, we must decrease the time for device manipulation.

Portal-systemic encephalopathy after Fontan-type operation in patient with polysplenia syndrome

An 18-year-old patient, who had polysplenia and single ventricle, presented with altered mental status 9 years after a Fontan-type operation and pacemaker implantation. He underwent replacement of common atrioventricular valve and aortic valve plasty 1 year previously and has been placed on multiple medications including beta-blocker for his poor ventricular function. Blood chemistry revealed hyperammonemia of 2420 microg/l as a cause of this altered mental status disturbance. Superior mesenteric arteriography revealed large portal-systemic shunts in venous phase as a cause of hyperammonemia. To control blood ammonia level, we placed him on low protein diet, oral polymixin B, and lactulose instead of closing shunt with device. This case illustrates that portal-systemic shunt may result in hyperammonemia leading to altered mental status long after a Fontan-type operation.

Three Cases of KCNT1 Mutations: Malignant Migrating Partial Seizures in Infancy with Massive Systemic to Pulmonary Collateral Arteries

KCNT1 mutations are gain-of-function mutations in potassium channels resulting in severe infantile epilepsy. Herein we describe 3 infants with malignant migrating partial seizures with KCNT1 mutations accompanied by massive systemic to pulmonary collateral arteries with life-threatening hemoptysis and heart failure.

Trans‐pulmonary echocardiography as a guide for device closure of patent ductus arteriosus

The aim of this study was to develop trans‐pulmonary echocardiography (TPE) to guide device closure of patent ductus arteriosus (DC‐PDA).

Circulating Myeloid Dendritic Cells is Decreased in the Acute Phase of Kawasaki Disease

Background: Kawasaki disease is the most prevalent vasculitis of children in the developed countries that affects middle-sized arteries. Though T-cells are known to be activated with ample production of cytokines in acute phase of Kawasaki disease, there is a paucity of data concerning dendritic cells (DCs), the most potent antigen presenting cells that initiates T-cell activation. This study examined change in circulating DCs in acute phase of Kawasaki disease. Methods: Using multi-color flow cytometry, we determined circulating myeloid DC (mDC), Lin-HLA-DR+CD11c+ cell, and plasmacytoid DC (pDC), Lin-HLA-DR+CD123+ cell in 33 patients with acute phase of Kawasaki disease (aKD), 24 febrile controls (FC), and 13 healthy controls (HC). Blood chemistry data including cytokines were determined at the same time. Numbers of DCs were compared among 3 groups and before and after immunoglobulin treatment in aKD. Correlation between numbers of circulating DCs and blood chemistry data were determined. Results: Number of circulating mDC was significantly lower in aKD on admission than in FC and HC [median (lower, upper quartile)=7260 (2463, 11550) vs. 12210 (9500, 22050) and 18600 (11520, 23460) cells/ml, p < 0.001]. This number of circulating DCs significantly correlated with disease severity represented by serum albumin (mDC, r=0.56, p < 0.0001; pDC, r=0.39, p < 0.02, respectively), C reactive protein (mDC, r=-0.42, p < 0.005), and interleukin-6 (mDC, r=-0.55, p < 0.007). Immunoglobulin treatment quickly restored number of mDC [7260 (2463, 11550) vs. 15200 (10840, 30965) after IVIG and 18600 (12950, 25510) cells/ml at convalescence, p < 0.001] in aKD. Conclusions: This study indicates that number of circulating mDCs is decreased in acute Kawasaki disease, and may be involved in the pathophysiology.

Entrapment of the Left Coronary Artery Ostium by the Aortic Valve Leaflet, Promoting Myocardial Ischemia

![Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4][![Graphic][5] ][5][![Graphic][6] ][6] A 13-year-old girl was referred to us after an episode of post-exercise ischemic heart event. Ascending aortography, left coronary angiography (Online Videos [1A][6]and [1B][7]),

Progressive left coronary stenosis after rotablator ablation appreciated by Doppler echocardiography.

We describe a patient with a history of Kawasaki disease who showed coronary jet flow in the giant aneurysm on color-Doppler echocardiography caused by progressive coronary stenosis. A 6-year-old boy who had bilateral giant coronary aneurysms (14.9 mm in diameter) of the left main trunk (Fig. 1a) as sequelae of Kawasaki disease presented to us with anginal chest pain. Two years ago he had undergone coronary artery bypass graft surgery using the bilateral internal mammary artery grafted to the left anterior descending and left circumflex artery. Coronary angiography this time showed obstruction of both right and left internal mammary arteries. A color-Doppler echocardiography showed an aliased jet originating from the coronary ostium and flowing into the left giant coronary aneurysm with an early peak diastolic flow of 1.6 m/s (normal: 0.18– 0.3) with acute deceleration, being much faster than peak systolic flow velocity of 1.0 m/s (Fig. 1b). Because it was difficult to repeat the bypass graft surgery, he underwent successful rotablator ablation of the left coronary stenosis using 1.5-mm burr and the left coronary stenosis was significantly alleviated with a decreased peak diastolic flow velocity of 1.0 m/s (Fig. 1c) and temporary resolution of anginal chest pain. However, 2 months later, he complained of refractory chest pain again, with accelerated diastolic left coronary flow velocity of 2.0 m/s and underwent another attempt at rotablator ablation, but, unfortunately, he fell into shock during the procedure and could not be saved with multiple resuscitations. This case illustrates that color-Doppler echocardiography is a useful tool for evaluating coronary artery stenosis with a giant coronary aneurysm caused by Kawasaki disease and also emphasizes the difficulty of treating these small children with coronary stenosis [1, 2].

Intra‑cardiac echocardiography‑guided stent implantation into stenosed superior vena cava in a patient with a history of contrast anaphylaxis

A 37-year-old patient, who suffered from a repeated superior vena cava (SVC) syndrome, was scheduled for stent implantation into SVC, but suffered from contrast anaphylaxis. To monitor the procedure, we used intra-cardiac echocardiography and successfully implanted a stent. Placing an intra-cardiac echocardiographic catheter in the main pulmonary artery and facing towards the right, we could readily visualize stenosis in the SVC and inflation of the stent. Also looking up from right atrium, we noted proximal obstruction of the stent and confirmed the relief of obstruction after additional balloon dilation. This report leads to new application of intra-cardiac echocardiography for intervention of structural and vascular diseases other than inter-atrial septum.