Search

Abstract. We report two young adult patients who had acute coronary syndrome after regression of coronary aneurysms caused by Kawasaki disease (KD). A 26 year-old man had acute anterior myocardial [...]

Abstract. We report the midterm results of percutaneous transluminal coronary balloon angioplasty (PCBA) for localized stenosis (LS) caused by Kawasaki disease (KD). Two 2-year-olds, a girl and a boy, underwent [...]

Abstract. To clarify the incidence of stenotic lesions according to the coronary arterial diameter in the acute phase. we investigated 190 patients with coronary arterial lesions who underwent an initial [...]

Byline: H. Ohuchi (1,3), H. Ohashi (1), H. Takasugi (1), O. Yamada (1), T. Yagihara (2), S. Echigo (1) Keywords: Fontan procedure; Hypoxia; Hypocapnia; Exercise; Ventilation Abstract: The objective of this study was evaluate the relationships between abnormal pulmonary circulation, lung function, and respiratory response during exercise in Fontan patients. Pulmonary function and cardiopulmonary exercise tests were performed in 101 Fontan patients and 122 controls. A small vital capacity (VC) with a high residual volume-to-total lung capacity ratio and a slight but significant low arterial saturation with hypocapnia were observed in Fontan patients. The number of surgical procedures determined VC. Total cavopulmonary connection, fenestration, higher pulmonary arterial wedge pressure, and smaller VC were independent determinants of low arterial saturation, which was the only determinant of hypocapnia. Arterial saturation decreased during exercise and resting arterial saturation correlated with that at peak exercise. Improvement in dead space ventilation was less in Fontan patients and was independently determined by resting arterial saturation. A steeper minute ventilation--carbon dioxide production slope was determined by resting arterial saturation, arterial carbon dioxide tension, and peak oxygen uptake. In Fontan patients, in addition to dead space ventilation, surgery-related reduced VC, the type of repair, and high pulmonary arterial wedge pressure cause arterial desaturation with subsequent hypocapnia, resulting in accelerated inefficient ventilation at rest and during exercise. Author Affiliation: (1) Department of Pediatrics, National Cardiovascular Center, 5-7-1,Fujishiro-dai, 565-8565, Suita, Osaka, Japan (2) Department of Thoracic surgery, National Cardiovascular Center, 5-7-1, Fujishiro-dai, 565-8565, Suita, Osaka, Japan (3) Physiological Imaging Labratory, A12-404 Mayo Clinic, 200 1 st Street S.W., Rochester, USA, MN 55905 Article History: Registration Date: 01/01/2003 Online Date: 12/05/2004

Byline: K. Watanabe (1), T. Nishikimi (2), M. Takamuro (1), K. Yasuda (1), Y. Ishikawa (1), S. Tanabe (1), O. Yamada (1), N. Nagaya (3), H. Matsuoka (2), K. Kangawa (4), S. Echigo (1) Keywords: Adrenomedullin; Congenital heart disease; Hypoxia; Pulmonary hypertension Abstract: To investigate the pathophysiological role of two forms of adrenomedullin (AM), a mature AM (AM-m) and a glycine-extended AM (AM-Gly), in congenital heart disease, we measured plasma levels of AM in patients with cyanotic heart disease, high pulmonary blood flow without pulmonary hypertension (PH), high pulmonary blood flow with PH, Fontan procedure, intracardiac repair without complication, and intracardiac repair with PH and control subjects. Plasma AM-m and AM-Gly were increased only for cyanotic heart disease (2. 5 +- 1.3 pmol/L, p &lt 0.001 13.1 +- 6.2 pmol/L, p &lt 0.05) and intracardiac repair with PH (2.3 +- 1.5 pmol/L, p &lt 0.01 13.0 +- 7.0 pmol/L, p &lt 0.05) compared with control (1.0 +- 1.4 and 8.6 +- 1.3 pmol/L, respectively). They were similarly correlated with mean systemic arterial pressure (r = -0.40 and -0.37 respectively p &lt 0.001), mixed venous oxygen saturation (r = -0.60 and -0.50 p &lt 0.0001), systemic arterial oxygen saturation (SA.sub.sat) (r = -0.56 and -0.46 p &lt 0.0001), and pulmonary arterial resistance (Rp) (r = 0.41 and 0.38 p &lt 0.005). Multiple regression analysis revealed that SA.sub.sat and Rp were independently correlated with AM. Interestingly, the venous AM-m level was significantly higher than the arterial AM-m, suggesting that the mature form is extracted in pulmonary circulation, whereas there were no venoarterial differences in AM-Gly. These results suggest that plasma AM-m and AM-Gly are similarly regulated and the main clearance site of AM-m is the lung in patients with congenital heart disease. Author Affiliation: (1) Department of Pediatrics, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan (2) Department of Hypertension and Cardiorenal Medicine, Dokkyo University School of Medicine, Mibu, Tochigi 321-0293, Japan (3) Division of Cardiology, Department of Medicine, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan (4) Research Institute, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan Article History: Registration Date: 01/01/2003 Online Date: 04/09/2003

Byline: E. Tsuda (), T. Kamiya (), K. Kimura (), Y. Ono (), S. Echigo () Abstract: We used intravascular ultrasound (IVUS) to compare the degree of coronary artery dilatation during the acute phase of Kawasaki disease with the extent of intima-medial thickening more than 10 years later. We wanted to determine if there was a threshold degree of dilatation that was highly predictive of later thickening. Twenty-eight patients with a mean age of 17.3 +- 1.7 years were studied the mean interval from the initial selective coronary angiography to the IVUS study was 15.0 +- 1.6 years. We measured the maximum intima-medial thickness of selected coronary arterial segments in IVUS images and measured the largest diameters of the corresponding coronary arterial segments in the initial coronary angiograms. A significant correlation was found between the initial diameters of the coronary arteries and the intima-medial thickness more than 10 years later in the right coronary, the left anterior descending coronary, and the left circumflex arteries. The coefficient of correlation was 0.77 (n = 120, p &lt 0.0001), and for the bifurcation of the left coronary artery it was 0.50 (n = 26, p &lt 0.01). For this study, abnormal intima-medial thickness was defined as more than 0.40 mm. When the initial coronary arterial dilatation exceeded 4.0 mm, the sensitivity was 28/31 (90%) and the specificity was 87/89 (98%) in the right coronary, the left anterior descending coronary, and the left circumflex arteries. For the bifurcation of the left coronary artery, the sensitivity was 14/21 (67%) and the specificity was 5/5 (100%). Author Affiliation: () Department of Pediatrics, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita-shi, Osaka 565-8565, Japan, JP () Department of Radiology, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita-shi, Osaka 565-8565, Japan, JP

Abstract. We report a case of isolated congenital complete atrioventricular block with left ventricular dysfunction after pacemaker implantation that improved after the pacing site was changed. During the neonatal period, [...]

Byline: A Miyazaki (1), Y. Ono (1), C. Yutani (2), S. Echigo (1) Keywords: Ventricular tachycardia; Exacerbation by fever; Autopsy Abstract: Primary ventricular tachycardia (VT) is rare in children. We report a child with VT exacerbated by fever which was ultimately fatal. His first episode occurred at 5 months old and recurred with fever, occasionally causing syncope. Episodes were difficult to control with antiarrhythmic agents during fever but remitted with antipyretics. He was admitted to hospital 23 times because of VT and died suddenly with fever when he was 17 years old. At autopsy, severe fatty replacement of the bundle of His and interstitial fibrosis of the sinus node was found. Author Affiliation: (1) Department of Pediatrics, National Cardiovascular Center, Suita, Osaka, Japan (2) Department of Pathology, National Cardiovascular Center, Suita, Osaka, Japan Article History: Registration Date: 01/01/2003 Online Date: 08/06/2004

Byline: K. Watanabe (1), K. Fukuchi (2), S. Echigo (1) Author Affiliation: (1) Department of Pediatrics, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka, 565-8565, Japan (2) Department of Radiology, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka, 565-8565, Japan Article History: Registration Date: 01/01/2004 Online Date: 12/05/2004

Byline: M. Kitano (1), K. Watanabe (1), T. Yagihara (2), S. Echigo (1) Keywords: Total anomalous pulmonary venous return; Common pulmonary vein; Asplenia Abstract: A rare case of total anomalous pulmonary venous return, in which the right and left peripheral pulmonary veins connected circularly and there was no central part of the pulmonary vein or the common pulmonary vein, is presented. To our knowledge, total anomalous pulmonary venous return with circular pulmonary venous connection has not been reported previously in the literature. It is thought that the complex connection between peripheral pulmonary veins with the absence of the central part of the pulmonary vein as well as the common pulmonary vein results from common pulmonary venous agenesis. Author Affiliation: (1) Department of Pediatrics, National Cardiovascular Center, 5-7-1, Fuzishirodai, Suita, Osaka, Japan (2) Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1, Fuzishirodai, Suita, Osaka, Japan Article History: Registration Date: 01/01/2003 Online Date: 02/04/2004