(2013 ) ACHD ACHD

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1 (0 ). ) ) ) ) ) ) (ACHD) / ( ) ACHD ACHD 0 6 (9 ) / ACHD ACHD Adult congenital heart disease, Pediatric cardiologists, Adult cardiologists, Emergency admission, Transfer of the patients (congenital heart disease: CHD) (adult CHD: ACHD),5) ACHD 5,8) ACHD CHD,0) CHD ) ) ACHD ACHD ACHD NYHA classiii-iv 6 (9 ) (CHD ) 0 ( ) 6 ( ) 0-9 ( ) (RCM) (DCM) Table 6 (ASD) (SV) (PA/VSD) ( (MAPCAs) ) (DORV) (TOF) (AVSD) (TrA) (c-tga) (RPA ) / (PDA) RCM DCM ASD (PH) (AMI) RCM CHD ( ) 8

2 (RPA /PDA:PDA ) (PA/VSD, MAPCAs ASD/PH) Fig. 9 ( ) ( ) ( ) 6 5 Fontan ( ) ( ) ( (PM) ) RCM AMI ( ) ASD PA/VSD, MAPCAs (ER) ( ASD/PH TrA) Table 9 8 (%) CHD ( ) (SIADH) (OMI) OMI AMI ASD ASD Amplatzer septal occluder (ASO) erosion erosion NT-proBNP ( pg/ml) ST (V-5) OMI (Sustained VT) (AED) ER (AF) (CAVB) PA/VSD,MAPCAs MAPCAs AVSD 6 trisomy Table Diagnosis of heart disease (n=6) ASD SV PA/VSD DORV TOF Absent RPA AVSD TrA c-tga RCM DCM(post MI: Kawasaki disease) ASD: atrial septal defect, AVSD: atrioventricular septal defect, c-tga: corrected transposition of great arteries, DCM: dilated cardiomyopathy, DORV: double outlet right ventricle, MI: myocardial infarction, PA: pulmonary atresia, RCM: restrictive cardiomyopathy, RPA: right pulmonary artery, SV: single ventricle, TOF: tetralogy of Fallot, TrA: truncus arteriosus, VSD: ventricular septal defect, PC+AC Adult Pallietive operation Other congenital disease 6 Fontan circulation Fig. Original charge department (n=9) AC: Adult cardiology, PC: pediatric Table Diagnosis of critical illness (n=9) Deterioration of CHD Suicide attempt SIADH OMI Arrhythmia sustained VT AF CAVB Myocarditis Respiratory failure Hemoperitoneum AF: atrial flatter, CAVB: complete atrioventricular block, CHD: congenital heart disease, OMI: old myocardial infarction, SIADH: syndrome of inappropriate secretion of antidiuretic hormone, VT: ventricular tachycardia, 8 Pediatric 5

3 (PT-INR.9-.) Table ER ER ER ASD/PH Table OMI 0 8 Fig. 9 ICU ( /RCM) 8 (88%) Table 5 (ICD)/ PM 8 6 ICU 9 6 (RCM RPA /PDA) (Fontan / AF) (PA/VSD,MAPCAs ) Table The department for first care (n=9) Pediatric Myocarditis OMI Hemoperitoneum Adult Arrhythmia SIADH Suicide attempt Respiratory failure OMI: old myocardial infarction, SIADH: syndrome of inappropriate secretion of antidiuretic hormone, Table The department for admission (n=9) Pediatric Myocarditis Hemoperitoneum Adult Arrhythmia SIADH Suicide attempt Respiratory failure OMI OMI: old myocardial infarction, SIADH: syndrome of inappropriate secretion of antidiuretic hormone, Intensive care unit Critical care center Fig. The ward 5 5 General ward Pediatrics 9 Gynecology The charge department Pediatric Adult 6

4 Fig. ( ) 9 8 (89%) Table 6 Fontan ( 9 ) ASD Table 5 The intensive treatment Mechanical ventilation Dialysis Brain hypothermia treatment PM/ICD implantation PCPS The charge department Pediatric Adult ICD: implantable cardioverter defibrillator, PCPS: percutaneous cardiopulmonary support, PM: Pacemaker PC Original First Care Admitt After discharge :dead AC PC+AC CS :dead ACHD 00,000 0,000 / 9) 00 CHD % CHD ACHD CHD 000 ACHD ACHD,9) ACHD ACHD,,0) ) Fig. The transition of charge department AC: Adult cardiology, CS: Cardiac surgery, PC: pediatric Table 6 The charge department after discharge and diagnosis of critical illness Pediatric Heart Failure Hemoperitoneum Suicide attempt PC+AC Arrhythmia SIADH(with ASD) OMI(with ASD) 6 5 (Fontan circulation ) (Chromosomal abnormality ) (Chromosomal abnormality ) (Palliative operation ) (Chromosomal abnormality ) (Palliative operation ) AF: atrial flutter, AC: adult cardiology, CAVB: complete atrioventricular block, OMI: old myocardial infarction, PC: pediatric cardiology, SIADH: Syndrome of inappropriate secretion of antidiuretic hormone, VT: ventricular tachycardia

5 ) ICU 8 ACHD,,8) ACC/AHA 008 ACHD 0) 0 0 CHD ACHD ACHD ( CHD ) ACHD Marelli ) ACHD (ACHD 9% ) 000 ACHD ACHD (ACHD ) Ochiai 6) Euro Heart Survey ACHD (Table ) - 9 ACHD ) ACHD ACHD 09 9% 6) ACHD ) ACC/AHA 008 ACHD 0) ACHD ACHD ACHD ACHD ACHD Table Recommendations for Optimal ACHD Care 6). The existence of an adult cardiology department that planned to treat all ACHD patients.. The presence of at least pediatric cardiac surgeons.. The presence of either an ACHD-specialized outpatient clinic or the existence of a plan to one in the near future.. The presence of at least pediatric cardiologist. 5. The center employ the adult cardiologists who specializing in cardiac catheterization, arrhythmia, or echocardiography. 6. There must be enough experiences of cardiac catheterization (>500per year), ablation (>0 per year), pacemaker insertions (>0per year), and ICD implantations (>0).. A fully equipped laboratory of three dimensional computed tomography, magnetic resonance imaging, CARTO and EnSite system are available. 8. The center employ obstetrician, psychiatrists and brain surgeons, and must have the intensive care unit. ICD: implantable cardioverter defibrillator (PM AMI ) ER ER ER ER ACHD 6 8

6 5 (Fig. ) ACHD ) Fontan ACHD ) CHD ACHD ACHD ACHD Amplatzer septal occluder Amplatzer duct occluder ACHD ACHD ACHD ACHD ACHD ACHD 6) ACHD ACHD ) ACHD 8) ACHD ACHD ACHD ACHD 5) ACHD 9) ACHD ( ) 6 / 9 ACHD ACHD 9

7 ACHD ACHD ACHD ) Child JS, Freed MD, Moodie DS, et al. Task Force 9: Training in the care of adult patients with congenital heart disease. JACC 008; 5: 89-9 ) Kaemmere H,Bauer U, Pensl U, et al. Management of emergencies in adults with congenital cardiac disease. Am J Cardiol. 008; 0: 5-55 ) Marelli AJ, Therrien J, Mackie AS, et al. Planning the specialized care of adult congenital heart disease patients: from numbers to guidelines; an epidemiologic approach. Am H J 009; 5: -8 ) 0; : -50 5) (00 ) (0 ) JCS0_niwa_h.pdf 6) Ochiai R, Yao A, Kinugawa K, et al. Stats and future needs of regiomal adult congenital heart disease centers in Japan. A nationwide survey-. Circulation J 0; 5:0- ) Reid GJ, Irvine MJ, McCrindle BW, et al. Prevalence and correlates of successful from pediatric to adult health care among a cohort of young adults with complex congenital heart defects. Pediatrics 00; : e9-e05 8) 0; ; ) Shiina Y, Toyoda T, Kawasoe Y, et al. Prevalence of adultpatients with congenital heart disease in Japan. Int J Cardiol 0; 6: -6. 0) Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 008 guidelines for the management of adults with congenital heart disease: a report of the American College of /American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 008; 8: e-e8 ) Webb GD, Williams RG. nd Bethesda Conference; Care of the adult with congenital heart disease: Introduction. J Am Coll Cardiol. 00;:6-69 ) (transition/ transfer). 0; : -9 0

8 The ideal method and the real state of transfer of the adult congenital heart disease patients: The consideration through the experiences of emergent admission. Naomi Nakagawa, Masahiro Kamada, Yukiko Ishiguchi Department of Pediatric Hiroshima City Hospital, Hiroshima, Japan Background: Despite the sharp rise in the number of adult congenital heart disease (ACHD) patients, the number of available specialist doctors and facilities are insufficient, meaning that in many cases, ACHD patients are treated by pediatric cardiologists (PC). Objective: We carried out a survey of ACHD patients who required emergency admission and investigated the ideal framework for collaboration between PCs and adult cardiologists (AC). Subjects and Methods: The subjects were 6 patients who required emergency admission on 9 occasions during the past 0 years. We surveyed the reasons for examination, the department where they were initially examined, the department to which they were admitted, hospital wards, prognoses, and departments responsible for post-discharge care. Results: The reason for examination in cases was heart failure, followed by arrhythmias in four cases. More than half of the cases were admitted to ICUs or emergency wards, and only one case to pediatric ward. For eight of the nine cases who underwent emergency examinations by ACs, it was their first examination by ACs. Of the ten cases in which patients were admitted to an adult cardiology department, six cases were jointly followed up by ACs and PCs and four cases only by PCs. Conclusions: PCs must have their patients see ACs actively. ACs must recognize the need of the treatment of ACHD patients. We must create a suitable environment here ACs and PCs cooperate in the treatment of ACHD patients.