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JCS/JSCS 2020 Guideline on Diagnosis and Management of Cardiovascular Sequelae in Kawasaki Disease

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JCS/JSCS 2020 Guideline on Diagnosis and Management of Cardiovascular Sequelae in Kawasaki Disease Advance Publication Circulation Journal JCS GUIDELINES doi: 10.1253/circj.CJ-19-1094 JCS/JSCS 2020 Guideline on Diagnosis and Management of Cardiovascular Sequelae in Kawasaki Disease Ryuji Fukazawa; Junjiro Kobayashi; Mamoru Ayusawa; Hiromichi Hamada; Masaru Miura; Yoshihide Mitani; Etsuko Tsuda; Hiroyuki Nakajima; Hiroyuki Matsuura; Kazuyuki Ikeda; Kazuhiko Nishigaki; Hiroyuki Suzuki; Kei Takahashi; Kenji Suda; Hiroshi Kamiyama; Yoshihiro Onouchi; Tohru Kobayashi; Hiroyoshi Yokoi; Kisaburo Sakamoto; Masami Ochi; Soichiro Kitamura; Kenji Hamaoka; Hideaki Senzaki; Takeshi Kimura on behalf of the Japanese Circulation Society Joint Working Group Table of Contents Introduction to the Revised Guidelines ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 2 IV. Treatment of Cardiovascular Sequelae ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 22 I. Epidemiology, Genetic Background, and 1. Pharmacotherapy ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 22 Severity Assessment of Kawasaki Disease (KD) ∙∙∙ 3 2. Nonpharmacological Therapy ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 29 1. Current Epidemiology ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 3 3. Summary of Treatment Options ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 33 2. Genetic Background of KD ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 5 V. Follow-up According to Life Stage ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 33 3. Severity Assessment of KD ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 7 1. Management at School ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 33 4. Diagnosis and Treatment of Incomplete KD ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 9 2. Management of the Adolescent/Young Adult (AYA) II. Pathology of Cardiovascular Sequelae and Generation (Transition Medicine) ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 38 Coronary Hemodynamics: Long-Term 3. Management of Adulthood ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 40 Prognosis ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 11 VI. Relationship Between Sequelae of Coronary 1. Histopathology of Coronary Artery Lesions (CAL) ∙∙∙∙∙∙∙∙∙∙ 11 Arteritis and Atherosclerosis ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 45 2. Coronary Hemodynamics in Patients With Coronary 1. Progression to Atherosclerosis (Pathological Point of Sequelae ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 11 View) ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 45 3. Long-Term Prognosis ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 12 2. Progression to Atherosclerosis (Clinical Point of III. Examinations and Diagnosis of View) ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 46 Cardiovascular Sequelae ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 14 VII. Summary ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 47 1. Blood Examination, Biomarkers and Arteriosclerosis ∙∙∙∙∙ 15 References ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 49 2. Electrocardiography ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 17 3. Diagnostic Imaging ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 17 Appendix 1 ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 59 4. Cardiac Catheterization ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 20 Appendix 2 ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 60 5. Summary of Examinations and Diagnosis ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙ 22 Abbreviations 2DE two-dimensional echocardiography ARB angiotensin II receptor blocker ACEI angiotensin converting enzyme inhibitor baPWV brachial-ankle pulse wave velocity ACS acute coronary syndrome BCG Bacille de Calmette et Guérin AMI acute myocardial infarction BMS bare metal stent AP angina pectoris BNP brain natriuretic peptide APV average peak velocity CAA coronary artery aneurysm(s) J-STAGE Advance Publication released online July 8, 2020 This document is an English version of JCS/JSCS 2020 Guideline on Diagnosis and Management of Cardiovascular Sequelae in Kawasaki Disease reported at the 84th Annual Scientific Meeting of Japanese Circulation Society performed in 2020 (Website: http://www.j-circ.or.jp/guideline/pdf/JCS2020_Fukazawa_Kobayashi.pdf). Joint Working Groups: the Japanese Circulation Society, the Japanese Society for Cardiovascular Surgery, Japanese Society of Kawasaki Disease, Japan Pediatric Society, Japanese Society of Pediatric Cardiology and Cardiac Surgery, Japanese College of Cardiology Mailing address: ScientificCommittee of the Japanese Circulation Society, 18F Imperial Hotel Tower, 1-1-1 Uchisaiwai-cho, Chiyoda-ku, Tokyo 100-0011, Japan. E-mail: [email protected] Refer to Appendix 1 for the details of members. ISSN-1346-9843 All rights are reserved to the Japanese Circulation Society. For permissions, please e-mail: [email protected] Advance Publication 2 FUKAZAWA R et al. CABG coronary artery bypass grafting LAD left anterior descending artery CAG coronary angiography LCA left coronary artery CAL coronary artery lesion(s) LDL-C low-density lipoprotein cholesterol cAMP cyclic adenosine monophosphate LMT left main trunk CCU coronary care unit LS localized stenosis CFR coronary flow reserve mAN medium aneurysm cIMT carotid artery intima-media thickness MDCT multi detector row computed tomography CK creatine kinase MI myocardial infarction CT computed tomography MLC myosin light chain CTA computed tomography angiography MLD minimum lumen diameter CTO chronic total occlusion MMP matrix metaroproteinase Cx circumflex MRA magnetic resonance angiography MRI magnetic resonance imaging DAPT dual antiplatelet therapy OCT optical coherence tomography DES drug-eluting stent oxLDL oxidative low-density lipoprotein DL dilated lesion PCI percutaneous coronary intervention DOAC direct oral anticoagulant PET positron emission tomography FFR fractional flow reserve POBA percutaneous old balloon angioplasty FMD flow-mediated dilation percutaneous transluminal coronary rotational PTCRA gAN giant aneurysm ablation GEA gastroepiploic artery PT-INR international normalized ratio of prothrombin time HDL-C high-density lipoprotein cholesterol PWV pulse wave velocity H-FABP heart-type fatty acid binding protein RA radial artery ICT intracoronary thrombolysis RAS renin-angiotensin system iFR instantaneous wave-free ratio RCA right coronary artery IHD ischemic heart disease RITA right internal thoracic artery ITA internal thoracic artery SVG saphenous vein graft IVIG intravenous immunoglobulin TC total cholesterol IVUS intravascular ultrasound TG triglyceride KD Kawasaki disease t-PA tissue plasminogen activator Introduction to the Revised Guidelines More than 50 years have passed since Kawasaki disease Table 1. Class of Recommendation (KD) was first reported.1 According to a nationwide survey, Conditions for which there is evidence for and/or the number of patients with KD who became adults was Class I general agreement that the procedure or treatment is useful and effective 136,960 in 2014 (45.9% of the total number of those with a history), and 15,000 adult patients with coronary sequelae Conditions for which there is conflicting evidence and/ Class II or a divergence of opinion regarding the usefulness/ (including regression cases) are presumed to exist. Even if efficacy of a procedure or treatment the KD coronary artery lesion (CAL) is a small or regressed aneurysm, the tissue is not normal, and most people diag- The procedure or treatment is likely to be useful and Class IIa nosed with coronary artery sequelae have a risk of coronary effective life event. This guideline is for the remote management of cases with such KD heart sequelae. Seven years have passed The procedure or treatment is not very well Class IIb established since the last revision of this guideline in 2013, and various changes have been made to the definition and management Conditions for which there is evidence and/or general of cardiovascular sequelae of KD. In particular, since the Class III agreement that the procedure or treatment is not AHA’s KD guideline2 announced in 2017 introduced the useful/effective and may in some cases be harmful Z-score of coronary artery diameter as a standard for remote management, established the evaluation of coronary aneurysms according to individual physique, and the stratification of management by Z-score became clearer. In Table 2. Levels of Evidence Japan, the Z-score can be calculated from the coronary Proven from multiple randomized interventions or 3 Level A artery diameter in children, and classification by Z-score meta-analyses has become possible, in addition to classification based on Proven in a single randomized clinical trial or a clinical Level B the absolute value of the coronary artery diameter. So, trial that is not a large, randomized intervention Z-score classification was also adopted in this guideline. In Consensus in an expert or small clinical trial (including Level C the classification based on absolute values, the lower limit retrospective studies and registration) values of medium and large aneurysms were combined as Advance Publication JCS/JSCS 2020 Guideline on Diagnosis and Management of Cardiovascular Sequelae in KD 3 “above”. In other words, an medium aneurysm was ≥4 mm evidence level. Unfortunately, the evidence in children is and <8 mm, and a giant aneurysm (gAN) was ≥8 mm. generally still poor, so we have omitted any that do not However, in children over 5 years old, the discrepancy have evidence in children. However, we list the class and between this absolute value standard
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