Abstract:Competing definitions and classifications of coronary artery abnormalities (CAAs) after Kawasaki disease (KD) have been arbitrarily defined based on clinical experience. We sought to propose a classification system for CAAs based only on coronary artery z-scores. All echocardiograms performed between 1990 and 2007 on patients with a previous history of KD were reviewed. Coronary artery luminal dimensions were converted to body-surface-area-adjusted z-scores and compared to current classification systems. A tot… Show more
“…Manlhiot et al proposed a classification scheme based solely on Z scores using the formulas provided in the study from the National Heart, Lung, and Blood Institute Pediatric Heart Network. 139,140 One potential limitation of this study is that regression formulas for the LAD were used to derive Z scores for the left circumflex branch (normal values for the circumflex are not available with the Z-score system that was used). A classification scheme based solely on Z scores was proposed, which has been adapted and recommended in these guidelines:…”
Section: Classification Of Coronary Artery Abnormalitiesmentioning
confidence: 99%
“…158,159,175 An even greater percentage of patients (30%) will be classified as having coronary artery dilation when a Z-score cut point of 2.0 is used to define dilation. 111,140,176 Additional therapies of potential benefit are discussed below, but optimal treatment awaits delineation of the specific agent(s)/trigger(s) and pathogenesis of KD. ; thus, ibuprofen generally should be avoided in children with coronary artery aneurysms taking ASA for its antiplatelet effects.…”
BACKGROUND:Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries.
METHODS AND RESULTS:To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and longterm outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up.
CONCLUSIONS:These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances. K awasaki disease (KD) is an acute, self-limited febrile illness of unknown cause that predominantly affects children <5 years of age. When initially described, the potential for coronary artery complications was not appreciated. KD is now the most common cause of acquired heart disease in children in developed countries. In the absence of pathognomonic tests, the diagnosis continues to rest on the identification of principal clinical findings and the exclusion of other clinically similar entities with known causes. Timely initiation of treatment with intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery aneurysms defined from absolute luminal dimensions from 25% to ≈4%. Ongoing studies with additional therapies have not substantially reduced this residual risk. The long-term prognosis is determined by the initial and current level of coronary artery involvement. Certain subsets of patients are at risk for myocardial ischemia from coronary artery thrombosis and stenoses. Medical management of such patients hinges on judicious use of thromboprophylaxis and vigilance to identify evolving stenoses. Invasive revascul...
“…Manlhiot et al proposed a classification scheme based solely on Z scores using the formulas provided in the study from the National Heart, Lung, and Blood Institute Pediatric Heart Network. 139,140 One potential limitation of this study is that regression formulas for the LAD were used to derive Z scores for the left circumflex branch (normal values for the circumflex are not available with the Z-score system that was used). A classification scheme based solely on Z scores was proposed, which has been adapted and recommended in these guidelines:…”
Section: Classification Of Coronary Artery Abnormalitiesmentioning
confidence: 99%
“…158,159,175 An even greater percentage of patients (30%) will be classified as having coronary artery dilation when a Z-score cut point of 2.0 is used to define dilation. 111,140,176 Additional therapies of potential benefit are discussed below, but optimal treatment awaits delineation of the specific agent(s)/trigger(s) and pathogenesis of KD. ; thus, ibuprofen generally should be avoided in children with coronary artery aneurysms taking ASA for its antiplatelet effects.…”
BACKGROUND:Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries.
METHODS AND RESULTS:To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and longterm outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up.
CONCLUSIONS:These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances. K awasaki disease (KD) is an acute, self-limited febrile illness of unknown cause that predominantly affects children <5 years of age. When initially described, the potential for coronary artery complications was not appreciated. KD is now the most common cause of acquired heart disease in children in developed countries. In the absence of pathognomonic tests, the diagnosis continues to rest on the identification of principal clinical findings and the exclusion of other clinically similar entities with known causes. Timely initiation of treatment with intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery aneurysms defined from absolute luminal dimensions from 25% to ≈4%. Ongoing studies with additional therapies have not substantially reduced this residual risk. The long-term prognosis is determined by the initial and current level of coronary artery involvement. Certain subsets of patients are at risk for myocardial ischemia from coronary artery thrombosis and stenoses. Medical management of such patients hinges on judicious use of thromboprophylaxis and vigilance to identify evolving stenoses. Invasive revascul...
“…We defined CAA by worst-ever z-scores: CA dimensions as standard deviation units normalized for basal surface area. 20, 21 We choose to define the CAA by their worst-ever score because even when the lumen of a previously affected coronary artery has returned to its normal size, the artery can still be damaged and thus the initial systemic vasculitis was clearly more severe when compared with children with normal-sized arteries who had never had any enlargement at all. CAA was defined as a coronary z-score ≥2.5, a giant aneurysm was defined as a z-score ≥10 or a diameter ≥8 mm.…”
“…Una lesión con un puntaje Z ≥ 10 debería tratarse como un aneurisma gigante por más que su valor absoluto sea menor de 8 mm. 37 Evaluación ecocardiográfica de lesiones no coronarias: se debe determinar el diámetro del VI en sístole y diástole, además de su fracción de acortamiento y de eyección. 38 La insuficiencia mitral se encuentra en un 27% de los pacientes; la insuficiencia aórtica, en 5%; la dilatación de la raíz aórtica, en 10%; 38 la pericarditis, en 5%; y algunos pueden desarrollar taponamiento cardíaco.…”
Section: Clasificación Ecocardiográfica De Las Lesiones Coronariasunclassified
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