Coronary Artery Dilation in Acute Kawasaki Disease and Acute Illnesses Associated With Fever

Bratincsák, András; Reddy, Venu; Purohit, Prashant; Tremoulet, Adriana H.; Molkara, Delaram; Frazer, Jeffrey R.; Dyar, Dan; Bush, Ruth A.; Sim, James Y.; Sang, New; Burns, Jane C.; Melish, Marian A.

doi:10.1097/inf.0b013e31826252b3

Cited by 40 publications

(29 citation statements)

References 19 publications

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“…Of note, febrile non-KD patients had lower white blood cell counts and ESR than KD patients. No febrile patients reported by Bratincsak et al 96 had a coronary artery Z score >2.5, but their duration of fever and degree of systemic inflammation were not described. Taken together, these studies suggest that cut points between 2.0 and 2.5 might reliably differentiate coronary artery involvement secondary to KD, with a Z score ≥2.5 differentiating KD with a 98% specificity.…”

Section: Impact Of Fevermentioning

confidence: 99%

See 1 more Smart Citation

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

McCrindle¹,

Rowley²,

Newburger³

et al. 2017

Circulation

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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...

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Section: Impact Of Fevermentioning

confidence: 99%

“…However, it is unclear whether such reductions in dimensions represent resolution of inflammatory changes in the arterial walls or hemodynamic or functional factors related to fever and circulating inflammatory mediators. 95,96 by guest on http://circ.ahajournals.org/…”

Section: Coronary Artery Abnormalitiesmentioning

confidence: 99%

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

McCrindle¹,

Rowley²,

Newburger³

et al. 2017

Circulation

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2,757

4,124

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“…Although mild transient coronary dilation may occur with some febrile illnesses, these typically do not reach z scores >2.5. 11,12 Thus, this case probably represents asymptomatic incomplete KD.…”

Section: Discussionmentioning

confidence: 99%

Asymptomatic Kawasaki Disease in a 3-Month-Old Infant

et al. 2016

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Kawasaki disease (KD) is the leading cause of acquired heart disease in children in the United States. It is a systemic vasculitis characterized by diffuse inflammation of medium and small blood vessels. If untreated it can lead to myocardial infarction, ischemic heart disease, or sudden death. Early recognition and treatment decrease the incidence of coronary consequences, resulting in improved clinical outcomes. Incomplete KD is much less likely to fulfill major clinical diagnostic criteria. Infants <12 months of age are more likely to have an incomplete presentation, and children <6 months of age are more likely to develop cardiac complications. We present a case of a 3-month-old, previously healthy white boy who was noted to have a new transient cardiac murmur during a routine health assessment. He was completely asymptomatic, and physical examination was otherwise within normal limits. An echocardiogram was performed and showed abnormal dilation of several coronary arteries, consistent with the coronary ectasia associated with KD. Laboratory evaluation was significant for values suggestive of systemic inflammation. Based on these results, a presumed diagnosis of incomplete KD was made and treatment administered. Close surveillance was undertaken, and serial laboratory studies and imaging showed gradual resolution of inflammatory markers and cardiac ectasia. This unique case of incomplete KD without any of the physical signs normally associated with the disease emphasizes the spectrum of presentation and the possibility of missing a diagnosis of incomplete disease, reinforcing the need to remain vigilant.

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“…24,25 Kawasaki disease should receive IVIg and high-dose aspirin before the 10th day of fever. 13,14 The finding of dilated coronary arteries in a febrile child is considered to be a good indicator of Kawasaki syndrome , as recommended by the American Heart Association and supported by Bratincsak et al 15,16 A comparison was made between 145 children with Kawasaki syndrome and 45 children with fever (36 with infectious illness and 9 with a self-limiting febrile condition); they found no children in the non-Kawasaki syndrome group with a coronary artery z score of $2.5 and a significant difference in overall z scores between the groups. In our patient with prolonged fever without an identifiable cause, the suspicion of incomplete Kawasaki syndrome was supported by the finding of mildly dilated proximal coronary arteries, which led to the clinical decision to treat with IVIg.…”

Section: Figurementioning

confidence: 99%

Periodic Fever in MVK Deficiency: A Patient Initially Diagnosed With Incomplete Kawasaki Disease

Thors¹,

Vastert²,

Wulffraat³

et al. 2014

Pediatrics

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Mevalonate kinase deficiency (MKD) is a rare autosomal recessive disorder causing 1 of 2 phenotypes, hyperimmunoglobulin D syndrome and mevalonic aciduria, presenting with recurrent fever episodes, often starting in infancy, and sometimes evoked by stress or vaccinations. This autoinflammatory disease is caused by mutations encoding the mevalonate kinase (MVK) gene and is classified in the group of periodic fever syndromes. There is often a considerable delay in the diagnosis among pediatric patients with recurrent episodes of fever. We present a case of an 8-week-old girl with fever of unknown origin and a marked systemic inflammatory response. After excluding infections, a tentative diagnosis of incomplete Kawasaki syndrome was made, based on the finding of dilated coronary arteries on cardiac ultrasound and fever, and she was treated accordingly. However, the episodes of fever recurred, and alternative diagnoses were considered, which eventually led to the finding of increased excretion of mevalonic acid in urine. The diagnosis of MKD was confirmed by mutation analysis of the MVK gene. This case shows that the initial presentation of MKD can be indistinguishable from incomplete Kawasaki syndrome. When fever recurs in Kawasaki syndrome, other (auto-)inflammatory diseases must be ruled out to avoid inappropriate diagnostic procedures, ineffective interventions, and treatment delay.

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Coronary Artery Dilation in Acute Kawasaki Disease and Acute Illnesses Associated With Fever

Abstract: Z-scores ≥ 2.5 SD units were not observed in our cohort of FC. Therefore, echocardiographic evidence of coronary artery dilation can be used to support the diagnosis of KD.

Cited by 40 publications

References 19 publications

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

Asymptomatic Kawasaki Disease in a 3-Month-Old Infant

Periodic Fever in MVK Deficiency: A Patient Initially Diagnosed With Incomplete Kawasaki Disease

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