Biopsy-proven myocardial sequels in Kawasaki disease with giant coronary aneurysms

Yonesaka, Susumu; Takahashi, Toru; Eto, Shuji; Sato, Takumi; Otani, Katuki; Ueda, Tomomi; Sato, Akira; Kitagawa, Yosuke; Konno, Yasuhiko; Kinjo, Manabu

doi:10.1017/s1047951109991132

Cited by 34 publications

(24 citation statements)

References 15 publications

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“…[294][295][296] In a more recent study of 16 patients with giant aneurysms, initial biopsy samples showed myocyte degeneration, hypertrophy, and inflammatory cell infiltration, whereas follow-up biopsy specimens showed myocyte disarray, interstitial fibrosis, and ongoing inflammatory cell infiltration. 297 The pathogenesis of the abnormalities was not clear, but the sites of the aneurysms were not related to the biopsy findings. Subsequent studies of myocardial characterization and function have reported variable findings.…”

Section: Myocardial 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

2,876

4,124

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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: Myocardial 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

2,876

4,124

View full text Add to dashboard Cite

show abstract

“…reported myocardial changes including hypertrophy, degeneration of myocytes, and interstitial fibrosis in the majority of biopsies obtained at least 3 years after, at various time points after the acute illness and their presence was more frequent in patients who had suffered coronary artery aneurysms during the acute phase. [19] Yutani et al . had previously found myocardial abnormalities including lymphocyte and plasma cell infiltration, myocardial fibrosis, and disarray of myocardial fibers in Kawasaki disease convalescent patients.…”

Section: Discussionmentioning

confidence: 99%

Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence

Bratis

Hachmann

Child

et al. 2017

Annals of Pediatric Cardiology

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Objective:The objective of this study was to determine whether left ventricular (LV) myocardial deformation indices can detect subclinical abnormalities in Kawasaki disease convalescence. We hypothesized that subclinical myocardial abnormalities due to inflammation represent an early manifestation of the disease that persists in convalescence.Background:Myocardial inflammation has been described as a global finding in the acute phase of Kawasaki disease. Despite normal systolic function by routine functional measurements, reduced longitudinal strain and strain rate have been detected by echocardiography in the acute phase.Methods and Results:Peak systolic LV myocardial longitudinal, radial, and circumferential strain and strain rate were examined in 29 Kawasaki disease convalescent patients (15 males; mean [standard deviation] age: 11 [6.6] years; median interval from disease onset: 5.8 [5.4] years) and 10 healthy volunteers (5 males; mean age: 14 [3.8] years) with the use of cardiac magnetic resonance (CMR) feature tracking. Routine indices of LV systolic function were normal in both groups. Comparisons were made between normal controls and (i) the entire Kawasaki disease group, (ii) Kawasaki disease subgroup divided by coronary artery involvement. Average longitudinal and circumferential strain at all levels was lower in patients compared to normal controls. In subgroup analysis, both Kawasaki disease patients with and without a history of coronary involvement had similar longitudinal and circumferential strain at all levels and lower when compared to controls. There were lower circumferential and longitudinal values in Kawasaki disease patients with persisting coronary artery lesions when compared to those with regressed ones.Conclusion:In this CMR study in Kawasaki disease convalescent patients with preserved routine functional indices, we detected lower circumferential and longitudinal strain values compared to normal controls, irrespective of the coronary artery status.

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“…In general, myocardial fibrosis is related to either replacement fibrosis after myocardial infarction or interstitial fibrosis as a sequela of myocardial inflammation, which is universally present during acute KD according to endomyocardial biopsy studies from Japan [4, 5, 28]. A systematic study of 29 KD patients who died within the first 40 days after fever onset demonstrated myocarditis with varying degrees of infiltration of inflammatory cells in all cases [29].…”

Section: Discussionmentioning

confidence: 99%

Cardiovascular pathology in 2 young adults with sudden, unexpected death due to coronary aneurysms from Kawasaki disease in childhood

Shimizu¹,

Sood²,

Lau³

et al. 2015

Cardiovascular Pathology

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Purpose Coronary artery aneurysms (CAA) may remain silent after Kawasaki disease (KD) until adulthood when myocardial ischemia can lead to sudden death. We postulated that there would be young adults with sudden, unexpected death due to CAA from KD who would have a state-mandated autopsy performed by the San Diego County Medical Examiner’s Office (SDCMEO). Methods We reviewed all autopsy cases <35 years of age from 1997–2012 at the SDCMEO with a cardiovascular cause of death (n=154). Results We found 2 cases meeting inclusion criteria. Case 1 was a 22-year-old Korean male with chronic ischemic changes due to a partially occluded and diffusely calcified 15mm aneurysm at the bifurcation of the left main coronary artery. Interview of the mother revealed that this patient had been diagnosed with KD complicated by giant aneurysms at age two years. Case 2 was a 30-year-old Hispanic male with myocardial infarction due to thrombosis of a calcified left anterior descending artery aneurysm. Histologic findings included diffuse myocardial fibrosis and a recanalized aneurysm in the right coronary artery. Interview of the family revealed a KD-compatible illness in childhood. Immunohistochemical staining showed expression of transforming growth factor β pathway molecules in the aneurysmal arterial wall. Conclusions In a medical examiner’s office serving a population of approximately 3 million people, 2 of 154 (1.3%) cardiovascular deaths in persons <35 years were attributed to cardiovascular complications of KD in childhood. Antecedent KD should be considered in the evaluation of all cases of sudden, unexpected death in young adults.

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Biopsy-proven myocardial sequels in Kawasaki disease with giant coronary aneurysms

Cited by 34 publications

References 15 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

Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence

Cardiovascular pathology in 2 young adults with sudden, unexpected death due to coronary aneurysms from Kawasaki disease in childhood

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