Mucocutaneous lymph node syndrome (Kawasaki disease): Delayed aortic and mitral insufficiency secondary to active valvulitis

Gidding, Samuel S.; Shulman, Stanford T.; Ilbawi, Michel N.; Crussi, Frank; Duffy, C. Elise

doi:10.1016/s0735-1097(86)80354-0

Cited by 48 publications

(17 citation statements)

References 9 publications

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“…Late-onset valvulitis of the mitral and aortic valves can occur very rarely and may require valve replacement. 263,287 MR that becomes severe or that persists into late phases of KD appears to occur with lower frequency and can result from persistent ischemia or, rarely, from more resistant inflammatory processes that result in fixed structural abnormalities of the valve apparatus. 287,288 This circumstance indicates more serious valvular pathology and may require surgical intervention.…”

Section: Valvular Regurgitationmentioning

confidence: 99%

“…263,287 MR that becomes severe or that persists into late phases of KD appears to occur with lower frequency and can result from persistent ischemia or, rarely, from more resistant inflammatory processes that result in fixed structural abnormalities of the valve apparatus. 287,288 This circumstance indicates more serious valvular pathology and may require surgical intervention.AR in KD is usually associated with aortic root dilation (as indicated by an increased aortic sinus Z score) and becomes apparent early in the course of the disease. AR is of lesser severity and appears to persist more consistently, and it is less reliably associated with other inflammatory markers.…”

mentioning

confidence: 99%

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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: Valvular Regurgitationmentioning

confidence: 99%

mentioning

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

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“…30 Mitral regurgitation without coronary arterial lesions may also occur during the acute or convalescent stage of Kawasaki disease, in which the histopathologic findings indicate the involvement of endocarditis and valvulitis. [31][32][33] In our patients, no signs of myocardial ischemia or infarction were detected. Endocarditis or valvulitis in Kawasaki is a potential cause of the chordal rupture.…”

Section: Causes Of the Diseasementioning

confidence: 62%

Acute Rupture of Chordae Tendineae of the Mitral Valve in Infants

et al. 2014

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Background— Recently, infant cases of acute heart failure attributable to rupture of the mitral chordae tendineae have been reported. However, little is known about the pathogenesis and clinical course of this condition. Methods and Results— Ninety-five children with rupture of mitral chordae tendineae were identified in nationwide surveys of Japan diagnosed from 1995 to 2013. The clinical manifestations, management strategies, and prognosis were investigated. Eighty-one (85%) patients were between 4 and 6 months (median, 5 months) of age. In 63 (66%) patients, rupture occurred during the spring or summer. The underlying conditions before rupture included Kawasaki disease (10 cases), maternally derived anti-SSA antibodies (2 cases), and infective endocarditis (1 case). Surgery was performed in 80 patients (94 operations), and the final operations included plasty of mitral chordae in 52 cases and mechanical valve replacement in 26 cases. The histopathologic examinations of the mitral valves and chordae (n=28) revealed inflammatory reactions with predominant mononuclear cell infiltration in 18 cases (64%) and increased fibrous and myxoid tissue in 11 cases (39%), suggesting that nonbacterial infectious or autoimmune endocarditis and myxoid changes are involved in the pathogenesis. Eight patients (8.4%) died before (n=6) and shortly after (n=2) the operation, and significant neurological complications persisted in 10 cases (11%). Conclusions— Acute heart failure attributable to rupture of the mitral chordae tendineae in infants is a unique disease resulting from diverse causes. This condition should be recognized as a significant cardiovascular disorder that may cause sudden onset of cardiogenic shock and death in infants.

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“…Other investigators have observed a much lower incidence of aortic regurgitation in the acute phase, 2 but late-onset aortic regurgitation has been reported as an exceedingly rare finding after Kawasaki disease and may be associated with the need for aortic valve replacement. 2,138,140 Approximately 4% of a consecutive series with Kawasaki disease had mild aortic regurgitation as seen by echocardiogram. 100…”

Section: Valvular Regurgitationmentioning

confidence: 99%

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association

Newburger¹,

Takahashi²,

Gerber³

et al. 2004

Pediatrics

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1,697

2,533

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ABSTRACT. Background. Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in ϳ15% to 25% of untreated children and may lead to ischemic heart disease or sudden death.Methods and Results. A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for >5 days and <4 classic criteria should undergo electrocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor-␣ antagonists, and abciximab. Long-term management of patients with Kawasaki disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac disease are classified according to risk strata.Conclusions. Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients. Pediatrics

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Mucocutaneous lymph node syndrome (Kawasaki disease): Delayed aortic and mitral insufficiency secondary to active valvulitis

Cited by 48 publications

References 9 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

Acute Rupture of Chordae Tendineae of the Mitral Valve in Infants

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association

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