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Giant coronary artery aneurysms in Kawasaki disease: the cost of a missed diagnosis

Published online by Cambridge University Press:  07 February 2013

Liliana Marta ,
Andreia Francisco  and
Rui Anjos
Liliana Marta*
Affiliation:
Department of Cardiology, Hospital de Santarém, Santarem, Portugal
Andreia Francisco
Affiliation:
Department of Pediatric Cardiology, Hospital de Santa Cruz, Lisbon, Portugal
Rui Anjos
Affiliation:
Department of Pediatric Cardiology, Hospital de Santa Cruz, Lisbon, Portugal
*
Correspondence to: Liliana Marta, Department of Cardiology, Hospital de Santarém, Avenida Bernardo Santareno, 2005-177 Santarém, Portugal. Tel: +351 243 300 872; Fax: +351 243 300 219; E-mail: liliana.marta@gmail.com
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Abstract

Giant coronary aneurysms secondary to Kawasaki disease are rare, but a very severe complication. Delayed diagnosis and appropriate treatment of the disease is a well-known risk factor for coronary aneurysms.

Keywords

Kawasaki diseasecoronary aneurysmgiant coronary aneurysm

Information

Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 23 , Issue 4 , August 2013 , pp. 608 - 609
Copyright
Copyright © Cambridge University Press 2013 

Case report

We report a case of a 2-year-old boy diagnosed with Kawasaki disease after 14 days of unremitting fever, maculopapular rash, bulbar conjunctivitis, cervical lymphadenopathy, and periungual peeling of fingers. He was seen on the 2nd day of fever by his paediatrician and treated with antibiotics. Diagnosis of Kawasaki disease and treatment with aspirin and immunoglobulin was only started on day 15 of the disease. Echocardiogram showed an aneurysm at the proximal right coronary artery (Fig 1) and left anterior descending artery. At the 9-month follow-up, cardiac catheterisation showed a giant fusiform aneurysm at the proximal segment of the left anterior descending artery (8 × 22 mm; Fig 2). The right coronary artery had a large fusiform aneurysm at the proximal segment (6 × 25 mm) and a saccular aneurysm (3.5 × 6.5 mm) more distally (Fig 2). There was no evidence of stenosis or thrombus formation. In the face of the very large dimensions of the aneurysms, we decided to treat him with long-term aspirin and warfarin. After 1 year of the initial episode, he is asymptomatic. Imaging follow-up with computed tomography angiography or repeat catheterisation is scheduled in 1–2 years.

Figure 1

Echocardiogram showing proximal right coronary artery aneurysm.

Figure 2

Coronary angiogram demonstrating a giant aneurysm at the proximal LAD and a large aneurysm at the proximal segment of the RCA and another aneurysm more distally. LAD, left anterior descending; RCA, right coronary artery.

Discussion

Kawasaki disease is an acute systemic vasculitis of unknown aetiology that affects mainly small- and medium-sized arteries, particularly the coronary arteries.Reference Newburger, Takahashi and Gerber 1 Giant coronary aneurysms are rare, occurring in 0.5–1% of adequately treated patients. Delayed treatment with intravenous immunoglobulin is a well-known risk factor for the development of coronary aneurysms. This case highlights the consequences of a missed diagnosis and late treatment, which certainly has contributed to these serious cardiovascular sequelae.

References

[1] Newburger, J, Takahashi, M, Gerber, M, et al. 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. Circulation 2004; 110: 2747–2771.CrossRefGoogle Scholar
Figure 0

Figure 1 Echocardiogram showing proximal right coronary artery aneurysm.

Figure 1

Figure 2 Coronary angiogram demonstrating a giant aneurysm at the proximal LAD and a large aneurysm at the proximal segment of the RCA and another aneurysm more distally. LAD, left anterior descending; RCA, right coronary artery.