| Abstract|| |
Although coronary artery aneurysms occur in Kawasaki disease, giant aneurysms are rare. We report a very large coronary artery aneurysm, measuring 25 mm and involving left anterior descending artery, in a 2-year-old child with Kawasaki disease. The challenges in management of such a patient have been highlighted.
Keywords: Coronary artery thrombus, giant aneurysm, Kawasaki′s disease
|How to cite this article:|
Patil S, Shirodkar S, Pinto RJ, Dalvi B. Giant coronary artery aneurysm with a thrombus secondary to Kawasaki disease. Ann Pediatr Card 2008;1:59-61
|How to cite this URL:|
Patil S, Shirodkar S, Pinto RJ, Dalvi B. Giant coronary artery aneurysm with a thrombus secondary to Kawasaki disease. Ann Pediatr Card [serial online] 2008 [cited 2023 Jan 27];1:59-61. Available from: https://www.annalspc.com/text.asp?2008/1/1/59/41059
| Clinical Summary|| |
A 2-year-old girl presented with fever, irritability, and a maculopapular rash of 7-day duration. On examination, she was febrile and had tachycardia, palmar and plantar erythema, cervical lymphadenopathy, bilateral conjunctival injection with congestion of oral mucosa and throat. Her systemic examination was normal. Her hemoglobin was 11.2 g/dl and total white blood cell count was 16,100/mm 3 with ESR of 60 mm at the end of 1 hour. Her platelets were 4.2 x 10 5 /mm 3 . A diagnosis of Kawasaki disease was made based on her clinical presentation and investigations. She was started on intravenous immunoglobulins (IVIg) and high-dose aspirin, after which she was noted to have symptomatic and clinical improvement. Her echocardiography done on admission revealed a giant aneurysm of proximal left anterior descending artery (LAD) measuring around 18 mm with evidence of a thrombus [Figure 1]. She was started on warfarin and the dose was adjusted to maintain INR of about 2.5. A follow-up echocardiogram, done 2 months later, revealed increase in the size of aneurysm (22 mm) with complete resolution of thrombus [Figure 2]. A subsequent echocardiogram after 6 months did not reveal any thrombus, but the aneurysm size increased to approximately 25 mm. She, however, remained asymptomatic throughout her follow-up period. In view of the progressive increase in the size of the aneurysm, coronary angiography was performed to delineate the coronary anatomy, which may prompt more aggressive therapeutic options. Her coronary angiography revealed a giant aneurysm (25 mm) just after the origin of LAD with swirling of contrast within the aneurysm [Figure 3]. There was a delayed and faint opacification of the distal LAD [Figure 4]. Left main coronary artery (LMCA) and left circumflex coronary artery (LCX) were normal; right coronary artery (RCA) was dominant and normal, supplying collaterals to the LAD [Figure 5].
| Discussion|| |
Cardiovascular sequelae of Kawasaki disease include asymptomatic coronary artery ectasia or aneurysm formation, giant coronary artery aneurysms with thrombosis, myocardial infarction, and sudden death. They have been reported in 15-25% of the untreated children.  With treatment using IVIg within first 10 days of the disease, the incidence drops down to about 5%.  In those who fail to respond to the initial treatment with IVIg, the administration of steroids is associated with an improvement in symptoms and absence of a significant progression in coronary artery abnormalities.  The development of myocarditis, congestive heart failure, pericarditis with pericardial effusion, mitral or aortic insufficiency, and arrhythmias is known to occur during the early course of the disease. Coronary artery dilatation can be seen as early as 4 days after the first appearance of fever. It peaks at approximately 4 weeks after the onset of illness.  In our patient, the coronary artery involvement was detected about 7 days after the onset of symptoms.
Aneurysms, which occur during the early phase of Kawasaki disease, are known to involve the proximal segments of the major coronary arteries. The commonest sites of aneurysms in the order of frequency include the proximal LAD as was seen in our patient, proximal RCA, followed by the LMCA, LCX, and finally the distal RCA. The aneurysms are classified as small (<5-mm internal diameter), medium (5-8 mm internal diameter), or giant (>8-mm internal diameter).  Approximately 50% of these lesions regress within 5 years. In the majority of those with small coronary artery aneurysms (3-4 mm), regression occurs within 2 years.  The risk of aneurysm is increased in patients with fever lasting for more than 16 days, those who have recurrence of fever after an afebrile period of at least 48 h, and those younger than 1 year. Some laboratory values predictive of aneurysm development include low hematocrit levels, thrombocytopenia, and elevated neutrophil/band counts. 
The resolution of coronary artery aneurysms depends on the initial size of aneurysm (<5 mm is more likely to resolve than >8 mm), age of the patient at the onset of disease (more likely to resolve in those <1 year age), morphology (fusiform more likely to regress than saccular aneurysm), and location of the aneurysm (distal more likely to involute as compared to the proximal). 
All patients with Kawasaki disease should undergo echocardiography on diagnosis and 6-8 weeks after the onset of the disease. Those with giant aneurysms may require a stress test and possibly coronary angiography to identify stenotic lesions.  Our patient was too young to perform a stress test. We proceeded with coronary angiography in view of progressive increase in the size of the aneurysm as was evident on serial echocardiography. In our patient, discrete narrowing of LAD was not very well appreciated due to massive aneurysm located just proximal to it. However, a significant reduction in the LAD flow was evident by the poor distal flow associated with delayed filling of the LAD. An extensive collateral supply from the RCA was also a pointer toward significant stenosis of the LAD.
The addition of warfarin to aspirin therapy has been recommended for those with giant aneurysms as was done in this patient. Attempts at excision or plication of the aneurysm have not been successful and have even caused deaths. The arterial graft patency rate in later adult life is still unknown. Percutaneous coronary intervention with placement of a covered stent was not feasible in our patient in view of small vessel size and potential for the artery to grow. In view of these limitations and in the absence of any obvious evidence of myocardial ischemia, it was decided to treat this patient conservatively at the present time. Management of such patients remains a dilemma due to rarity of such giant aneurysms and a very limited data available on the surgical and catheter interventions.
| References|| |
|1.||Barron K. Kawasaki disease: Etiology, pathogenesis and treatment. Cleve Clin J Med 2002;69:SII69-78. |
|2.||Newburger J, Takahashi M, Gerber M, Gewitz M, Tani L, Burns J, 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 young American Heart Association. Circulation 2004;110:2747-71. |
|3.||Wallace C, French J, Kahn S, Sherry D. Initial intravenous gamma-globulin treatment failure in Kawasaki disease. Pediatrics 2000;105:E78. |
|4.||Kato H, Sugimura T, Akagi T. Long form consequences of Kawasaski disease: A 10 to 21 year follow up of 594 patients. Circulation 1996;94:1379-85. |
|5.||Brogan PA, Bose A, Burgner D, Shingadia D, Tulloh R, Michie C, et al. Kawasaki disease: An evidence based approach to diagnosis, treatment and proposals for future research. Arch Dis Child 2002;86:286-90. [PUBMED] [FULLTEXT]|
|6.||Shulman ST, Inocencio J, Hirsch R. Kawasaki disease. Pediatr Clin North Am 1995;42:1205-22. [PUBMED] |
|7.||Daniels SR, Specker B, Capannari TE, Schwartz DC, Burke MJ, Kaplan S. Correlates of coronary artery aneurysm formation in patients with Kawasaki disease. Am J Dis Child 1987;141:205-7. [PUBMED] |
|8.||Takahashi M, Mason W, Lewis AB. Regression of coronary aneurysms in patients with Kawasaki syndrome. Circulation 1987;75:387-94. [PUBMED] |
|9.||Newburger JW. Kawasaki disease: Current treatment options. Cardiovasc Med 2000;2:227-36. |
Robin J Pinto
Glenmark Cardiac Centre, 10, Nandadeep, 209 D, Dr Ambedkar Road, Matunga (E), Mumbai - 400 019, Maharashtra
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]