British Journal of Radiology (2006) 79, e133-e136
© 2006 British Institute of Radiology
doi: 10.1259/bjr/16077689
Giant coronary artery aneurysm secondary to Kawasaki disease: diagnosis in an adult by multi-detector row CT coronary angiography
N E Manghat, MBChB, MRCP, FRCR
1
G J Morgan-Hughes, MBChB, MRCP, MD
2
I D Cox, MA, MD, MRCP
2 and
C A Roobottom, MBChB(Hon), MRCP, FRCR
1
Departments of 1Clinical Radiology, 2Cardiology, Derriford Hospital, Plymouth, Devon PL6 8DH, UK
 |
Abstract
|
|---|
We present the case of an adult female patient with a giant coronary artery aneurysm secondary to Kawasaki disease diagnosed for the first time, as far as the authors are aware, on multi-detector row computed tomography (MDCT). The long-term complications relate to the persistence of these aneurysms with giant coronary aneurysms having the lowest regression rate, the highest risk of stenosis and strongest association with myocardial infarction. MDCT coronary angiography represents an ideal, alternative non-invasive imaging modality for the diagnosis and follow-up of the coronary arterial complications of Kawasaki disease, thereby avoiding invasive coronary imaging, and its use in the management algorithm should be considered. We also aim to contribute to the expanding clinical role of MDCT coronary imaging.
|
Case report
|
|---|
A 41-year-old woman presented with a 9-month history of tightness around the chest that she felt was related to stress. Symptoms were particularly noted at rest in bed at night and during exertion. She was otherwise fit and well, had a positive family history of coronary disease, although it was noted that she had a previous history of Kawasaki disease diagnosed when she was 6 years old; there was no available history suggestive of subsequent complications and no previous follow-up.
Blood pressure was 108/68 mmHg with a regular pulse rate of 63 beats per minute. An electrocardiogram (ECG) demonstrated sinus rhythm with a short PR interval.
Since we have established the use of multi-detector computed tomographic (MDCT) coronary angiography in our institution, the decision was made to screen this patient for coronary artery aneurysm disease using MDCT coronary angiography to avoid initial diagnostic invasive coronary angiography.
The MDCT coronary angiogram (
Figures 1 and 2) revealed a left coronary artery with two aneurysms. The largest was in the distal left main stem measuring 9 mm in diameter, saccular and partially calcified; it involved the origins of both the left anterior descending artery and the circumflex artery. A second aneurysm was present at the origin of the first diagonal artery measuring 6 mm in diameter. There were no distal aneurysms and the right coronary artery was dominant and normal.
View larger version (64K):
[in this window]
[in a new window]
|
Figure 1. (a) Axial section contrast-enhanced CT demonstrating a giant (9 mm) aneurysm of the distal left main stem artery and a further moderate-sized (6 mm) aneurysm of the left anterior descending and circumflex artery origins. (b) A "curved multiplanar reformation (MPR)" helps to more accurately demonstrate the calcified, saccular nature of the giant aneurysm and morphology of the more distal aneurysm.
|
|
View larger version (54K):
[in this window]
[in a new window]
|
Figure 2. This volume rendered(VR) three-dimensional (3D) coronary arterial tree aids the localization and morphological evaluation of the aneurysms
|
|
The patient is currently stable on medical management with a 6 month follow-up MDCT planned.
|
Discussion
|
|---|
Background
Kawasaki disease is a generalized vasculitis of unknown aetiology. In 1967, Tomisaku Kawasaki described this illness, also termed "mucocutaneous lymph node syndrome", which had been affecting infants and young children in Japan [1]. Most cases occur between the ages of 6 months and 8 years with an incidence of 40–150 cases per 100 000 in those <5 years in Japan and 1:10 000 in the UK [2].
Aetiology is believed to be infectious although no specific agent has been consistently isolated. Diagnosis requires fever and four out of five principal clinical features (conjunctivitis; erythematous lips, oral cavity, palms and soles; polymorphous exanthema of the body trunk; swelling of the cervical lymph nodes) or if fewer than four, patients should also have documented coronary artery disease diagnosed by echocardiography or coronary angiography [3].
|
Cardiac involvement
|
|---|
Cardiac manifestations of Kawasaki disease in the acute phase include pericarditis, myocarditis, endocarditis, inflammation of the conduction system and coronary artery involvement, with 1–2% of patients presenting with sudden death due to cardiac failure [4]. Myocardial infarction is also a potential complication usually occurring in the first year and at post-mortem; these infants are found to have coronary arteritis with associated thrombosis and aneurysm formation. In a large Japanese study, 25% of patients with acute Kawasaki disease were shown by coronary angiography to have coronary aneurysms [5].
In the chronic phase, the long-term complications relate to the persistence of these aneurysms, the development of thrombotic occlusion, the risk of ischaemic heart disease and premature atherosclerosis [5]. Aneurysms detected after the acute stage regress in 
50% of cases with small (<5 mm) to moderate (5–8 mm) sized aneurysms more likely to regress, with approximately 1% of patients who recover from acute Kawasaki disease developing giant coronary artery aneurysms (>8 mm in diameter) or coronary artery obstruction due to thrombosis or stenosis [6]. Giant coronary aneurysms have the lowest regression rate, the highest risk of stenosis and strongest association with myocardial infarction [7, 8].
Treatment of the acute stages includes the use of salicylates and intravenous gamma globulin therapy, which when instituted before the 10th day of illness has reduced both the morbidity of Kawasaki disease and the apparent incidence of coronary artery abnormalities from approximately 25% to less than 5% at 6–8 weeks after initiation of therapy [9]. In most patients, coronary ectasia or aneurysms regress within 1–2 years [10]. Systemic aneurysms develop in 2% of patients, most notably the axillary and iliac arteries.
|
Follow-up
|
|---|
Children with suspected Kawasaki disease should have their coronary arteries screened as soon as possible with echocardiography and ECG to look for ischaemic or dysrhythmic changes, and repeat coronary imaging at 10–14 days to look for new lesions [11]. It has been previously uncertain as to whether children who have had Kawasaki disease are predisposed to atherosclerosis in adulthood since there is an absence of prospective or retrospective long-term follow-up data. Long-term management of patients with Kawasaki disease depends on the degree of coronary arterial involvement.
American Heart Association (AHA) guidelines for long-term management of these patients include appropriate interval echocardiography, exercise stress testing of patients with known coronary disease and coronary angiography on those with a positive stress test [11]. However, visualization of coronary arteries on echocardiography becomes progressively more difficult as a child grows. The preliminary examination establishes a baseline for longitudinal follow-up.
The usefulness of coronary angiography is limited in that it does not specifically detect intramural changes in the coronary artery; with post-mortem examinations of some patients with angiographically documented regression of coronary artery aneurysms revealing intimal proliferation and fibrosis not apparent on angiogram [12, 13]. Intravascular imaging of the coronary arteries may allow more detailed visualization of coronary wall morphology and the healing process.
The frequency of follow-up visits for patients with a giant or medium solitary aneurysm or multiple aneurysms is based on the patient's clinical condition. If non-invasive studies or clinical symptoms suggest myocardial ischaemia, then radioisotope myocardial perfusion scanning, coronary angiography, or both, are indicated.
Patients with no evidence of aneurysm or ectasia at the 1-year evaluation are not likely to benefit from further repeated echocardiography, although patients with transient coronary artery ectasia early in the illness may be followed up at 3–5 year intervals [11].
MDCT is currently not part of the management algorithm in Kawasaki disease. Over the last 2 years, MDCT cardiac imaging has evolved from the research setting to become a useful clinical tool. The improvements in spatial and temporal resolution, sophisticated ECG gating and post-processing software algorithms allow motion-free, fast, accurate and detailed contrast-enhanced coronary imaging that rivals the accuracy of traditional invasive and non-invasive diagnostic techniques [14–17]. MDCT not only has the ability to demonstrate changes in luminal calibre, it can also assess intramural changes, plaque morphology and characterize plaque [18].
MRI can also assess coronary morphology with low temporal resolution (20–50 ms). However, the three-dimensional spatial resolution achievable is marginal for coronary arterial imaging and is therefore not yet reliable [19, 20]. Nonetheless, at centres with appropriate expertise and resources, the exclusion of severe proximal multivessel coronary artery disease in selected patients, Kawasaki disease and coronary aneurysm identification are among those for whom coronary MR angiography has demonstrated clinical usefulness [21, 22]. It may prove to be of value for the serial non-invasive evaluation of these patients, particularly in the paediatric population where MDCT poses a significant radiation burden.
|
Conclusion
|
|---|
This case illustrates for the first time, as far as the authors are aware, that coronary artery aneurysms associated with Kawasaki disease can be elegantly demonstrated by MDCT coronary angiography. Although probably unsurprising, given its increasingly prominent clinical role, MDCT coronary angiography represents an ideal, alternative non-invasive imaging modality for the diagnosis and follow-up of the coronary arterial complications of Kawasaki disease in adults, thereby avoiding invasive coronary imaging, and its use in the management algorithm should be considered. We also aim to contribute to the expanding clinical role and pathological imaging features of MDCT coronary angiography.
|
Acknowledgments
|
|---|
"The Royal College of Radiologists Research Fellowship Scheme 2004/5" funds the first author to study "The Clinical Applications of Cardiac CT". General Electric Medical Systems for providing the cardiac software package on our 16-detector row "Lightspeed" scanner. Thanks to the Medical Photography Department, Derriford Hospital for help in image preparation.
Received for publication August 2, 2005.
Revision received November 30, 2005.
Accepted for publication December 1, 2005.
|
References
|
|---|
- Kawasaki T. Pediatric acute mucocutaneous lymph node syndrome: clinical observation of 50 cases. Jpn J Allerg 1967;16:178–222.
- Harnden A, Alves B, Sheikh A. Rising incidence of Kawasaki disease in England: analysis of hospital admission data. Br Med J 2002;324:1424–5.[Free Full Text]
- Kawasaki T, Kosaki F, Okawa S, et al. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics 1974;54:271–6.[Abstract/Free Full Text]
- Fujiwara H, Hamashima Y. Pathology of the heart in Kawasaki disease. Pediatrics 1978;61:100–7.[Abstract/Free Full Text]
- Kato H, Sugimura T, Agaki T, et al. Long-term consequences of Kawasaki Disease. A 10- to 21-year follow-up study of 594 patients. Circulation 1996;94:1379–85.[Abstract/Free Full Text]
- Suzuki A, Kamiya T, Yasuo O, Kuroe K. Extended long term follow-up study of coronary arterial lesions in Kawasaki disease. J Am Coll Cardiol 1991;17:33A
- Kato H, Ichinose E, Yoshioka F, Takechi T, Matsunaga S, Suzuki K, et al. Fate of coronary aneurysms in Kawasaki disease: serial coronary angiography and long-term follow-up study. Am J Cardiol 1982;49:1758–66.[CrossRef][Medline]
- Suzuki A, Kamiya T. Visualization of coronary artery lesions in Kawasaki disease by angiography. Cardiol Young 1991;1:225–33.
- Newburger JW, Takahashi M, Burns JC, Beiser AS, Chung KJ, Duffy CE, et al. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med 1986;315:341–7.[Abstract]
- Akagi T, Rose V, Benson LN, Newman A, Freedom RM. Outcome of coronary artery aneurysms after Kawasaki disease. J Pediatr 1992;121:689–94.[CrossRef][Medline]
- Dajani AS, Taubert KA, Takahashi M, et al. Guidelines for long-term management of patients with Kawasaki disease. Circulation 1994;89:916–22.[Abstract/Free Full Text]
- Suzuki A, Kamiya T, Ono Y, Kohata T, Kimura K, Takamiya M. Follow-up study of coronary artery lesions due to Kawasaki disease by serial selective arteriography in 200 patients. Heart Vessels 1987;3:159–65.[CrossRef][Medline]
- Naoe S, Takahashi K, Masuda H, Tanaka N. Coronary findings post Kawasaki disease in children who died of other causes. In: Shulman ST, editor. Kawasaki Disease. New York, NY: Alan R. Liss, 1987:341–6
- Mollet NR, Cademartiri F, Nieman K, et al. Multislice spiral computed tomography coronary angiography in patients with stable angina pectoris. J Am Coll Cardiol 2004;43:2265–70.[Abstract/Free Full Text]
- Kuettner, Trabold T, Schroeder S, et al. Noninvasive detection of coronary lesions using 16-detector multislice spiral computed tomography technology: initial clinical results. J Am Coll Cardiol 2004;44:1230–7.[Abstract/Free Full Text]
- Martuscelli E, Romagnoli A, D'Eliseo A, et al. Accuracy of thin-slice computed tomography in the detection of coronary stenoses. Eur Heart J 2004;12:1043–8.
- Morgan-Hughes GJ, Roobottom CA, Owens PE, Marshall AJ. Highly accurate coronary angiography using sub-millimetre computed tomography. Heart 2005;91:308–13.[Abstract/Free Full Text]
- Schroeder S, Kopp AF, Baumbach A, et al. Non-invasive detection and evaluation of atherosclerotic plaques with multislice CT. J Am Coll Cardiol 2001;37:1430–5.[Abstract/Free Full Text]
- Wielopolski PA, van Geuns RJM, de Feyter PJ, Ouderk M. Coronary arteries. Eur Radiol 1998;8:873–85.[CrossRef][Medline]
- Appelbaum E, Botnar RM, Yeon SB, Manning WJ. Coronary magnetic resonance imaging: current state-of-the-art. Coron Artery Dis 2005;16:345–53.[CrossRef][Medline]
- Danias PG, Stuber M, Botnar RM, Kissinger KV, Yeon SB, Rofsky NM, et al. Coronary MR angiography clinical applications and potential for imaging coronary artery disease. Magn Reson Imaging Clin N Am 2003;11:81–99.[CrossRef][Medline]
- Mavrogeni S, Papadopoulos G, Douskou M, Kaklis S, Seimenis I, Baras P, et al. Magnetic resonance angiography is equivalent to X-ray coronary angiography for the evaluation of coronary arteries in Kawasaki disease. J Am Coll Cardiol 2004;43:649–52.[Abstract/Free Full Text]
Top
Abstract
Case report
