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Table of Contents
Year : 2022  |  Volume : 11  |  Issue : 2  |  Page : 39-42

Lateral wall ischemia secondary to funnel-shaped left main coronary artery aneurysm with stenotic osteoproximal left circumflex coronary artery in a child with recent recovery from kawasaki disease: A case report and review of literature

Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

Date of Submission29-Dec-2021
Date of Decision01-Mar-2022
Date of Acceptance08-Mar-2022
Date of Web Publication27-Jul-2022

Correspondence Address:
Dr. Debasish Das
Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar - 751 019, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/rcm.rcm_70_21

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We present a case of funnel-shaped aneurysmal dilatation of left main coronary artery (LMCA) with aggressive stenosis of osteoproximal left circumflex coronary artery presenting with lateral wall coronary ischemia in a 6-year-old school-going child who recovered from Kawasaki disease (KD) 1 month back. Although aneurysmal dilatation of LMCA during first 2 months of recovery from KD is well known, with median duration for the development of stenotic lesion in coronaries after KD is approximately 6 months and all the cases of coronary stenosis described so far have been described in the presence of large coronary artery aneurysm, our case is rare to illustrate aggressive stenosis of osteoproximal left circumflex coronary artery just after 1 month of recovery in spite of the presence of small coronary artery aneurysm.

Keywords: Aneurysm, Kawasaki disease, left circumflex coronary left main coronary artery, stenosis

How to cite this article:
Das D, Banerjee A, Das T, Dixit M, Kumar A, Singh S. Lateral wall ischemia secondary to funnel-shaped left main coronary artery aneurysm with stenotic osteoproximal left circumflex coronary artery in a child with recent recovery from kawasaki disease: A case report and review of literature. Res Cardiovasc Med 2022;11:39-42

How to cite this URL:
Das D, Banerjee A, Das T, Dixit M, Kumar A, Singh S. Lateral wall ischemia secondary to funnel-shaped left main coronary artery aneurysm with stenotic osteoproximal left circumflex coronary artery in a child with recent recovery from kawasaki disease: A case report and review of literature. Res Cardiovasc Med [serial online] 2022 [cited 2022 Dec 9];11:39-42. Available from: https://www.rcvmonline.com/text.asp?2022/11/2/39/352500

  Introduction Top

Kawasaki disease (KD) presents with fever more than 5 days, generalized vasculitis, and lymphadenopathy under the children of 5 years of age.[1] Children present with red eyes, palm and soles, and within 3 weeks typical desquamation of palm and sole occurs. Even with adequate treatment of KD with aspirin and immunoglobulin, 5% of children with KD develop transient coronary dilatation and 1% of children develop coronary artery aneurysm.[2],[3],[4] Coronary artery aneurysm is first detected at a mean of 10 days of illness, and peak frequency of coronary dilatation or aneurysm occurs within 4 weeks of onset. Development of coronary stenosis occurs as a process of healing which develops later in the course of the disease with a mean time of development at 6 months. Literature report exists about the development of beaded appearance of stenosis in coronary arteries much later in the course of the disease, but osteoproximal stenosis of the left circumflex coronary artery within 1 month of recovery from illness has not yet been described.

  Case Top

A 6-year-old boy diagnosed case of KD presented to the cardiology outpatient department with complaint of chest pain while playing with peers for the last 15 days. He was admitted with fever, strawberry tongue, red palm, and soles with cervical lymphadenopathy 1 month back and was treated with aspirin and immunoglobulin. There was no previous episode of rashes or redness of eye with fever. He had a good response to intravenous immunoglobulin therapy at discharge. Erythrocyte sedimentation rate came down to 18 mm/h from 78 mm/h, TLC came down to 8600/cmm from 18,000/cmm, C-reactive protein came down to 6 mg/L from 24 mg/L, and platelet count came down to 3.4 Lacs/cmm from 5.6 Lacs/cmm. At discharge, he had dilatation of left main coronary artery (LMCA) with normal caliber left anterior descending (LAD) and left circumflex coronary artery. Electrocardiogram (ECG) revealed the presence of ST elevation in I and aVL [Figure 1] suggestive of high lateral wall coronary ischemia which normalized later with optimal medical therapy (OMT) [Figure 2]. QRS/QTc ratio was 0.27 and was within the normal limit. Cardiac troponin was negative. Echocardiography revealed no regional wall motion abnormality with normal left ventricular systolic function. Parasternal short-axis view revealed aneurysmal osteoproximal LMCA with progressive narrowing and LMCA appearing a typical funnel shape with osteoproximal narrowing of the proximal left circumflex coronary artery (LCX) (60%–70%) stenosis [Figure 3]. The diameter of the LMCA midway between the ostium and bifurcation was 3.4 mm. It was a small coronary artery aneurysm with Z score of 2.89 (2.5–5). LAD coronary artery was normal in caliber [Figure 4]. There was no presence of intraluminal thrombus in LMCA, LAD, or LCX. In view of intermediate stenosis of LCX, the child was managed with baby aspirin 5 mg/kg, i.e., 75 mg/day, beta-blocker metoprolol 12.5 mg twice daily, and nitroglycerine 1.3 mg twice daily with strict advice to follow-up after 1 month. We did not proceed for coronary angiogram immediately for detailed anatomic characteristics and it was the limitation as the stenosis was intermediate, he was not on OMT before it, follow-up ECG normalized and he responded well to optimal antianginal therapy.
Figure 1: Lateral wall ischemia (ST elevation in lead I and aVL) in a 6-year-old child with Kawasaki disease

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Figure 2: Electrocardiogram showing normalization of ST-T segment postoptimal medical therapy

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Figure 3: Aneurysmal left main coronary artery with osteoproximal left circumflex coronary stenosis in modified parasternal short-axis view

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Figure 4: Aneurysmal left main coronary artery with normal caliber left anterior descending without stenosis or aneurysm

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  Discussion Top

KD is a generalized vasculitis of unknown etiology. Tomisaku Kawasaki in 1967 described the illness as “mucocutaneous lymph node syndrome” affecting the infants and young children in Japan.[5] KD affects the children between 6 months and 8 years. Diagnosis requires fever for more than 5 days and four out of five principal clinical features (conjunctivitis; erythematous lips, oral cavity; palms and soles; and polymorphous exanthema of the trunk and cervical lymphadenopathy) or fewer than four in case of documented coronary artery disease diagnosed by echocardiography or coronary angiography.[6]

Cardiac, particularly coronary artery complications in KD occur in the form of aneurysm, stenosis, or thrombosis in the coronary artery. Aneurysmal dilatation and thrombosis are an early process, while the development of stenosis is a late one in the pathogenesis of coronary involvement of KD. Mandal et al.[7] described a case of left circumflex coronary artery stenosis in a 19-year-old male who had KD at the age of 4 years and a case of LMCA stenosis filled by collateral from right coronary artery in a 7-year-old boy who got KD at the age of 1 year, suggesting the fact that presence of stenotic lesion occurs at a late phenomenon in the disease process.

Patel et al.[8] described coronary stenosis occurs in 34.6% of cases with KD and the median period to detect the same is 190 days or more than 6 months. They described that the children with giant coronary aneurysms develop stenosis, and children under 6 months of age are more likely to develop stenosis which occurs in more than 50% of children who had KD under 6 months of age.

Friedman and Newburger.[9] concluded that children are having low body surface area (BSA) <0.5 m2 are at a higher risk of developing giant coronary aneurysm and subsequent stenosis than the children with BSA >0.5 m2 they coined it as “size matters.” Interestingly, the index child had a BSA of more than >0.5 m2 in spite he developed significant coronary stenosis without the presence of giant coronary aneurysm. They also proposed the definition of giant coronary aneurysm should be brought down to 6 mm in spite of 8 mm in conventional practice. Kato et al.[10] described 26 patients with giant coronary aneurysm, 12 patients developed coronary stenosis all of whom had giant coronary aneurysm, and no case of giant coronary aneurysm regressed with time.

Echocardiography is the first-line imaging for delineating coronary anatomy in patients with KD as endorsed by the American Heart Association[11] with reported sensitivity and specificity are in the ranges 80%–85% and 97%–98%, respectively, for identifying coronary stenosis.[12] Invasive coronary angiography is an important modality in assessing the coronary arteries with higher diagnostic accuracy than echocardiography.[13] Computed tomography (CT) coronary angiography is another useful non-invasive modality for assessing coronary luminal stenosis with simultaneous coronary ectasia.[14],[15] The measurement of coronary artery ectasia is statistically similar between invasive angiography and magnetic resonance angiography.[16]

The index child had intermediate coronary stenosis as evidenced in echocardiography, was not having angina refractory to drugs and also did not have left ventricular systolic dysfunction for which we did not proceed for coronary angiography in the form of CT coronary angiography or invasive coronary angiography. In the possibility of exacerbation of symptoms in future, we would like to do invasive coronary angiography for proper delineation of the coronary lesion.

So far as the coronary artery aneurysms are concerned, most commonly they occur in the proximal LAD and proximal right coronary arteries, followed in frequency by the LMCA, circumflex coronary artery, distal right coronary artery, and at the take-off of the posterior descending coronary artery from the right coronary artery.[17] Beaded appearance of coronary arteries pertains to KD as they develop multifocal aneurysm and stenosis together.

In a single-institution retrospective study[18] of 1073 patients with KD followed for a median of 6.7 years, myocardial ischemia, acute myocardial infarction, or death occurred in 13 patients (48%) with giant aneurysms, 1 patient (2%) with a medium aneurysm, and no patients with small aneurysms[19] suggesting the fact that small coronary aneurysm never becomes lethal as noted in our case. Suda et al.[20] followed 70 patients with giant coronary aneurysms and reported a survival rate of 88% at 30 years.

Reassurance can be given to the patient during follow-up only from the absence of late coronary artery calcification.[15] With respect to medications, beta-blocker therapy is used in the patients with giant coronary aneurysms which retard the progression of aneurysm expansion,[21],[22] and statins are also beneficial for their pleotropic anti-inflammatory effects. Giant aneurysms are treated with a combination of antiplatelet therapy and anticoagulation to prevent coronary thrombosis.[23]

Indications of percutaneous coronary intervention (PCI) in children with stenotic lesions include ischemic symptoms, inducible ischemia, or more than 75% stenosis in LAD coronary artery. Contraindications to PCI include the presence of complex aneurysms with multiple, ostial, or long-segment stenosis in coronary arteries.[24] In our case, it was a case of ostial left circumflex artery stenosis, if at all, it will progress to critical ostial lesion in future, we would like to plan for coronary artery bypass graft (CABG) rather than PCI after the age of 12 years as graft longevity is better after the age of 12 years. Comparing the outcomes after PCI versus CABG, the primary composite endpoint of mortality or Q-wave myocardial infarction is similar across both groups, but repeat target vessel revascularization (TLR) is significantly higher in the PCI arm.[25] When considering stent placement, cardiologists should be aware that the walls of a stenotic artery may be composed of considerable thrombus; intravascular ultrasound should be used to assess the true lumen diameter.

Aspirin therapy may be stopped at 4–8 weeks, once inflammatory markers have normalized and no coronary abnormalities are present. However, in our patient, the child has lateral wall coronary ischemia secondary to aneurysmal LMCA with ostial stenosis of LCX for which we decided to put the child on long-term aspirin. Aneurysmal segment in LMCA has a natural drop in coronary perfusion pressure secondary to local dilatation and this perfusion pressure further drops in the poststenotic segment of left circumflex coronary artery attributing toward coronary ischemia in this index child. A lipid profile should be checked annually in each patient with KD. Ten-year event-free survival is 100%, 94%, and 52% in men and 100%, 100%, and 75% in women for small, medium, and large aneurysms, respectively.[26] Predictors of adverse coronary events include large aneurysms, male sex, and resistance to intravenous immunoglobulin therapy.

  Conclusion Top

Our case is a rare illustration of paradoxical presence of osteoproximal left circumflex coronary artery stenosis in the presence of small funnel-shaped aneurysm of LMCA causing obligatory coronary ischemia in a 6-year-old child early (i.e., 1 month) after recovery from KD. It reflects aggressive negative coronary remodeling (stenosis) in place of positive coronary remodeling (aneurysm) in the very early phase of recovery from KD. Although coronary artery aneurysm predominates early and stenosis prevails late in the course of KD, the child had the reversal of the same in the natural history of coronary involvement in KD.

Ethical clearance

Institutional ethical committee (IEC) clearance has been obtained.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Durongpisitkul K, Gururaj VJ, Park JM, Martin CF. The prevention of coronary artery aneurysm in Kawasaki disease: A meta-analysis on the efficacy of aspirin and immunoglobulin treatment. Pediatrics 1995;96:1057-61.  Back to cited text no. 2
Terai M, Shulman ST. Prevalence of coronary artery abnormalities in Kawasaki disease is highly dependent on gamma globulin dose but independent of salicylate dose. J Pediatr 1997;131:888-93.  Back to cited text no. 3
Dajani AS, Taubert KA, Takahashi M, Bierman FZ, Freed MD, Ferrieri P, et al. Guidelines for long-term management of patients with Kawasaki disease. Report from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 1994;89:916-22.  Back to cited text no. 4
Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Arerugi 1967;16:178-222.  Back to cited text no. 5
Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics 1974;54:271-6.  Back to cited text no. 6
Mandal S, Pande A, Mandal D, Sarkar A, Kahali D, Panja M. Various coronary artery complications of Kawasaki disease: Series of 5 cases and review of literature. J Cardiovasc Dis Res 2012;3:231-5.  Back to cited text no. 7
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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