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Ten days after receiving the first dose of coronavirus disease vaccine, a 22-year-old woman in South Korea experienced myocarditis, myopathy, pericarditis, and gastroenteritis; rash subsequently developed. There was no evidence of prior infection with severe acute respiratory syndrome coronavirus 2. The diagnosis was multisystem inflammatory syndrome resulting from coronavirus disease vaccination.

Kawasaki disease (KD) is an acute, self-limited vasculitis, mainly affecting children younger than 5 years old, with accompanying fever and signs of mucocutaneous inflammation. Intravenous immunoglobulin (IVIG) is the standard treatment for KD; however, ~15% of patients are resistant to IVIG treatment. To identify protein coding genetic variants influencing IVIG resistance, we re-analyzed our previous genome-wide association study (GWAS) data from 296 patients with KD, including 101 IVIG non-responders and 195 IVIG responders. Five nonsynonymous SNPs (nsSNPs) in five immune-related genes, including a previously reported SAMD9L nsSNP (rs10488532; p.Val266Ile), were associated with IVIG non-response (odds ratio [OR] = 1.89–3.46, P = 0.0109–0.0035). In a replication study of the four newly-identified nsSNPs, only one in the interleukin 16 (IL16) gene (rs11556218, p.Asn1147Lys) showed a trend of association with IVIG non-response (OR = 1.54, P = 0.0078). The same IL16 nsSNP was more significantly associated with IVIG non-response in combined analysis of all data (OR = 1.64, P = 1.25 × 10−4). Furthermore, risk allele combination of the IL16 CT and SAMD9L TT nsSNP genotypes exhibited a very strong effect size (OR = 9.19, P = 3.63 × 10−4). These results implicate IL16 as involved in the mechanism of IVIG resistance in KD.

Kawasaki disease (KD) is a systemic vasculitis affecting infants and children; it manifests as fever and signs of mucocutaneous inflammation. Intravenous immunoglobulin (IVIG) treatment effectively attenuates the fever and systemic inflammation. However, 10–20% patients are unresponsive to IVIG. To identify genetic variants influencing IVIG non-response in KD, a genome-wide association study (GWAS) and a replication study were performed using a total of 148 IVIG non-responders and 845 IVIG-responders in a Korean population. rs28662 in the sterile alpha motif domain-containing protein 9-like (SAMD9L) locus showed the most significant result in the joint analysis of GWAS and replication samples (odds ratio (OR) = 3.47, P = 1.39 × 10−5). The same SNP in the SAMD9L locus was tested in the Japanese population, and it revealed a more significant association in a meta-analysis with Japanese data (OR = 4.30, P = 5.30 × 10−6). These results provide new insights into the mechanism of IVIG response in KD.

Kawasaki disease (KD) is a systemic vasculitis that mainly affects younger children. Intravenous immunoglobulin (IVIG) resistant cases are at increasing risk for coronary artery complications. The strategy on prediction of potential nonresponders and treatment of IVIG-resistant patients is now controversial. In this review the definition and predictors of IVIG-resistant KD and current evidence to guide management are discussed.

A number of microorganisms were hypothesised as an aetiology of the Kawasaki disease. Unfortunately, no specific agent that provides reproducible evidence has yet been reported. We report two cases of extremely rare Kawasaki disease with tsutsugamushi disease. These case reports suggest that Kawasaki disease can rarely occur concurrently or immediately after a rickettsial illness such as tsutsugamushi disease.

Background and Objectives We investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens. Methods Between March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.3±19.9 months, 72 males) who received infliximab at any time after first IVIG treatment failure were enrolled. Data were retrospectively collected using a questionnaire. Results Subjects were divided into two groups according to the timing of infliximab administration. Early treatment (group 1) had shorter fever duration (10.5±4.4 days) until infliximab infusion than that in late treatment (group 2) (16.4±4.5 days; p5). Overall response rate to infliximab was 89/102 (87.3%) and the incidence of significant CAA was lower in group 1 than in group 2 (1/42 [2.4%] vs. 17/60 [28.3%], p

Background and objectives Patients with Kawasaki disease (KD) are clinically heterogeneous because its diagnosis is based solely on clinical observation and there are no definitive biomarkers. We dissected the clinical heterogeneity of KD patients using the KD-associated genetic variants. Methods We performed a genetic association analysis in several KD subgroups categorized by clinical characteristics using the KD-associated variants of the B lymphoid tyrosine kinase (BLK; rs6993775) and Fc gamma receptor II a (FCGR2A; rs1801274) in a large number of case (n=1,011) and control (n=4,533) samples. Results BLK and FCGR2A were very significantly associated with KD in Korean KD patients (odds ratio [OR],1.48; p=4.63×10⁻¹¹ for BLK, and OR, 1.26; p=1.42×10⁻⁴ for FCGR2A). However, in KD subgroup analysis, we found that neither BLK nor FCGR2A were associated with either incomplete Kawasaki disease (iKD) type patients or those older than 5 years of age (p>0.2), suggesting that patients with iKD or those older than 5 years of age are a unique subgroup of KD. In genetic association analysis after excluding iKD patients and those older than 5 years old, we found that BLK was associated with all KD subgroups, whereas FCGR2A was specifically associated with male KD patients younger than 1 year of age (OR, 2.22; p=2.35×10⁻⁵). Conclusions KD is a clinically and genetically heterogeneous disease. These findings will provide new insights into the clinical and genetic heterogeneity of KD.

Background and Objectives Kawasaki disease (KD) is an acute systemic vasculitis that affects the coronary arteries. Abnormal immune reactions are thought to contribute to disease pathogenesis. The effect of immunoglobulin (Ig) isotype (IgG, IgA, IgM, and IgE) on inflammatory data and clinical outcomes of patients with KD was examined. Methods Ig levels in 241 patients with KD were measured during the acute, subacute, convalescent, and normal phases of the disease. Results Compared with reference Ig values, IgG, IgA, and IgM levels were significantly higher in the subacute phase, while IgE levels were elevated in 73.9% (178/241) of patients with KD in all clinical phases. However, high IgE levels were not associated with clinical outcomes, including intravenous immunoglobulin unresponsiveness and coronary artery lesions (CALs). Significantly more CALs were observed in the high IgA group than in the normal IgA group (44.7% vs. 20.8%, respectively; p

Kawasaki disease (KD) is a self-limited systemic vasculitis of an unknown pathogenesis mainly affecting children

Kawasaki disease (KD), a systemic vasculitis of infants and children, manifests as fever and mucocutaneous inflammation. Although its etiology is largely unknown, the epidemiological data suggest that genetic factors are important in KD susceptibility. To identify genetic variants influencing KD susceptibility, we performed a genome-wide association study (GWAS) and replication study using a total of 915 children with KD and 4553 controls in the Korean population. Six single-nucleotide polymorphisms (SNPs) in three loci were associated significantly with KD susceptibility (P

PURPOSE We conducted a study to determine which factors may be useful as predictive markers in identifying Kawasaki disease (KD) patients with a high risk of resistance to intravenous immunoglobulin (IVIG) and developing coronary artery lesions (CAL). METHODS We enrolled 287 patients in acute phase of KD at a single center. The demographic, clinical and laboratory data were collected retrospectively. RESULTS There were 34 patients in the IVIG resistant group. The IVIG resistant group had significantly higher serum N-terminal-pro-brain natriuretic protein (NT-proBNP) levels (P

Kawasaki disease (KD) is the most common cause of acquired heart disease in children. Intravenous immunoglobulin (IVIG) is the standard therapy for KD, but more than 10% of KD patients do not respond to IVIG and are at high risk for the development of coronary artery lesions (CALs). To identify clinical and genetic risk factors associated with CAL development and IVIG nonresponsiveness, this study analyzed the clinical data for 478 Korean KD patients. Multivariate logistic regression analysis showed that incomplete KD, IVIG nonresponse, fever duration of 7 days or longer, and the CC/AC genotypes of the rs7604693 single nucleotide polymorphism (SNP) in the PELI1 gene were significantly associated with the development of CALs, with odds ratios (ORs) ranging from 2.06 to 3.04. The risk of CAL formation was synergistically increased by the addition of individual risk factors, particularly the genetic variant in the PELI1 gene. Multivariate analysis also showed that a serum albumin level of 3.6 g/dl or lower was significantly associated with nonresponsiveness to IVIG [OR, 2.76; 95% confidence interval (CI), 1.34-5.68; P = 0.006]. Conclusively, incomplete KD, IVIG nonresponsiveness, long febrile days, and the rs7604693 genetic variant in the PELI1 gene are major risk factors for the development of CALs, whereas low serum albumin concentration is an independent risk factor for IVIG nonresponsiveness.

Kawasaki disease is a systemic vasculitis of unknown etiology, with clinical observations suggesting a substantial genetic contribution to disease susceptibility. We conducted a genome-wide association study and replication analysis in 2,173 individuals with Kawasaki disease and 9,383 controls from five independent sample collections. Two loci exceeded the formal threshold for genome-wide significance. The first locus is a functional polymorphism in the IgG receptor gene FCGR2A (encoding an H131R substitution) (rs1801274; P = 7.35 × 10(-11), odds ratio (OR) = 1.32), with the A allele (coding for histadine) conferring elevated disease risk. The second locus is at 19q13, (P = 2.51 × 10(-9), OR = 1.42 for the rs2233152 SNP near MIA and RAB4B; P = 1.68 × 10(-12), OR = 1.52 for rs28493229 in ITPKC), which confirms previous findings(1). The involvement of the FCGR2A locus may have implications for understanding immune activation in Kawasaki disease pathogenesis and the mechanism of response to intravenous immunoglobulin, the only proven therapy for this disease.

Purpose: Croup is known to have epidemics in seasonal and biennial trends, and to be strongly associated with epidemics of para influenza virus. However, seasonal and annual epidemics of croup have not been clearly reported in Korea. This study aimed to ex amine the seasonal/annual patterns and etiologies of childhood croup in Korea during a consecutive 6-year period. Methods: Pediatric croup data were collected from 23 centers in Korea from 1 January 2010 to 31 December 2015. Electronic medi cal records, including multiplex reverse transcription polymerase chain reaction (RT-PCR) results, demographics and clinical informa tion were cross-sectionally reviewed and analyzed. Results: Overall, 2,598 childhood croup patients requiring hospitalization were identified during the study period. Among them, a total of 927 who underwent RT-PCR were included in the analysis. Males (61.5%) predominated, and most (63.0%) of them were younger than 2 years of age (median, 19 months; interquartile range, 11-31 months). Peak hospitalization occurred in 2010 and 2012 in even-numbered years, and parainfluenza virus (PIV, 39.7%) was the most common cause of childhood croup requiring hos pitalization, followed by respiratory syncytial virus (14.9%), human rhinovirus (12.5%), Mycoplasma pneumonaie (10.6%), and human coronavirus (7.3%). Conclusion: It is concluded that croup hospitalization has a biennial pattern in even-numbered years. PIV may be the most com mon cause of childhood croup; however, croup epidemics could be attributed to other viruses. (Allergy Asthma Respir Dis 2019;7:78-85)

Kawasaki disease (KD) is an acute self-limited vasculitis of infants and children that manifests as fever and signs of mucocutaneous inflammation. Coronary artery aneurysms develop in approximately 15-25% of untreated children. Although the etiology of KD is largely unknown, epidemiologic data suggest the importance of genetic factors in the susceptibility to KD. In order to identify genetic variants that influence KD susceptibility, we performed a genome-wide association study (GWAS) using Affymetrix SNP array 6.0 in 186 Korean KD patients and 600 healthy controls; 18 and 26 genomic regions with one or more sequence variants were associated with KD and KD with coronary artery lesions (CALs), respectively (p

Purpose Studies have been conducted to identify predictive factors of resistance to intravenous immunoglobulin (IVIG) for Kawasaki disease (KD). However, the results are conflicting. This study aimed to identify laboratory factors predictive of resistance to high-dose IVIG for KD by performing meta-analysis of available studies using statistical techniques. Methods All relevant scientific publications from 2006 to 2014 were identified through PubMed searches. For studies in English on KD and IVIG resistance, predictive factors were included. A meta-analysis was performed that calculated the effect size of various laboratory parameters as predictive factors for IVIG-resistant KD. Results Twelve studies comprising 2,745 patients were included. Meta-analysis demonstrated significant effect sizes for several laboratory parameters: polymorphonuclear leukocytes (PMNs) 0.698 (95% confidence interval [CI], 0.469–0.926), C-reactive protein (CRP) 0.375 (95% CI, 0.086–0.663), pro-brain natriuretic peptide (pro-BNP) 0.561 (95% CI, 0.261–0.861), total bilirubin 0.859 (95% CI, 0.582–1.136), alanine aminotransferase (AST) 0.503 (95% CI, 0.313–0.693), aspartate aminotransferase (ALT) 0.436 (95% CI, 0.275–0.597), albumin 0.427 (95% CI, –0.657 to –0.198), and sodium 0.604 (95% CI, –0.839 to –0.370). Particularly, total bilirubin, PMN, sodium, pro-BNP, and AST, in descending numerical order, demonstrated more than a medium effect size. Conclusion Based on the results of this study, laboratory predictive factors for IVIG-resistant KD included higher total bilirubin, PMN, pro-BNP, AST, ALT, and CRP, and lower sodium and albumin. The presence of several of these predictive factors should alert clinicians to the increased likelihood that the patient may not respond adequately to initial IVIG therapy.

Background : Clinical and epidemiologic features suggest infectious agents as a possible cause of Kawasaki disease; however, the etiology of Kawasaki disease still remains unknown. A number of microorganisms were hypothesized as an etiology of the illness. This is the first reported case of Kawasaki disease with tsutsugamushi disease. Case presentation: We report the case of a 4-year-old boy who presented with fever of 7 days duration and skin rash and bilateral conjunctival injection. He had a history of visiting a rural area with his grandmother. On admission, he had fever of 39.4 °C. His heart rate was 90/minute and his blood pressure was 90/60 mmHg. His pharynx was slightly injected and there was red lip. His neck was swollen with cervical lymphadenitis. He had erythematous macular rash on her trunk. Examination of his skin revealed an eschar on penile base of right scrotum. His laboratory results showed WBC 4,720/mm 3 , 42% polymorphonuclear leucocytes, 39% lymphocytes, hemoglobin 10.3 gm/dL, platelet count 148,000/mm 3 , CRP 3.23mg/dl, pro-BNP 316.5 pg/ml. The respiratory viruses using a multiplex real-time-PCR kit (Adenovirus, Influenza A, Influenza B, Metapneumovirus, Rhino A virus, Respiratory syncytiai virus, Parainfluenza ) were all negative. Mycoplasma pneumonia IgM was negative. R.tsutsugamushi Ab was positive. Echocardiographic findings 1 day after admission was mild dilatation of LCA (RCA=1.8mm, LCA=3mm). He was treated on oral roxithromycin for presumptive diagnosis of tsutsugamushi disease along with clinical features of Kawasaki disease which resolved after therapy with intravenous immune globulin and aspirin. Over the next 48 hours, he became afebrile and his rash improved. He was placed on low-dose aspirin for 8 weeks. His echocardiogram were within normal limit (RCA= 1.9mm, LCA= 2.7mm) at 2 months after the onset of his illness. Conclusion: This case report suggests that Kawasaki disease can rarely occur concurrently or immediately after a rickettsial illness such as tsutsugamushi disease.

BACKGROUND AND OBJECTIVES Approximately 10-15% of children with Kawasaki disease (KD) do not respond to initial intravenous immunoglobulin (IVIG) and have higher risk for coronary artery lesion (CAL). The aim of this study was to identify predictive factors from laboratory findings in patients who do not respond to IVIG treatment and develop CAL from KD. METHODS We retrospectively collected nationwide multicenter data from the Korean Society of Kawasaki Disease and included 5,151 patients with KD between 2012 and 2014 from 38 hospitals. RESULTS Among 5,151 patients with KD, 524 patients belonged to the IVIG-resistant group. The patients in the IVIG-resistant group had a significantly higher serum N-terminal pro-brain natriuretic peptide (NT-proBNP) level (1,573.91±3,166.46 vs. 940.62±2,326.10 pg/mL; p

Kawasaki disease (KD) is an acute self-limiting form of vasculitis that afflicts infants and children and manifests as fever and signs of mucocutaneous inflammation. Children with KD show various laboratory inflammatory abnormalities, such as elevations in their white blood cell (WBC) count, C-reactive protein (CRP) level, and erythrocyte sedimentation rate (ESR). We here performed a genome-wide association study (GWAS) of 178 KD patients to identify the genetic loci that influence 10 important KD laboratory markers: WBC count, neutrophil count, platelet count, CRP, ESR, hemoglobin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and total protein. A total of 165 loci passed our arbitrary stage 1 threshold for replication (p < 1 × 10(-5)). Of these, only 2 SNPs (rs12068753 and rs4786091) demonstrated a significant association with the CRP level in replication study of 473 KD patients (p < 0.05). The SNP located at the CRP locus (rs12068753) demonstrated the most significant association with CRP in KD patients (beta = 4.73 and p = 1.20 × 10(-6) according to the stage 1 GWAS; beta = 3.65 and p = 1.35 × 10(-8) according to the replication study; beta = 3.97 and p = 1.11 × 10(-13) according to combined analysis) and explained 8.1% of the phenotypic variation observed. However, this SNP did not demonstrate any significant association with CRP in the general population (beta = 0.37 and p = 0.1732) and only explained 0.1% of the phenotypic variation in this instance. Furthermore, rs12068753 did not affect the development of coronary artery lesions or intravenous immunoglobulin resistance in KD patients. These results indicate that common variants in the CRP promoter can play an important role in the CRP levels in KD.

To assess the recent epidemiologic features of Kawasaki disease (KD) in South Korea from the nationwide survey conducted between 2009 and 2011.We collected data regarding the incidence, symptoms, treatment and coronary complications associated with acute KD by sending questionnaires to the 100 hospitals that have pediatric residency programs from 2009 to 2011.We received complete responses from 73 hospitals and partial responses from 14 hospitals. A total of 13,031 patients of KD were reported from the 87 hospitals (3941 in 2009, 4635 in 2010 and 4455 in 2011). The male to female ratio was 1.44:1, and the median age at diagnosis was 28 months. From the questionnaires with complete responses, we noted that the incidence of KD per 100,000 children5 years of age was 115.4 in 2009, 132.9 in 2010 and 134.4 in 2011 (average rate, 127.7). KD occurred more frequently during summer (June, July and August) and during winter (December and January). The recurrence rate was 3.83%. The standard dose of intravenous immunoglobulin was administered to 93.6% of the patients, and nonresponder rate was 11.6%. Coronary aneurysm occurred in 1.9% of the patients and giant aneurysm developed in 26 patients (0.26%) over 3 years, and 2 patients had myocardial infarction. No mortality was reported.The average annual incidence of KD in South Korea has continuously increased to 134.4 per 100,000 children5 years of age in 2011, which is the second highest incidence of KD worldwide, following its incidence in Japan.

Kawasaki disease (KD) is often complicated by coronary artery lesions (CALs), including aneurysms. Because of the complications associated with KD, this disorder is the leading cause of acquired heart disease in children from developed countries. To identify genetic loci that confer a higher risk of developing CALs, we performed a case-control association study using previous genome-wide association study data for samples from KD cases only (n=186) by grouping KD patients without CALs (control: n=123) vs KD patients with extremely large aneurysms (diameter5 mm) (case: n=17). Twelve loci with one or more sequence variants were found to be significantly associated with CALs (P1 × 10(-5)). Of these, an SNP (rs17136627) in the potassium intermediate/small conductance calcium-activated channel, subfamily N, member 2 (KCNN2) at 5q22.3 was validated in 32 KD patients with large aneurysms (diameter5 mm) and 191 KD patients without CALs (odds ratio (OR)=12.6, P(combined)=1.96 × 10(-8)). This result indicates that the KCNN2 gene can have an important role in the development of coronary artery aneurysms in KD.

High-dose intravenous immunoglobulin (IVIG) therapy is the highly effective and standard treatment for Kawasaki disease (KD). However, ~20 % of KD patients have persistent fever or recurrence of fever after the initial IVIG treatment, which increases the risk for coronary artery lesions (CALs). Furthermore, the mechanism of IVIG resistance in KD patients still is unknown. The number of CC chemokine ligand 3-like 1 (CCL3L1) gene copies is reported to be associated with KD and IVIG resistance in Japanese patients. In addition, the authors observed significant upregulation of the CCL3L1 gene expression after in vitro immunoglobulin treatment in B cell lines derived from KD patients. Therefore, this study of 459 KD patients and 496 healthy control subjects tested whether the number of CCL3L1 gene copies is associated with a risk of KD, CALs, and/or IVIG resistance in Korean KD patients. However, the number of CCL3L1 gene copies was not associated with KD (P = 0.18), CAL formation (P = 0.062), or the IVIG resistance (P = 0.90). Therefore, the results indicate that the number of CCL3L1 gene copies does not have a role in susceptibility to KD or CALs nor with IVIG resistance in Korean KD patients.

T-cell-dependent immunity in the central nervous system (CNS) is beneficial for neuroprotection, neurogenesis and even behavior. As a highly specialized site, the CNS is speculated to possess the means to maintain T-cell immune responses through its own resident cells. Therefore, we investigated whether microglia, the most potent antigen-presenting cells residing in the CNS, could sustain T-cell responses in vitro. We showed that interferon-gamma (IFN-gamma)-activated microglia (MG(IFN-gamma)) inducibly expressed an important immune co-stimulatory molecule, OX40 ligand (OX40L). Co-culture of activated CD4(+) T cells with MG(IFN-gamma) significantly increased T-cell proliferation and decreased apoptosis, and these effects were markedly inhibited by addition of a neutralizing anti-OX40L monoclonal antibody. In addition, ligation of OX40L in MG(IFN-gamma) enhanced their production of insulin-like growth factor I (IGF-I). These results suggest that the expression of OX40L in microglia provides a molecular basis for the maintenance of T-cell survival, expansion of T cells and increased secretion of remedial growth factor from MG(IFN-gamma), which may contribute to the protective effect in the CNS.

Turner syndrome is well known to be associated with significant cardiovascular abnormalities. This paper studied the incidence of cardiovascular abnormalities in asymptomatic adolescent patients with Turner syndrome using multidetector computed tomography (MDCT) instead of echocardiography. Twenty subjects diagnosed with Turner syndrome who had no cardiac symptoms were included. Blood pressure and electrocardiography (ECG) was checked. Cardiovascular abnormalities were checked by MDCT. According to the ECG results, 11 had a prolonged QTc interval, 5 had a posterior fascicular block, 3 had a ventricular conduction disorder. MDCT revealed vascular abnormalities in 13 patients (65%). Three patients had an aberrant right subclavian artery, 2 had dilatation of left subclavian artery, and others had an aortic root dilatation, aortic diverticulum, and abnormal left vertebral artery. As for venous abnormalities, 3 patients had partial anomalous pulmonary venous return and 2 had a persistent left superior vena cava. This study found cardiovascular abnormalities in 65% of asymptomatic Turner syndrome patients using MDCT. Even though, there are no cardiac symptoms in Turner syndrome patients, a complete evaluation of the heart with echocardiography or MDCT at transition period to adults must be performed.

Background and Objectives This was a multicenter study to evaluate the usefulness of the tumor necrosis factor-alpha (TNF-α) blocker infliximab for treatment of Korean pediatric patients with refractory Kawasaki disease (KD). Subjects and Methods Data from 16 patients throughout Korea who were diagnosed with refractory KD and received infliximab were collected retrospectively. Results Complete response to therapy with cessation of fever occurred in 13 of 16 patients. C-reactive protein (CRP) concentrations decreased following infliximab infusion in all 14 patients in whom it was measured before and after treatment. There were no infusion reactions or complications associated with infliximab except in 1 case with acute hepatitis occurring during treatment followed by calculous cholecystitis 4 months later. Fifteen patients had coronary artery (CA) abnormalities before infliximab therapy. Three had transient mild dilatation and 9 had CA aneurysms, with subsequent normalization in 4 patients, persistent mild dilatation in 3, persistent aneurysm in 2, and there were 3 cases (2 with CA aneurysm, 1 with mild CA dilatation) without follow-up echocardiography. Conclusion The results of this study suggest that infliximab may be useful in the treatment of refractory KD, and it appears that there is no significant further progression of CA lesions developing after infliximab treatment. Multicenter trials with larger numbers of patients and long-term follow-up are necessary to assess the clinical efficacy and safety of infliximab in refractory KD.

A case of a single coronary artery complicated with a coronary artery fistula (CAF) to the right ventricle is extremely rare, and its management strategy and prognosis are not clear. A 5-year-old boy was hospitalized for evaluation of a continuous heart murmur. Transthoracic echocardiography suggested a CAF to the right ventricle, with an enlarged left coronary artery. Cardiac catheterization confirmed the CAF terminating at the right ventricle and the absence of a right coronary artery. The fistula was ligated at the right ventricular side under cardiopulmonary bypass. At follow-up 18 months later, the child was clinically asymptomatic, and coronary angiogram showed no recurrence of the fistula.