Coronary Arteries In Children Research Articles

Emerging role of computed tomography coronary angiography in evaluation of children with Kawasaki disease.

Coronary artery abnormalities are the most important complications in children with Kawasaki disease (KD). Two-dimensional transthoracic echocardiography currently is the standard of care for initial evaluation and follow-up of children with KD. However, it has inherent limitations with regard to evaluation of mid and distal coronary arteries and, left circumflex artery and the poor acoustic window in older children often makes evaluation difficult in this age group. Catheter angiography (CA) is invasive, has high radiation exposure and fails to demonstrate abnormalities beyond lumen. The limitations of echocardiography and CA necessitate the use of an imaging modality that overcomes these problems. In recent years advances in computed tomography technology have enabled explicit evaluation of coronary arteries along their entire course including major branches with optimal and acceptable radiation exposure in children. Computed tomography coronary angiography (CTCA) can be performed during acute as well as convalescent phases of KD. It is likely that CTCA may soon be considered the reference standard imaging modality for evaluation of coronary arteries in children with KD.

Clinical characteristics of anomalous aortic origin of a coronary artery in children

Objective: To analyze the clinical characteristics, diagnosis and treatment of anomalous aortic origin of a coronary artery (AAOCA) in children. Methods: There were 17 children diagnosed with AAOCA from January 2013 to January 2022 in Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine.Their clinical manifestations, laboratory and imaging data, treatment and prognosis were retrospectively analyzed. Results: These 17 children included 14 males and 3 females, with the age of (8.7±3.5) years. There were 4 anomalous left coronary artery (ALCA) and 13 anomalous right coronary artery (ARCA). Seven children presented with chest pain or chest pain after exercise, three patients presented with cardiac syncope, one complained chest tightness and weakness, and the other six patients had no specific symptoms. Cardiac syncope and chest tightness occurred in patients with ALCA. Fourteen children had the dangerous anatomical basis of myocardial ischemia caused by coronary artery compression or stenosis on imaging. Seven children had coronary artery repair, of whom two were ALCA and five were ARCA. One patient had received heart transplantation because of heart failure. The incidence of adverse cardiovascular events and poor prognosis in ALCA group was higher than that in ARCA group (4/4 vs. 0/13, P<0.05). They were followed up in the outpatient department regularly for 6 (6, 12) months; except for the one who lost visit, the rest of the patients had a good prognosis. Conclusions: Cardiogenic syncope or cardiac insufficiency usually occurs in ALCA, and adverse cardiovascular events and poor prognosis are more common in ALCA than in ARCA. Early surgical treatment should be considered for children with ALCA and ARCA accompanied by myocardial ischemia.

Pathology of coronary arteries in children and its clinical significance

Annotation. Coronary insufficiency and associated ischemic heart disease are most common among adult patients, while life-threatening myocardial ischemia can develop in childhood and clinically debut as a sudden death syndrome. In children and young adults, the most frequent cardiac causes of sudden death syndrome include hypertrophic cardiomyopathy, coronary artery anomalies, other phenotypes of myocardial pathology, and myocarditis. Anomalies of the coronary arteries cause significant difficulties in diagnostics because they have a wide clinical polymorphism – from asymptomatic variants to severe myocardial dysfunction due to ischemia, and also require special knowledge and skills when using modern methods of imaging the heart and its structures. The work is devoted to the analysis of the etiology of coronary artery disease in childhood and is aimed at increasing the awareness of the medical community about this problem. The Cochrane Library, Pub Med and Google Scholar reference databases were used for the review. 84 articles were studied and analyzed, 29 sources were included in the review. Myocardial ischemia in childhood and associated severe myocardial dysfunction are most often the result of congenital anomalies of coronary artery development, coronary artery pathologies in the composition of other congenital heart defects, coronary artery damage in Kawasaki disease, and coronary insufficiency in patients with hypertrophic cardiomyopathy. It is important to note that coronary vessel anomalies can often be asymptomatic and not detected during routine clinical examination, but manifest themselves in young and even adult life in the form of acute myocardial ischemia and cardiogenic shock against the background of complete well-being. During echocardiographic screenings it is necessary to pay attention to the anatomy of the coronary arteries. Also a multidisciplinary approach to the management of children with diseases in which coronary artery damage is possible is necessary.

Outcomes of a protocolized approach for surgical unroofing of intramural anomalous aortic origin of coronary artery in children and adults

ObjectiveManagement of anomalous aortic origin of coronary arteries has been variable, and limited data are available on early and late outcomes. MethodsWe report a single institution's experience with a protocolized approach to 148 consecutive patients who underwent surgical unroofing of intramural anomalous aortic origin of coronary arteries (June 2003 to December 2020). The management algorithm included preoperative and postoperative cross-sectional and echocardiographic imaging, exercise testing, and a standardized operative technique. ResultsThe median age of the cohort was 44.4 years (range, 4 months to 83 years); 130 patients had an anomalous right coronary artery, and 19 patients had an anomalous left coronary artery. Surgical unroofing was an isolated procedure in 118 patients (80%) and a concomitant procedure in 30 patients (20.3%). There were 2 (1%) early deaths; both were reoperations for aortic root or valve replacement. Over a median follow-up of 9.5 (interquartile range, 5-12.3) years, there were 5 late deaths, 3 due to noncardiac causes and 2 due to unknown cause. Late survival after anomalous aortic origin of coronary arteries repair at 10 and 15 years was 94.5% and 94.5%, respectively. There were no early or late deaths in the pediatric cohort with a median follow-up of 10.9 years (interquartile range, 6.9-12.1). At a median clinical follow-up of 3.9 years (interquartile range, 1.1-9.5), 36 patients had chest pain but none with evidence of ischemia related to the unroofing. ConclusionsSurgical unroofing of anomalous aortic origin of coronary arteries can be performed safely with low early mortality, even in the setting of concomitant procedures. Late survival is excellent, with the vast majority being symptom free.

Anomalous Aortic Origin of Coronary Arteries in Children: Postoperative High-risk Anatomic Features

BackgroundThis study aimed to assess postoperative presumed high-risk anatomic features (HRAFs) by using computed tomographic angiography (CTA) in patients with anomalous aortic origin of a coronary artery (AAOCA) after surgical unroofing vs transection and reimplantation (TAR) if unroofing was thought to provide unsatisfactory results. MethodsThe study included 62 children with postoperative CTA performed at a median of 3 months (interquartile range, 3-4 months) after unroofing (n = 45) and TAR (n = 17). HRAFs included slitlike ostium, intramural course, acute angle takeoff (<45o), interarterial course, proximal stenosis >50%, or course through a thickened intercoronary pillar. ResultsMedian age at surgery was 13.8 years (interquartile range, 10.5-15.8 years). None of the patients had a slitlike ostium or an intramural course on postoperative CTA. Acute takeoff was seen in 100% after unroofing and in 2 of 17 (12%) after TAR (P < .001). After unroofing, the interarterial course improved to 35 of 45 (78%) from 43 of 45 (96%) (P = .003), and a thickened intercoronary pillar improved to 10 of 45 (22%) from 22 of 45 (49%) (P = .0001), compared with none seen after TAR. Preoperative intramural length <5 mm was associated with a postoperative thickened intercoronary pillar in right AAOCA after unroofing (P = .0004). Severe coronary stenosis occurred in 2 of 17 (12%) after TAR, and both patients needed urgent revision procedures. All patients except 2 (97%) returned to exercise activities at a median follow-up of 4.9 years (range, 0.6-9.2 years). ConclusionsThe slitlike ostium and intramural course resolved in all patients. Residual acute angle takeoff, an interarterial course, and mild coronary narrowing related to a thickened intercoronary pillar were common after unroofing. TAR allows resolution of all HRAFs, although severe narrowing requiring surgical revision happened only in TAR. Long-term studies are needed to understand the clinical significance of these residual presumed HRAFs.

Surgery for Anomalous Aortic Origin of a Coronary Artery (AAOCA) in Children and Adolescents: A Meta-Analysis.

We conducted a systematic review and meta-analysis of the literature to assess the outcomes of surgery for the anomalous aortic origin of a coronary artery in children and young adults (<30 years). Thirteen publications were selected, including a total of 384 patients. Unroofing of the intramural segment was adopted in 92% (95% CI: 81%-98%) of cases, with pooled early and late mortality of 0% (95% CI: 0%-0.3%) and 0.1% (95% CI: 0%-1%), respectively. Reoperation for aortic regurgitation was anecdotal. Surgical management of anomalous aortic origin of coronary arteries can be achieved with excellent results in pediatric patients, but concerns remain about the durability of surgery.

Evaluation of coronary arteries in congenital heart disease in children: diagnostic comparison of electrocardiogram-gated and non-electro-cardiogram-gated computed tomography cardiac angiography.

To compare the visualization and anatomy of coronary arteries in children (≤ 2 years) with congenital heart disease (CHD) on non-electrocardiogram (ECG)-gated and ECG-gated computed tomography angiography (CTA). In this retrospective study, approved by the Ethics Committee of our institute, evaluation of coronary arteries in CHD was performed in 40 children on non-ECG-gated CTA and in 42 children on ECG-gated CTA. The origin and course of the right coronary artery (RCA), left main coronary artery (LMCA), left anterior descending (LAD) artery, and left circumflex (LCX) artery were evaluated by 2 paediatric radiologists independently. ECG-gated CT scans yielded increased (additional) visualization of all the coronary arteries, when compared to non-ECG-gated CT scans. The RCA, LMCA, LAD artery, and LCX artery were visualized in 47.5%, 62.5%, 55%, and 32.5% of children, respectively, on non-ECG-gated studies, while they were visualized in 64.3%, 92.8%, 80.9%, and 62% children, respectively, on ECG-gated studies. The coronary artery anatomical variations were also supplementarily detected more in the ECG-gated group (23.8%) than in the non-ECG gated group (2.5%). ECG-gated CT cardiac angiography studies yield enhanced diagnostic outcomes for the evaluation of the coronary arteries in comparison to non-ECG-gated studies.

Coronary computed tomography angiography in children

Imaging the coronary arteries of children, with their faster heart rates, small vessel size and common inability to lie still or breath-hold, has been a major challenge. With numerous advances in technology, CT examinations can now be performed quickly, often with children free-breathing and with much lower radiation doses than previously. This has led to increased use in children. Care must be taken with technique and choice of electrocardiogram (ECG)-gating technique to obtain adequate imaging for a diagnosis while keeping radiation dose as low as reasonably achievable (ALARA). In this paper, we discuss techniques and tips for CT imaging of the coronary arteries in children, including use of dual-source- and ultrawide-detector CT scanners.

Carotid Intima-Media Thickness and Ankle-Brachial Index and Their Correlation with Coronary Artery Dilatation in Children with Kawasaki Disease.

Objective To investigate the correlation between carotid intima-media thickness (IMT), ankle-brachial index (ABI), and coronary artery dilatation (CAD) in children with Kawasaki disease (KD) and to evaluate the effectiveness of CAD. Methods A total of 68 children diagnosed with KD from January 2019 to January 2021 in our hospital were included. According to the results of cardiac color Doppler ultrasound, the children with KD were divided into a noncoronary artery dilation group (NCAD), with 41 children with KD who did not have coronary artery lesions, and a coronary artery dilation group (CAD), with 27 children with KD who had coronary artery dilation. 27 healthy children undergoing physical examination in our hospital at the same time were selected as the normal control group. Laboratory index of all subjects was measured individually. The carotid IMT, ABI, and coronary artery diameter of all subjects were measured and compared. Pearson correlation was used to analyze the correlation between carotid IMT, ABI, and the severity of coronary artery disease. The ROC curve was used to evaluate the efficacy of carotid IMT and ABI in predicting coronary artery disease. Results The ALB of children in the CAD group was lower than that in the NCAD group (P < 0.05). The IMT of carotid artery and the diameter of coronary artery in children of the CAD group and the NCAD group were higher than those of the normal control group, and the IMT of the CAD group was higher than that of the NCAD group. The ABI of children in the CAD group and the NCAD group was lower than that of the normal control group, and the ABI of children in the CAD group was lower than that of the NCAD group (P < 0.05). Correlation analysis showed that carotid artery IMT of children with KD was positively correlated with coronary artery diameter, while ABI was negatively correlated with coronary artery diameter. The AUC of carotid IMT for CAD in children with KD was 0.668 (95% CI: 0.538–0.797), that of ABI for CAD in children with KD was 0.646 (95% CI: 0.513–0.780), and that of the combination of carotid IMT and ABI for CAD was 0.874 (95% CI: 0.785–0.963). Conclusion The changes of carotid artery IMT and ABI in children with KD have a certain correlation with CAD, and the joint detection of carotid artery IMT and ABI can provide clinical reference value for predicting the degree of coronary artery disease in children with KD.

Flow Dynamics in Anomalous Aortic Origin of a Coronary Artery in Children: Importance of the Intramural Segment

This study aims to assess the differences in pressure, fractional flow reserve (FFR) and coronary flow (with increasing pressure) of the proximal coronary artery in patients with anomalous aortic origin of a coronary artery with a confirmed ischemic event, without ischemic events, and before and after unroofing surgery, and compare to a patient with normal coronary arteries. Patient-specific flow models were 3D printed for 3 subjects with anomalous right coronary arteries with intramural course, 2 of them had documented ischemia, and compared with a patient with normal coronaries. The models were placed in the aortic position of a pulse duplicator and precise measurements to quantify FFR and coronary flow rate were performed from the aortic to the mediastinal segment of the anomalous right coronary artery. In an ischemic model, a gradual FFR drop (emulating that of pressure) was shown from the ostium location (∼1.0) to the distal intramural course (0.48). In nonischemic and normal patient models, FFR for all locations did not drop below 0.9. In a second ischemic model prior to repair, a drop to 0.44 was encountered at the intramural and mediastinal intersection, improving to 0.86 postrepair. There is a difference in instantaneous coronary flow rate with increasing aortic pressure in the ischemic models (slope 0.2846), compared to the postrepair and normal models (slope >0.53). These observations on patient models support a biomechanical basis for ischemia and potentially sudden cardiac death in aortic origin of a coronary artery, with a drop in pressure and FFR in the intramural segment, and a decrease in coronary flow rate with increasing aortic pressure, with both improving after corrective surgery.

Comprehensive pathogen detection in sera of Kawasaki disease patients by high-throughput sequencing: a retrospective exploratory study

BackgroundKawasaki disease (KD) is an idiopathic systemic vasculitis that predominantly damages coronary arteries in children. Various pathogens have been investigated as triggers for KD, but no definitive causative pathogen has been determined. As KD is diagnosed by symptoms, several days are needed for diagnosis. Therefore, at the time of diagnosis of KD, the pathogen of the trigger may already be diminished. The aim of this study was to explore comprehensive pathogens in the sera at the acute stage of KD using high-throughput sequencing (HTS).MethodsSera of 12 patients at an extremely early stage of KD and 12 controls were investigated. DNA and RNA sequences were read separately using HTS. Sequence data were imported into the home-brew meta-genomic analysis pipeline, PATHDET, to identify the pathogen sequences.ResultsNo RNA virus reads were detected in any KD case except for that of equine infectious anemia, which is known as a contaminant of commercial reverse transcriptase. Concerning DNA viruses, human herpesvirus 6B (HHV-6B, two cases) and Anelloviridae (eight cases) were detected among KD cases as well as controls. Multiple bacterial reads were obtained from KD and controls. Bacteria of the genera Acinetobacter, Pseudomonas, Delfita, Roseomonas, and Rhodocyclaceae appeared to be more common in KD sera than in the controls.ConclusionNo single pathogen was identified in serum samples of patients at the acute phase of KD. With multiple bacteria detected in the serum samples, it is difficult to exclude the possibility of contamination; however, it is possible that these bacteria might stimulate the immune system and induce KD.

Cardiac Magnetic Resonance to Evaluate Percutaneous Pulmonary Valve Implantation in Children and Young Adults.

Experience with cardiac magnetic resonance to evaluate coronary arteries in children and young adult patients is limited. Because noninvasive imaging has advantages over coronary angiography, we compared the effectiveness of these techniques in patients who were being considered for percutaneous pulmonary valve implantation. We retrospectively reviewed the cases of 26 patients (mean age, 12.53 ± 4.85 yr; range, 5-25 yr), all of whom had previous right ventricular-to-pulmonary artery homografts. We studied T2-prepared whole-heart images for coronary anatomy, velocity-encoded cine images for ventricular morphology, and function- and time-resolved magnetic resonance angiographic findings. Cardiac catheterization studies included coronary angiography, balloon compression testing, right ventricular outflow tract, and pulmonary artery anatomy. Diagnostic-quality images were obtained in 24 patients (92%), 13 of whom were considered suitable candidates for valve implantation. Two patients (8%) had abnormal coronary artery anatomy that placed them at high risk of coronary artery compression during surgery. Twelve patients underwent successful valve implantation after cardiac magnetic resonance images and catheterization showed no increased risk of compression. We attempted valve implantation in one patient with unsuitable anatomy but ultimately placed a stent in the homograft. Magnetic resonance imaging of coronary arteries is an important noninvasive study that may identify patients who are at high risk of coronary artery compression during percutaneous pulmonary valve implantation, and it may reveal high-risk anatomic variants that can be missed during cardiac catheterization.

Evaluation of Cardiac Coronary Artery in Children with Kawasaki Disease by Echocardiography

&lt;p&gt;Kawasaki disease is an acute, unknown etiology of vascular inflammatory response syndrome, heart coronary artery changes as one of the main clinical manifestations of Kawasaki disease. How to determine the effective changes of coronary heart disease in children has become a lot of research scholars concern. With the continuous development of medical technology in recent years, echocardiography is widely used in the clinical diagnosis of various diseases and for the effective development of clinical treatment had a positive impact. A large number of research scholars found that by echocardiography, it can effectively achieve the diagnosis of coronary heart disease in children with Kawasaki disease, in order to lay a good foundation for the development of clinical treatment.&lt;/p&gt;

The role of echocardiography in Kawasaki disease.

Kawasaki disease (KD) is an acute, self-limited vasculitis affecting young children. It can result in coronary artery abnormalities in a significant proportion of patients, especially if the diagnosis is missed or treatment gets delayed. Echocardiography is the imaging modality of choice for detection of coronary artery abnormalities and assessment of myocardial function. It is also useful for characterization and risk stratification of patients with KD. Echocardiography should be performed at the time of diagnosis and then again at 1-2 weeks and 4-6 weeks after treatment, for uncomplicated cases who do not have significant coronary artery involvement. Use of a standardized imaging protocol is necessary to detect and characterize coronary artery abnormalities, including standardization of measurements (Z scores). For patients with evolving abnormalities, more frequent assessment is necessary in order to detect thromboses in aneurysms. Long-term prognosis and management is dependent on both the maximal and current Z scores of aneurysms. Patients with large or giant aneurysms (i.e., Z score ≥ 10) are at the highest risk of both thrombosis and stenosis. Such patients need careful follow-up for subsequent cardiovascular events. Many of them would be candidates for advanced cardiovascular imaging and may require revascularization therapy. Serial echocardiography plays a key role in surveillance. In addition, stress echocardiography has proven useful as a modality to assess for inducible myocardial ischemia. Intravascular ultrasound has been recommended for functional and structural assessment of coronary arteries in children with KD.

Computed Tomography Coronary Angiography for Evaluation of Children With Kawasaki Disease

We sought to assess the feasibility of computed tomography coronary angiography (CTCA) on a 128-slice, dual source scanner in children with acute and convalescent phase Kawasaki disease (KD). A prospective study of 49 children with KD (12 at presentation and 37 in the convalescent phase) was conducted between November 2013 and April 2015. CTCA was performed with either prospective (n = 37) or retrospective (n = 12) electrocardiographic gating. A radiologist blinded to clinical profile and echocardiogram evaluated each scan. Median age (36 boys and 13 girls) was 7 years. Median dose-length product value and median effective CT radiation dose was 32mGycm (interquartile range [IQR]: 21-74) and 0.54 miliSieverts (mSv) (IQR: 0.77-3.2) for all scans, and 27mGycm (IQR: 18.5-33.75) and 0.48mSv (IQR: 0.18-1.17) for prospectively triggered scans (n = 37). Fourteen subjects (30 coronary segments) showed abnormalities by CTCA including aneurysms (n = 27) and stenoses (n = 3). In the acute phase (n = 12), aneurysms were detected in 5 children (18 segments). CTCA allows comprehensive evaluation of coronary arteries in children with KD.

Abnormalities of the Coronary Arteries in Children: Looking beyond the Origins.

Coronary arterial abnormalities are uncommon findings in children that have profound clinical implications. Although anomalies of the coronary origins are well described, there are many other disease processes that affect the coronary arteries. Immune system-mediated diseases (eg, Kawasaki disease, polyarteritis nodosa, and other vasculiditides) can result in coronary arterial aneurysms, strictures, and abnormal tapering of the vessels. Because findings at imaging are an important component of diagnosis in these diseases, the radiologist's understanding of them is essential. Congenital anomalies may present at varying ages, and findings in hemodynamically significant anomalies, such as fistulas, are key for both diagnosis and preoperative planning. Pediatric heart surgery can result in wide-ranging postoperative imaging appearances of the coronary arteries and also predisposes patients to a multitude of complications affecting the heart and coronary arteries. In addition, although rare, accidental trauma can lead to injury of the coronary arteries, and awareness and detection of these conditions are important for diagnosis in the acute setting. Patients with coronary arterial conditions at presentation may range from being asymptomatic to having findings of myocardial infarction. Recognition of the imaging findings is essential to direct appropriate treatment. ©RSNA, 2017.

Arterial immune protein expression demonstrates the complexity of immune responses in Kawasaki disease arteritis.

A more complete understanding of immune-mediated damage to the coronary arteries in children with Kawasaki disease (KD) is required for improvements in patient treatment and outcomes. We recently reported the transcriptional profile of KD coronary arteritis, and in this study sought to determine protein expression of transcriptionally up-regulated immune genes in KD coronary arteries from the first 2 months after disease onset. We examined the coronary arteries of 12 fatal KD cases and 13 childhood controls for expression of a set of proteins whose genes were highly up-regulated in the KD coronary artery transcriptome: allograft inflammatory factor 1 (AIF1), interleukin 18 (IL-18), CD74, CD1c, CD20 (MS4A1), Toll-like receptor 7 (TLR-7) and Z-DNA binding protein 1 (ZBP1). Immunohistochemistry and immunofluorescence studies were performed to evaluate protein expression and co-localization, respectively. AIF1 was expressed transmurally in KD arteritis and localized to macrophages and myeloid dendritic cells. CD74, which interacts with major histocompatibility complex (MHC) class II on antigen-presenting cells, localized to the intima-media. CD1c, a marker of myeloid dendritic cells, was expressed in a transmural pattern, as were IL-18 and CD20. ZBP1 and TLR-7 were up-regulated compared to controls, but less highly compared to the other proteins. These findings provide evidence of antigen presentation and interferon response in KD arteritis. In combination with prior studies demonstrating T lymphocyte activation, these results demonstrate the complexity of the KD arterial immune response.

Inositol-Triphosphate 3-Kinase C Mediates Inflammasome Activation and Treatment Response in Kawasaki Disease.

Kawasaki disease (KD) is a multisystem vasculitis that predominantly targets the coronary arteries in children. Phenotypic similarities between KD and recurrent fever syndromes point to the potential role of inflammasome activation in KD. Mutations in NLRP3 are associated with recurrent fever/autoinflammatory syndromes. We show that the KD-associated genetic polymorphism in inositol-triphosphate 3-kinase C (ITPKC) (rs28493229) has important functional consequences, governing ITPKC protein levels and thereby intracellular calcium, which in turn regulates NLRP3 expression and production of IL-1β and IL-18. Analysis of transcript abundance, protein levels, and cellular response profiles from matched, serial biospecimens from a cohort of genotyped KD subjects points to the critical role of ITPKC in mediating NLRP3 inflammasome activation. Treatment failure in those with the high-risk ITPKC genotype was associated with the highest basal and stimulated intracellular calcium levels and with increased cellular production of IL-1β and IL-18 and higher circulating levels of both cytokines. Mechanistic studies using Itpkc-deficient mice in a disease model support the genomic, cellular, and clinical findings in affected children. Our findings provide the mechanism behind the observed efficacy of rescue therapy with IL-1 blockade in recalcitrant KD, and we identify that regulation of calcium mobilization is fundamental to the underlying immunobiology in KD.

GW27-e0947 Image quality of coronary computed tomography angiography with 320-Row area detector computed tomography in children with congenital heart disease

To assess factors affecting image quality of 320-row computed tomography angiography (CTA) of coronary arteries in children with congenital heart disease (CHD). We retrospectively reviewed 28 children up to 3 years of age with CHD who underwent prospective electrocardiography (ECG)-gated 320-row

Novel Echocardiographic Indices for Assessing the Left Main Coronary Artery in Children With Kawasaki Disease.

BackgroundKawasaki disease (KD) is the most common cause of acquired myocardial infarction in children. Coronary artery involvement is the most serious feature of this vasculitis syndrome. Timely diagnosis of coronary artery involvement is of utmost importance since it can prevent long-term morbidity and mortality. The current methods for the diagnosis of coronary artery dilation in KD are inconsistent and are also not capable of detecting all the abnormal coronary arteries or the so-called occult dilations present.ObjectivesThe aim of this study was to determine the sensitivity and specificity of three novel allometric indices for the diagnosis of left main coronary artery (LMCA) involvement in KD.MethodsWe performed a prospective case-control study in 69 children (38 with KD and 31 healthy children). All the followed up patients underwent two complete echocardiographic examinations at the time of admission and 4 - 6 weeks later. We measured the size of the LMCA, coronary sinus (CS) and aorta (A) and calculated the LMCA/CS, LMCA/A and LMCA/CS/A ratios. We also calculated the cut-off scores for each index using receiver operating characteristic curves both in the acute phase and 4 - 6 weeks later.ResultsIn the acute phase, the cut-off scores for the LMCA/A ratio was > 0.23; LMCA/CS, > 0.44; and LMCA/CS/A, > 0.03. This implied 60% sensitivity and 80% specificity for the detection of abnormal LMCA in KD. Four to six weeks after the acute phase, the LMCA/A cut-off score was > 0.23; LMCA/CS, > 0.73; and LMCA/CS/A, > 0.73. This implied 100% sensitivity and 100% specificity for the detection of abnormal LMCA. There was a significant decrease in the size of the CS in comparison with the control group (1.92 ± 0.363 mm; P = 0.007 and 0.023).ConclusionsThe LMCA/A, LMCA/CS and LMCA/CS/A ratios seem to provide simple and patient-specific indices for the detection of abnormal LMCA in KD, both in the acute and subacute phase. Further, a decrease in the size of the CS may imply a decrease in coronary artery flow in the acute and subacute phases of KD.