Research advances in genetic polymorphisms in Kawasaki disease / 中国当代儿科杂志

Kawasaki disease (KD) is a systemic inflammatory vascular disorder that predominantly affects children and is the leading cause of acquired heart disease in children. Although the etiology of this disease remains unclear, genome-wide association and genome-wide linkage studies have shown that some susceptible genes and chromosomal regions are associated with the development and progression of KD. With the advancement of high-throughput DNA sequencing techniques, more and more genomic information related to KD is being discovered. Understanding the genes involved in the pathogenesis of KD may provide novel insights into the diagnosis and treatment of KD. By analyzing related articles and summarizing related research advances, this article mainly discusses the T cell activation-enhancing genes that have been confirmed to be closely associated with the development and progression of KD and reveals their association with the pathogenesis of KD and coronary artery lesions.

Role and mechanisms of CHI3L1 in coronary artery lesions in a mouse model of Kawasaki disease-like vasculitis / 中国当代儿科杂志

OBJECTIVES@#To explore the role and potential mechanisms of chitinase-3-like protein 1 (CHI3L1) in coronary artery lesions in a mouse model of Kawasaki disease (KD)-like vasculitis.@*METHODS@#Four-week-old male SPF-grade C57BL/6 mice were randomly divided into a control group and a model group, with 10 mice in each group. The model group mice were intraperitoneally injected with 0.5 mL of lactobacillus casei cell wall extract (LCWE) to establish a mouse model of KD-like vasculitis, while the control group mice were injected with an equal volume of normal saline. The general conditions of the mice were observed on the 3rd, 7th, and 14th day after injection. Changes in coronary artery tissue pathology were observed using hematoxylin-eosin staining. The level of CHI3L1 in mouse serum was measured by enzyme-linked immunosorbent assay. Immunofluorescence staining was used to detect the expression and localization of CHI3L1, von Willebrand factor (vWF), and α-smooth muscle actin (α-SMA) in coronary artery tissue. Western blot analysis was used to detect the expression of CHI3L1, vWF, vascular endothelial cadherin (VE cadherin), Caspase-3, B cell lymphoma-2 (Bcl-2), Bcl-2 associated X protein (Bax), nuclear factor κB (NF-κB), and phosphorylated NF-κB (p-NF-κB) in coronary artery tissue.@*RESULTS@#The serum level of CHI3L1 in the model group was significantly higher than that in the control group (P<0.05). Compared to the control group, the expression of CHI3L1 in the coronary artery tissue was higher, while the expression of vWF was lower in the model group. The relative expression levels of CHI3L1, Bax, Caspase-3, NF-κB, and p-NF-κB were significantly higher in the model group than in the control group (P<0.05). The relative expression levels of vWF, VE cadherin, and Bcl-2 were lower in the model group than in the control group (P<0.05).@*CONCLUSIONS@#In the LCWE-induced mouse model of KD-like vasculitis, the expression levels of CHI3L1 in serum and coronary arteries increase, and it may play a role in coronary artery lesions through endothelial cell apoptosis mediated by inflammatory reactions.

Platelet-to-lymphocyte ratio as a biomarker for predicting coronary artery lesions in Chinese children with Kawasaki disease: a Meta analysis / 中国当代儿科杂志

OBJECTIVES@#To systematically evaluate the value of the platelet-to-lymphocyte ratio (PLR) in predicting coronary artery lesions (CAL) in Chinese children with Kawasaki Disease (KD).@*METHODS@#A comprehensive search was conducted in databases including PubMed, Embase, Cochrane Library, Web of Science, China National Knowledge Infrastructure, Wanfang Data, China Biomedical Literature Database, and China Science and Technology Journal Database from inception to December 2022. The quality of the included literature was assessed using the Newcastle-Ottawa Scale, and a Meta analysis was performed using Stata 15.1.@*RESULTS@#A total of ten published reports, involving 3 664 Chinese children with KD, were included in this Meta analysis, of whom 1 328 developed CAL. The Meta analysis revealed a sensitivity of 0.78 (95%CI: 0.71-0.83), specificity of 0.71 (95%CI: 0.61-0.80), overall diagnostic odds ratio of 8.69 (95%CI: 5.02-15.06), and an area under the curve of the summary receiver operating characteristic of 0.82 (95%CI: 0.78-0.85) for PLR in predicting CAL in the children with KD. The sensitivity, specificity, and area under the curve of summary receiver operating characteristic were lower for PLR alone compared to PLR in combination with other indicators. Sensitivity analysis demonstrated the stability of the Meta analysis results with no significant changes upon excluding individual studies. However, a significant publication bias was observed (P<0.001).@*CONCLUSIONS@#PLR demonstrates certain predictive value for CAL in Chinese children with KD.

Protection of side-branch ostium by the jailed balloon technique validated by three-dimensional optical coherence tomography / 中华心血管病杂志

Objective: To evaluate the protective effect of jailed balloon technique on side branch (SB) ostium using three-dimensional optical coherence tomography(OCT). Methods: This is a retrospective study. Consecutive coronary disease patients with coronary artery bifurcation lesions who underwent percutaneous coronary intervention (PCI) and completed pre-and post-procedural OCT examinations at the Chinese People's Liberation Army General Hospital from September 2019 to March 2022 were enrolled. Patients were divided into the jailed balloon technique group and the unprotected group according to the options applied for the SB. The SB ostium area difference was calculated from OCT images (SB ostium area difference=post-PCI SB ostium area-pre-PCI SB ostium area). The SB ostium area differences were compared between the two groups and compared further in the subgroup of true bifurcation lesions and non-true bifurcation lesions. In the jailed balloon group, the SB ostium area difference was compared between the active jailed balloon technique and the conventional jailed balloon technique, between the jailed balloon>2.0 mm diameter and the jailed balloon≤2.0 mm diameter, and between the higher balloon pressure (>4 atm, 1 atm=101.325 kPa) and the lower balloon pressure (≤4 atm). Multivariate linear regression analysis was used to explore the correlation between the technical parameters of the jailed balloon technique and the SB protection effect. Results: A total of 176 patients with 236 bifurcation lesions were enrolled, aged (60.7±9.3) years, and there were 128 male patients (72.7%). There were 67 patients in the jailed balloon technique group with 71 bifurcation lesions and 123 patients in the unprotected group with 165 bifurcation lesions. Fourteen patients had 2 to 3 lesions, which were treated in different ways, so they appeared in the unprotected group and the jailed balloon technique group at the same time. The area difference in SB ostium was greater in the jailed balloon group than in the unprotected group (0.07 (-0.43, 1.05)mm2 vs.-0.33 (-0.83, 0.26)mm2, P<0.001), and the results were consistent in the true bifurcation lesion subgroup (0.29 (-0.35, 0.96)mm2 vs.-0.26 (-0.64, 0.29)mm2, P=0.004), while the difference between the two groups in the non-true bifurcation lesion subgroup was not statistically significant (P=0.136). In the jailed balloon technique group, the SB ostium area difference was greater in patients treated with the active jailed balloon technique than in those treated with the conventional jailed balloon technique ((0.43±1.36)mm2 vs. (-0.22±0.52)mm2, P=0.013). The difference in SB ostium area was greater in those using>2.0 mm diameter jailed balloons than in those using≤2.0 mm diameter jailed balloons (0.25 (-0.51, 1.31) mm2 vs.-0.01 (-0.45, 0.63) mm2, P=0.020), while SB ostium area difference was similar between those endowed with higher balloon pressure (>4 atm) compared to those with lower balloon pressure (≤4 atm) (P=0.731). Multivariate linear regression analysis showed that there was a positive correlation between jailed balloon diameter and SB ostium area difference (r=0.344, P=0.019). Conclusions: The jailed balloon technique significantly protects SB ostium, especially in patients with true bifurcation lesions. The active jailed balloon technique and larger diameter balloons may provide more protection to the SB.

Dissecção espontânea da artéria coronária em gestação gemelar no terceiro trimestre: relato de caso / Spontaneous coronary artery dissection in a twin pregnancy in the third trimester: a case report

O infarto agudo do miocárdio no período gravídico-puerperal é uma condição rara em que a principal causa é a dissecção espontânea da artéria coronária. É um evento comumente subdiagnosticado, com pouca literatura disponível e elevado índice de morbimortalidade. Esse relato descreve o caso de uma gestante de 36 semanas de gravidez gemelar, monocoriônica-diamniótica, com infarto agudo do miocárdio secundário à dissecção espontânea da artéria coronária. As equipes de cirurgia cardíaca e obstetrícia optaram pela realização de parto cesariano e histerectomia subtotal, seguido da revascularização da artéria mamária descendente anterior. Discutem-se as orientações adotadas na dissecção espontânea da artéria coronária, bem como a abordagem terapêutica e a conduta obstétrica, quando essa condição ocorre durante a gravidez.(AU)

Acute myocardial infarction in the pregnancy-puerperal period is a rare condition the main cause of which is the spontaneous coronary artery dissection. A commonly underdiagnosed event with little available literature and a high rate of morbidity and mortality. This case reports a 36-week pregnant woman of mono-chorionic-diamniotic pregnancy who had a myocardial infarction secondary to a spontaneous coronary artery dissection. In a joint discussion between the team of cardiac surgery and obstetrics, it was decided to perform a cesarean delivery and subtotal hysterectomy followed by revascularization of the mammary anterior descendant. It discusses the conducts to be adopted in a case of spontaneous coronary artery dissection as well as therapeutic approaches and obstetric conducts to be taken in a case of dissection during pregnancy.(AU)

Association of liver damage with coronary artery lesion and no response to intravenous immunoglobulin in the acute stage of Kawasaki disease / 中国当代儿科杂志

OBJECTIVES@#To summarize the clinical features of liver damage in children in the acute stage of Kawasaki disease (KD), and to investigate the clinical value of liver damage in predicting coronary artery lesion and no response to intravenous immunoglobulin (IVIG) in children with KD.@*METHODS@#The medical data were collected from 925 children who were diagnosed with KD for the first time in Beijing Children's Hospital from January 1, 2016 to December 31, 2017. According to the presence or absence of abnormal alanine aminotransferase (ALT) level on admission, the children were divided into a liver damage group (n=284) and a non-liver damage group (n=641). A logistic regression analysis was used to investigate the clinical value of the indicators including liver damage in predicting coronary artery lesion and no response to IVIG in children with KD.@*RESULTS@#Compared with the non-liver damage group, the liver damage group had a significantly earlier admission time and significantly higher serum levels of inflammatory indicators (P<0.05). The liver damage group had a significantly higher incidence rate of coronary artery lesion on admission than the non-liver damage group (P=0.034). After initial IVIG therapy, the liver damage group had a significantly higher proportion of children with no response to IVIG than the non-liver damage group (P<0.001). In children with KD, coronary artery lesion was associated with the reduction in the hemoglobin level and the increases in platelet count, C-reactive protein, and ALT (P<0.05), and no response to IVIG was associated with limb changes, the reduction in the hemoglobin level, the increases in platelet count, C-reactive protein, and ALT, and coronary artery lesion (P<0.05).@*CONCLUSIONS@#Compared with those without liver damage, the children in the early stage of KD with liver damage tend to develop clinical symptoms early and have higher levels of inflammatory indicators, and they are more likely to have coronary artery lesion and show no response to IVIG treatment.

Pediatric expert consensus on the application of aspirin in Kawasaki disease / 中国当代儿科杂志

Kawasaki disease (KD) is one of the common acquired heart diseases in children aged <5 years and is an acute systemic vasculitis. After nearly 60 years of research, intravenous immunoglobulin combined with oral aspirin has become the first-line treatment for the prevention of coronary artery lesion in acute KD; however, there are still controversies over the role and optimal dose of aspirin. The consensus was formulated based on the latest research findings of KD treatment in China and overseas and comprehensive discussion of pediatric experts in China and put forward recommendations on the dose, usage, and course of aspirin treatment in the first-line treatment of KD.

Fístula coronário-cavitária da artéria coronária circunflexa para o átrio esquerdo como provável etiologia de insuficiência cardíaca: relato de caso / Coronary­cavitary fistula of the circumflex coronary artery to the left atrium as a probable etiology of heart failure: a case report

Fístulas de artérias coronárias têm incidência baixa, sendo diagnosticadas mais frequentemente pelo ecocardiograma ou angiotomografia coronariana, embora a cineangiocoronariografia seja o método padrão-ouro. Têm origem mais comumente na artéria coronária direita, sendo a drenagem para câmaras de baixa pressão o mais habitual. O tratamento pode ser expectante, cirúrgico ou percutâneo. Este relato descreve o caso de uma rara apresentação de insuficiência cardíaca, dada por fístula da coronária circunflexa com drenagem para o átrio esquerdo. (AU)

Coronary artery fistulas have a low incidence and are often diagnosed by echocardiography or coronary computed tomography angiography, although coronary angiography is the gold standard. They commonly originate in the right coronary artery, with drainage to low-pressure chambers being the most frequent finding. Treatment can be expectant, surgical, or percutaneous. This report describes a case of a rare presentation of heart failure due to a fistula of the circumflex coronary artery with drainage into the left atrium. (AU)

Coronary arteritis as a cause of sudden cardiac death in a young girl

A case of probable coronary arteritis in a young girl who died suddenly and unexpectedly is presented. The histologic presentation of the disorder is discussed, especially the differential diagnosis of arteritis of the coronary arteries with an emphasis on tuberculosis (TB). TB myocarditis with or without concomitant lung involvement is rare, and tubercular coronary arteritis without underlying pulmonary Koch's disease is all the rarer. We herein describe a case where the cause of death was ascertained on post-mortem examination.

Clinical features of children with recurrent Kawasaki disease: a Meta analysis / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features of children with recurrent Kawasaki disease (KD).@*METHODS@#PubMed, Web of Science, Embase, CNKI, Wanfang Med Online, and Weipu Data were searched for case-control studies on the clinical features of initial and recurrent KD. The articles were screened according to the inclusion and exclusion criteria. RevMan 5.3 software was used to perform the Meta analysis. Effect models were selected based on the results of heterogeneity test, and then pooled @*RESULTS@#A total of 9 case-control studies were included, with 12 059 children with KD in total, among whom 206 children had recurrent KD (127 boys/61.7%; 79 girls/38.3%). The results of the Meta analysis showed that compared with the initial KD onset, the children with recurrent KD had a shorter duration of fever (@*CONCLUSIONS@#Current evidence shows that children with recurrent KD tend to have a shorter duration of fever and a lower incidence of swelling of the hands and feet. KD recurrence is more common in boys. Current evidence does not show an increased risk of developing coronary artery lesions in children with recurrent KD.

Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso / Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

Correlação entre a complexidade das lesões coronarianas e os níveis de Troponina ultrassensível em pacientes com Síndrome Coronariana Aguda / Correlation between the complexity of coronary lesions and high-sensitivity Troponin levels in patients with Acute Coronary Syndrome

Fundamento: As doenças cardiovasculares são a principal causa de morte no Brasil. Marcadores bioquímicos possuem importância diagnóstica e prognóstica nas síndromes coronarianas agudas (SCAs), sendo a troponina o biomarcador preferido. Estudos já demonstram relação positiva entre elevação da troponina ultrassensível (TnUs) e prognóstico. Entretanto, poucos relacionam seus níveis com a complexidade das lesões coronárias. Objetivos: Comparar níveis de TnUs com a complexidade das lesões coronarianas pelo escore SYNTAX e relacionar os escores TIMI e GRACE com os níveis desse biomarcador em pacientes com SCA. Métodos: Estudo retrospectivo, transversal com 174 indivíduos com SCA. A correlação entre as variáveis foi avaliada pelo teste de correlação linear não paramétrico de Spearman e a análise estatística realizada pelo programa SPSS, com nível de significância de 5%. Resultados: A média de idade foi 63 anos, predominando o sexo feminino (52,9%). A maioria dos pacientes era hipertensa, não diabética e não tabagista. Dos pacientes avaliados, 19,0% apresentaram IAM com SST, 43,1% IAM sem SST e 36,8% angina instável. A maioria encontrava-se em Killip 1 (82,8%). A mediana de TnUs foi de 67pg/ml. As medianas dos escores de risco foram de 3, 121 e 3 pontos nas escalas TIMI, GRACE e SYNTAX, respectivamente. Houve correlação da taxa de TnUs com os escores SYNTAX (p < 0,001, r = 0,440), TIMI (p < 0,001, r = 0,267) e GRACE (p = 0,001, r = 0,261). Conclusão: Encontrada correlação linear positiva entre os níveis de TnUs e complexidade das lesões coronarianas, assim como entre esse biomarcador e os escores clínicos TIMI e GRACE

Background: Cardiovascular diseases are the leading cause of death in Brazil. Biochemical markers have diagnostic and prognostic importance in acute coronary syndromes (ACSs), with troponin as the preferred biomarker. Studies have already demonstrated a positive relationship between increased levels of high-sensitivity troponin (hsTn) and prognosis. However, few studies have correlated hsTn levels with the complexity of coronary lesions. Objectives: To compare hsTn levels with the complexity of coronary lesions according to the SYNTAX score, and to correlate the levels of this biomarker with the TIMI and GRACE scores in patients with ACS. Methods: Retrospective, cross-sectional study with 174 patients with ACS. The correlation between variables was assessed by the nonparametric Spearman's rank correlation, and statistical analysis was performed by the SPSS program, with a significance level of 5%. Results: Mean age was 63 years, and most patients were women (52.9%), hypertensive, non-diabetic and non-smokers. Nineteen percent of the patients had STEMI, 43.1% NSTEMI, and 36.8% unstable angina. Most were in Killip 1 (82.8%). Median hsTn was 67 pg/mL. Median risk scores were 3, 121 and 3 in the TIMI, GRACE and SYNTAX scores, respectively. There was a correlation of hsTn with SYNTAX (p <0.001, r = 0.440), TIMI (p < 0.001, r = 0.267) and GRACE (p = 0.001, r = 0.261) scores. Conclusion: A positive linear correlation was found of hsTn levels with the complexity of coronary lesions, and with the TIMI and GRACE

Reporte de un caso de puente intramiocárdico en arteria coronaria derecha / A case report of intramyocardial bridge on right coronary artery

Los puentes intramiocárdicos son una anomalía de la circulación coronaria. Estos suelen presentarse en el trayecto de la arteria descendente anterior y se clasifican en superficiales o profundos. Reportamos el hallazgo de un puente intramiocárdico en el trayecto de la arteria coronaria derecha, incluida su rama sinoauricular y descendente posterior durante la disección de rutina de un cadáver. El paciente al parecer no presentó signos relativos a insuficiencia coronaria y falleció debido a una insuficiencia respiratoria aguda causada por neumonía aspirativa. Debido a la presencia limitada de casos sobre puente intramiocárdico de la arteria coronaria derecha, realizamos una revisión de la literatura al respecto y planteamos algunas conclusiones.

Intramyocardial bridges are a coronary circulation anomaly. They usually occur on the path of the anterior descending artery and are classified as superficial or deep. During a routine autopsy, we found­and report­an intramyocardial bridge in large part of the right coronary artery pathway, including the sinoatrial and posterior descending branch. The patient did not show signs of coronary insufficiency, and died from an acute respiratory failure caused by aspiration pneumonia. Due to the infrequency of cases of intramyocardial bridges of the right coronary artery without concomitant clinical symptoms or signs, we conducted a literature review in this regard.

Clinical Outcomes of Low-Dose Methotrexate Therapy as a Second-Line Drug for Intravenous Immunoglobulin-Resistant Kawasaki Disease

PURPOSE: Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease (KD). However, there is still no standard treatment for IVIG-resistant KD. This study aimed to evaluate the efficacy of low-dose methotrexate (MTX) as a treatment for IVIG-resistant KD. MATERIALS AND METHODS: We retrospectively analyzed 10-year data for patients with IVIG-resistant KD who were administered MTX at Severance Children's Hospital. RESULTS: The subjects included 75 patients with KD aged 5 months to 9.2 years who had been administered MTX. Their maximum body temperatures decreased significantly within 24 h of therapy. The patients' C-reactive protein levels were significantly lower 1 week after administering the first dose of MTX than those before treatment. No adverse effect for MTX was observed. CONCLUSION: MTX treatment of IVIG-resistant KD resulted in rapid defervescence, improvement of clinical symptoms, and normalization of acute-phase reactants in all patients. Thus, MTX could be a candidate treatment for IVIG-resistant KD.

A case of intramural coronary amyloidosis associated with hemodialysis

Dialysis-related amyloidosis predominantly occurs in osteo-articular structures and dialysis-related amyloid (DRA) substances also deposit in extra-articular tissues. Clinical manifestations of DRA include odynophagia, gastrointestinal hemorrhage, intestinal obstruction, kidney stones, myocardial dysfunction, and subcutaneous tumors. The pathological characteristics of DRA in the heart of hemodialysis patients have rarely been reported. We report the case of a 73-year-old female with a history of cerebral palsy and end-stage renal disease status post two failed renal transplants who had been on hemodialysis for 30 years. The patient was admitted with the working diagnosis of pneumonia. An echocardiography showed markedly reduced biventricular function manifested by low blood pressure with systolic in the 70s and elevated pulmonary artery pressure of 45 mmHg, which did not respond to therapy. Following her demise, the autopsy revealed bilateral pulmonary edema and pleural effusions. There was cardiac amyloid deposition exclusively in the coronary arteries but not in the perimyocytic interstitium. Amyloids were also found in pulmonary and intrarenal arteries and the colon wall. Previous case reports showed that beta 2-microglobulin amyloid deposits in various visceral organs but less frequently in the atrial and/or the ventricular myocardium. In the present case, amyloids in the heart were present in the intramural coronary arteries causing myocardial ischemia and infarction, which was the immediate cause of death.