Coronary Artery Abnormalities Research Articles

Multiple coronary artery microfistulae are common and progress in adult patients late after Fontan operation

Abstract Background Coronary angiograms of adult Fontan patients sometimes show the abnormal multiple coronary artery microfistulae (mCAFs) that drain directly into the ventricular chamber. They look like coronary artery fistulae or Thebesian vein. Angiographically detectable mCAFs are a rare finding sometimes associated with myocardial ischemia due to coronary steal and volume overload of ventricles in non-CHD adults. However, there are limited data concerning these mCAFs in the setting of CHD patients. Purpose The aim of this study is to demonstrate presence, character, and changes with growth of mCAFs in adult patients late after Fontan operation. Methods This study is single-center retrospective review of adults (16 years old or older) with Fontan circulation patients who had undergone cardiac catheterization for routine Fontan surveillance between 2009-2022. We assessed the presence of angiographically detectable mCAFs from ascending aortography. After identification of the characteristic cases, we examined the ventricular morphology, the portion of drainage, and compared with previous findings of angiograms. SaO2, EF of main ventricle, CVP, EDP of main ventricle, BNP, and use of pulmonary vasodilators were compared between mCAFs(+) group and mCAFs(-) group. We also reviewed clinical manifestation and findings of exercise stress ECG of the patients. Pulmonary atresia with intact ventricular septum was excluded because abnormal coronary arterial communications have been well documented in this condition. Results Among 90 Fontan patients who had undergone catheterization, significant mCAFs were observed in a total of 18 cases (20%). There were 10 right-dominant single ventricle, 4 left-dominant single ventricle, and 4 unbalanced two ventricles. In all cases, including left-dominant single ventricle, all fistulae drained into right ventricle cavity, never into left ventricle. The patients who underwent serial catheterizations demonstrated that the dilation and tortuosity of fistulae progressed over time. The comparison of two groups showed SaO2 was low, BNP and CVP was high significantly in mCAFs(+) group. There were no significant differences in other factors including age at catheterization and the use of pulmonary vasodilators. Among 10 patients who had underwent exercise stress ECG, only 1 patient showed ST segment depression. None had clinical history of chest pain, and arrhythmia, and ischemic event. Conclusions MCAFs are common and progress in adult patients late after Fontan operation. Although the clinical significance and etiology of these findings remains uncertain, particular attention should be given to subclinical Fontan failure caused by hhigh CVP, volume overload, and myocardial ischemia for the future of adult Fontan patients who have prominent mCAFs.

Fully automated quantitative stress perfusion cardiac MRI to assess myocardial perfusion in adult patients with convalescent Kawasaki disease and coronary aneurysms: an age and sex-matched analysis

Abstract Introduction Coronary aneurysms are a well-documented complication of childhood Kawasaki disease. They may persist into adulthood predisposing to impaired myocardial blood flow, symptoms, and adverse cardiovascular events. Preliminary reports suggest that Kawasaki disease may also be associated with coronary microvascular dysfunction. The effect of coronary involvement from Kawasaki disease on myocardial perfusion in convalescent asymptomatic adults has not been studied using fully automated inline quantitative perfusion MRI (CMR). Purpose To assess the effect of coronary artery abnormalities on quantitative myocardial perfusion in adult patients with convalescent Kawasaki disease compared to an age and sex-matched control cohort. Methods Retrospective single centre cohort analysis of adult patients with convalescent Kawasaki disease who have undergone fully automated inline quantitative myocardial stress perfusion CMR. All patients had coronary involvement during their acute childhood illness. An age and sex-matched group (n=14) with normal CMRs was selected for comparison. Rest MBF was corrected for heart rate by dividing by the scan heart rate and multiplying by the mean resting heart rate among all subjects. A per-vessel analysis was performed using a linear mixed effects model with pairwise comparisons to assess the effect of coronary abnormalities (normal, aneurysmal with or without thrombosis, revascularised) on myocardial perfusion reserve (MPR). Results 28 patients were included (14 Kawasaki; 14 controls). All patients in the Kawasaki cohort had assessment of coronary anatomy either by CT (93%) or invasive (7%) coronary angiography. A total of 84 vessels were analysed. Mean age and sex were well matched (Kawasaki: 27±6.2 years; 13 males : 1 female vs. controls: 27±6.4 years; 13 males : 1 female). In patients with Kawasaki disease, thrombosed aneurysmal arteries were associated with a significantly impaired MPR compared to normal (mean Δ: -0.817; P=0.003) and aneurysmal (mean Δ: -0.957; P<0.001) arteries. MPR in aneurysmal and revascularised arteries were not significantly different from normal arteries in patients with Kawasaki disease. There was no significant difference in MPR between normal coronary arteries in patients with Kawasaki compared to controls. Conclusions Thrombosis in coronary aneurysms was associated with a significant impairment of myocardial perfusion in adult patients with convalescent Kawasaki disease. There was no significant reduction in perfusion in territories supplied by normal or aneurysmal arteries which does not support coronary microvascular dysfunction in these patients. Further studies are needed to confirm these preliminary findings.

Abstract 15506: Pattern of Use of Anakinra for Patients With Kawasaki Disease versus Multisystem Inflammatory Syndrome in Children Associated With COVID-19

Background/Aims: Anakinra (interleukin-1 blocker) has emerged as a potential adjunctive therapy for treating resistant acute Kawasaki disease (KD) and those with evolving aneurysms, and has been used as both primary and adjunct therapy for treating Multisystem Inflammatory Syndrome in Children (MIS-C), both in the absence of randomized trials. We sought to determine patterns of use, adverse events and evidence of benefit. Methods: The International KD Registry contemporaneously enrolled 727 patients with KD (site diagnosis confirmed by AHA criteria) and 1476 with MIS-C (site diagnosis confirmed by CDC criteria) from 39 sites in 7 countries from 01/2020 to 01/2023. Data collected included demographics, clinical features and presentation, management, laboratory values, and outcomes. Results: Anakinra was used at 22 (56%) sites for 11 (1.5%) KD patients (median duration 21 days) and 257 (17.4%) MIS-C patients (8 days; 11% discharged on anakinra). For KD, anakinra was used for treatment resistance for 5 sites, evolving aneurysms 3, macrophage activation syndrome (MAS) 1 and not-specified for 2. For MIS-C, anakinra use was determined by protocol for 3 sites, on a case-by-case basis for 10, reserved for treatment failure for 3 and not-specified for 6 sites. Reported indications for use (may be multiple) included routine use for ICU patients for 34% of treated MIS-C patients, worsening/persistent lab abnormalities 28%, critical decompensation (primarily cardiac) 21%, persistent/recurrent fever 21%, treatment failure 9%, coronary artery abnormalities 8%, and cytokine storm/MAS for 4%. Adverse events in treated MIS-C patients were noted for 23 (9%) and included neutropenia in 9, transaminitis 8, injection site pain/rash 4 and one each with anemia and thrombocytopenia. Extreme patient heterogeneity and confounding by indication precluded a formal analysis of impact on outcomes, although sites reported evidence of clinical improvement relevant to reported indications for all but one patient. Conclusions: Greater anakinra use for MIS-C vs KD likely reflects greater perceived indication, primarily greater clinical and inflammatory severity. Subjective evidence of benefit and a low prevalence of adverse events suggest an ongoing role for anakinra.

Abstract 15834: The Utility of Intra-Operative Coronary Doppler Profiles in Predicting Early and Late Major Adverse Cardiovascular Events After Arterial Switch Operation

Introduction: Coronary artery transfer is the most important technical point in the arterial switch operation (ASO). Intra-operative echocardiography identifies aberrancies in coronary artery Doppler profiles guiding immediate surgical management. Objective: We sought to determine whether abnormal intra-operative coronary artery Doppler profiles are associated with major adverse cardiovascular events (MACE; death, transplantation, heart failure, arrhythmia, need for coronary re-intervention) following ASO at 30-days, 5 & 10 years. Methods: Single centre retrospective review of patients undergoing ASO between 2009 - 2022. Transesophageal or epicardial intra-operative coronary artery Doppler profiles were analysed. Patient variables were abstracted from records. Freedom from MACE was estimated using Kaplan-Meier methods. Results: 347 patients were included. Coronary anatomy was usual (1LCx2R) in 237 (68%), single sinus ostia in 24 (7%), intramural in 14 (4 %). Overall 30-day MACE was 11.2% [8.3%, 15.1%], increasing to 25% [12.1, 47.4%] with single sinus ostia & 35.7% [16.7%, 65.7%] with intramural. Overall 5 & 10-year MACE was 16.5% [12.9, 20.9%], 33.8% [18.5%, 56.4%] with single sinus ostia & 50.0% [27.8%, 77.1%] with intramural. A higher probability of MACE was seen with coronary Doppler reverse flow, absence of normal pattern (Table 1) and a higher peak velocity (Figure 1). Overall 30-day mortality was 1.2% [95%CI 0.4%, 3.0%] and at 5 & 10 years was 6.5% [4.3%, 9.8%]. Conclusions: Intra-operative abnormal coronary artery Doppler profiles during ASO may identify those at highest risk of early and late adverse events, guiding follow-up surveillance strategies.

Kawasaki Disease Associated Acute Abdomen: Most Require No Surgery.

To summarize the clinical features and treatment experiences of patients with Kawasaki disease (KD)-associated acute abdomen (KD-AA). We conducted a retrospective case-control study of patients with KD-AA treated at our hospital between January 2006 and November 2022. Of the 917 children with KD, 43 (4.7%) presented with AA. Of these, 33 with complete information were included in the KD-AA group. Patients with KD-AA were significantly older, with higher neutrophil rate, C-reactive protein, procalcitonin, and alanine transaminase levels and lower hemoglobin, albumin, and serum sodium levels. Additionally, more patients with KD-AA presented with aseptic meningitis and KD shock syndrome than those with KD alone (all p<0.05). The two groups did not differ in the incidence of intravenous immunoglobulin (IVIG) resistance, incomplete KD, or coronary artery abnormalities. All patients received aspirin and IVIG therapy, with nine receiving a second dose of IVIG and 11 receiving corticosteroids. Only two patients with KD-AA underwent surgery, and the prognosis of all patients with KD-AA was good. KD-AA should be suspected in febrile children with abdominal symptoms. Prompt diagnosis of KD-AA is important for early effective treatment to avoid unnecessary surgical harm. KD, complicated by acute abdomen, has a good prognosis.

Narrative Review of Anomalous Origin of Coronary Arteries: Pathophysiology, Management, and Treatment.

Coronary artery anomalies (CAA) are a diverse group of congenital anomalies and are the second most common cause of sudden cardiac death in the young population after Hypertrophic Cardiomyopathy (HCM). Symptoms range from chest pain, syncope, or sudden cardiac arrest to completely asymptomatic. The prevalence of congenital coronary artery anomalies in the general population is estimated to be between 1% and 2%. CAA often gets underdiagnosed due to the lack of knowledge of the disease process. Approximately 5% of patients with acute myocardial infarction do not have atherosclerotic coronary artery disease or luminal narrowing due to other causes. Congenital coronary artery anomalies account for 50-60% of this 5% of patients. Most patients are asymptomatic for most of their lives, and chest pain is the most common symptom in symptomatic patients when referred for coronary angiography, typically when the diagnosis is typically made. The malignant coronary artery is a rare presentation of a coronary anomaly when associated with atherosclerotic coronary artery disease or valvular heart disease. Patients with symptoms of an abnormal coronary artery origin will receive medical treatment/observation, exercise restriction, coronary angioplasty with stent deployment, or surgical repair.

Clinical Features and Determinants of Coronary Artery Dilatation in Pediatric Patients with Complete and Incomplete Kawasaki Disease: A Single-Center Experience Over 15 Years in Hubei, China

Abstract Objective This study is aimed to elucidate age-associated clinical and echocardiography-defined coronary artery (CA) abnormalities features and to identify independent determinants of CA dilatation in pediatric patients with complete Kawasaki disease (cKD) and incomplete KD (iKD) over 15 years in a tertiary hospital in China. Methods This study comprised 241 patients with KD hospitalized in the pediatric department between 2003 and 2018. Patients were divided into three groups according to age at diagnosis: ≤12, 13 to 35, and ≥36 months. Results A total of 167 (69.3%) patients were defined as cKD (aged 24 months, male 60.5%) and 74 (30.7%) patients as iKD (aged 18 months, male 59.5%). The prevalence of cervical lymphadenopathy significantly increased with age in both cKD and iKD patients. In iKD patients, the prevalence of rash significantly decreased with age. CA dilatation most frequently occurred in patients with cKD and iKD aged ≤12 months. The absence of conjunctivitis or oral changes and higher serum creatinine levels were independent determinants of CA dilatation in KD patients aged ≤12 months, regardless of age, sex, and the type of KD. Conclusion Present study underlines the age-stratified disease characteristics, and the factors associated with CA dilatation in cKD and iKD patients. The absence of conjunctivitis or oral changes, higher serum creatinine levels, and mycoplasma, cytomegalovirus, and adenovirus infections might be associated with an increased risk of CA dilatation in KD patients. These findings provide additional hints for pediatricians in the early diagnosis and treatment of KD, especially for individual medical care of cKD and iKD patients in different age groups.

Efficacy and safety of coronary computed tomography angiography in diagnosing coronary lesions in children.

Identification of paediatric coronary artery abnormalities is challenging. We studied whether coronary artery CT angiography can be performed safely and reliably in children. Retrospective analysis of consecutive coronary CT angiography scans was performed for image quality and estimated radiation dose. Both factors were assessed for correlation with electrocardiographic-gating technique that was protocoled on a case-by-case basis, radiation exposure parameters, image noise artefact parameters, heart rate, and heart rate variability. Sixty scans were evaluated, of which 96.5% were diagnostic for main left and right coronaries and 91.3% were considered diagnostic for complete coronary arteries. Subjective image quality correlated significantly with lower heart rate, increasing patient age, and higher signal-to-noise ratio. Estimated radiation dose only correlated significantly with choice of electrocardiographic-gating technique with median doses as follows: 2.42 mSv for electrocardiographic-gating triggered high-pitch spiral technique, 5.37 mSv for prospectively triggered axial sequential technique, 3.92 mSv for retrospectively gated technique, and 5.64 mSv for studies which required multiple runs. Two scans were excluded for injection failure and one for protocol outside the study scope. Five non-diagnostic cases were attributed to breathing motion, scanning prior to peak contrast enhancement, or scan acquisition during the incorrect portion of the R-R interval. Diagnostic-quality coronary CT angiography can be performed reliably with a low estimated radiation exposure by tailoring each scan protocol to the patient's body habitus and heart rate. We propose coronary CT angiography is a safe and effective diagnostic modality for coronary artery abnormalities in children.

Cardiac involvement in children with paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 (PIMS-TS): data from a prospective nationwide surveillance study.

Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) may occur 4 to 8 weeks after SARS-CoV-2 infection. The acute presentation of PIMS-TS has been well described, but data on longer-term outcomes, particularly cardiac, is scarce. This prospective nationwide surveillance study included children and adolescents less than 18 years of age who were hospitalised with PIMS-TS in Switzerland between March 2020 and March 2022. Data was collected from all 29 paediatric hospitals through the Swiss Paediatric Surveillance Unit (SPSU) during hospitalisation and approximately six weeks after discharge. The data was analysed after categorising the participants into three groups based on their admission status to the intensive care unit (ICU) (non-ICU, ICU-moderate) and the requirement for invasive ventilatory and/or inotropic support (ICU-severe). Overall, 204 children were included of whom 194 (95.1%) had follow-up data recorded. Median age was 9.0 years (interquartile range [IQR] 6.0-11.5) and 142 (69.6%) were male. In total, 105/204 (51.5%) required ICU admission, of whom 55/105 (52.4%) received inotropic support and 14/105 (13.3%) mechanical ventilation (ICU-severe group). Echocardiography was performed in 201/204 (98.5%) children; 132 (64.7%) had a cardiac abnormality including left ventricular systolic dysfunction (73 [36.3%]), a coronary artery abnormality (45 [22.4%]), pericardial effusion (50 [24.9%]) and mitral valve regurgitation (60 [29.9%]). Left ventricular systolic dysfunction was present at admission in 62/201 (30.8%) children and appeared during hospitalisation in 11 (5.5%) children. A coronary artery abnormality was detected at admission in 29/201 (14.2%) children and developed during hospitalisation or at follow-up in 13 (6.5%) and 3 (1.5%) children, respectively. None of the children had left ventricular systolic dysfunction at follow-up, but a coronary abnormality and pericardial effusion were found in 12 (6.6%) and 3 (1.7%) children, respectively. School absenteeism at the time of follow-up was more frequent in children who had been admitted to the ICU (2.5% in the non-ICU group compared to 10.4% and 17.6% in the ICU-moderate and ICU-severe group, respectively) (p = 0.011). Cardiac complications in children presenting with PIMS-TS are common and may worsen during the hospitalisation. Irrespective of initial severity, resolution of left ventricular systolic dysfunction is observed, often occurring rapidly during the hospitalisation. Most of the coronary artery abnormalities regress; however, some are still present at follow-up, emphasising the need for prolonged cardiac evaluation after PIMS-TS.

Study Progress of Coronary Artery Damage in Kawasaki Disease

Kawasaki Disease (KD) is a vasculitis of unknown etiology commonly seen in infants and children. Children with Kawasaki disease are at risk for serious cardiovascular sequelae, especially coronary artery abnormalities, which can lead to myocardial ischemia, myocardial infarction, and sudden death. Coronary artery lesions (CAL) caused by Kawasaki disease has become one of the common acquired heart diseases in children in some countries and regions. This article reviews the current research progress on the mechanism, risk factors and treatment of coronary artery damage in Kawasaki disease, aiming to improve the long-term prognosis of vascular health management in children with KD, in order to provide references for clinicians.

Comparison of Demographic, Clinical, and Echocardiographic Features Between Complete and Incomplete, and Early and Late Presenters of Kawasaki Disease: A 10-Year Single-Center Experience.

The diagnosis of Kawasaki disease(KD)is based mainly on clinical findings and supported by laboratory tests. Complete KD fulfills the main clinical criteria, while incomplete KD includes patients with fewer main criteria and compatible laboratory or echocardiographic findings. The study compares the demographic, clinical, laboratory, and echocardiographic parameters between the complete and incomplete KD and early and late presenters. Moreover, it describes the coronary manifestations of the study population. A retrospective review of all patients admitted with a diagnosis of KD during the period from January 2010 to September 2020 was conducted. Clinical presentation, laboratory features, echocardiographic observations, and follow-up data were examined. Moreover, the patients were further classified as early presenters (presented within 10 days of fever onset) and late presenters (presented after 10 days of disease onset). A comparison between complete and incomplete KD and early and late presenters was performed for demographic, clinical, and echocardiographic features. A total of 76 patients were admitted with a diagnosis of KD.The median age of presentation was 28 months, with a range of five to 144months, and the median timing was seven days, with a range of one to 30days. The median follow-up period was six weeks, with a range of one to 192weeks. Complete KD was present in 38 patients (50%), and 38 (50%) had incomplete KD.Skin manifestations, oral mucosal changes, skin desquamation, conjunctivitis, and lymphadenopathy were present more in patients with complete KD than incomplete ones. Complete and incomplete diseases did not differ regarding coronary artery lesions. Of the patients,53 (70%) presented 10 days or less after the onset of fever, and 23 (30%) presented after the 10th day of disease onset. Comparison between early and late presenters revealed significantly greater mucus membrane changes and lymphadenopathy manifestations among the early presenters and coronary artery lesions among the late presenters. The clinical features of KD should prompt early referral for evaluation, echocardiography, and early administration of intravenous immunoglobulin to prevent coronary artery complications. The complete form of Kawasaki does not have more frequent coronary artery lesions than the incomplete form. Additionally, late presenters may be at increased risk for coronary artery abnormalities than early presenters.

CD40 gene polymorphism and its expression in children with Kawasaki disease from North India: a preliminary case-control study and meta-analysis.

CD40 gene single-nucleotide polymorphisms (SNPs) have been associated with susceptibility and development of coronary artery abnormalities (CAAs) in children with Kawasaki disease (KD) in Japanese, Chinese, and Taiwanese populations. However, data on SNPs of the CD40 gene in patients with KD from the Indian subcontinent are not available. We studied the CD40 gene polymorphisms and its expression in children with KD from North India. SNPs of the CD40 gene (rs4810485, rs1535045) were studied using Sanger sequencing. CD40 expression was studied by flow cytometry. Meta-analysis was carried out to assess the role of both SNPs of the CD40 gene in KD. GRADEpro GDT software (v.3.2) was used to assess the "certainty of evidence." Forty-one patients with KD and 41 age-, sex-matched febrile controls were enrolled. However, none of the alleles and genotypes of the CD40 gene were found to be associated with KD. CD40 expression was higher in KD and in KD with CAAs compared to controls, but it failed to reach statistical significance. In a meta-analysis, the T allele of rs153045 was found to be significantly associated with KD (OR = 1.28; 95% confidence interval (: 1.09-1.50; p = 0.002). The GRADE of evidence for this outcome, however, is of " very low certainty." The present study found no association between SNPs (rs4810485 and rs153045) and susceptibility to KD. This could be a reflection of a modest sample size. CD40 expression was higher in KD and in KD with CAAs. In the meta-analysis, the T allele of rs153045 was significantly associated with KD. Our study confirms a significant genetic heterogeneity in KD among different ethnicities.

Radiation dose analysis of computed tomography coronary angiography in Children with Kawasaki disease.

There is evolving role of computed tomography coronary angiography (CTCA) in non-invasive evaluation of coronary artery abnormalities in children with Kawasaki disease (KD). Despite this, there is lack of data on radiation dose in this group of children undergoing CTCA. To audit the radiation dose of CTCA in children with KD. Study (December 2013-February 2018) was performed on dual source CT scanner using adaptive prospective electrocardiography-triggering. The dose length product (DLP in milligray-centimeters-mGy.cm) was recorded. Effective radiation dose (millisieverts-mSv) was calculated by applying appropriate age adjusted conversion factors as per recommendations of International Commission on Radiological Protection. Radiation dose was compared across the groups (0-1, 1-5, 5-10, and > 10 years). Eighty-five children (71 boys, 14 girls) with KD underwent CTCA. The median age was 5 years (range, 2 mo-11 years). Median DLP and effective dose was 21 mGy.cm, interquartile ranges (IQR) = 15 (13, 28) and 0.83 mSv, IQR = 0.33 (0.68, 1.01) respectively. Mean DLP increased significantly across the age groups. Mean effective dose in infants (0.63 mSv) was significantly lower than the other age groups (1-5 years 0.85 mSv, 5-10 years 1.04 mSv, and > 10 years 1.38 mSv) (P < 0.05). There was no significant difference in the effective dose between the other groups of children. All the CTCA studies were of diagnostic quality. No child required a repeat examination. CTCA is feasible with submillisievert radiation dose in most children with KD. Thus, CTCA has the potential to be an important adjunctive imaging modality in children with KD.

Interaction of dietary patterns with rs28362491 on severity of coronary artery stenosis in patients undergoing coronary angiography

Coronary artery disease (CAD) is one of the most important cardiovascular diseases. Lifestyle and genetic factors play important roles in the development of CAD. The aim of the study is to examine the interaction of dietary patterns and genes on the likelihood of abnormal lipid profile and coronary artery stenosis in Iranians undergoing coronary angiography. This cross-sectional study was performed on 440 patients who underwent coronary angiography. The factor analysis method was used to extract dietary patterns. Commercial kits have been used to assess biochemical parameters. The detection of the rs28362491 genotype was carried out by the method of restriction fragment length polymorphism. Traditional (TDP) and western dietary pattern (WDP) were extracted. We observed an interaction of adherence to TDP and rs28362491 on the odds of having a high Gensini score. These interactions indicated that higher adherence to TDP was associated with higher odds of having a high Gensini score for patients with DD genotype than for those with II genotype. (OR 2.33, 95%CI 1.00–5.44; P = 0.05). These interactions remained statistically significant even after confounder variables. We observed an interaction between higher adherence to TDP and rs28362491 variants on the odds of high low-density lipoprotein cholesterol levels (P = 0.04) in the unadjusted model. We found a significant interaction of this polymorphism and higher adherence to WDP on the odds of having a high Gensini score in the unadjusted model (P = 0.04). This study provides a basis for future research on NF-KB1 gene and diet interaction. More large-scale longitudinal studies are needed to validate these findings.

Approach to resistant IVIG Kawasaki disease in children

Kawasaki disease (KD) is an acute febrile illness of early childhood characterized by vasculitis of the medium-sized arteries. There is no diagnostic test for KD because the cause is unknown, so diagnosis relies on the recognition of the diagnostic clinical criteria. The most feared sequela of KD is development of coronary artery abnormalities, which occur in 20-25% of untreated children. IVIG reduces this risk in 2-5% when administrated within 10 days from the onset of fever. Here is reported the case of an infant which resulted resistant to 2 consecutive doses of IVIG. Glucocorticoids are recommended as an appropriate therapy in IVIG resistant Kawasaki disease.

Quadruple-Rule-Out Computed Tomography Angiography (QRO-CT): A Novel Dual-Energy Computed Tomography Technique for the Diagnostic Work-Up of Acute Chest Pain.

Computerized tomography (CT) has been increasingly utilized in the differential diagnosis of acute chest pain. Combining the triple rule out CT angiography (TRO-CT) approach with dual-energy CT (DECT) can enhance the diagnostic capability by identifying myocardial perfusion deficiencies. This combination can yield a quadruple-rule-out computed tomography angiography (QRO-CT) technique. The aim of this study is to determine the efficacy of the QRO-CT. Intraluminal diseases and abnormalities in the main coronary arteries and branches were investigated. The myocardial dark spots on the color-coded iodine map were identified as perfusion deficiencies. Pulmonary arteries and aorta were also evaluated. The study population consisted of 211 patients. The sensitivity, specificity, and positive and negative predictive values of QRO-CT for pulmonary embolism were 93.5%, 100%, 100%, and 95.3%, respectively. For obstructive coronary artery disease, the values were 96.1%, 93.4%, 89.2%, and 97.7%, respectively. For myocarditis, the values were 69.2%, 100%, 100%, and 93.6%, respectively. the QRO-CT method may successfully evaluate myocardial perfusion deficits, hence expanding the differential diagnosis capabilities of the standard TRO-CT method for myocarditis. It can provide useful information on myocardial perfusion, which may influence the choice to perform invasive catheterization in cases of coronary artery obstruction.

Retrospective analysis of children diagnosed with Kawasaki disease.

The aim of our study was to evaluate the long-term impacts of Kawasaki disease on our patients regarding coronary involvement demographic characteristics, treatment regimens, and clinical course. Our study included 104 patients diagnosed and hospitalized with Kawasaki disease in our center, from January 2004 to January 2019. In our study, patients were divided into three groups according to coronary artery involvement. Patients in group 1 had no echocardiographic findings, while the ones in group 2 had coronary artery dilatation and ones in group 3 had coronary artery aneurysm (CAA). Among 104 patients, the median age was 9.15 (3.0-22.0) years, and 61 of the patients were male while 43 of the patients were female. With a wide range of 1.50-16.50 years of follow-up time, the median diagnosis age of our patients was 31 months (3.0-164.0). Fever duration (median day 10 (5-21), p = 0.025) was statistically significantly higher in group 3. Blood C-reactive protein (CRP) levels, white blood cell (WBC) counts, and neutrophil counts were significantly higher in group 3. There was a statistically significant difference between patients in group 3 and group 2 in which the lowest strain deformation values were in the patients of group 3. In contrast to group 1, the time for initiation of IVIG therapy is significantly prolonged both in group 2 (median: 9.5 days, p = 0.028) and group 3 (median: 10 days, p = 0.036). In our study, serum CRP levels, WBC count, and neutrophil count were higher in patients with coronary artery abnormalities, in agreement with the previous studies. In the light of our results, we consider that the most important determining factor for the development of coronary artery aneurysm is the time of intravenous immunoglobulin (IVIG) administration.

Sex Differences in Systemic and Coronary Arterial Hemodynamics in Heart Failure With Preserved Ejection Fraction

Heart failure (HF) with preserved ejection fraction (HFpEF) predominantly affects females. Systemic and coronary arterial abnormalities are present in HFpEF and may contribute to HFpEF in females. We performed a cross-sectional study of 32 participants with HFpEF and 26 controls. Arterial hemodynamics were noninvasively assessed by combining arterial tonometry with echocardiography. Coronary microvascular function was assessed by rubidium-82 positron emission tomography as the myocardial flow reserve. Coronary vascular resistance (CVR) at rest and vasodilator stress were calculated using positron emission tomography. CVR reserve was calculated as stress-rest CVR. Multivariable linear regression assessed the associations of female sex with arterial hemodynamics in participants with and without HF, and the association of HF with arterial hemodynamics within each sex stratum. Demographics and left ventricular systolic and diastolic function were similar between males and females. Among those with HFpEF, females had a higher steady and pulsatile arterial load and more impaired (less negative) CVR reserve than males. Conversely, in controls, females had similar hemodynamics to males. We then divided the sample based on sex. Femaleswith HFpEF had a higher pulsatile arterial load and higher stress CVR than control females. Among males, arterial hemodynamics were similar, regardless of HFpEF status. The measures of early pulsatile arterial load were independently associated with higher E/e' and lower myocardial flow reserve in females only. In conclusion, despite similar left ventricular function between sexes, older females with HFpEF are characterized by additional systemic and coronary arterial hemodynamic abnormalities compared with males with HFpEF and similarly aged females without HFpEF.

Sox7-positive endothelial progenitors establish coronary arteries and govern ventricular compaction.

The cardiac endothelium influences ventricular chamber development by coordinating trabeculation and compaction. However, the endothelial-specific molecular mechanisms mediating this coordination are not fully understood. Here, we identify the Sox7 transcription factor as a critical cue instructing cardiac endothelium identity during ventricular chamber development. Endothelial-specific loss of Sox7 function in mice results in cardiac ventricular defects similar to non-compaction cardiomyopathy, with a change in the proportions of trabecular and compact cardiomyocytes in the mutant hearts. This phenotype is paralleled by abnormal coronary artery formation. Loss of Sox7 function disrupts the transcriptional regulation of the Notch pathway and connexins 37 and 40, which govern coronary arterial specification. Upon Sox7 endothelial-specific deletion, single-nuclei transcriptomics analysis identifies the depletion of a subset of Sox9/Gpc3-positive endocardial progenitor cells and an increase in erythro-myeloid cell lineages. Fate mapping analysis reveals that a subset of Sox7-null endothelial cells transdifferentiate into hematopoietic but not cardiomyocyte lineages. Our findings determine that Sox7 maintains cardiac endothelial cell identity, which is crucial to the cellular cross-talk that drives ventricular compaction and coronary artery development.

Comparison of the effects of minimally invasive cardiopulmonary bypass and conventional cardiopulmonary bypass on atrial fibrillation after coronary artery bypass grafting and application of magnetic nanoparticles chemiluminescence immunoassay to detect serum indexes

To compare the effects of minimally invasive cardiopulmonary bypass coronary artery bypass grafting (MICB-CABG) and conventional cardiopulmonary bypass (CCPB)-CABG on the occurrence of atrial fibrillation (AF), in this research, totally 86 patients with CABG, admitted in our hospital from March 2020 to October 2021, were selected and divided equally into MICB-CABG and CCPB-CABG groups, according to the random number table method. The incidence of AF and left atrial volume index (LAVI) were compared between the two groups. In total, 104 patients with symptoms such as chest tightness, breathlessness, chest pain, and precordial discomfort were included in the study. All patients were examined by nano-CT and coronary angiography. However, time of ventilator use and time of fever were shorter in the MICB-CABG group. AF is closely related to LAVI, and levels of inflammatory factors, such as high-sensitivity (hs)-CRP, IL-6, and tumor necrosis factor-α (TNF-α), in the CCPB-CABG group were significantly higher than those in the MICB-CABG group. Taking the results of selective coronary angiography as the gold standard, the sensitivity and specificity of nano-CT in detecting coronary artery abnormalities were 99.17% and 97.93%, respectively, suggesting that nano-CT can display coronary artery variations precisely. Compared with the CCPB-CABG group, the incidence of AF after surgery and the risk indicators related to the occurrence of AF were significantly lower in the MICB-CABG group. Compared with traditional CCPB-CABG, MICB-CABG interferes less with the physiological functions of the body, reduces atrium damage, and improves the therapeutic effect. In addition, magnetic nanoparticle CLIA can meet the clinical need for detecting serum indexes and has good potential for clinical use.