Coronary Artery Abnormalities Research Articles

A systematic review and meta-analysis to determine the biological sex ratio in monogenic genetic and non-genetic dilated cardiomyopathy

Abstract Background Dilated cardiomyopathy (DCM) is characterised by left ventricular or biventricular systolic dysfunction and dilatation, in the absence of abnormal loading conditions or coronary artery disease (1). Up to 30% of cases have an identified monogenic cause, usually with autosomal dominant (AD) inheritance (2). The disease appears to be twice as common in males, suggesting either a sex specific penetrance or a potential difference in expressivity. This sex ratio has not been systematically evaluated for genetic forms of DCM. Purpose To assess the sex ratio in patients with all-cause DCM compared to genetic (monogenic) and gene-elusive subtypes, defined by the presence or absence of a pathogenic variant in DCM-associated genes. Methods A literature search was conducted to identify cohorts of DCM patients with identifiable sex ratios. Exclusion criteria were patients with syndromic, X-linked, mitochondrial, or autosomal recessive (AR) DCM. Studies with a small cohort size (n<100), a paediatric population, unidentifiable sex ratio, or ischaemic and peripartum DCM were excluded. 98 studies with a total of 36,662 participants were included in this study. A random-effects meta-analysis generated a pooled proportion of female participants with a 95% confidence interval (95% CI) for an overall DCM cohort as well as for the subtypes – all genetic DCM, individual gene level for 10 key DCM genes (including titin, TTN, and lamin, LMNA), and gene elusive DCM. Results The overall DCM cohort had a 0.30 female proportion (95% CI: 0.28-0.32; Figure 1). This corresponds to a male:female (M:F) ratio of 2.38:1. This ratio did not significantly change in patients with an identified causative DCM variant (0.31 [95% CI 0.26-0.36]; M:F ratio of 2.22:1; p=0.563). There was also no significant difference when compared to patients who had been genetically tested with no identified variant (0.30 [95% CI 0.24-0.37]; M:F ratio of 2.29:1; p=0.814). Furthermore, the ratio within participants with AD gene variants was not significantly different for the specific genes of TTN (0.28 [95% CI 0.22-0.36]; M:F ratio of 2.51:1) and LMNA (0.35 [95% CI 0.27-0.44]; M:F ratio of 1.84:1). Overall, the sex ratio amongst patients with these AD gene variants was similar to the all-cause group (0.34 [95% CI 0.28-0.40]; M:F ratio of 1.98:1; p=0.18); Figure 2. Conclusions This meta-analysis suggests that DCM is twice as prevalent in males than females, even in pooled monogenic genetic subtypes with an equally prevalent genetic risk factor, indicating a difference in penetrance between the sexes. Further studies are required to determine the drivers of this sex specific penetrance.Forest plots: overall DCM cohortForest plots: gene-specific DCM cohort

The role of stress echocardiography in patients with congenital abnormalities of coronary arteries: two tertiary cardiac centres experience

Abstract Background Coronary anomalies (CA) include anomalous aortic origin of a coronary artery (AAOCA), anomalous coronary artery from the pulmonary artery and coronary fistulae. While outcome of natural history of anomalous left coronary artery from the pulmonary artery (ALCAPA) is poor, assessment of stress-induced ischaemia and/or arrhythmia are warranted for clinical decision making in AAOCA. Purpose The aim of this study was to establish the prevalence of ischaemia during exercise stress echocardiography (ESE) in patients with different congenital CA. Methods Patients with CA who had stress exercise echocardiography from two large tertiary cardiac centres were retrospectively recruited. Treadmill/bike exercise stress echo tests were performed and analysed according to ESC guidelines. Computed tomography (CT), magnetic resonance imaging (MRI), myocardial perfusion scintigraphy, positron emission tomography (PET) and invasive angiogram with intravascular ultrasound (IVUS) were used for CA morphology and myocardial perfusion assessment. Patients with sclerotic stenosis of >50% in any of the coronary arteries were excluded from this study. Results Total 59 patients, including 8 ALCAPA, 4 coronary fistula and 47 AAOCA (12 anomalous aortic origin of the left coronary artery (AAOLCA) and 35 anomalous aortic origin of the right coronary artery (AAORCA)) formed the study cohort. The mean age was 44±17 years. Cardiac symptoms at rest were present in 5 (63%) ALCAPA, 31 (66%) patients with with AAOCA, and none in patients with coronary fistula. Nine patients (7 ALCAPA, 1 coronary fistula and 1 AAOLCA) had undergone previous repair. Out of 47 patients with AAOCA patients, 31 (66%) had interarterial course of coronary arteries, and 8 (17%) had intramural course. A positive ESE presents in 2 (25%) ALCAPA, 1 (25%) coronary fistula patient and 1 (2%) AAOCA patient. Myocardial perfusion was assessed in 12 of 59 (20%) patients, of which 9 were AAOCA. Myocardial perfusion studies were negative in 11 patients with negative ESE tests, while only in one patient with AAOLCA myocardial perfusion defects were found on MRI and PET despite negative ESE. No major adverse cardiovascular events were observed during follow-up of patients with negative ESE. Following ESE, surgical repair of CA has been performed in 3 patients with AAORCA and in 2 patients with AAOLCA based on either symptoms attributable to anomalous coronary, evidence of myocardial ischaemia on myocardial perfusion assessment or high-risk anatomy.While 4 out of 5 repaired patients were symptomatic before repair, they became asymptomatic following repair. Conclusions Incidence of ischaemia on exercise stress echo is extremely low in AAOCA patients despite symptoms at rest and malignant anatomical features. Improving stress echo methodology by using myocardial perfusion assessment might shed a light on this discrepancy between ESE results and symptomatic status/high-risk anatomy.

Primary Adjunctive Corticosteroids in the Acute Management of High-Risk Kawasaki Disease in a North American Cohort.

Primary adjunctive therapy with corticosteroids have been shown to reduce coronary artery abnormalities in high-risk Kawasaki Disease (KD) patients in Japan (RAISE study). We evaluated their effect on outcomes in North American patients with high-risk KD. We performed a single-center retrospective review of high-risk KD patients between 2010 - 2023. From 2017-2023, adjunctive corticosteroids in a modified RAISE regimen (mRAISE) were given to high-risk patients as primary adjunctive therapy with intravenous gammaglobulin (IVIG) and aspirin. We compared CA outcomes in these patients and those presenting from 2010-2016, when mRAISE therapy was not administered. A total of 221 high-risk KD patients were treated at our institution between 2010-2023. Among these, 83 received the mRAISE regimen and 138 did not (no corticosteroid, n=82, corticosteroid in a non-mRAISE regimen, n=56). There were no significant differences in coronary artery outcomes in the mRAISE and non-mRAISE groups. Patients receiving the mRAISE regimen were significantly less likely to receive more than one dose of IVIG when compared to those who did not receive this regimen (11% vs 33%, p<0.001). Use of adjunctive primary therapy with corticosteroids in a mRAISE regimen in high-risk KD patients resulted in significantly decreased IVIG retreatment.

Kawasaki Disease: A Single-Center Study from a Tertiary Care hospital in Dhaka, Bangladesh

Background: Kawasaki Disease (KD) is recognized as the leading cause of acquired heart disease in children in developed countries. This is a pioneer study from Bangabandhu Sheikh Mujib Medical University (BSMMU), documenting the common epidemiological, clinical, laboratory and echocardiographic findings, including management and immediate outcome of KD children. Method: This retrospective study includes all patients with a diagnosis of KD, who attended the pediatric rheumatology division at BSMMU from January 2015 to December 2019. Data were collected from medical records at diagnosis and immediately after management. Relevant statistical tests were done and a p value &lt; 0.05 was considered as statistically significant. Result: A total of 129 cases were diagnosed with KD, among them complete KD cases were 62% and incomplete KD was 38%. The mean age at onset was significantly higher in incomplete KD than complete KD. The majority of the cases (67%) were between 1 to 5 years of age and male (72%) children were predominant. Fever was the most common presenting feature (100%), followed by bilateral conjunctivitis, mucosal involvement, rash, changes in extremities, cervical lymphadenopathy and others. Coronary artery dilatation was present in 30.5% cases with no significant differences between complete and incomplete KD cases. Aspirin was given to all the cases and IVIG was given to only 46.6% children. Rests of the children were treated with methyl prednisolone. All the cases improved after treatment and there was no immediate mortality. Conclusion: It seems that KD is not an uncommon disease and incomplete KD cases are relatively more prevalent. About 30% KD cases had coronary artery abnormalities. Immediate outcome of management is excellent but needs long term follow-up. BANGLADESH J CHILD HEALTH 2023; VOL 47 (1) : 9-16

Electrocardiographic Changes in Acute Kawasaki Disease in the Era of Immunoglobulin Therapy.

Kawasaki disease (KD), an acute, febrile, self-limiting, multisystem vasculitis of unknown etiology, often involves the cardiac complications of coronary artery aneurysm development, myocarditis, pericarditis, and valvulitis. However, there are insufficient epidemiological data on electrocardiographic (ECG) changes in acute KD in the IVIG era, and little is known about the association between ECG changes and the prognosis. The present study aimed to determine the type of ECG changes found in acute KD and to clarify the course of these changes as well as their relationship to disease severity. The present, retrospective, observational study analyzed 201 patients aged < 15years with Kawasaki disease who received IVIG from April 2021 to March 2023. In the 156 eligible patients analyzed (male: 62.8%; median age on admission: 24months), the most common abnormalities were T wave changes (n = 141; 90.4%) on any lead followed by T wave inversions (n = 63; 40.4%), tachycardia (n = 36; 23.1%), and a prolonged PR interval (n = 23; 14.7%). PR prolongation, abnormal Q wave, QTc prolongation, and ST changes were less common in the present cohort, and arrhythmia occurred less frequently than in previous studies. No significant association was found between T wave inversion and disease severity. T wave changes occurred frequently in the acute phase ECG in KD but were not associated with clinical features, such as unresponsiveness to IVIG therapy or coronary artery abnormalities.

Incidence of Kawasaki disease among children in Chandigarh, India during 2015–2019: a trend analysis

Only limited information exists regarding the epidemiology of Kawasaki disease (KD) in low-income and middle-income countries. The present study provides the incidence of KD during 2015-2019 in Chandigarh, north India. Our centre follows the largest KD cohort in India. Children with KD at Chandigarh diagnosed during January 2015-December 2019 were enrolled in the study. Annual incidence rates were determined using decadal growth rates of the National Census 2011. We computed the incidence of KD in children aged <5, and <15 years. We also undertook linear trend analysis using our incidence data from 1994 to2019. During 2015-2019, 83 patients (66 males, 17 females) were diagnosed with KD in Chandigarh. Incidence rates during these 5 years were 5.64, 9.25, 9.11, 9.87, and 9.72/100,000 in children aged <5 years, and 2.65, 4.44, 3.86, 5.07, 4.74/100,000 in children aged <15 years. The median age at diagnosis was 48 months (range: 12 days to 15 years). Compared to previous data (2009-2014), there was a 53.1% increase in annual incidence of KD in children aged <5 years, and a 53.7% increase in children aged <15 years. Coronary artery abnormalities during acute phase were noted in 16.9%, and in 7.2% of patients at 6 weeks of follow-up. The trend analysis indicated a monthly rise of 0.002 cases per 100,000 children aged <5 years, and 0.0165 cases per 100,000 children aged <15 years. The incidence of KD has continued to show an upward trend in Chandigarh over the period 2015-2019. This may indicate a true rise in the occurrence of KD or may reflect better disease ascertainment as a result of greater awareness about KD amongst healthcare professionals. None.

Analysis of ultrasound coronary parameters and blood red cell distribution width and N-terminal pro-brain natriuretic peptide concentrations following coronary lesions in children with Kawasaki disease.

Aims/Background Kawasaki disease is an acute inflammatory condition primarily affecting the young children. It can lead to coronary artery abnormalities, which can worsen the prognosis. Early diagnosis of coronary disease is crucial for the effective treatment and the prognosis evaluation. To explore the clinical significance of ultrasound examination characteristics, peripheral blood red cell distribution width, and changes in N-terminal pro-brain natriuretic peptide levels for the early detect coronary artery abnormality in children with Kawasaki disease. Methods The case-control study was conducted. 85 Kawasaki disease patients diagnosed in our hospital from January 2020 to December 2023 were selected as the Kawasaki disease group. 100 healthy children who received physical examination in the Department of Child Healthcare during the same period were selected as control group. The cardiac ultrasound indicators, erythrocyte sedimentation rate, C-reactive protein, white blood cell, neutrophil percentage, platelet count, D-dimer, red cell distribution width, N-terminal pro-brain natriuretic peptide of two groups were compared. The Kawasaki disease group was further divided into the coronary artery lesion group and the non-coronary artery lesion group based on whether coronary artery lesions occurred in the Kawasaki disease patients. The differences of above indicators were compared. Results The left main coronary artery, left anterior descending branch, and right coronary artery Z-scores of the Kawasaki disease group were all higher than those of the control group (p < 0.05). There was no significant difference in left ventricular ejection fraction between Kawasaki disease group and control group (p > 0.05). The erythrocyte sedimentation rate, C-reactive protein, neutrophil percentage, platelet count, D-dimer, red cell distribution width, and N-terminal pro-brain natriuretic peptide of Kawasaki disease group were all higher than those of control group (p < 0.05). The left main coronary artery, left anterior descending branch, and right coronary artery Z-scores of Kawasaki disease patients with coronary artery lesions were all higher than those of Kawasaki disease patients without coronary artery lesions (p < 0.05). The left ventricular ejection fraction of Kawasaki disease patients with coronary artery lesions was lower than that of Kawasaki disease patients without coronary artery lesions (p < 0.05). The erythrocyte sedimentation rate, C-reactive protein, white blood cell, neutrophil percentage, platelet count, D-dimer, red cell distribution width, and N-terminal pro-brain natriuretic peptide of Kawasaki disease patients with coronary artery lesions were all higher than those of Kawasaki disease patients without coronary artery lesions, and the differences were statistically significant (p < 0.05). After treatment, the left main coronary artery, left anterior descending branch, and right coronary artery Z-scores of Kawasaki disease patients with coronary artery lesions significantly decreased (p < 0.05), and the left ventricular ejection fraction significantly increased (p < 0.05). The erythrocyte sedimentation rate, C-reactive protein, white blood cell, neutrophil percentage, platelet count, D-dimer, red cell distribution width, and N-terminal pro-brain natriuretic peptide of Kawasaki disease patients with or without coronary artery lesions significantly decreased after treatment compared with before treatment in the same group (p < 0.05). Conclusion Kawasaki disease patients with coronary artery lesions exhibit significantly increased coronary artery vessel diameter, as well as elevated red cell distribution width and N-terminal pro-brain natriuretic peptide concentration. The combined use of ultrasound combined with red cell distribution width and N-terminal pro-brain natriuretic peptide examination can assist in determining whether Kawasaki disease patients have coronary artery lesions and assessing the clinical treatment effect.

The changes of coagulation profiles in Kawasaki disease and its associations with clinical classification, intravenous immunoglobulin responsiveness and coronary artery involvement

Coagulation disorders are common in Kawasaki disease (KD). The main objectives of the present study were to probe the associations of coagulation profiles with clinical classification, IVIG responsiveness, coronary artery abnormalities (CAAs) in the acute episode of KD. A total of 313 KD children were recruited and divided into six subgroups, including complete KD (n = 217), incomplete KD (n = 96), IVIG-responsive KD (n = 293), IVIG-nonresponsive KD (n = 20), coronary artery noninvolvement KD (n = 284) and coronary artery involvement KD (n = 29). Blood samples were collected within 24-h pre-IVIG therapy and 48-h post-IVIG therapy. Coagulation profiles, conventional inflammatory mediators and blood cell counts were detected. Echocardiography was performed during the period from 2- to 14-day post-IVIG infusion. In addition, 315 sex- and age-matched healthy children were enrolled as the controls. (1) Before IVIG therapy, coagulation disorders were more prone to appear in KD patients than in healthy controls, and could be overcome by IVIG therapy. FIB and DD significantly increased in the acute phase of KD, whereas reduced to normal levels after IVIG therapy. (2) PT and APTT were significantly longer in patients with complete KD when compared with their incomplete counterparts after IVIG therapy. (3) The larger δDD, δFDP and the smaller δPT, δINR predicted IVIG nonresponsiveness. (4) The higher δDD and δFDP correlated with a higher risk for CAAs (DD: r = −0.72, FDP: r = −0.54). Coagulation disorders are correlated with complete phenotype, IVIG nonresponsiveness and CAA occurrence in the acute episode of KD, and can be rectified by synergistic effects of IVIG and aspirin.

Angiographic Profile of Coronary Artery Disease in Patients of Heart Failure Attending in BPKIHS, Nepal

INTRODUCTION Coronary Angiography in heart failure is one of the diagnostic modalities that acts as complementary tool in the management. Very few studies are conducted in Nepal regarding prevalence of CAD in HF. CAD has been one of the etiologies in 18% patients of Heart failure.1 On categorizing heart failure patients into ischemic and non-ischemic groups and knowing any anomalous anatomy would be feasible to intervene as essential and decrease in recurrent hospital admissions and improve quality of life. Our current study aimed to evaluate various characteristics and profiles of Coronary Arteries in the heart failure subjects after Coronary Angiography. METHODS It was a single center hospital based descriptive cross-sectional study conducted at BPKIHS from October 2022 to October 2023 in heart failure patients who underwent CAG. Heart failure patients were selected based on Framingham criteria. RESULTS Out of total 78 patients 64.15 were males and mean age was 62.4±13.69 years. There were 47.4% patients with HFrEF, 28.2% with HFmrEF and 24.4% were having HFpEF. Significant CAD was found in 70.5%, normal Coronary artery was present in 2.8%, 2.5% had anomalous coronary artery, minor CAD was found in 2.6% while 5.1% had moderate CAD. Prevalence of significant CAD was found in 70.3% patients with HFrEF, 68.2% in HFmrEF whereas HFpEF had significant CAD in 73.7% subjects. Significant CAD in males and females were 48.7% and 70.5% respectively. Smoking was the most common risk factor present in our study population. Most common presentation were Basal Lung Crepitations, Raised JVP, Hepatomegaly and Leg Swelling. CONCLUSION Our result suggests higher prevalence of CAD in HFpEF (73.7%) followed by HFrEF (70.3%) and HFmrEF (68.2%). Coronary anomaly was seen in 2.5% while angiographic profile revealed abnormal coronary artery to be more frequent in HF subjects which was quite comparable to other similar studies of Heart failure.

Successful percutaneous coronary intervention in a congenital single right coronary artery with acute myocardial infarction: A case report and literature review.

Single coronary artery (SCA) is a rare coronary artery malformation. SCA combined with atherosclerotic plaques can cause severe and widespread myocardial ischemia and infarction, leading to hemodynamic instability and even sudden death. A 48-year-old Chinese man was admitted for treatment of persistent chest tightness and panic for 5 hours. The patient was a lorry driver with high work intensity and mental stress, with body mass index of 33.78, history of smoking and alcohol consumption, but no history of hypertension and diabetes. Admission examination showed Troponin was 183.083 µg/L and CK-MB value was >300 µg/L. The patient was diagnosed with a congenital single right coronary artery (RCA) with acute myocardial infarction (AMI) by coronary angiography (CAG). Due to atherosclerotic plaques rupture, a complete occlusion of the proximal RCA with thrombolysis in myocardial infarction grade 0 of distal blood flow were found. The patient was treated with thrombus aspiration and thrombolytic therapy by percutaneous coronary intervention under the support of intra-aortic balloon pump. Postoperative the chest tightness and panic were relieved, and CAG revealed that the proximal thrombus of the RCA was reduced, and distal blood flow was restored to thrombolysis in myocardial infarction grade 3. After 2 weeks of intensive antithrombotic and lipid-regulating drug therapy, the patient was successfully discharged. Follow-up for 6 months, the patient was able to live and work normally without experiencing chest tightness and chest pain. Computed tomography angiography (CTA) confirmed a congenital single RCA with patent lumen and no severe stenosis. The congenital single RCA is very rare, and it is fatal in conjunction with acute coronary syndrome. Early detection and appropriate treatment is critical for AMI patient with single RCA. CAG is the gold standard for diagnosis of single RCA, and CTA is a necessary to describe the anatomical course of abnormal coronary arteries.

Clinical Diagnostic Workflow of Nondilated Left Ventricular Cardiomyopathy: A Case-based Review

Abstract Dilated cardiomyopathy is defined as left ventricular (LV) or biventricular dysfunction and dilatation not explained by abnormal loading conditions or coronary artery disease. However, the existing definition does not cover a range of disorders that manifest as a spectrum of electrical, structural, and functional myocardial abnormalities that change over time. With the rising use of echocardiographic speckle tracking strain imaging and cardiac magnetic resonance imaging, subtle myocardial structural or functional pathology can be detected very early in the natural history of the myocardial disease. The 2023 European Society of Cardiology guidelines for cardiomyopathy have coined a new terminology “nondilated LV cardiomyopathy (NDLVC)” to help in early risk stratification and better patient surveillance and therapeutic management. This article is a case series of NDLVC with an emphasis on systematic clinical workup for early diagnosis and better management of this pathology.

Importance of Cardiovascular Magnetic Resonance Applied to Congenital Heart Diseases in Pediatric Age: A Narrative Review.

Congenital heart diseases (CHDs) represent a heterogeneous group of congenital defects, with high prevalence worldwide. Non-invasive imaging is essential to guide medical and surgical planning, to follow the patient over time in the evolution of the disease, and to reveal potential complications of the chosen treatment. The application of cardiac magnetic resonance imaging (CMRI) in this population allows for obtaining detailed information on the defects without the necessity of ionizing radiations. This review emphasizes the central role of CMR in the overall assessment of CHDs, considering also the limitations and challenges of this imaging technique. CMR, with the application of two-dimensional (2D) and tri-dimensional (3D) steady-state free precession (SSFP), permits the obtaining of very detailed and accurate images about the cardiac anatomy, global function, and volumes' chambers, giving essential information in the intervention planning and optimal awareness of the postoperative anatomy. Nevertheless, CMR supplies tissue characterization, identifying the presence of fat, fibrosis, or oedema in the myocardial tissue. Using a contrast agent for angiography sequences or 2D/four-dimensional (4D) flows offers information about the vascular, valvular blood flow, and, in general, the cardiovascular system hemodynamics. Furthermore, 3D SSFP CMR acquisitions allow the identification of coronary artery abnormalities as an alternative to invasive angiography and cardiovascular computed tomography (CCT). However, CMR requires expertise in CHDs, and it can be contraindicated in patients with non-conditional devices. Furthermore, its relatively longer acquisition time and the necessity of breath-holding may limit its use, particularly in children under eight years old, sometimes requiring anesthesia. The purpose of this review is to elucidate the application of CMR during the pediatric age.

EFFICACY OF INTRAVENOUS IMMUNOGLOBULIN ALONE ON CORONARY ARTERY LESION REDUCTION IN KAWASAKI DISEASE.

Though Aspirin and intravenous immunoglobulin (IVIG) remain the standard treatments for Kawasaki Disease (KD) to minimize coronary artery damage, the duration and dosage of aspirin are inconsistent across hospitals. However, the lack of multi-center randomized trials prevents definitive answers to the impact of high-dose aspirin. This clinical trial was structured as a prospective, evaluator-blinded, multi-center randomized controlled trial with two parallel arms, aiming to assess the effectiveness of IVIG as a standalone primary therapy of KD in comparison to the combination of IVIG with high-dose aspirin therapy. KD patients were enrolled between September, 2016 and August, 2019. A final cohort of 134 patients were randomly assigned to the standard and test groups with 69 and 65 patients, respectively. The Standard group received IVIG (2 g/kg) along with aspirin (80-100 mg/kg/day) until fever subsided for 48 hours. The test group received IVIG (2 g/kg) alone. Following the initial treatment, both groups received a daily aspirin dose (3-5 mg/kg) for six weeks. The primary outcome measure was the occurrence of coronary artery lesions (CAL) at the 6-8 weeks mark. The secondary outcome is IVIG resistance. The overall rate of CAL in test group decreased from 10.8% at diagnosis to 1.5% and 3.1% at 6 weeks and 6 months, respectively. The CAL rate of standard group declined from 13.0% to 2.9% and 1.4%, with no statistically significant difference (P>0.1) in the frequency of CAL between the two groups. Furthermore, no statistically significant differences were found for treatment (P>0.1) and prevention (P>0.1) effect between the two groups. This marks the first prospective multi-center randomized controlled trial comparing the standard treatment of KD using IVIG plus high-dose aspirin against IVIG alone. Our analysis indicates that addition of high-dose aspirin during initial IVIG treatment is neither statistically significant nor clinically meaningful for CAL reduction. URL: http://www.clinicaltrials.gov ; identifier: NCT02951234. This study represents the first multi-center randomized controlled trial investigating the efficacy of high-dose aspirin or intravenous immunoglobulin (IVIG) during the acute stage of KD. This study assessed the impact of discontinuing high-dose aspirin (80-100 mg/kg/day) on the occurrence of CAL during the acute phase treatment of Kawasaki Disease.No significant differences were observed between high-dose aspirin plus IVIG treatment and IVIG alone treatment in terms of the frequency of abnormal coronary artery abnormalities. Additionally, our analysis revealed no statistically significant differences in either the treatment effect (the number of cases successfully treated) or prevention effect (the prevention of new cases) between these two treatments. Comparison analysis indicated the non-inferiority between two groups with or without high-dose aspirin.Administering the standard 2 g/kg/day IVIG without high-dose aspirin (80-100 mg/kg/day) during the acute phase therapy for KD does not increase the risk of coronary artery lesions, which are a primary cause of morbidity and mortality in KD patients.Addition of high-dose aspirin during initial IVIG treatment is not statistically significant or clinically meaningful.

Clinico-Laboratory Profile and Outcome of Kawasaki Disease in Children in a Tertiary Care Hospital in Kathmandu

Introduction: Kawasaki disease (KD) is one of the most common vasculitis of childhood. It contributes significantly to acquired heart disease in children and results in coronary artery abnormalities including aneurysm in 25% of untreated children. This study aims to retrospectively assess the clinical course, laboratory results, and presenting symptoms of patients with complete and incomplete KD treated at tertiary care hospital in Kathmandu.Method: This retrospective study was conducted in the Department of Pediatrics. Data from January 2020 to December 2020 were retrieved from medical records department of the hospital and analyzed using Microsoft excel.Result: The total number of cases evaluated in our study was 9. The median age at the time of the diagnosis of KD was 24 months (median age for incomplete KD-21 months, complete KD-37 months). Male to female ratio was 1.2:1. The peak season of KD occurrence was Spring (March – May). Mean duration of stay in the hospital was 7 days. All children presented with fever, with an mean fever duration of 7 days at the time of presentation. Fever was present in every case. The most common principal symptom was a polymorphous skin rash, observed in 88% of the cases. Elevated CRP levels (≥3 mg/dl) were noted in all 9 cases. Cardiovascular complications were present in 88% of the patients, with coronary artery aneurysm (CAA) detected in 11%, coronary artery dilation in 22%, and mild pericardial effusion documented in 66% of the cases. Eight patients (88%) received IVIG, all 9 patients (100%) received aspirin, and 2 patients received steroids. There were no fatalities.Conclusion: KD is a common vasculitis in children which do not have significant sex predilection in oppose to most of the studies. Majority of cases occurred in spring season. The most common symptom was polymorphous rash. A high level of suspicion for diagnosing KD is essential, and early treatment is crucial to prevent complications and reduce the future economic burden on families and the nation.

Speckle Tracking Echocardiography in Patients with Non-Ischemic Dilated Cardiomyopathy Who Undergo Cardiac Resynchronization Therapy: A Narrative Review.

Non-ischemic dilated cardiomyopathy (DCM) represents a significant cause of heart failure, defined as the presence of left ventricular (LV) dilatation and systolic dysfunction unexplained solely by abnormal loading conditions or coronary artery disease. Cardiac resynchronization therapy (CRT) has emerged as a cornerstone in the management of heart failure, particularly in patients with DCM. However, identifying patients who will benefit the most from CRT remains challenging. Speckle tracking echocardiography (STE) has garnered attention as a non-invasive imaging modality that allows for the quantitative assessment of myocardial mechanics, offering insights into LV function beyond traditional echocardiographic parameters. This comprehensive review explores the role of STE in guiding patient selection and optimizing outcomes in CRT for DCM. By assessing parameters such as LV strain, strain rate, and dyssynchrony, STE enables a more precise evaluation of myocardial function and mechanical dyssynchrony, aiding in the identification of patients who are most likely to benefit from CRT. Furthermore, STE provides valuable prognostic information and facilitates post-CRT optimization by guiding lead placement and assessing response to therapy. Through an integration of STE with CRT, clinicians can enhance patient selection, improve procedural success rates, and ultimately, optimize clinical outcomes in patients with DCM. This review underscores the pivotal role of STE in advancing personalized management strategies for DCM patients undergoing CRT.

Should we be afraid of long-term cardiac consequences in children with multisystem inflammatory syndrome? Experience from subsequent waves of COVID-19.

The purpose of the study was to assess and compare short- and long-term cardiac complications of the multisystem inflammatory syndrome in children (MIS-C) by predominant severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) variants throughout the pandemic. The analysis of prospectively collected data comparing cardiac complications of MIS-C during and after hospitalization across the original/alpha, delta, and omicron waves. Cardiac complications were defined as cardiac failure with systolic function impairment or hypotension or abnormalities in echocardiographic findings (decrease in LVEF, FS, valvular insufficiency, pericardial effusion, or coronary artery abnormalities). A total of 120 patients with MIS-C admitted to the Children's Hospital of Krakow between November 1, 2020, and May 5, 2023, were included in the study (74 during original/alpha dominance, 31 delta, and 15 omicron). Patients in the omicron group were found to be younger than those in the alpha and delta groups (37 vs. 75 vs. 80months, p = 0.03). The frequency of cardiac failure with systolic function impairment or hypotension was diagnosed more frequently in the original/alpha and delta groups than in the omicron group (44.59% vs. 41.94% vs. 13.33%, p = 0.08) also echocardiographic abnormalities changed, with rates of 60.8%, 35.5%, and 13.3% (p < 0.001) accordingly. The multivariable regression revealed an older age (OR = 1.19, 95% CI = 1.07-1.33, p = 0.002) as the only independent factors of cardiac failure with systolic function impairment or hypotension. In all patients, signs of cardiac failure resolved during the hospitalization. Moreover, in 98.3% of patients, all echocardiagraphic abnormalities resolved completely during the observation period. Conclusion: The cardiac complications of MIS-C appeared to advance less severely in younger children during the Omicron outbreak. In long-term observation, symptoms of cardiac failure resolve completely. Similarly, also echocardiographic abnormalities normalize in the vast majority of patients. What is Known: • Knowledge about the long-term cardiac complications of MIS-C is still evolving and uncertain. • The greatest concern of MIS-C is cardiac complications, including cardiac failure and coronary artery dilatation. What is New: • Long-term observations revealed complete resolution of cardiac complications in the vast majority of patients with MIS-C, irrespective of the dominant variant. • Cardiac complications of MIS-C were less common in younger children during subsequent pandemic waves in our patient population.

Different dose aspirin plus immunoglobulin (DAPI) for prevention of coronary artery abnormalities in Kawasaki disease: Study protocol for a multi-center, prospective, randomized, open-label, blinded end-point, non-inferiority trial

BackgroundKawasaki disease is a pediatric acute systemic vasculitis that specifically involves the coronary arteries. Timely initiation of immunoglobulin plus aspirin is necessary for diminishing the incidence of coronary artery abnormalities (CAAs). The optimal dose of aspirin, however, remains controversial. The trial aims to evaluate if low-dose aspirin is noninferior to moderate-dose in reducing the risk of CAAs during the initial treatment of Kawasaki disease. MethodsThis is a multi-center, prospective, randomized, open-label, blinded endpoint, noninferiority trial to be conducted in China. The planned study duration is from 2023 to 2026. Data will be analyzed according to intention-to-treat principles. Participants are children and adolescents under the age of 18 with Kawasaki disease, recruited from the inpatient units. A sample size of 1,346 participants will provide 80% power with a one-sided significance level of 0.025. Qualifying children will be randomized (1:1) to receive either intravenous immunoglobulin (2 g/kg) plus oral moderate-dose aspirin (30-50 mg·kg−1·d−1) until the patient is afebrile for at least 48 hours, or immunoglobulin plus low-dose aspirin (3-5 mg·kg−1·d−1) as initial treatment. The primary outcome will be the occurrence of CAAs at 8 weeks after immunoglobulin infusion. Independent blinded pediatric cardiologists will assess the primary endpoint using echocardiography. ConclusionsThere is a shortage of consensus on the dose of aspirin therapy for Kawasaki disease due to the lack of evidence. The results of our randomized trial will provide more concrete evidence for the efficacy and adverse events of low- or moderate-dose aspirin in the acute phase of Kawasaki disease. Trial Registrationwww.chictr.org.cn: ChiCTR2300072686.

Markers of Endothelial Dysfunction in Kawasaki Disease: An Update.

Kawasaki disease (KD) is a medium vessel vasculitis that has a special predilection for coronary arteries. Cardiovascular complications include the development of coronary artery abnormalities (CAAs) and myocarditis. Endothelial dysfunction (ED) is now recognized to be a key component in the pathogenesis of KD and is believed to contribute to the development of CAAs. ED has been evaluated by several clinical parameters. However, there is paucity of literature on laboratory markers for ED in KD. The evaluation of ED can be aided by the identification of biomarkers such as oxidative stress markers, circulating cells and their progenitors, angiogenesis factors, cytokines, chemokines, cell-adhesion molecules, and adipokines. If validated in multicentric studies, these biomarkers may be useful for monitoring the disease course of KD. They may also provide a useful predictive marker for the development of premature atherosclerosis that is often a concern during long-term follow-up of KD. This review provides insights into the current understanding of the significance of ED in KD.

Diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery

Objective: To analyze the diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery (AAOCA). Methods: This is a retrospective case series study. From January 2016 to July 2023, 24 cases of high-risk AAOCA underwent surgical treatment in Department of Cardiac Surgery, Guangdong Provincial People's Hospital. There were 18 males and 6 females, operatively aged (M (IQR)) 13 (26) years (range: 0.3 to 57.0 years). They were confirmed by cardiac ultrasound and cardiac CT, all of which had anomalous coronary running between the aorta and the pulmonary artery. There were 15 cases of the right coronary artery from the left aortic sinus of Valsalva, 6 cases of left coronary artery from the right aortic sinus of Valsalva, 3 cases of the sigle coronary artery. Only 3 patients had no obvious related symptoms (2 cases were complicated with a positive exercise stress test and 1 case with other intracardiac malformations), 21 cases had a history of chest tightness, chest pain, or syncope after exercise. Three patients suffered syncope after exercise and underwent cardiopulmonary resuscitation (2 cases were treated with an extracorporeal membrane oxygenerator (ECMO)). The gap from the first symptom to the diagnosis was 4.0 (11.5) months (range: 0.2 to 84.0 months). The detection rate of coronary artery abnormalities suggested by the first cardiac ultrasound was only 37.5% (9/24). Seven patients were complicated with other cardiac diseases (4 cases with congenital heart defects, 2 cases with coronary atherosclerotic heart disease, 1 case with mitral valve disease). Results: All 24 patients underwent surgical treatment (23 cases underwent abnormal coronary artery unroofing, 1 case underwent coronary artery bypass grafting), and 5 patients underwent other intracardiac malformation correction at the same time. There were no death or surgery related complications in the hospital for 30 days after the operation. A patient with preoperative extracorporeal cardiopulmonary resuscitation was continuously assisted by ECMO after emergency AAOCA correction and had complications such as limb ischemia necrosis and renal dysfunction after the operation. During the follow-up of 2.2 (3.3) years (range: 1 month to 7.2 years), one patient who previously underwent percutaneous transluminal coronary angioplasty with a stent implant experienced significant postoperative symptomatic relief, and the other discharged patients had no related symptoms. Conclusions: The accurate rate of initial diagnosis for high-risk AAOCA is still low, but the risk of cardiovascular accidents is high. For sports-related chest pain and other symptoms, more attention should be paid to the detection of AAOCA, especially for adolescents. Exercise stress testing can be helpful in evaluating the cardiovascular risk of asymptomatic AAOCA. Instant surgical treatment can achieve satisfactory curative effects.

64-Slice ECG-gated computed tomographic angiography for assessment of coronary arteries in brachycephalic dogs with pulmonary stenosis.

Brachycephalic dogs with pulmonary stenosis are known to have a higher incidence of concurrent coronary artery abnormalities than non-brachycephalic breeds, which increases risk when performing balloon valvuloplasty. The use of ECG-gated CT angiography has been reported for the evaluation of coronary arteries in normal dogs and dogs with pulmonary stenosis. The purpose of this study was to report findings of coronary artery origination and morphology of main branches using ECG-gated CT angiography in brachycephalic dogs with pulmonary stenosis. An ECG-gated CT angiographic protocol was used to image coronary artery anatomy in nine brachycephalic dogs with pulmonary stenosis. Images were assessed for quality as well as coronary artery morphology by one veterinary radiologist, one veterinary cardiologist and one veterinary radiology resident. All nine dogs had good to excellent image quality. Coronary artery anomalies were identified in three of nine dogs: one R2A anomaly, one L2A anomaly and one L2C anomaly. Two dogs were assessed to be poor balloon valvuloplasty candidates based on CT angiographic images. Coronary artery morphology was not confirmed via postmortem examination in all patients. ECG-gated CT angiography is a minimally invasive imaging modality capable of diagnosing various coronary artery anomalies in brachycephalic dogs with pulmonary stenosis and aiding in the determination of patient candidacy for balloon valvuloplasty.