Epidemiology and Risk Factors for Resistance to Treatment of Kawasaki Disease in Cyprus.

Background: Kawasaki disease (KD) is one of the most common vasculitides of early childhood and the first cause of acquired heart disease in developed countries. There are no previous studies on KD in Cyprus. The aim of this study was to evaluate the epidemiology of KD in Cyprus, risk factors for resistance to treatment and the development of cardiac complications. Lastly, the Kobayashi and Egami scoring systems were evaluated in our population.Methods: This is a retrospective multicenter study of pediatric patients with KD hospitalized between January 2000 and-December 2019. The data were collected from medical records. Results: A total of 136 patients with KD were included in the study. 83% of patients were <5 years of age and 10% were <6 months. Median duration of fever before treatment was 6 days. 108/129 patients responded to initial treatment with IVIG. Thirty patients (22%) developed coronary artery lesions. Serum sodium ≤133mmol/L, albumin ≤ 3.2 g/dl, ALT≥ 80 U/L and neutrophils percentage≥ 80% at diagnosis, were identified as risk factors for resistance to IVIG. The Kobayashi and Egami scoring systems had low sensitivity of 57% and 62% respectively and a medium specificity of 76% and 76% respectively in predicting IVIG resistance.Conclusion: Clinical and epidemiological characteristics of KD in Cyprus population were similar to those reported in the literature. Although the majority of cases received appropriate treatment in time, cardiac complications still occurred. The Kobayashi and Egami scoring systems cannot be used in our population in order to predict responsiveness to IVIG.

Effect of COVID-19 on Kawasaki Disease: Decrease Age of Onset and Increase Skin Manifestation

Background Kawasaki Disease (KD) is the most common childhood vasculitis and cause of acquired heart disease for no apparent reason. There is some evidence indicating infectious agents as possible triggers for KD. During the COVID-19 pandemic, vasculitis has been a presentation of COVID-19 in children. We performed this study to assess the association between KD and COVID-19. We evaluated KD hospitalized children during February to September 2020 for COVID-19 (group one) and compared their demographic, clinical, laboratory, and echocardiographic findings with KD patients from the same period time in 2019 (group two). We also compared the same data in COVID-19 positive and COVID-19 negative KD patients in 2020 pandemic period in Shiraz Namazi referral hospital at southwest of Iran. Results Thirty-two patients in group one compared with 44 patients in group two. Sixty-eight percent of group one KD patients were positive for COVID-19 during the pandemic period. KD Age of onset in the group one was lower than group two (4.38 years VS 5.5 years, P-value = 0.044). There was no difference in the demographic, clinical, laboratory, and echocardiographic features of the patients during and before the COVID-19 pandemic (p-value > 0.05). Moreover, Comparing COVID-19 positive and negative the incidence of rash was higher within COVID-19 positive cases (p < 0.05), and coronary artery abnormalities were more prevalent in COVID-19 negative cases (p < 0.05). Conclusion Admission rate of KD was almost similar during the COVID-19 pandemic but 68% of KD admitted patient were COVID-19 positive. Age of onset for KD during the COVID-19 pandemic was lower and skin manifestation was higher than the same period time in last year.

Anti-inflammatory Treatment of Kawasaki Disease: Comparison of Current Guidelines and Perspectives

Kawasaki disease (KD), an acute, generalized vasculitis, is associated with an increased risk of coronary heart disease and is the most common cause of acquired heart disease in childhood. The incidence of KD is increasing worldwide. There are numerous international treatment guidelines. Our study aims to perform the first one so far comparison of them. While the gold standard therapy remains still the same (intravenous immunoglobulins and aspirin), there is currently a lack of evidence for choosing optimal treatment for high-risk patients and refractory KD. In this review, we also discuss the treatment of complications of KD and Kawasaki-like phenotypes, present an anti-inflammatory treatment in the light of new scientific data, and present novel potential therapeutic targets for KD.

Abstract 17147: Multidisciplinary-team Management Of A Severely-ill Pregnant Covid-19 Patient With A Newly Diagnosed Severe Mitral Stenosis

A 30-year-old female who was 26 weeks pregnant, presented to an outside facility with acute hypoxic respiratory failure and was initially treated as an asthma exacerbation. Her chest X-ray and CT revealed extensive bilateral airspace disease of ground glass opacities (Figure 1A) and a main pulmonary artery diameter of 3.5 cm. Transesophageal echocardiogram revealed severe mitral stenosis (MS) with a high Wilkins-Abascal score of 11/16, a mean mitral gradient of 19 mmHg, and a right ventricular systolic pressure of 94 mmHg consistent with severe pulmonary hypertension (Figure 1B, 1C). Rapid COVID test was positive, patient clinically deteriorated and was emergently transferred to our facility. She rapidly required intubation and vasopressor support. Our multi-disciplinary team (MDT) decided to perform an emergent rescue percutaneous balloon mitral valvuloplasty, which led to an improvement of mitral stenosis, but with a resultant significant MR. Intraoperative decision was taken to proceed with an emergency cesarean section and a live male was delivered. Postoperatively, she was treated supportively for COVID pneumonia with rapid improvement in clinical status. She was extubated on the 3 rd postoperative day (POD), and was ambulating without any need for oxygen by the 7 th POD, and discharged home on the 11 th POD. Her infant continued to improve and was discharged 1 month later. Discussion: Rheumatic MS constitutes a major cause of acquired heart disease complicating pregnancy in the developing world. This case report features some of the challenges in the diagnosis and management of a complex condition in the pandemic environment and highlights the importance of MDT approach.

Analysis of Heart Diseases in Pregnancy

Introduction: Cardiac diseases complicate 1% of all pregnancies. It is one of the 3 major indirect causes of maternal mortality in India. Methodology: This qualitative study was conducted at Saveetha medical college and hospital. Out of 1320 women who delivered between June 2020 to June 2021, 15 women had heart disease and they were included in the study. All the 15 women were asymptomatic. Results: A total of 15 pregnant women were included in the study. There were no twin pregnancies. In total, 2 women presented with congenital heart disease and 13 women with acquired heart disease. Out of the 15 women, 4 delivered vaginally and the remaining 11 had undergone LSCS. Discussion: During pregnancy, major changes occur in the cardiovascular system to meet the increasing metabolic needs of the mother and fetus. If these changes are not met with, it can lead to maternal and fetal morbidity. Failure to make normal adjustments can worsen an existing heart condition or early manifestations of a previously unknown condition, and therefore pregnancy is a natural stress test. The changes that occur during pregnancy and the physiology of pregnancy itself will enhance some pathological processes of the heart. Understanding these changes is important, and adjustments may vary from person to person. Conclusion: A developing cardiovascular disorder should be detected as early as possible and also it should be a priority in pregnancy monitoring, and if a disorder is identified, the cardiologist team should be involved immediately. Many groups dedicated to managing heart problems during pregnancy, is recommended as it leads to better clinical outcomes.

Unexplained Syncope: The Importance of the Electrophysiology Study

Syncope of cardiac origin may be associated with an increased risk of sudden cardiac death if not treated in a timely and appropriate manner. The diagnostic approach of syncope imposes a significant economic burden on society. The investigation and elucidation of the pathogenetic mechanism of syncope are of great clinical importance, as both prognosis and appropriate therapeutic approaches depend on these factors. The responsible mechanism of presyncope or syncope can only be revealed through the patient history, baseline clinical examination and electrocardiogram. The percentage of patients who are diagnosed with these tests alone exceeds 50%. In patients with a history of organic or acquired heart disease or/and the presence of abnormal findings on the electrocardiogram, a further diagnostic electrophysiology inclusive approach should be followed to exclude life threatening arrhythmiological mechanism. However, if the patient does not suffer from underlying heart disease and does not show abnormal electrocardiographic findings in the electrocardiogram, then the probability in the electrophysiology study to find a responsible cause is small but not absent. The role of a two-step electrophysiology study inclusive risk stratification approach for the effective management of the former is thoroughly discussed in this review.

Kawasaki Disease and General Anesthesia for Dental Treatment: A Case Report

Kawasaki disease (KD) is an acute vasculitis of childhood and is the leading cause of acquired heart disease in children in developed countries. Failure to quickly diagnose and treat patients with KD can result in severe cardiac sequelae, especially coronary artery aneurysms (CAAs). Patients with a prior diagnosis of KD who require general anesthesia (GA) may present unique challenges depending on the severity of any cardiovascular sequelae. This case report describes the perioperative management of a 5-year-old male patient previously diagnosed with incomplete KD approximately 1 year before presenting to Stony Brook University Hospital for full mouth dental rehabilitation under GA. Most uniquely, the patient was at high risk for coronary artery thrombosis due to a giant CAA of his right coronary artery and a small CAA of his left anterior descending artery. The discussion also includes the implications of dental treatment under GA for patients with a history of KD.

The Immune and Inflammatory Basis of Acquired Pediatric Cardiac Disease

Children with acquired heart disease face significant health challenges, including a lifetime of strict medical management, multiple cardiac surgeries, and a high mortality risk. Though the presentation of these conditions is diverse, a unifying factor is the role of immune and inflammatory responses in their development and/or progression. For example, infectious agents have been linked to pediatric cardiovascular disease, leading to a large health burden that disproportionately affects low-income areas. Other implicated mechanisms include antibody targeting of cardiac proteins, infection of cardiac cells, and inflammation-mediated damage to cardiac structures. These changes can alter blood flow patterns, change extracellular matrix composition, and induce cardiac remodeling. Therefore, understanding the relationship between the immune system and cardiovascular disease can inform targeted diagnostic and treatment approaches. In this review, we discuss the current understanding of pediatric immune-associated cardiac diseases, challenges in the field, and areas of research with potential for clinical benefit.