BACKGROUND: Kawasaki disease (KD) is a type of vasculitis in which giant coronary artery aneurysms (CAAs) can occur. There are no specific guidelines for managing giant CAAs that develop quickly and are at risk of rupture. Regarding cardiovascular drugs, only beta-blockers are formally recommended in the acute phase of KD.
METHODS: A 6-month-old male patient with multiresistant Kawasaki disease and giant CAAs that continued to enlarge after controlling systemic inflammation was examined. The patient required three doses of intravenous immunoglobulin, methylprednisolone pulses, and anakinra and infliximab to normalize systemic inflammation. Due to the rapid increment of aneurysms\' dimensions and the risk of rupture, we introduced anticoagulant therapy and propranolol plus captopril, and titration doses were introduced according to a tolerated decrease in heart rate and arterial pressure. CAAs increment stabilized and slowly reduced their dimensions.
CONCLUSIONS: The authors describe an atypical case of multiresistant KD with giant rapidly increasing CAAs even after controlling systemic inflammation. The introduction of a beta-blocker and an angiotensin-converting enzyme (ACE) inhibitor was demonstrated to be useful for stabilizing giant CAAs growth and reducing the potential risk of rupture.

A 59-year-old woman, smoker, hypertensive, without a previous history of coronary artery disease referred several episodes of epigastric pain, after exercise electrocardiogram was referred to coronary angiography. It revealed extensive coronary calcification, with a suboccluded left anterior descending (LAD) and a calcified aneurysm of the right coronary artery (RCA), partially filled with thrombus. Coronary-computed tomography showed aneurysmal saccular dilatation of the proximal LAD entirely thrombosed with subocclusion, and a fusiform aneurysm in the proximal RCA, partially thrombosed. The patient was referred for surgical treatment. In our patient, congenital etiology of the aneurysms was unlikely, since the patient did not present congenital heart disease or known genetically inherited disorders. Among acquired aneurysms, the most common cause is represented by atherosclerosis. Other potential causes are connective tissue disorders, trauma, infections, iatrogenic, and Kawasaki syndrome. Usual complications include myocardial ischemia and infarction, embolism, rupture, fistulization, and thrombosis (clearly represented in our case). Current recommendations about management strategies of coronary artery aneurysms (CAAs) are focused on small case series and based on aneurysm\'s location and morphology, patient\'s characteristics, and clinical presentation. Medical treatment strategies include antiplatelet therapy or anticoagulant. Other therapeutical options are percutaneous coronary intervention (PCI) and coronary artery bypass graft. In our case, the heart team opted for surgical treatment due to the subocclusion of the proximal LAD and considering stable angina as admitting diagnosis. Moreover, the CAAs were placed in proximal segments, with a large amount of thrombus, so related with high risk for complications if PCI was performed.

Kawasaki disease is an acute self-limiting systemic vasculitis in childhood, resulting in arterial swelling or inflammation and eventually leading to cardiovascular problems, such as coronary artery aneurysms. Based on previous studies, serum sodium ≤133 mmol/L, albumin ≤3.2 g/dL, alanine transaminase ≥80 U/L, and neutrophil percentage ≥80% at diagnosis are risk factors for intravenous immunoglobulin (IVIg). However, the prevalence of resistance to Ig among children with Kawasaki disease varies among different countries due to diversity in evaluation, treatment, and diagnosis. Approximately, 10% to 20% of patients have IVIg-resistant Kawasaki disease. As the probability of coronary artery damage associated with IVIg-resistant Kawasaki disease is higher than that with IVIg-sensitive Kawasaki disease, the early detection and appropriate treatment of IVIg-resistant Kawasaki disease can decrease the probability of damage to coronary arteries and hospital lengths of stay and cost. Kawasaki disease in early infancy is uncommon, and sometimes it occurs with thrombosis and peripheral gangrene. A positive genetic background may play a role in susceptibility to thrombosis. We herein describe a patient suffering from an IVIg-resistant Kawasaki disease with severe coronary artery thrombosis and positive genetic mutation. Medical treatment resolved the thrombosis, but the coronary arteries remained dilated.

Kawasaki disease is a self-limiting systemic vasculitis that can lead to various cardiac complications, including coronary dilatation and aneurysms. However, systemic aneurysms are uncommon and only occur in rare cases. In this instance, we present the case of an eight-week-old infant who presented to the emergency department with fever, loose motion, and neck swelling, ultimately diagnosed with Kawasaki disease accompanied by multiple systemic arterial aneurysms. This case highlights the potential for Kawasaki disease to cause systemic aneurysms and emphasizes the importance of recognizing and monitoring this rare complication in patients diagnosed with Kawasaki disease.

Kawasaki illness is an inflammatory condition of small- to medium-sized vessels that primarily affects children. It affects the lymph nodes, skin, mucous membranes, and heart, especially the coronary arteries. Patients who lack the comprehensive clinical manifestations of classic Kawasaki disease (KD) are typically evaluated for incomplete KD. Such patients have persistent fever and lack one or more characteristic clinical signs. Here, we present a case of a 16-month-old baby presented with fever for nine days, excessive crying and irritability for four days, and refusal to feed for one day with pallor and developed lip cracking, mucositis, bilateral edema, and redness in the palms and soles followed by periungual desquamation. Lab evaluations revealed anemia, elevated white cell count, and c-reactive protein along sterile pyuria. Since the child became afebrile after ten days of illness, inflammatory marker levels decreased, and no coronary artery abnormalities were detected on 2D echocardiography, and the child was diagnosed with incomplete KD based on the clinical, laboratory, and radiological evaluations after ruling out all other possible causes. He was managed conservatively with low-dose aspirin, and the child was doing well on a two-month follow-up.

UNASSIGNED: Due to the increasing prevalence of Kawasaki Disease (KD) in adulthood, the number of women considering pregnancy is growing. There are limited data on the course of pregnancy in KD with coronary artery involvement.
UNASSIGNED: We report on the pregnancy outcome of a 30-year-old woman with KD who was successfully resuscitated for ventricular tachycardia 3 years before. At that time, bypass surgery and later implantable cardioverter-defibrillator implantation were performed because of thrombotically occluded calcified giant coronary aneurysms. The pregnancy course was initially uncomplicated, however, at 31 weeks of gestation, left-sided breast cancer was diagnosed. Weighing maximum therapeutic efficacy against acceptable foetal and maternal cardiotoxic risk, our multidisciplinary team decided on neoadjuvant chemotherapy. The mother and foetus tolerated the therapy well. However, at 36 weeks of gestation, due to HELLP (haemolysis, elevated liver, low platelets) syndrome, a caesarean section had to be performed. The newborn was healthy with good APGAR (appearance, pulse, grimace, activity, respiration) scores. Three weeks after delivery, chemotherapy was restarted and at Week 4 after the caesarean section, the tumour was no more detectable.
UNASSIGNED: We discuss data on pregnancy and KD and outline that pregnancy can be considered if the clinical condition is good and left ventricular function is preserved. We also address possible therapeutic approaches and care for breast cancer in pregnancy and coexisting cardiovascular disease. The extraordinary importance of interdisciplinary cooperation between different disciplines in such complex clinical disease conditions is emphasized.

UNASSIGNED: Pediatric inflammatory multisystem syndrome (PIMS) is a complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children that resembles Kawasaki syndrome and places them at high risk of cardiorespiratory instability and/or cardiac damage. This study aims to describe the clinical presentation and outcomes of patients with PIMS in Mexico City.
UNASSIGNED: This was an observational study of children hospitalized for PIMS based on the Centers for Disease Control and Prevention case definition criteria, in a single tertiary care pediatric center in Mexico City between May 1, 2020, and September 30, 2021. Demographic characteristics, epidemiological data, medical history, laboratory tests, cardiologic evaluations, treatment, and clinical outcomes were analyzed.
UNASSIGNED: Seventy-five cases fulfilled the case definition criteria for PIMS [median age: 10.9 years, Interquartile range (IQR): 5.6-15.6]. Fifteen (20%) patients had a severe underlying disease, 48 (64%) were admitted to the intensive care unit, 33 (44%) required invasive mechanical ventilation and 39 (52%) received vasopressor support. The patients were clustered through latent class analysis based on identified symptoms: Cluster 1 had rash or gastrointestinal symptoms (n = 60) and cluster 2 were those with predominantly respiratory manifestations (n = 15). Two patients (2.7%) died, and both had severe underlying conditions. Five patients (6.7%), all from cluster 1, developed coronary aneurysms.
UNASSIGNED: There were a high proportion of patients with severe respiratory involvement and positive RT-PCR SARS-CoV-2 and very few cases of coronary aneurysms in our study which suggests that a high proportion of the children had severe acute COVID-19. The clinical manifestations and outcomes are comparable to previously reported international studies.

Isolated coronary arteritis without systemic involvement in adults is exceedingly rare. A 60-year-old patient developed recurrent non-ST-segment elevation myocardial infarctions for 1 year. After an initial coronary angiogram that was normal, serial angiograms showed de novo aneurysm formation. The patient responded favorably to corticosteroids, supporting the diagnosis of isolated coronary arteritis. (Level of Difficulty: Intermediate.).

UNASSIGNED: Multiple coronary-to-pulmonary artery fistulas (CPAFs) with giant coronary aneurysms (CAs) are extremely rare. The appropriate therapeutic indication and strategy for CPAFs have not been established.
UNASSIGNED: Herein, we report the case of an asymptomatic 74-year-old woman with multiple CPAFs associated with giant CAs that had gradually developed over a 4-year period. After heart team discussion, we were successfully treated by minimally invasive intervention using transcatheter coil embolization and coronary stent implantation to prevent ruptures.
UNASSIGNED: Coronary-to-pulmonary artery fistulas required evaluation of the appropriate timing of therapy initiation with reference to the presence of symptoms and fistula and aneurysm sizes, and determination of the optimal therapeutic approach with reference to the anatomy of the fistula with aneurysm and patient background characteristics.

Granulomatosis with polyangiitis (GPA) is characterized by necrotizing vasculitis of small and medium sized vessels and is rarely present in the pediatric population. Cardiac manifestations in pediatric patients with GPA are extremely uncommon, with only two known reported cases associated with coronary artery aneurysms (Rehani and Nelson in Pediatrics 147:e20200932, 2021, https://doi.org/10.1542/peds.2020-0932 ;Aghaei Moghadam et al. in Case Rep Cardiol 2020:3417910, 2020, https://doi.org/10.1155/2020/3417910 ). We report a case of a 14-year-old male who presented with a 1 month history of fatigue and shortness of breath. He ultimately was found to have multiple giant coronary aneurysms in both the left and right coronaries including a giant aneurysm in the posterior descending; this has not been previously reported. The case highlights the need for complete multi-modality imaging of the coronary arteries in patients with GPA.