Coronary thrombosis and myocardial ischemia in Kawasaki disease: a case report.

BACKGROUND: Coronary artery thrombosis and myocardial ischemia caused by giant coronary aneurysms are the main causes of death in children with Kawasaki disease. The use of thrombolytic therapy in children with Kawasaki disease who have coronary thrombosis is a controversial topic, especially with respect to the timing of treatment. CASE PRESENTATION: In this article, we report a case of a child aged two years and nine months with Kawasaki disease whose coronary arteries had no involvement in the acute phase. However, by only one week after discharge, the patient returned because we found giant coronary aneurysms complicated by thrombosis via echocardiography. Despite aggressive thrombolytic therapy, the child developed myocardial ischemia during thrombolytic therapy. Fortunately, because of timely treatment, the child's thrombus has dissolved, and the myocardial ischemia has resolved. CONCLUSIONS: This case suggests that for patients at high risk of coronary artery aneurysms, echocardiography may need to be reviewed earlier. Low-molecular-weight heparin should be added to antagonize the early procoagulant effects of warfarin when warfarin therapy is initiated. In the case of first-detected coronary thrombosis, aggressive thrombolytic therapy may be justified, particularly during the acute and subacute phases of the disease course.

A case of large aneurysms of the coronary arteries and abdominal aorta in an infant.

Aneurysms of the coronary arteries and abdominal aorta are extremely rare in infancy. Due to the rarity of the anomaly, there are no existing guidelines on management of these aneurysms. We describe the challenges in diagnosis, evaluation and management of an infantile Marfan with this rare presentation.

Life-Saving Management of Traumatic Coronary Artery Dissection and Acute Myocardial Infarction in a 21-Year-Old Motorcyclist: A Case Report.

BACKGROUND A traumatic coronary artery dissection is a rare but severe complication of chest trauma that can result in blockage of the coronary artery. The clinical symptoms can vary considerably, from asymptomatic arrhythmia to acute myocardial infarction and sudden death. This report describes a young man with coronary artery dissection following blunt chest trauma from a motorcycle accident presenting with ventricular fibrillation due to acute myocardial infarction, which was treated with percutaneous transluminal coronary angioplasty and extracorporeal membrane oxygenation. CASE REPORT We present a 21-year-old man with chest contusion from a motorcycle accident who experienced sudden collapse due to ventricular fibrillation and acute myocardial infarction. The patient was resuscitated with extracorporeal membrane oxygenation, and 12-lead electrocardiogram showed sinus tachycardia with a hyperacute T-wave and ST elevation in leads V2-V6. Percutaneous coronary intervention revealed dissection from the ostial to proximal portion of the left anterior descending artery, and traumatic coronary artery dissection was confirmed. He was successfully treated with percutaneous transluminal coronary angioplasty, in which a drug-eluting stent was inserted to enhance blood flow in the left anterior descending artery, resulting in TIMI 2 flow restoration. After 16 days of intensive care, he was discharged and was well at a 3-month follow-up. CONCLUSIONS This report describes a case with the rare association between blunt chest trauma and coronary artery dissection and highlights that coronary artery dissection can result in ST-elevation myocardial infarction. Extracorporeal membrane oxygenation can protect the patient's circulation for coronary angioplasty. Therefore, early detection and intensive resuscitation can prevent disastrous outcomes.

Angiotomography in the diagnosis of coronary aneurysm secondary to Kawasaki disease

CASE REPORT: A 13yo patient, with a history of Kawasaki disease at the age of 2yo, diagnosed at the time with coronary artery aneurysm, treated with immunoglobulin and continued dual antiplatelet. Without follow-up, remained asymptomatic until January 2024, when has began to develop sporadic episodes of short-term chest pain, without well-identified triggering factors. He underwent Cardiac Computed Tomography Angiography (CCTA) demonstrating partially thrombosed aneurysm and calcified walls in the middle segment of the Right Coronary Artery (RCA), measuring 23 mm length and diameters of 12 x 10.5mm, with slight luminal reduction (Figure 1); There was also evidence of an aneurysm at the origin of the Circumflex Artery (ACx) with diameters of 7 x 6 mm without signs of a thrombus (Figure 2). After the test results, antithrombotic therapy and outpatient follow-up care was estabilished. In subsequent evaluations, the patient reported absent of new episodes of chest pain. DISCUSSION: An aneurism of coronary artery is an uncomun lesion, wich is defined as a 50% or greater increase in coronary artery diameter compared with an adjacent arterial segment. The number of complications related to coronary aneurysm are many, and includes embolic phenomenon, thrombosis, spasm, arteriovenous fistulization, and rupture. Kawasaki desease is a granulomatous inflammatory vasculitis wich can affect different anatomic regions of the heart, including the coronary arteries in 20-40% of the cases leading to stenosis or aneurysms. To date, there is no consensus on the most assertive method for diagnosing Kawasaki disease when transthoracic echocardiography is limitated. However, CCTA has been shown to be highly specific and sensitive for the accurate detection of coronary artery aneurysms. It can be considered a better and safer strategy, since it doesn't expose this population, pediatric in most cases, to the risk of possible procedural and vascular complications associated with invasive coronary angiography; and it has been also shownd to be superior to transthoracic echocardiogram. CONCLUSION: Based on the results demonstrated by imaging exams illustrated in the case described, we can highlight that CCTA can be considered a superior diagnostic instrument. As it is a non-invasive method, it offers a better safety profile for the diagnosis and long-term follow-up of patients with coronary artery aneurysm, to quickly guide appropriate management in order to avoid complications.

[Giant Coronary Artery Aneurysm in the Main Trunk of the Coronary Artery].

The case is a 76-year-old woman. She was admitted to the hospital because of chest and back pain. Coronary angiography revealed a 62-mm giant coronary artery aneurysm originating from the left main trunk( LMT), and urgent surgery was performed. Coronary artery-pulmonary artery fistula along with coronary artery aneurysm was completely removed by surgery. In this case, the reconstruction strategy for the LMT was crucial. The aneurysm wall was completely resected, allowing the coronary artery to return to its original course, and the length of the LMT defect was <2 cm. We determined that anatomical reconstruction of the LMT was optimal and succeeded in replacing a short great saphenous vein corresponding to the length of the defect. The patient was discharged without any complications.

Evaluation of Coronary Circulation by 13N-Ammonia Myocardial Perfusion Positron Emission Tomography in Patients with Right Coronary Artery Occlusion Due to Kawasaki Disease.

BACKGROUND: Although occlusion of the right coronary artery (RCA) is common in the remote stages of Kawasaki disease, revascularization of the RCA is challenging in children and is usually managed by observation without intervention. METHODS: Using adenosine-stress 13N-ammonia myocardial perfusion positron emission tomography, we evaluated coronary circulation in 14 patients (12 males) with RCA occlusion to identify ischemia (myocardial flow ratio < 2.0) in the RCA region and examined hemodynamics, cardiac function, and coronary aneurysm diameter. These variables were also compared in patients with/without RCA segmental stenosis (SS). RESULTS: There were five cases of ischemia in the RCA region. RCA myocardial blood flow (MBF) at rest was higher in patients with ischemia than in those without ischemia, but the difference was not significant (1.27 ± 0.21 vs. 0.82 ± 0.16 mL/min/g, p = 0.2053). Nine patients presented with RCA SS, and age at onset of Kawasaki disease tended to be lower in those with SS. The maximum aneurysm diameter of RCA was significantly smaller in patients with SS (10.0 ± 2.8 vs. 14.7 ± 1.6, p = 0.0239). No significant differences in other variables were observed between patients with/without ischemia and SS. CONCLUSIONS: At rest, MBF in the RCA region was relatively well preserved, even in patients with RCA occlusion, and there was no progressive deterioration in cardiac function. Adenosine stress showed microcirculatory disturbances in only half of the patients, indicating that it is reversible in children with Kawasaki disease.

Right coronary sinus aneurysm repair.

In this case report, we describe the surgical treatment of a right coronary sinus aneurysm. A 69-year-old male patient was screened because of palpitations. He was finally diagnosed with an aneurysm of the sinus of Valsalva of the right coronary cusp. According to current aortic guidelines, surgical reconstruction was proposed. The patient underwent a cardiac operation through a median sternotomy under routine cardiopulmonary bypass. After aortic cross-clamping, the aorta was opened and the connection between the aorta and the aneurysm was clearly visualized, underneath the ostium of the right coronary artery. After excision of the right coronary button and the remaining right coronary sinus wall, this sinus was reconstructed with a Dacron graft, with subsequent coronary reimplantation. The postoperative course was uneventful. The patient was discharged on postoperative day 7. A complete sinus reconstruction was preferred over local patching of the defect because of the proximity of the aneurysm sac to the right coronary artery and the fragile, thin aortic tissue just underneath the coronary ostium.

Lymphocyte-C-reactive protein ratio combined with albumin upon admission predicts coronary artery dilation and aneurysm formation in pediatric patients with Kawasaki disease: a retrospective cohort study.

BACKGROUND: We aimed to explore simple and effective clinical parameters or combinations to predict coronary artery dilation and aneurysm formation in pediatric patients with Kawasaki disease (KD). DESIGN AND METHODS: This retrospective cohort study included pediatric patients with KD from January, 2013 to December, 2022. Multiple demographic and clinical data were collected, collated, and calculated from the medical records. Then they were divided into the coronary artery dilation and aneurysm formation group or the non-coronary artery dilation and aneurysm formation group. Lymphocyte-C-reactive protein ratio (LCR) was transformed into its natural logarithm and expressed as lnLCR. RESULTS: A total of 64 pediatric patients with KD were enrolled in this cohort study after 1:3 propensity score matching (PSM). For each unit increase in lnLCR, the possibility of coronary artery dilation and aneurysm formation decreased to 0.419 times the original value. The areas under the receiver operating characteristic (ROC) curves of lnLCR combined with albumin (ALB), ALB, and lnLCR to classify pediatric patients with KD into the coronary artery dilation and aneurysm formation group were 0.781, 0.692, and 0.743, respectively. CONCLUSION: LCR combined with ALB upon admission is a promising predictor of coronary artery dilation and aneurysm formation in pediatric patients with KD.

Percutaneous intervention of a giant native coronary artery aneurysm using self-expanding stents.

In the absence of standardized management guidelines, coronary artery aneurysms (CAAs) present therapeutic challenges. Percutaneous coronary intervention (PCI) is rarely explored, especially in giant aneurysms with persistent angina, where surgery might be presumed as a preferred option. We describe the technical aspects and feasibility of PCI using Gore Viabahn expanded polytetrafluoroethylene (ePTFE)-covered nitinol self-expanding stents in a 66-year-old woman with a complex medical history and an enlarging, symptomatic right coronary artery aneurysm. The case was complicated by endoleak after the first stent, but intravascular ultrasound guidance enabled the precise deployment of additional stents, resulting in the successful exclusion of the aneurysm. This case demonstrates steps to successful CAA PCI with Gore Viabahn ePTFE-covered nitinol self-expanding stents and emphasizes that in unsuitable surgical candidates, PCI might be a potential alternative for symptomatic CAAs.

Coronary Artery Aneurysms and Acute Coronary Syndrome: An Interventionalist's Dilemma.

We report three cases of coronary artery aneurysm (CAA) in adults who presented with acute coronary syndrome. Two of these patients did not have traditional coronary artery disease risk factors. Management of CAA poses a significant challenge to interventionalists. We discuss the etiologic mechanisms, risk factors, pathophysiology, and diagnosis using angiography, intravascular ultrasound, and coronary computed tomography. We also highlight management options, including medical therapy and catheter-based interventions such as stenting, coil embolization, stent-assisted coil embolization, and surgical exclusion.

Giant coronary artery aneurysm in the atrial septum.

A man in his 60s with paroxysmal atrial fibrillation was scheduled for a catheter ablation but was admitted to our department after contrast-enhanced CT showed a large homogeneous right atrial mass (52×52 mm) as well as a dilated right coronary artery (RCA). Coronary artery angiography showed a large fistula from the RCA to the mass in the right atrium. A giant coronary artery aneurysm was suspected and a surgical resection was performed. The mass was attached to the atrial septal wall and was palpated in the right atrium with a feeding artery from the RCA. The final diagnosis was an extremely rare case of giant coronary artery aneurysm originating from the RCA. The surgery was successful, and the patient was discharged 30 days later.

[Surgical treatment of coronary artery to pulmonary artery fistula with a saccular aneurysm]. / Trattamento chirurgico di una grande fistola coronarica tra arteria discendente anteriore e arteria polmonare, di grosso aneurisma sacculare e di coronaropatia severa trivasale.

A 60-year-old man with hypercholesterolemia and hypertension presented with acute coronary syndrome (SCA). The ECG showed lateral ischemia (T-wave inversion in V4-V6, D1 and aVL) and echocardiography showed normal left ventricular wall motion. Coronary angiography showed critical atherosclerotic lesions in the distal part of the left circumflex artery (LCx, culprit lesion), chronic total occlusion of the right coronary artery (RCA), significant but not critical stenosis in the middle part of left anterior descending artery (LAD), and a coronary artery to pulmonary artery (PA) fistula originating from the proximal part of the LAD and emptying into the PA via a coronary saccular aneurysm (12 x 12 x 10 mm). A multidetector row computed tomography angiography (CTA) confirmed the coronary artery fistula, which was treated with surgical approach. The patient underwent aneurysmorrhaphy with CAF closure and coronary artery bypass grafting on the RCA and LCx. The postoperative course was uneventful and the patient was discharged on postoperative day 14. CTA was useful for understanding the spatial relation of the CAF and the connection with the PA.

Serum NLRP3: A potential marker for identifying high-risk coronary arterial aneurysm in children with Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is a vasculitis of unknown etiology in children aged under 5 years. Coronary arterial aneurysm (CAA) is the major complication of KD. It is no longer though to be a self-limiting disease because its cardiovascular sequelae might persist into adulthood. NLRP3 is a key protein of the NLRP3 inflammasome that participates in sterile inflammatory disease. This study investigated the serum levels of NLRP3 in patients with KD at different stages to explore the relationships between serum NLRP3 and clinical parameters. METHODS: A total of 247 children enrolled in this study. There were 123 patients in the acute stage of KD, and 93 healthy children made up the healthy control (HC) group. Among the acute KD patients, 52 had coronary arterial aneurysm (KD-CAA) and 71 did not (KD-NCAA). 36 patient samples were collected after IVIG and aspirin treatment. Additionally, 29 patients were in the cardiovascular sequelae stage. Enzyme-linked immunosorbent assay was used to measure serum NLRP3 levels in all subjects. RESULTS: Serum NLRP3 was elevated in the KD group and was even higher in the KD-CAA subgroup than in the KD-NCAA subgroup of acute-stage patients. Serum NLRP3 declined when the patients were treated with IVIG and aspirin, but during the convalescent (coronary sequelae) stage, serum NLRP3 re-increased. Serum NLRP3 was higher in the ≥ 6-mm-coronary-arterial-diameter group than that the < 6-mm-diameter group. The ROC curve of serum NLRP3 indicated its utility in the prediction of both KD and KD-CAA. CONCLUSIONS: NLRP3 may be involved in the development of KD and CAA in children with KD. Targeting NLRP3 might mitigate CAA, thereby reducing the risk of cardiovascular events in adulthood.

[Giant coronary aneurysm incidentally detected on transthoracic echocardiography in a patient with previous Bentall procedure]. / Riscontro incidentale di aneurisma coronarico gigante tramite ecocardiogramma transtoracico in esiti di pregresso intervento di Bentall.

Coronary artery aneurysms represent a rare pathology (0.2-4.9% of patients undergoing coronary angiography) that may reach considerable size. The clinical presentation is various, manifesting as acute coronary syndrome or, conversely, remaining silent lifelong. We here report the case of an incidental finding by transthoracic echocardiography of a paracardiac mass of considerable size in a patient with vasculopathy that underwent a Bentall procedure for acute aortic dissection 18 years earlier. On thoracic computed tomography angiography, a 62 mm-sized giant aneurysm located in the proximal right coronary artery was evidenced. The optimal treatment of patients affected by coronary artery aneurysms remains debated; therefore, the therapeutic strategy should be individualized considering the etiology, clinical presentation, anatomical characteristics and concomitant presence of obstructive coronary artery disease.

Coronary Artery Aneurysm with Fistula Associated with Tricuspid Valve Regurgitation.

ABSTRACT: Aneurysmal dilation of coronary arteries is a rare condition detected during coronary angiography. Due to their poorly elucidated underlying mechanisms, their variable presentations, and the lack of large-scale outcome data on their various treatment modalities, coronary artery aneurysms, and coronary ectasia pose a challenge to the managing clinician. This case presentation provides insight into the challenges regarding the management of the coronary artery aneurysm during the perioperative period.

Autopsy report of a sudden infant death that was strongly suspicious of Kawasaki disease.

We conducted an autopsy on a 3-month-old boy in whom Kawasaki disease (KD) was strongly suspected based on the autopsy findings. The infant had a fever and was brought to a nearby clinic, where he was prescribed antipyretics and kept under observation. However, 15 days after onset of the fever, he suddenly died in bed. He exhibited no obvious redness of the lips, tongue, or conjunctiva. Membranous desquamation was present on his distal fingers. Vasculitis was observed in the coronary arteries, renal artery, splenic artery, and pulmonary vein. In addition, coronary artery aneurysms were present in the right coronary artery and left anterior descending artery. Thrombotic occlusion was observed in one aneurysm in the right coronary artery, resulting in acute myocardial infarction. The coronary artery wall showed infiltration of numerous macrophages and neutrophils. This case was classified as incomplete KD because the coronary artery aneurysm could not be demonstrated before death and was only recognized at autopsy. Pathologists and forensic scientists need to be aware that there are cases in which KD goes undiagnosed and untreated, leading to coronary artery aneurysm formation and sudden death.