First-in-Human Percutaneous Use of a Cerebral Flow-Diverting Stent in a Large Coronary Aneurysm.

A 58-year-old man presenting with angina was found to have a large coronary aneurysm on angiography. After coronary bypass and multiple ST-elevation myocardial infarctions over the following months, the decision was made to exclude the aneurysm with a flow-diverting stent, which reduced flow to the aneurysm and left the patient asymptomatic since the procedure. This is the first reported use of a cerebral flow-diverting stent for treatment of a coronary aneurysm.

Risk Factors for Intravenous Immunoglobulin Non-responsiveness and Coronary Ectasia in Korean Patients With Kawasaki Disease.

We aimed to determine the risk factors for non-responsiveness to intravenous immunoglobulin (IVIG) and coronary ectasia in Korean children with Kawasaki disease (KD) and compare the efficacy of previously published Japanese and Chinese risk scoring systems in the same cohort. We retrospectively reviewed 459 KD cases diagnosed from January 1, 2013, to December 31, 2022. Age (odds ratio [OR]: 0.983; 95% confidence interval [CI]: 0.968-0.999), change in extremities (OR: 3.308; 95% CI: 1.530-7.151), neutrophils (OR: 1.078; 95% CI: 1.049-1.108), and alanine aminotransferase (OR: 1.002; 95% CI: 1.000-1.004) were identified as independent risk factors for IVIG non-responsiveness, and age (OR: 0.945; 95% CI: 0.902-0.989), C-reactive protein (OR: 1.092; 95% CI: 1.004-1.188), and creatinine kinase (OR: 1.004; 95% CI: 1.001-1.006) were identified as independent risk factors for coronary ectasia. Among previously published risk scoring systems, the Egami (area under the receiver operating characteristics curve [AUC]: 0.695; 95% CI: 0.651-0.737) for IVIG non-responsiveness and the Tang score (AUC: 0.726; 95% CI: 0.578-0.874) for coronary ectasia showed the highest predictive value for our study cohort.

Etiology and prognosis of non-Kawasaki disease induced coronary aneurysms in children: a retrospective case series study.

While Kawasaki disease (KD) induced coronary artery aneurysms (KD CAAs) in children are well studied, the features and prognosis of non-KD induced CAAs (non-KD CAAs) in the pediatric population are poorly documented. This case series study is to analyze the etiology and prognosis of non-KD CAAs in children and compare the characteristics of non-KD CAAs and KD CAAs. Non-KD CAA and KD CAA cases at our department from January 2022 to December 2023 were retrospectively collected. Etiologies and prognosis of non-KD CAAs were analyzed. Furthermore, demographic data, biochemical parameters and outcomes between children with Non-KD CAAs and children with KD CAAs were comparatively studied. Fifteen children with non-KD CAAs with a median age of 6 years and 117 children with KD CAAs with a median age of 2.0 years (p = 0.022) were included in this study. The causes of non-KD CAAs include: unknown etiologies (2 cases), coronary artery structural abnormalities (4), Takayasu arteritis (2), virus infection (2), cardiomyopathy (2), aplastic anemia with agranulocytosis (1), ANCA-associated vasculitis (1), and mucopolysaccharidosis (1). In the non-KD CAA group, there were a total of 19 CAAs with 3 being giant, 5 medium, and 11 small; 4 patients had complete CAA regression; an infant with a fistula between the right coronary artery and the coronary sinus complicated with cardiac enlargement died of heart failure. The KD group had significantly higher levels of CRP, white cells counts and ESR with zero mortality. Non-KD CAA cases had a significantly lower regression rate than KD-CAA cases (26.7% vs 66.7%, p = 0.004), and the probability of CAA regression in non-KD patients was 0.341 of that in KD patients (p = 0.006, OR = 0.341, 95% CI: 0.179-0.647). CONCLUSIONS: Various etiologies for Non-KD CAAs are identified. Patients with Non-KD CAAs were observed to have lower inflammatory indexes but poorer recovery than patients with KD CAAs. Therapeutic strategies different than those for KD may be needed for non-KD CAAs. WHAT IS KNOWN: • Coronary artery aneurysm (CAA) in children is most commonly induced by Kawasaki disease (KD CAA), with a 50 ~ 70% regression rate in 1 to 2 years. • CAA induced by diseases other than KD (non-KD CAA) in children is rare and its prognosis remains largely unknown. WHAT IS NEW: • Most non-KD CAA cases are caused by coronary artery structural malformations. • Non-KD CAA in children has poorer prognosis and lower regression rate compared with KD CAA. • In addition to guideline directed anti-platelet and anti-coagulant therapies, treatments targeting the causal factor are necessary for non-KD CAA.

Advancing Kawasaki Disease Research in the Arab World: Scoping Literature Review Analysis with Emphasis on Giant Coronary Aneurysms.

To evaluate giant aneurysms (GiAn) prevalence in Arab countries and examine contributing factors; and to review Kawasaki disease (KD) publication trends and collaborations among Arab nations. A scoping literature review was conducted to analyze the publications across the Arab world, spanning 16 countries from 1978 to 2023. The collected articles were a combination of database search with a call on Kawasaki Disease Arab Initiative (Kawarabi) members to share non-PubMed publications. Over 45 years, 50 articles originated from the Arab Countries with a 30% average annual growth rate in KD research output. Publications were evenly split between case reports (42%) and institutional series (52%). Research productivity lagged in developing nations with UAE, KSA and Egypt, contributed to 64% of total publications. Among 26 institutional series, 256 coronary artery aneurysms (CAA) from a total of 1264 KD cases were reported. Of those, 25 CAA were GiAn (prevalence 1.43% [range 0-12.5%]). The initial KD misdiagnosis rate was 4%, and incomplete KD (iKD) averaged 10.6%. Series (38.5%) that did not report iKD correlated with a higher prevalence of CAA, but not of GiAn. Longer fever duration emerged as a pivotal factor for GiAn (OR 5.06, 95%CI 1.51-17). This review unveils the research landscape of KD in the Arab world over 45 years. Initial misdiagnosis, untreated cases, delayed diagnosis and underreporting of iKD are contributing factors for an underestimated epidemiology, explaining the higher GiAn prevalence. This calls for strategic interventions to enhance KD research in these countries, aligning with Kawarabi's mission.

Anterior STEMI in a 25-year-old with Cogan syndrome.

Cogan syndrome (CS) is a chronic inflammatory disorder primarily affecting young adults. It is characterized by interstitial keratitis, vestibuloauditory dysfunction, and, rarely, systemic vasculitis and aortitis. In this case report, we present a rare instance of a young male with anterior ST-elevation myocardial infarction in the context of aneurysmal coronary vessels and proximal occlusion of the left anterior descending artery. This case underscores the importance of recognizing cardiac risk factors in the setting of CS, as they can be life-threatening. Regular follow up is necessary, especially for patients showing signs of vasculitis or experiencing cardiac symptoms. A multidisciplinary approach to management can help optimize the prognosis for these complex patients. Learning objective: Comprehend Cogan syndrome (CS): Grasp the clinical manifestations and potential cardiovascular complications of CS. Diagnostic approach in CS: Differentiate cardiac symptoms in CS from typical coronary diseases, emphasizing the role of heart catheterization. Multidisciplinary management: Recognize the importance of integrating rheumatologists, cardiologists, and surgeons for optimal CS patient outcomes.

Mycotic Coronary Aneurysm Associated With Biventricular Fistula.

The incidence of coronary artery aneurysm is between 1.4% and 4.9% based on autopsy or angiographic series. Mycotic coronary arteries aneurysms are very rare and represent less than 3% of all coronary aneurysms. We report the case of a patient who presented with multiple coronary mycotic aneurysms.

ST-segment elevation myocardial infarction in Nail-Patella syndrome with anomalous coronary anatomy and aneurysms: a case report.

Background: Nail-Patella syndrome (NPS) is an autosomal-dominant pleiotropic condition characterized by pelvic and skeletal abnormalities and most commonly affecting a tetrad of nails, knees, elbows, and iliac horns, the iliac horns being pathognomonic for the condition. The most well-documented extra-skeletal manifestation is renal involvement with alteration in Type III collagen. No documented cases of NPS with anomalous coronary arteries or aneurysms, acute coronary occlusion, or successfully coronary interventions exist in the medical literature. Case summary: A 62-year-old female with a medical history significant for NPS diagnosed 50 years ago presented to the emergency department with a chief complaint of chest pain. She recently developed end-stage renal disease managed with peritoneal dialysis within the last year. Angiography revealed 100% right coronary artery occlusion with an anomalous take-off from the left circumflex artery. She demonstrated diffuse coronary aneurysms in the right coronary artery, mid-left anterior descending artery, and other epicardial vessels. Two drug-eluting stents were placed in overlapping fashion. Following careful apposition, the aneurysmal segment was successfully stented without complication. The patient was discharged without complication 2 days later. Discussion: Our case shows the first reported case of coronary vascular anomalies and successful coronary revascularization in a patient with NPS in the medical literature. Given the recently reported vascular anomalies and known collagen alterations seen in patients with the genetic disorder, clinicians should suspect further systemic vascular anomalies with their own unique therapeutic challenges when encountering patients with this rare genetic syndrome.

Multimodality Imaging of Bi-Coronary and Aortic to Pulmonary Fistulas With Saccular Aneurysms.

A 69-year-old man had fistulas arising from the left main, conus and aortic arch complicated by 2 saccular aneurysms with one draining into the pulmonic trunk seen during computed tomography and invasive angiography. These were treated conservatively but required repeat computed tomography and cardiac magnetic resonance imaging 2 years later for new heart failure.

Giant coronary artery aneurysm occluded completely by a thrombus.

A coronary artery aneurysm is an uncommon vascular disorder, and it can be a life-threatening disease when associated with rupture or an embolism. A 52-year-old man was found to have a 50-mm coronary artery aneurysm at the right coronary artery, and the aneurysm was completely occluded by a thrombus. He had no symptoms after arriving at our hospital, and his hemodynamics was stable. Therefore, initially, we administered anticoagulation therapy involving heparin. After therapy, the distal coronary artery was detected when the thrombus dissolved, and elective surgery was planned. Coronary artery bypass grafting, ligation of the inflow and outflow vessels, and resection of the aneurysm were performed. Early anticoagulation therapy and surgical aneurysm resection were effective for treating the completely occluded coronary artery aneurysm. We herein report this rare case of a giant coronary artery aneurysm occluded completely by a thrombus and treated successfully by anticoagulation therapy and surgical aneurysm resection.

Sex-Specific Susceptibility Loci Associated With Coronary Artery Aneurysms in Patients With Kawasaki Disease.

BACKGROUND AND OBJECTIVES: Kawasaki disease (KD) is an acute vasculitis that primarily affects children under age 5 years. Approximately 20-25% of untreated children with KD and 3-5% of those treated with intravenous immunoglobulin therapy develop coronary artery aneurysms (CAAs). The prevalence of CAAs is much higher in male than in female patients with KD, but the underlying factors contributing to susceptibility to CAAs in patients with KD remain unclear. This study aimed to identify sex-specific susceptibility loci associated with CAAs in KD patients. METHODS: A sex-stratified genome-wide association study (GWAS) was performed using previously obtained GWAS data from 296 KD patients and a new replication study in an independent set of 976 KD patients by comparing KD patients without CAA (controls) and KD patients with aneurysms (internal diameter ≥5 mm) (cases). RESULTS: Six male-specific susceptibility loci, PDE1C, NOS3, DLG2, CPNE8, FUNDC1, and GABRQ (odds ratios [ORs], 2.25-9.98; p=0.00204-1.96×10-6), and 2 female-specific susceptibility loci, SMAD3 (OR, 4.59; p=0.00016) and IL1RAPL1 (OR, 4.35; p=0.00026), were significantly associated with CAAs in patients with KD. In addition, the numbers of CAA risk alleles additively contributed to the development of CAAs in patients with KD. CONCLUSIONS: A sex-stratified GWAS identified 6 male-specific (PDE1C, NOS3, DLG2, CPNE8, FUNDC1, and GABRQ) and 2 female-specific (SMAD3 and IL1RAPL1) CAA susceptibility loci in patients with KD.

Complete surgical repair for enlarging coronary aneurysm with coronary-pulmonary artery fistula.

A 46-year-old male patient was referred to our hospital due to the presence of a coronary aneurysm showing a tendency to enlarge. Subsequent coronary angiography revealed a diagnosis of coronary aneurysm with a concomitant coronary-pulmonary artery fistula. The patient underwent a successful surgical repair, and postoperatively, experienced an uneventful recovery with no residual shunt or aneurysm.

A successful surgical management for a thrombosed giant left coronary aneurysm with right ventricular fistula in a young patient.

Giant coronary aneurysm with ventricular fistula is rare. Due to the limited data from randomised clinical trials, therapeutic strategies for coronary aneurysms predominantly rely on on case series and anecdotal evidences. Reporting cases that provide practical experience in managing these aneurysms is therefore crucial. In this article, we report a rare case of a successful surgical management for a thrombosed giant left coronary aneurysm with right ventricular fistula, which is larger than any previously reported cases.

Right Coronary Artery-to-Right Atrial Fistula Accompanied by Multiple Right Coronary Artery Aneurysms: A Case Report.

A coronary artery aneurysm (CAA) denotes a localized dilation of the coronary artery, while a coronary artery fistula signifies an aberrant connection between a coronary artery and a cardiac chamber or adjacent vessel. Here, we present a case study of a 68-year-old female with a previously diagnosed right coronary artery-to-right atrial fistula concomitant with multiple right coronary artery aneurysms. Initially asymptomatic, the patient subsequently manifested atrial fibrillation. Management involved augmenting the patient's home regimen with metoprolol tartrate, followed by successful cardioversion and restoration of sinus rhythm. Given the stability of the fistula and the absence of symptomatic exacerbation, no further interventional measures were undertaken. The patient was discharged with an adjusted metoprolol regimen and scheduled follow-up with her cardiologist. Subsequent imaging assessments unveiled progressive fistula expansion alongside the development of concurrent CAA, inciting deliberations concerning optimal treatment modalities.

Transcatheter closure of a coronary artery to pulmonary artery fistula with two sequential giant aneurysms.

A 73-year-old female presented with exertional dyspnea and was found to have a coronary artery to pulmonary artery fistula with 2 sequential giant aneurysms. Her chest radiograph showed a mass above the cardiac silhouette.

Giant Coronary Artery Aneurysms Presenting As Posterior Myocardial Infarction.

A coronary artery aneurysm (CAA) is defined as the dilatation of a vessel with a diameter of ≥1.5 times that of the adjacent normal vessel. Occasionally, aneurysms can be large enough to be characterized as giant CAAs, but there is no universally accepted definition. We discuss the case of a 45-year-old male patient who presented to the hospital with substernal chest pain. His ECG revealed ST depression and T wave inversions in precordial leads. Cardiac biomarkers were within normal limits. Due to concerns about coronary artery disease, cardiac catheterization was done, which revealed CAAs in the distribution of the right coronary artery (RCA), left anterior descending (LAD) and left circumflex (LCX) artery. The patient was at high risk for surgical intervention given coexisting severe pulmonary hypertension. Therefore, medical treatment was initiated with beta-blockers, high-intensity statin, and anticoagulation with warfarin. In a two-month follow-up, the patient remained asymptomatic without any residual symptoms. A CAA can present as an acute coronary syndrome. The treatment still evolves, involving medical management and/or percutaneous or surgical interventions.