Left Upper Extremity Pain, Right Coronary Artery Culprit: A Puzzling Path to Aneurysm Discovery.

Giant coronary artery aneurysm (GCA) is a rare disease afflicting 0.2% of the population. It is primarily attributed to atherosclerosis in adults and Kawasaki disease in children. Other uncommon etiologies include Takayasu arteritis and post-percutaneous coronary intervention.1,2 GCA lacks a universally accepted definition, with proposed criteria including a diameter exceeding 2 cm, 5 cm, or four times the normal vessel size.3 While the majority of GCAs are asymptomatic, a subset of patients present with angina, myocardial infarction from embolization or compression, heart failure due to fistula formation, or even sudden death.1 We report a case of an adult harboring a GCA involving the right coronary artery.

Kawasaki disease: patients' transition journey and recommendations for adult care.

BACKGROUND: Children who develop coronary artery aneurysms after Kawasaki disease are at risk for cardiovascular morbidity, requiring health care transition and lifelong follow-up with an adult specialist. Follow-up losses after health care transition have been reported but without outcome and patient experience evaluation. OBJECTIVE: The Theoretical Domains Framework underpinned our aim to explore the required self-care behaviours and experiences of young adults' post-health care transition. METHODS: A qualitative description approach was used for virtual, 1:1 interviews with 11 participants, recruited after health care transition from a regional cardiac centre in Ontario. Directed content analysis was employed. RESULTS: Health, psychosocial, and lifestyle challenges were compounded by a sense of loss. Six themes emerged within the Theoretical Domains Framework categories. Participants offered novel health care transition programme recommendations. CONCLUSIONS: The realities of health care transition involve multiple, overlapping stressors for young adults with Kawasaki disease and coronary artery aneurysms. Our findings will inform a renewed health care transition programme and will include outcome evaluation.

Solitary recurrence of IgG4-related giant coronary aneurysm: Case report and review of the literature focusing on treatment strategies and complications.

Coronary periarteritis is a dangerous manifestation of IgG4-related disease, because it forms coronary artery aneurysms, which may cause sudden cardiac death. We report the case of a 78-year-old woman with IgG4-related coronary periarteritis and a coronary aneurysm, which showed progressive enlargement despite maintenance therapy for Type 1 autoimmune pancreatitis. This case was unique, in that coronary periarteritis was the only active lesion that recurred. Low-dose glucocorticoids suppressed the progression of periarterial lesions but led to rapid thinning of the aneurysmal wall and an increase in the size of mural thrombi, which pose a risk of myocardial infarction. Our systematic literature review including 98 cases of 86 articles was performed to examine its treatment strategies and complications. Among the cases in which the effect of immunosuppressive therapy could be followed radiologically, 33 of 37 (89.1%) cases showed improvement in wall thickening/periarterial soft tissue, while 6 of 13 (46.2%) showed worsening increase in the outer diameter of the coronary aneurysms. We propose a draft treatment algorithm and suggest that immunosuppressive therapy for IgG4-related coronary periarteritis with coronary aneurysms should be conducted only after the therapeutic benefit has been determined to outweigh the risks. Because coronary periarteritis can occur without other organ involvement, as in our case, all cases of IgG4-related disease require careful monitoring of coronary artery lesions.

Optical Coherence Tomography in a 9-Year-Old Kawasaki Disease Patient with Giant Coronary Artery Aneurysms and Acute Myocardial Infarction.

BACKGROUND Kawasaki disease (KD), a systemic vasculitis, affects children aged <5 years and is the leading acquired cardiovascular disease in developed countries. Although intravenous immunoglobulin is an effective treatment for KD and decreases the rate of cardiovascular complications, some patients still develop coronary sequelae, including coronary aneurysms and myocardial infarction. CASE REPORT A 9-year-old boy was diagnosed with KD at the age of 6 years. For coronary sequelae of a giant coronary artery aneurysm (CAA) 8.8 mm in diameter, he was prescribed aspirin and warfarin. At 9 years old, he visited the Emergency Department because of acute chest pain. Electrocardiography revealed an incomplete right bundle branch block and ST-T change over right and inferior leads. Also, troponin I was elevated. Coronary angiography found acute thrombotic occlusion of the right CAA. We performed aspiration thrombectomy with intravenous tirofiban. Coronary angiography and optical coherence tomography (OCT) images later found white thrombi, calcification, destruction of media layer, irregular intimal thickening, and uneven intima edge. We prescribed antiplatelet therapy and warfarin, and he was doing well at a 3-year follow-up. CONCLUSIONS OCT is a promising modality that can impact the clinical care in patients with coronary artery disease. This report presents treatment management and OCT images of KD complicated with a giant CAA and acute myocardial infarction. We used aspiration thrombectomy in combination with medical treatments as the initial intervention strategy. Afterward, the OCT images showed vascular wall abnormalities, which were helpful for defining the future risk and decision making of further coronary interventions and medical treatments.

In-hospital outcomes and conditions in patients with acute coronary syndrome and coronary artery aneurysms who undergo percutaneous coronary intervention.

BACKGROUND: Coronary artery aneurysms (CAA) are uncommon, often incidental findings in patients with acute coronary syndrome (ACS) that represent a management challenge due to as there is a paucity of literature in this area. METHODS: We analyzed the National Inpatient Sample database from 2016 to 2020 by including all patients with the admission diagnosis of the ACS who underwent percutaneous coronary intervention (PCI). We sought to evaluate the association of CAA with other relevant systemic conditions and determine the impact of CAA on in-hospital outcomes. RESULTS: Among 1,733,655 hospital admission with ACS who underwent PCI, 2675 had CAA. There was a 2-fold increase in odds of CAA if the patient had coronary artery dissection (OR 2.05 95%CI 1.12-3.75, p = 0.020) or extracoronary aneurysm (OR 2.47 95%CI 1.46-4.16, p = 0.001) and a 3-fold increase in odds if they had a systematic inflammatory disorder (OR 3.24 95%CI 2.08-5.07, p < 0.001). CAA was not associated with increased odds of mortality (OR 1.22 95%CI 0.76-1.95, p = 0.42), bleeding (OR 1.29 95%CI 0.86-1.95, p = 0.22), acute stroke (OR 0.91 95%CI 0.40-2.07, p = 0.83), major adverse cardiac and cerebrovascular events (OR 1.08 95%CI 0.72-1.61, p = 0.71) or cardiac complications (OR 0.85 95%CI 0.49-1.47, p = 0.55). However, it was significantly associated with increased odds of vascular complications (OR 2.17 95%CI 1.47-3.19, p < 0.001). CONCLUSIONS: For patients with ACS who undergo PCI, the presence of CAA is associated with greater odds of vascular complications but after adjustments there was no difference in mortality or other complications. In this population, CAA is more prevalent in patients with coronary dissection, extracoronary aneurysms and systemic inflammatory disorders.

French national diagnostic and care protocol for Kawasaki disease.

Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up.

Percutaneous treatment of multiple fistulas associated with a giant aneurysm.

Coronary fistulas are not common in patients undergo coronary angiography. It is described, even less frequently, that coronary fistulas may be associated with giant aneurysms along their course. These cases classically are treated surgically. We present a case treated fully percutaneously in a novel fashion.

Use of the PK Papyrus covered coronary stent in the treatment of Kawasaki disease-associated giant coronary artery aneurysms.

Kawasaki disease (KD) is an acute vasculitis that can cause coronary artery inflammation and aneurysm formation leading to early obstructive disease. We describe the use of PK Papyrus covered stents (Biotronic, Inc.) in three pediatric patients to exclude coronary artery aneurysms (CAA) from the circulation and relieve aneurysm associated stenoses. Follow-up angiography at 11-17 months postprocedure demonstrated persistent exclusion of CAA and varying degrees of in-stent restenosis (ISR). Two patients required percutaneous coronary intervention with drug eluting stent (DES) implantation to relieve in-stent stenosis. Our findings suggest that CAA exclusion with the PK Papyrus stent is possible and may be a valuable tool in simultaneously treating stenotic and thrombogenic CAA in pediatric KD patients. ISR of these non-DES remains an issue and may require additional interventions within the short-term to maintain vessel patency.

A retrospective cohort study of major adverse cardiac events in children affected by Kawasaki disease with coronary artery aneurysms in Thailand.

Kawasaki disease (KD) is a common form of vasculitis in children that can be complicated by coronary artery aneurysms (CAAs). Data of long-term outcomes and major adverse cardiac events (MACE) in children with CAAs following KD in developing country are limited. Our aims were to determine the rates of MACE and identify risk factors associated with MACE in children with KD and CAAs in Thailand. We performed a retrospective analysis of data from 170 children diagnosed with KD and CAAs in two tertiary hospitals between 1994 and 2019. During a median (range) follow-up of 5.4 years (22 days to 23 years), 19 patients (11.2%) experienced MACE, that included 12 coronary artery bypass grafting, 2 percutaneous coronary intervention and 5 children with evidence of myocardial ischemia and coronary occlusion. Coronary interventions were performed at a median time of 4 years (0.01 to 9.5 years) after KD diagnosis. Forty-nine patients (28.8%) had giant CAAs. No MACE was reported in children with small CAAs. Independent risks of MACE were from the absence of intravenous immunoglobulin treatment (HR 7.22; 95% CI 2.21 to 23.59; p = 0.001), the presence of giant aneurysms (HR 13.59; 95% CI 2.43 to 76.09; p = 0.003), and CAAs that involved bilateral branches of coronary arteries (HR 6.19; 95% CI 1.24 to 30.92; p = 0.026). Among children with giant CAAs, the intervention-free rate was 93.8%, 78.7% and 52.2%, at 1, 5 and 10 years, respectively. Of note, 81% of the small CAAs regressed to a normal size, and for medium CAAs, 50% regressed to normal size. Overall, ~10% of children with CAAs following KD experienced MACE in this cohort. Timely IVIG treatment in children with KD following symptom onset will reduce the risk of MACE. Cautious surveillance to identify cardiac complications should be recommended for children once medium or giant CAAs develop. Trial registration: TCTR20190125004.

Impact of Z score system on the management of coronary artery lesions in Kawasaki disease.

BACKGROUND: Coronary artery aneurysms are well-described in Kawasaki disease and the Multisystem Inflammatory Syndrome in Children and are graded using Z scores. Three Z score systems (Boston, Montreal, and DC) are widely used in North America. The recent Pediatric Heart Network Z score system is derived from the largest diverse sample to-date. The impact of Z score system on the rate of coronary dilation and management was assessed in a large real-world dataset. METHODS: Using a combined dataset of patients with acute Kawasaki disease from the Children's Hospital at Montefiore and the National Heart, Lung, and Blood Institute Kawasaki Disease Study, coronary Z scores and the rate of coronary lesions (Z ≥ 2.0) and aneurysms (Z ≥ 2.5) were determined using four Z score systems. Agreement among Z scores and the effect on Kawasaki management were assessed. RESULTS: Of 333 patients analysed, 136 were from Montefiore and 197 from the Kawasaki Disease Study. Age, sex, body surface area, and rate of coronary lesions did not differ between the samples. Among the four Z score systems, the rate of acute coronary lesions varied from 24 to 55%. The mean left anterior descending Z scores from Pediatric Heart Network and Boston had a large uniform discrepancy of 1.3. Differences in Z scores among the four systems may change anticoagulation management in up to 22% of a Kawasaki population. CONCLUSIONS: Choice of Z score system alone may impact Kawasaki disease diagnosis and management. Further research is necessary to determine the ideal coronary Z score system.

Outcomes of Patients Who Undergo Elective Covered Stent Treatment for Coronary Artery Aneurysms.

BACKGROUND: Coronary artery aneurysms (CAA) are reported in up to 5% of patients undergoing coronary angiography. Treatment of CAAs with covered stents has been reported in several case reports, however there is limited evidence available on the effectiveness and safety of this interventional practice. PURPOSE: To evaluate the current practice and outcomes of elective treatment of coronary artery aneurysms with covered stents. METHODS: We conducted a systematic review of published case reports and case series of patients presenting with CAA that have been treated with covered stents in a non-emergency setting. RESULTS: A total of 63 case reports and 3 case series were included in the final analysis comprising data from 81 patients. The treated CAA was situated in a native coronary artery in 92.6%, and in a saphenous vein graft in 7.4%. Procedural success was achieved in 95.1%. The types of stents used were mainly polytetrafluoroethylene (75.3%) and Papyrus (11.1%). In 11.0% of cases additional abluminal drug eluting stents (DES) and in 6.8% additional adluminal DES were implanted. After a mean follow up of 13.4 months overall major adverse cardiovascular events (MACE), mortality, myocardial infarction, stroke, stent thrombosis and target lesion revascularization were reported in 26.2, 0.0, 7.6, 0.0, 4.6 and 18.5% of cases, respectively. CONCLUSIONS: The use of covered stents for elective treatment of CAA appears to be effective and reasonably safe. Nevertheless, it is associated with higher MACE rate, driven mainly by higher target lesion revascularization. Further studies, particularly in form of randomized trials and controlled registries are warranted to identify patients who might profit the most from this procedure.