Percutaneous coronary interventions for aneurysmatic right coronary artery in acute coronary syndrome: RIGHTMARE registry outcomes.

BACKGROUND: The optimal strategy during percutaneous coronary intervention (PCI) of aneurysmatic right coronary artery (ARCA) remains uncertain and has never been tested in the acute setting. OBJECTIVES: To compare the in-hospital and long-term outcomes of immediate and staged PCI strategies for ARCA as culprit lesions during acute coronary syndrome (ACS). METHODS: Among 102.376 PCIs performed in 18 European centers, a total of 85 patients presenting with acute coronary syndrome undergoing ARCA PCI were finally included in the analysis. PCI strategy (stenting performed during the immediate vs staged procedure) and pharmacological approach adopted were collected. The primary outcome was procedural success (technical success without in-hospital MACE). RESULTS: The primary outcome occurred in 48.2 % of cases, with no significant differences observed between the immediate and staged PCI groups (50.9 % vs 43.3 %, p = 0.504). Patients in the staged-PCI group had a significantly higher rate of intravenous anticoagulant use (83.3 % vs 48.1 %, p = 0.002), BARC type 3 and 5 bleedings (12.9 % vs 1.9 %, p = 0.037), and longer in-hospital stay (7.40 ± 5.11 vs 9.5 ± 5.25 days, p = 0.049). After multivariate analysis, no independent predictors for procedural success were found in either group. Target lesion failure occurred in 24.1 % of cases without differences between groups at a median follow-up of three years. CONCLUSIONS: Among patients undergoing ARCA PCI in the setting of ACS, immediate or staged PCI were associated with similar in-hospital and long-term outcomes. However, staged PCI was associated with a higher risk of major bleeding events and longer length of stay compared to immediate PCI strategy.

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.

Coronary changes and cardiac events in children diagnosed with kawasaki disease without initial coronary aneurysm: A multicenter retrospective cohort study.

Background: Kawasaki disease (KD) is a systemic vasculitis affecting young children, which may lead to coronary artery aneurysm (CAA). The optimal timing of serial echocardiography in patients with uncomplicated KD is debated. Objectives: To assess changes in coronary artery Z-scores from the initial diagnosis, two weeks, eight weeks, and one year following diagnosis and adverse cardiac events in children diagnosed with KD without initial CAA. Methods: Retrospective chart reviews of four referral centers in Thailand were conducted of all children who were diagnosed with KD without initial CAA (coronary artery Z-score < 2.5) between 2017 and 2020. Eligibility criteria included the absence of congenital heart disease and patients with available echocardiographic evaluations at baseline and at eight weeks of illness. The two-week and one-year echocardiographies were reported. Adverse cardiac events at one year from diagnosis were explored. The primary outcome was a maximal coronary Z-score on the follow-up echocardiography at eight weeks and one year. Results: Of 200 patients diagnosed with KD, 144 patients (72%) did not have CAA. A total of 110 patients were included in the study. The median age was 23 months (IQR, 2-39 months) and 60% were male. Fifty patients (45.5%) had incomplete KD, and four (3.6%) received a second intravenous immunoglobulin treatment. Of 110 patients, 26 patients (23.6%) had coronary ectasia (Z-score of 2-2.49) on their initial echocardiographic examination. Sixty-four patients were evaluated in two-week echocardiographic studies, which showed four new small CAAs and five coronary ectasia. At 8 weeks, 110 patients had undergone complete echocardiographic studies. No patient had residual CAAs. Only one patient had persistent coronary ectasia that regressed to normal within one year. At one-year follow-up (n = 90), no cardiac events were reported. Conclusion: New CAA in-patients with KD who had no previous CAA in their initial echocardiography are rare. In addition, patients who had normal echocardiographic follow-up at two weeks or eight weeks mostly continued to be normal at one year. The optimal timing of the echocardiographic follow-up should be at two to eight weeks in patients without initial CAA, who still have a coronary artery Z-score < 2 at the second echocardiography.Trial registration: TCTR20210603001.

Effect of Beta Blockers on the Hemodynamics and Thrombotic Risk of Coronary Artery Aneurysms in Kawasaki Disease.

This study aims to simulate beta blockers' (BB) effects on coronary artery aneurysms' (CAA) hemodynamics and thrombotic risk in Kawasaki disease (KD). BB are recommended in cases of large aneurysms due to their anti-ischemic effect. Coronary blood flow (CBF) was simulated in KD patient-specific CAA models using computational fluid dynamics. Hemodynamic indices that correlate with thrombotic risk were calculated following two possible responses to BB: (1) preserved coronary flow (third BB generation) and (2) reduction in coronary flow (first and second BB generations) at reduced heart rate. Following CBF reduction scenario, mean TAWSS and HOLMES significantly decreased compared to normal conditions, leading to a potential increase in thrombotic risk. Preserved CBF at lower heart rates, mimicking the response to vasodilating BBs, does not significantly affect local CAA hemodynamics compared with baseline, while achieving the desired anti-ischemic effects. Different BB generations lead to different hemodynamic responses in CAA.

Predicting hemodynamic indices in coronary artery aneurysms using response surface method: An application in Kawasaki disease.

BACKGROUND AND OBJECTIVES: Coronary artery aneurysms (CAA), such as those in Kawasaki Disease (KD), induce hemodynamic alterations associated with thrombosis and atherosclerosis. Current clinical routines assess the risk level of the CAA cases based on the Z-Score, which considers the body surface area (BSA) and the CAA's diameter. A full geometric characterization and impact on hemodynamic metrics and their correlation with thrombotic risks have not been systematically investigated. The goal of this study was to investigate the effect of CAA shape indices on local hemodynamics using the response surface method (RSM) through considering KD applications. METHODS: Transient computational fluid dynamics (CFD) simulations have been performed on idealized CAA geometries defined by geometrical ratios combining neck diameter, CAA diameter and CAA length. The results were used to develop full quadratic regression models of the indices using the response surface method (RSM). Validation using patient-specific KD models was performed. RESULTS: The results indicated that the aneurysm diameter is the main determining factor in the thrombotic risk of CAA patients, which is consistent with clinical guidelines. Furthermore, it was observed that in most CAA cases having the same diameter, the one with the shorter length experiences higher RRT values, indicating flow stagnation and circulation. CONCLUSIONS: The developed regression models can be used to ultimately assess the thrombotic risk of CAA cases from the hemodynamic perspective. The applicability of these models was tested on 2 KD patient specific models, with close values achieved between the models and the patient-specific results.

Evaluation of comprehensive myocardial contractility in children with Kawasaki disease by cardiac magnetic resonance in a large single center.

BACKGROUND: Children with Kawasaki disease (KD) and coronary artery lesions (CALs) can develop myocardial ischemia, fibrosis, and abnormal contractility. We aimed to assess the association between myocardial mechanical deformation with myocardial fibrosis, ischemia, and CALs. METHODS: In total, 76 KD and 20 healthy volunteers received cardiac magnetic resonance (CMR). Peak systolic left ventricular (LV) longitudinal, radial, and circumferential strain and strain rate [LV strain longitudinal (LVSL), LV strain radial (LVSR), LV strain circumferential (LVSC), LV strain rate longitudinal (LVSRL), LV strain rate radial (LVSRR), and LV strain rate circumferential (LVSRC)], along with late gadolinium enhancement (LGE), perfusion deficit, and CALs in related segments were analyzed. The KD group was subdivided by CALs, perfusion, and LGE results, and strain results were compared with controls and in subgroups. RESULTS: Cardiac fibrosis and ischemia were not confined to the territory of CALs. In a global analysis, strain and strain rates were lower in the KD group, especially in the subgroup with LGE and perfusion deficit. In segmental analysis, LVSR, LVSC, LVSL, and LVSRR decreased in the giant aneurysm group, and a lower LVSR (20.369%±10.603% vs. 26.071%±12.349%) and LVSC (-13.37%±5.365% vs. -15.847%±5.778%) were observed in thrombosed segments. The strain and strain rate were all lower in segments with LGE and perfusion deficit, and no obvious difference was found between groups with and without stenosis. LVSR had a better ability to identify giant aneurysm, thrombosis, stenosis, perfusion deficit, and LGE. CONCLUSIONS: We detected lower strain values in KD patients, which was more pronounced in segments with aneurysm, thrombi, LGE, and perfusion deficit. LVSR is useful to discern patients with higher risk.

Asymptomatic giant right coronary artery aneurysm in Kawasaki disease: A case report.

Incidentally diagnosed giant coronary artery aneurysms (gCAA) exceeding 50 mm in diameter are extremely rare and carry increased risks of cardiovascular morbidity and mortality. Currently, surgery is the preferred treatment for such gCAA.

Difference in inflammation, atherosclerosis, and platelet activation between coronary artery aneurysm and coronary artery ectasia.

BACKGROUND: Coronary artery aneurysm (CAA) and coronary artery ectasia (CAE) may be two different types of coronary artery dilatation with unknown etiology. This study aimed to compare the differences between CAA and CAE and to investigate their pathogenesis and the necessity of antiplatelet therapy. METHODS: One hundred patients each with confirmed CAA, CAE, and normal coronary artery (NCA) from September 2017 to July 2019 were included. All patients completed examinations of the ankle-brachial index (ABI), pulse wave rate, and carotid ultrasonography; and were tested for routine blood, lipid, and immune parameters. Blood samples were collected 1 week after the withdrawal of antiplatelet drugs, and vascular inflammatory indexes, platelet activation indexes, thromboelastography, and the platelet aggregation rate were measured. Analysis of variance and the chi-square or Fisher exact test were used for statistical analysis. RESULTS: The perinuclear anti-neutrophil cytoplasmic antibody (ANCA), endothelial-1, matrix metalloproteinase-9, and tumor necrosis factor-α were significantly higher in CAE than in NCA, while cytoplasmic ANCA was appreciably higher in CAE than in CAA (P<0.05). Myeloperoxidase and growth/differentiation factor-15 were significantly higher in CAE than in CAA and NCA (P<0.05). ABI was significantly lower in CAA and CAE than in NCA (P<0.05), low-density lipoprotein/high-density lipoprotein was significantly higher in CAA than in NCA (P<0.05), and the detection rate of carotid artery thickening was significantly higher in CAA than in CAE and NCA (P<0.05). The Gensini and SYNTAX scores were significantly higher in CAA than in CAE (P<0.05). The percentages of CD62P and PAC-1 were higher in CAA and CAE than in NCA (P<0.05). The arachidonic acid aggregation rate in CAA and adenosine 5'-diphosphate aggregation rate in CAE were significantly higher than in NCA (P<0.05). The values of thrombin formation time and reaction time were significantly lower in CAE than in NCA (P<0.05), and the α angle was significantly higher in CAE than in NCA. CONCLUSIONS: CAE was closely related to inflammation, whereas CAA was closely related to atherosclerosis. Platelet activation was present in both diseases; therefore, antiplatelet therapy is recommended.

Chronic active Epstein-Barr virus infection manifesting as coronary artery aneurysm and uveitis.

BACKGROUND: Chronic active Epstein-Barr virus (CAEBV) infection is a type of lymphoproliferative disorder characterized by chronic or recurrent infectious mononucleosis (IM)-like symptoms, which can have less-frequent clinical presentations. The prognosis of CAEBV is poor, and hematopoietic stem cell transplantation (HSCT) has been shown to be the only potentially effective treatment. In this article, we present a special CAEBV case of a patient who had no typical IM-like symptoms at the early stage, but manifested with severe and progressive coronary artery aneurysm (CAA), abdominal aortic lesions, and severe uveitis. These manifestations were uncommon features and could only be blocked by HSCT. CASE PRESENTATION: A 4-year-old girl with no special medical history complained of decreased vision for 10 months and cough after physical activities for three months. The blurred vision grew rapidly worse within one month, until only light perception remained. She was diagnosed with uveitis and cataract, and received prednisone and ciclosporin A treatment. However, her vision did not improve. Physical examination showed slight hepatosplenomegaly. Ultrasonic cardiogram showed bilateral CAA (5.0 mm and 5.7 mm for inner diameters), and abdominal CT scan revealed a thickened aortic wall, as well as stenosis and dilation of the segmental abdominal aorta. Other significant findings were increased EBV-DNA (3.29 × 104 copies/mL) from peripheral blood, positive EBV antibodies (EBV-CA-IgG, EBV-EA-IgA, and EBV-NA-IgG), and positive EBV-encoded small RNAs found by bone marrow biopsy. Based on her clinical manifestations and evidence for EBV infection, we diagnosed CAEBV. She received allogeneic HSCT, and the cataract operation was performed after HSCT. EBV-DNA could not be detected in peripheral blood after HSCT. Her CAAs did not progress, and uveitis was well controlled. Her vision recovered gradually over the 3 years after HSCT. CONCLUSIONS: We present a rare CAEBV case of a patient who suffered from uncommon and severe cardiovascular and ocular involvement that was relieved by HSCT. Therefore, early recognition and diagnosis of CAEBV are of vital importance to improve its prognosis. In summary, this atypical CAEBV case could help us recognize similar cases more easily, make the right diagnosis as early as possible, and deliver proper and timely treatment.

Association of autosomal dominant polycystic kidney disease, asymptomatic multiple giant coronary arteries aneurysms and abdominal aortic aneurysm: a case report.

Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disease characterized by the formation of multiple cysts in several organs. The formation of aneurysms accompanying this disease is being increasingly reported in the literature, and mutations in PKD-1 and PKD-2 are suspected in this etiology. Although the association between ADPKD and multiple coronary arteries aneurysms (CAA) was reported several times, we are presenting a case with the combination of ADPKD, multiple giant CAAs, abdominal aortic aneurysms and a suspected intracranial aneurysm, which has never been reported. The asymptomatic presentation of these multiple aneurysms might support the recommendations for further diagnostic investigations in these patients.

Multi-vessel giant coronary artery aneurysm in an elderly female.

Coronary artery aneurysm (CAA) is a rare anomaly. The right coronary artery is the most commonly affected, followed by the left circumflex (LCX), or the left anterior descending artery (LAD). Three-vessel disease or left main (LM) involvement is extremely rare. A giant coronary artery aneurysm (GCAA) has an extremely low incidence and refers to an aneurysm that is 20 mm or greater in size. Most CAAs occur as a consequence of atherosclerosis. Most patients with CAA are males, have three-vessel disease, and a history of myocardial infarction (MI). Thrombosis within the aneurysm can lead to distal embolization and MI. Depending on the severity of coronary stenosis, management of patients with LM CAAs is either surgical or medical.

Coronary Artery Anomalies: When You Need to Worry.

PURPOSE OF REVIEW: There is a broad spectrum of coronary artery anomalies that cardiologists may encounter either incidentally or during evaluation for cardiac symptoms. These anomalies include anomalous coronary arteries arising from the opposite sinus of Valsalva (ACAOS), coronary fistulae, and coronary artery aneurysms. This manuscript outlines the unique features, diagnostic characteristics, and treatment considerations for these lesions. RECENT FINDINGS: Intravenous ultrasound (IVUS), computed tomographic angiography (CTA), and magnetic resonance imaging (MRI) are becoming more sophisticated and will be increasingly used to facilitate the optimal treatment approach for coronary anomalies. There are a wide variety of coronary artery anomalies and their clinical ramifications range from benign to potentially fatal. Coronary anomalies often have complex anatomy and require advanced imaging modalities for comprehensive characterization. Due to the heterogeneity in lesion characteristics and outcomes, physicians should consider clinical and imaging features to create individualized management plans, along with referral to adult congenital heart disease centers.

Distribution of Kawasaki Disease Coronary Artery Aneurysms and the Relationship to Coronary Artery Diameter.

We investigated how the diameter of coronary artery aneurysm (CAA) relates to the distribution immediately after Kawasaki disease (KD). Two hundred and four pts (155 males and 49 females) who had undergone selective coronary angiography (CAGs) less than 100 days after the onset of KD were studied. We measured the maximum diameter of each artery segment in the initial CAGs. We analyzed the relationship between the maximum diameters and the distribution of CAA. We divided the patients into four groups based on the maximum CAA diameter in each patient (large(L) ≥8 mm, medium(M) ≥6 and <8 mm, small(S) ≥4 and <6 mm, very small(VS) <4 mm) and counted the affected segments. There were 87, 61, 36, and 20 patients in groups L, M, S, VS, respectively. The number of segments with CAA in each group was L 6 ± 2, M 4 ± 2, S 2 ± 2, VS 2 ± 1. The number of affected segments in L was significantly more than M, and a large value for L indicated that involvement was significantly more likely to be bilateral. The larger the maximum diameter of CAA, the more extensive disease involvement and the more likely to be bilateral. A large maximum CAA can also indicate coronary involvement in the longitudinal directions. It is an important charcteristic in distribution of CAA caused by KD vasculitis.

Association between SRC-1 gene polymorphisms and coronary artery aneurysms formation in Taiwanese children with Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) patients who experience a cardiovascular complication known as a coronary artery aneurysm (CAA) are at high risk of developing ischemic heart disease, which may lead to sudden death. The etiology of CAA in KD patients is unclear, and this study aims to clarify the relationship between steroid receptor coactivator-1 (SRC-1) gene polymorphisms and CAA pathogenesis. METHODS: We investigated four SRC-1 gene polymorphisms (rs11894248, rs17791703, rs7572475, and rs9309308) and their correlation with KD with CAA susceptibility in 327 Taiwanese people (279 KD patients without CAA and 48 KD patients with CAA). RESULTS: The results indicated a statistically significant difference in genotype and allele frequency distributions at the SRC-1 four single nucleotide polymorphisms (SNPs) between KD patients with and without CAA (P < 0.01). Additionally, Smad3 gene polymorphism (rs12901071) is well known to be associated with KD patients. In our results, Smad3 SNP did not provide a statistically significant difference between KD patients with and without CAA. CONCLUSION: Our data show that SRC-1 polymorphisms may be the underlying cause of CAA; therefore, the polymorphisms examined in this study warrant further investigation.