Coronary Artery Involvement Research Articles

Epidemiological Features and Clinical Study of Kawasaki Disease in Iran

Kawasaki disease (KD) is an acute febrile systemic vasculitis of unknown etiology and the major cause of pediatric acquired cardiac disease worldwide, particularly in developed countries. This study characterizes the epidemiologic and clinical features of KD in the Pediatric Rheumatology Department service in a general hospital. 120 patients with the diagnosis of KD between 1990 and 2009 were enrolled. We investigated the epidemiologic and clinical features of coronary artery involvement of the patients. Frequency of many parameters including age, sex, season, clinical and laboratory findings, response to treatment, and complications of the patients determined. During the 20-year study period, 120 patients <15 years of age were admitted for KD. Among them, 39.2% were at the extremes of the age spectrum, with 2.5% <6 months and 36.7% >5 years of age, male to female ratio of 1.3:1 and the classic KD to incomplete KD ratio of 3.1:1. KD recurred in 5% of all cases. It occurred most frequently in the winter and least frequently in the summer. The occurrence of coronary artery abnormalities (CAA) was 4.2%. Kawasaki disease should be considered in any pediatric patients with a prolonged refractory febrile illness in order to prevent CAA formation.

Midterm Follow-Up of Multisystem Inflammatory Syndrome in Children From Pakistan

Background: There is very limited data on the follow-up of multisystem inflammatory syndrome in children (MIS-C). We aim to describe the early and up-to 16 months follow-up of MIS-C, with special reference to cardiac involvement.Methods: This cohort non-interventional descriptive study included patients Findings: Forty-one patients predominantly males (73%), at a median age of 7(0.2-16) years fulfilled the World Health Organisation criteria for MIS-C. Cardiac involvement was seen in 15 (36.5%) patients; impaired left ventricle (LV) function in 5 (12.2%), coronary artery involvement in 10 (24.4%), and pericardial effusion in 6 (14.6%) patients. No arrhythmia was noted in any case. There were 2 hospital deaths (4.9%), both in MIS-C shock subgroup (20%). At one-month there was persistent LV dysfunction in 2/5, coronary artery abnormalities in 7/10 and pericardial effusion resolved completely in all patients. By 6-months LV function returned to normal in all but coronary abnormalities persisted in 2 patients. At last follow-up [median 9.8 (interquartile range, 2-16) months], in 36/38 (94.7%) patients, coronary artery dilatation was persistent in 2 (20%) patients. Interpretations: Children with MIS-C have a good early outcome although MIS-C with shock, if diagnosed late, has a high mortality. On midterm follow-up, there is normalisation of LV function in all and recovery of coronary abnormalities in 80% of patients. Longer-term follow-up will define the extended natural history of coronary artery involvement in MIS-C.Funding Information: None.Declaration of Interests: No potential conflicts of interest.Ethics Approval Statement: The institutional review board of the Children’s Hospital approved the study and an informed consent was taken from the parents.

Prognostic value of modified Syntax Score in high- and medium-risk patients with multiple-vessel coronary artery disease and involvement of the left main coronary artery

Prognostic value of modified Syntax Score in high- and medium-risk patients with multiple-vessel coronary artery disease and involvement of the left main coronary artery

The Clinical Utility of the Two-Week Echocardiogram in Assessing Coronary Artery Changes in children with Kawasaki Disease

Kawasaki disease (KD) is the leading cause of paediatric acquired heart disease in developed countries due to cardiovascular sequelae from coronary artery (CA) involvement, especially if inadequately treated. Current international guidelines recommend all patients diagnosed with KD should undergo echocardiograms at baseline, 2-weeks and 6-weeks following diagnosis [1]. We aimed to assess the utility of the 2-week echocardiogram in guiding management of KD patients.

Clinical Impact of Medical Therapy Versus Revascularization in Patients With Chronic Coronary Total Occlusions.

Chronic total occlusions (CTOs) are prevalent angiographic findings in patients with suspected coronary artery disease. Conflicting results of randomized controlled trials and registries have not clarified the therapeutic approach for patients with CTO in clinical practice. Therefore, we sought to analyze variables influencing the decision-making process and their relationship with clinical outcomes according to the type of selected therapy. A total of 1248 consecutive patients with at least 1 CTO were identified between 2010 and 2014 at our institution. Clinical and angiographic variables were collected to allow the calculation of several predictive scores. Primary outcome was all-cause death at the longest follow-up available. Other endpoints of interest included cardiac mortality and myocardial infarction. Medical therapy (MT) alone was indicated in 719 patients (58%), whereas percutaneous coronary intervention (PCI) or coronary artery bypass grafting (CABG) were used in 240 (19%) and 298 (24%), respectively. Age, acute myocardial infarction, previous CABG, and age, creatinine, ejection fraction (ACEF) score were independently associated with the decision for MT alone. Conversely, the presence of multivessel disease, left main coronary artery involvement, and high SYNTAX score favored the decision for CABG. At a median follow-up of 4.3 years, revascularization strategies were independently associated with all-cause mortality and cardiac mortality (hazard ratio [HR], 0.42; 95% confidence interval [CI] 0.27-0.65; P<.001 and HR, 0.32; 95% CI, 0.17-0.60; P<.001, respectively) for PCI and (HR, 0.39; 95% CI, 0.26-0.58 and HR, 0.51; 95% CI, 0.32-0.81; P<.01, respectively) for CABG. Several clinical and angiographic parameters influence the decision-making process of patients with CTO. CTO revascularization with either PCI or CABG appeared to be associated with improved clinical outcomes at long-term follow-up as compared with MT alone.

Association of NFE2L2 Gene Polymorphisms with Risk and Clinical Characteristics of Acute Type A Aortic Dissection in Han Chinese Population.

The present study is aimed at investigating the association of NFE2L2 gene polymorphisms with risk and clinical characteristics of acute type A aortic dissection (AAAD) in a Han Chinese population. Six SNPs (rs1806649, rs13001694, rs2364723, rs35652124, rs6721961, and rs2706110) in NFE2L2 were genotyped using SNaPshot Multiplex Kit in 94 adult patients diagnosed with AAAD at our hospital, and 208 healthy Han Chinese subjects from the 1000 Genomes Project were served as the control group. The CC genotype of rs2364723 (CC versus (GC+GG), OR = 2.069, 95% CI: 1.222-3.502, p = 0.006) and CC genotype of rs35652124 (CC versus (CT+TT), OR = 1.889, 95% CI: 1.112-3.210, p = 0.018) were identified as risk factors for AAAD. Multivariable linear regression analysis revealed that the CC genotype of rs2364723 (β = 5.031, 95% CI: 1.878-8.183, p = 0.002) and CC genotype of rs35652124 (β = 4.751, 95% CI: 1.544-7.958, p = 0.004) were associated with increased maximum ascending aorta diameter of AAAD. Patients carrying rs2364723 CC genotype had a higher incidence of coronary artery involvement (31% vs. 12%, p = 0.027), while patients carrying rs35652124 CC genotype had a higher incidence of brain ischemia (9% vs. 0%, p = 0.045). In conclusion, NFE2L2 gene polymorphisms were correlated with risk and severity of AAAD in Han Chinese population.

EXTENSIVE ANTERIOR WALL ST ELEVATED MYOCARDIAL INFARCTION FOLLOWING STEERING WHEEL IMPACT AND BLUNT CHEST TRAUMA IN A ROAD TRAFFIC ACCIDENT: A RARE CASE REPROT

Background: Blunt trauma chest may rarely lead to acute myocardial infarction. Shear force generated from trauma causes tearing, laceration of coronary vascular intima and results in intraluminal thrombosis. Left anterior descending (LAD) artery is the most common to be involved secondary to its proximity to anterior chest wall. Case Presentation: We report a case of 38year old hypertensive male presented in emergency room with complaints of left sided chest pain &amp; diaphoresis for one hour following trauma to his chest from steering wheel following a collision between two four wheeler. During primary survey as per ATLS guideline 12 ECG revealed acute extensive anterior wall STEMI. Other associated injuries were left frontal non hemorrhagic contusion, bilateral minimal pneumothorax, multiple bilateral rib fractures, mild hemoperitoneum with small hematoma in retroperitoneum and serosal surface of stomach. Urgent coronary angiography done by cardiologist on call and patient was diagnosed with single vessel coronary artery disease involving LAD with severe systolic LV dysfunction. Percutaneous transmural coronary angioplasty (PTCA) was done by a drug eluting stent in LAD. Conclusion: An emergencyphysician should consider cardiac complications in patients with chest trauma including myocardial infarction, early diagnosis of which is critical to save the myocardium.Any delay in diagnosis can be detrimental. What we already know? There are quite a few case reports that already mentioned about possibility of acute myocardial infarction after blunt chest trauma following road traffic accident secondary to intimal laceration of coronary artery and intraluminal thrombosis. Most coomonly left anterior descending artery is involved. What this paper adds?This is rare case report of acute &amp; extensive anterior wall myocardial infarction secondary to single vessle coronary artery involvement after blunt chest trauma by steering wheel in a road traffic accident. We specifically points towards the fact that noobvious Clinical guideline to manage these kind of cases mentioned in literature.Hence proper clinical guidelines to manage such kind of cases. Also, in acute myocardial infarction post blunt trauma chest, PCI seems to be a better option than thrombolysis keeping in mind of other injuries.

Aerococcus urinae : A Rare Cause of Endocarditis Presenting With Acute Stroke

Aerococcus urinae is a rare organism infrequently isolated from cultures. Mostly known to cause urinary tract infection, it can cause bacteremia leading to severe urosepsis and infective endocarditis. Embolization is frequently reported with Aerococcus urinae endocarditis (AUE); hence, the presentation is highly variable. Sequelae such as various central nervous system manifestations, sepsis, valvular regurgitation with heart failure and even coronary artery involvement have been reported. We report a case of a 58-year-old man with AUE of the aortic valve, severe aortic regurgitation and embolic stroke as a result of embolization from AUE and ultimately required aortic valve replacement. Our case highlights this rare cause of endocarditis and offers insight into the variability of patient presentation and risk factors to consider. J Med Cases. 2021;12(2):65-70 doi: https://doi.org/10.14740/jmc3612

Angiographic Studies of Coronary Artery Disease in Rangpur Medical College Hospital

Background: Coronary artery disease (CAD), one of the leading causes of death is increasing globally. The number of CAD is also increasing in Bangladesh. Rangpur Medical College &amp; Hospital is also providing cardiovascular services to populations from urban, semiurban and rural population of northern region of the country. It started coronary angiography services from June 2011. This paper aims to analyze pattern of coronary artery occlusion in patients undergoing coronary angiography during January to October 2019.&#x0D; Methods: This was a retrospective observational study conducted in the cardiology department. There were a total of 308 cases of diagnostic angiography and coronary interventions done in Rangpur Medical College &amp; Hospital from January to October 2019. Among them 308 cases of coronary angiography done for Acute Coronary Syndrome and Stable Angina, were analyzed using SPSS (Statistical Package for Social Science), version 17.&#x0D; Results: Males were higher in number than females and majority of the patients were at or above 50 years of age. Out of 308 cases 225 had Acute Coronary Syndrome (ACS) and 83 had Stable Angina. 54 out of 308 were found to have normal coronaries. 25 patient with ACS had normal coronary. Out of all the patients with coronary stenosis, 19 had left main disease, 59 had SVD, 62 has DVD, 71 patient had TVD. 9 patients had CTO. 159 patients had severe coronary stenosis. 117 out of 225 ACS patient had more than one coronary artery involved, which is significantly higher than the stable angina group ( p&lt;0.01). Severe stenosis was found to be more common in ACS group (p&lt;0.003) when compared to the stable angina group.&#x0D; Conclusion: There has been a change with regard to clinical presentation and onset of risk factors for CAD at young age, but the load of atherosclerotic burden and pattern of involvement of coronary arteries have not changed in Elder group. Coronary angiography is a useful diagnostic and therapeutic tool for CAD. Coronary status is significantly different in ACS and stable angina. ACS has more chance of having multivessel stenosis whereas stable angina has single vessel, less severe or normal coronaries. Severity of stenosis is also high in ACS than in stable angina.&#x0D; University Heart Journal Vol. 17, No. 1, Jan 2021; 55-59

Characteristics and Outcomes of Coronary Artery Involvement in Polyarteritis Nodosa

BackgroundCoronary artery involvement is a severe but uncommon manifestation of polyarteritis nodosa (PAN), so clinicians have little knowledge of it. Our aim was to investigate the clinical characteristics, risk factors and outcomes of patients with PAN complicated with coronary artery lesions. MethodsData from 145 patients with PAN who were admitted to Peking Union Medical College Hospital from January 2000 to September 2019 were retrospectively collected. ResultsNineteen patients (13.1%) had coronary artery lesions due to PAN. The age at the onset of PAN was 32.3 ± 11.8 years. There were no significant differences in common risk factors for coronary arterial atherosclerosis between the patients with coronary artery involvement and those without. Affected branches of the coronary arteries were left anterior descending branch (15 patients), right coronary artery (14 patients), and left circumflex branch (9 patients). Eleven of the 19 patients exhibited multivessel lesions. Multivariate logistic regression analysis showed that celiac artery involvement (odds ratio [OR] 3.722, 95% confidence interval [CI] 1.115-12.427; P = 0.033) and new-onset hypertension (OR 6.668, 95% CI 1.936-22.961; P = 0.003) were risk factors for coronary artery involvement in patients with PAN. Stent placement was performed for 2 patients, and in-stent restenosis occurred in 1 of those patients a year later. ConclusionsPAN with coronary artery involvement exhibits more combined involvement of arteries of other organs and more severe diseases. PAN should be considered when treating young adults with an unknown origin of coronary artery lesions. In addition to systemic immunosuppressive treatment, other measures including antiplatelet and anticoagulation therapy should be initiated; however, determining the optimal time to perform procedures such as intervention or surgery is still challenging.

Pericarditis masking a localised thoracic aorta dissection: Making a point for point-of-care ultrasound

Early detection of aortic dissection (AD) remains essential due to high mortality rates. We report a case of localised thoracic AD initially misdiagnosed as pericarditis, but subsequently diagnosed through point-of-care ultrasound (POCUS). A 35-year-old gentleman presented with sudden-onset jaw and chest pain. An initial electrocardiogram revealed changes consistent with pericarditis, and high-sensitive troponin levels on admission were not raised. An initial diagnosis of pericarditis was made. However, due to persistent symptoms despite treatment, an urgent POCUS was performed, revealing a prominently dilated aortic root, with evidence of an intimal flap near the coronary cusps. Computed tomography imaging revealed a focal aortic root dissection confined within the sinus of Valsalva, with no involvement of coronary arteries or descending thoracic or abdominal aorta. This case highlights how commonly a misdiagnosis of AD can occur and how concurrent pericarditis can often mask AD. Our report highlights the need for better incorporation of POCUS in the initial assessment of acute chest pain, as studies have shown both high sensitivity and specificity in diagnosing AD, with no additional burden on treatment start-up time and mortality.

Preoperative Imaging Risk Findings for Postoperative New Stroke in Patients With Acute Type A Aortic Dissection.

Background: Stroke is a common postoperative complication in patients with acute type A aortic dissection (ATAAD). We aimed to explore the preoperative imaging risk findings for postoperative new stroke in patients with ATAAD.Methods: From January 2015 to December 2018, 174 patients with ATAAD who underwent preoperative aortic computed tomography angiography (CTA) and cerebral diffusion-weighted imaging (DWI) as well as postoperative brain CT were included, and divided into DWI (+) and DWI (–) groups. Pre- and intraoperative variables were collected, and logistic regression analysis was used to determine the independent risk predictors of postoperative new stroke.Results: The incidence of postoperative new stroke was 18.4% (32/174) in patients with ATAAD. Postoperative stroke was detected in 13 (31.0%) patients in the DWI (+) group and in 19 (14.4%) patients in the DWI (–) group with significant difference (P = 0.016). In the DWI (+) group, the lesions of the cerebral infarction located in the unilateral cerebral hemisphere and distributed more than three lobes (P = 0.007) were an independent risk factor for postoperative new stroke. Hypotension (P = 0.002), retrograde ascending aorta dissection with thrombosis of the false lumen (P = 0.010), aortic arch entry (P = 0.035), and coronary artery involvement (P = 0.001) were independent risk factors for postoperative stroke in the DWI (–) cohort.Conclusions: Patients with ATAAD with cerebral infarction are more likely to develop postoperative new stroke; thus, a preoperative DWI examination may be necessary. DWI lesions distributed more than 3 lobes in the unilateral hemisphere suggest a high possibility of postoperative stroke. For patients with ATAAD with normal brain, particular attention should be given to the CTA findings of false lumen thrombosis, aortic arch entry, and coronary artery involvement to avoid postoperative stroke.

An unusual presentation requiring an unusual intervention

Stanford type A aortic dissection is associated with significant morbidity and mortality. Acute myocardial infarction is a rare complication and can occur in 3% of patients due to retrograde extension of the dissection leading to coronary malperfusion. Involvement of the right coronary artery is more common. On the other hand, Left Main Coronary Artery (LMCA) occlusion is unusual and if unrecognised, can result in rapid hemodynamic deterioration with cardiogenic shock and eventually death. Emergent surgical repair is the definitive treatment. Intra-Aortic Balloon Pump (IABP) is frequently used in the setting of cardiogenic shock due to LMCA stenosis. In this case it was inserted before the diagnosis of type A aortic dissection was made. This represents a unique case of type A aortic dissection resulting in severe ostial left main coronary artery stenosis in which the IABP was successfully used as a bridge therapy to definitive surgical repair.

Revascularization Versus Medical Therapy in Takayasu's Arteritis Patients with Coronary Artery Involvement.

IntroductionTreatment strategies to improve clinical outcomes in Takayasu's arteritis (TA) with coronary lesions have ranged from pharmacological therapy to invasive procedures, such as coronary angioplasty, stenting, and surgery. However, the therapeutic strategy for this kind of patient is still unclear. This study aimed to investigate the clinical characteristics and influence of revascularization versus medical therapy in TA patients with coronary artery involvement.MethodsWe analyzed the medical records of 806 TA patients between January 2008 and December 2019. Clinical features and treatment were analyzed, and patients were categorized into medical treatment and revascularization. Additionally, patients were sorted as percutaneous coronary intervention (PCI) and coronary artery bypass surgery (CABG) based on the strategy of revascularization.ResultsNinety cases with coronary lesions induced by TA were enrolled. Among 90 cases, 39 patients adopted conservative treatment, and 51 patients received revascularization (28 subjects with PCI, 23 participants with CABG). The median follow-up time was 63 (45–91) months. There is no significant difference in cardiovascular death between medical treatment and revascularization (2/39, 5.1% vs. 5/51, 9.8%, P = 0.971). The analysis of subgroup indicated that the mortality caused by cardiovascular disease was also similar in the CABG and PCI (2/28, 7.1% vs. 3/23, 13.0%, P = 0.772). However, the proportion of restenosis is much higher in the PCI compared with that of CABG (39.3%, 8.7%, P = 0.022, respectively). Heart failure is an independent predictor of death in these patients.ConclusionsThere is no significant difference in cardiovascular death between medical treatment and revascularization. The analysis of the subgroup indicated that the mortality caused by cardiovascular disease was also similar in the CABG and PCI, but the restenosis is much higher in the PCI compared with that of CABG. Heart failure is an independent predictor of death in these patients.

Abstract 13574: A Rare Case of Giant Cell Arteritis With Coronary Artery Involvement

Introduction: Coronary artery involvement in patients with systemic vasculitis is relatively rare, but can be life threatening. There is a well-known occurrence of coronary artery vasculitis in Kawasaki disease and Takayasu arteritis, however, coronary arteritis caused by giant cell arteritis (GCA) has been documented in only a handful of reports. Case report: A 65-year-old woman with a history of GCA complicated by cerebrovascular stenosis s/p bypass and coronary artery disease (CAD) s/p DES to mid-left anterior descending (LAD) 3 years prior, without cardiac risk factors, who presented with shortness of breath and 2-3 hours of progressive non-radiating pressure like midsternal chest pain. Vital signs were stable with an unremarkable physical exam. EKG showed new T-wave inversions in V4-V6, I, II, aVL. Labs were significant for Trop T 0.17 and CRP 2.02. Echocardiogram showed EF 30% with wall motion abnormalities (ECHO 9 months prior showed EF 69% with no wall motion abnormalities). Coronary angiography revealed 99% in-stent-restenosis (ISR) of mid-LAD, 50% tubular OM1 stenosis, and 100% RCA occlusion with right-to-right collateral (figure 2). The patient underwent CABG with a left internal mammary artery graft to LAD and saphenous vein graft to RCA. Post-operative EF was 50%, and she was discharged on post-operative day 5. Discussion: In patients with systemic vasculitis and no identifiable CAD risk factors presenting with acute coronary syndrome, there has to be a high suspicion for coronary arteritis. Our case strongly suggests that GCA can involve the coronary arteries given the rapid progression of ISR with triple vessel disease in the absence of cardiac risk factors. Although advancements in imaging techniques have led to improvements in the diagnosis of systemic vasculitis, standardized imaging for evaluating coronary vasculature is lacking. Larger retrospective case studies are needed in order to clearly delineate the association between GCA and CAD.

Abstract 15484: Combined Coronary and Carotid Artery Involvement in a Caucasian Patient With Moyamoya Disease

Introduction: Moyamoya disease (MMD) is a rare, progressive steno-occlusive disease of the intracranial carotid arteries. This is primarily a cerebrovascular disease and is mostly seen in young Japanese patients. We report a case of severe stenosis in the distal left circumflex artery (dLCx) in a non-Japanese patient with previously diagnosed MMD. Case presentation: A 33-year-old Caucasian female with history of MMD and 2 CVAs presented with chest pain for 2 days. Her only medication at home was aspirin. There was no history of hypertension, diabetes mellitus, or tobacco use. There was no family history of early CAD. Physical examination was positive for tachycardia but negative for carotid or femoral bruits. Neurologic examination revealed decreased touch sensation and diminished deep tendon reflexes on the left side. ECG demonstrated tachycardia with T wave inversions in leads III and ST depression in lead II. Acute CVA was ruled out with an MRI. Cardiac troponins were minimally elevated with peak of 0.12 ng/dl. An initial diagnosis of NSTEMI was made; patient was loaded with aspirin, clopidogrel and started on a heparin drip. Cardiac catheterization on the following day revealed single vessel CAD in the dLCx, with 80% stenosis and TIMI flow 2. A drug eluting stent was successfully placed in the dLCx. Angiogram post-stent placement showed optimal angiographic result with TIMI 3 flow. The patient was discharged home on dual antiplatelet therapy, statin, beta-blocker and ACE inhibitor. Discussion: MMD is thought to affect the coronary arteries from fibrous intimal thickening and histopathology of these coronary lesions show a homogenous, soft intimal proliferation with minimum lipid deposition and without substantial inflammatory cell infiltration. Although the combined involvement of carotid and coronary artery stenosis is rare, coronary involvement should be considered as one of the causes of ischemic heart disease in young patients with MMD. To the best of our knowledge, this is the first reported case of combined involvement of carotid and coronary artery stenosis in a Caucasian patient with previously diagnosed MMD. It is imperative to educate patients with MMD about possible CAD so that they seek immediate medical attention if cardiac symptoms occur.

Abstract 17092: Coronary Artery Aneurysms Are More Common in Post-COVID-19 Multisystem Inflammatory Syndrome in Children (MIS-C) Than Pre-Pandemic Kawasaki Disease

Introduction: Multisystem Inflammatory Syndrome in Children (MIS-C) is thought to be a delayed reaction to SARS-CoV-2 exposure. Coronary artery aneurysms (CAA) have been described in the MIS-C diagnostic criteria, with many symptoms mimicking Kawasaki disease (KD). Our institution has seen a significant rise in KD-like illness during the current COVID-19 pandemic. Objectives: We sought to describe the variation in coronary artery (CA) involvement between traditional KD and post-COVID-19 pandemic KD/MIS-C cases. Methods: We identified children admitted to our center with KD from April to June 2016 - 2017 and those with MIS-C/KD from April 1 - June 6, 2020, with review of clinical and echocardiogram data. Presence of CAA (any CA z-score ≥ +2.5), z-scores of the left main (LMCA), left anterior descending (LAD), and right coronary artery (RCA), and presence of cardiac and valvar dysfunction were evaluated. Nonparametric Wilcoxon rank sum test was used to compare the groups. Results: There were 26 patients in the 2016-17 KD group and 24 in the 2020 KD/MIS-C group; results are shown in Table 1. The groups had similar median age, and 2016-17 KD patients were more likely to be male. 2020 KD/MIS-C patients were more likely to have CAA than 2016-17 KD patients (54% vs 26%, p=0.05). The LAD had larger median z-score in 2020 KD/MIS-C than KD (p=0.017). RCA and LMCA z-scores of 2020 KD/MIS-C patients tended to be larger than 2016-17 KD but did not reach statistical significance (p=0.097, p=0.07 respectively). More 2020 KD/MIS-C patients had cardiac dysfunction, not statistically significant (13% vs 0%, p=NS), with no differences in valve function or effusion. Conclusions: Our spring 2020 cohort of MIS-C/KD patients had higher incidence of CAA, particularly larger LAD z-scores than those with KD pre-COVID-19 pandemic. Coronary arteries should be thoroughly assessed in patients presenting with MIS-C symptoms. Future studies are needed to determine long term outcomes in this cohort.

Resistant Kawasaki Disease in an Infant Causing Giant Coronary Aneurysms with Thrombosis

Giant coronary artery aneurysms that occur in 0.5 to 1% of patients with Kawasaki disease can be fatal if associated with thrombosis. Some patients may show persistent inflammation and fever despite treatment with repeated doses of intravenous immunoglobulin (IVIG), steroids, and aspirin. This report describes an infant boy with resistant Kawasaki disease who presented with extensive coronary artery involvement and coronary thrombosis. His inflammation was not controlled with multiple doses of IVIG, parenteral and oral steroids, or high-dose aspirin, and he finally needed infliximab, a monoclonal antibody against tumor necrosis factor alpha.

Patterns of coronary vascular involvement in patients with heart failure due to cardiac amyloidosis

Abstract Background Amyloidosis is a group of protein-folding disorders characterised by organ deposits, derived from one of several amyloidogenic precursor proteins. The involvement of coronary arteries has been previously described in amyloidosis, however the pattern of the disease in the coronaries is still unknown. Purpose The aim of this study is to characterise the pattern and severity of coronary artery lesions in cardiac amyloidosis. Methods We retrospectively compared patients with heart failure who tested positive (i.e. biopsy or gene tests – HF/CA+) against those that tested negative (HF/CA−) for cardiac amyloidosis. Groups were compared demographically and angiographically for qualitative and quantitative variables to determine patterns of involvement in the major epicardial coronary vessels. Results In total, 110 heart failure patients were included in the study, of whom 55 were HF/CA+ patients (88 lesions) and 55 were HF/CA− patients (66 lesions). Despite the advanced age in the HF/CA+, (74.53±11.02 vs 54.11±15.08; p=0.05), severe calcification was reported in HF/CA− group (4.5% vs to 0.0%; p=0.018). The HF/CA+ group also had fewer ostial lesions (3.4% vs. 15.15%, p=0.0095), reduced TIMI flow grade (83% vs 76%; p=0.21) and a higher TIMI frame count (30±12 Vs 27±11 frames; p=0.06). In the HF/CA+ group, compared to women, men had a significant number of tandem lesions (14.55% vs 0.0%, p=0.02). Men trended to have more ulcerations in comparison to women (9.09% vs 0.0%; p=0.15). Conclusion Overall, patients with HF/CA+ were older but found to have lesser calcified lesions, ostial involvement and a reduced anterograde blood flow. This is the first report outlining the coronary lesions in patients with HF/CA+. Funding Acknowledgement Type of funding source: None

Coronary arteries in fibromuscular dysplasia. 3-Dimensional coronary CT, case-control study. The ARCARDIA-POL BIS study

Abstract Background Fibromuscular dysplasia (FMD) is a non-atherosclerotic arterial disease characterized by the presence of string-of-beads or focal stenosis. Other FMD-related lesions include arterial dissections, aneurysms, and tortuosity. However, limited data are available on the involvement of coronary arteries in pts diagnosed with “extra-coronary” FMD. Purpose To examine FMD-related coronary artery involvement, coronary artery lesions and coronary tortuosity in patients with a diagnosis of “extra-coronary” FMD. Methods In a case control study we enrolled 103 (age 45.7±13.2, 82 women) consecutive patients with a diagnosis of FMD, in whom atherosclerotic coronary artery disease was excluded on the basis of coronary CTA, and 96 (age 47.3±12.2, 75 women) sex- and age-matched controls without coronary atherosclerosis based on CTA imaging (Siemens Force 2x192 scanner). Detailed analysis of coronary arteries was assessed, with detailed analysis of arterial tortuosity, defined as curves &amp;gt;30 OR &amp;gt;45 OR &amp;gt;90 degrees. Tortuosity Index (TI) was defined as number of curves x artery length (centerline) / vector artery start-end. Each arterial tortuosity evaluation was performed per coronary segment AND per artery. Size (area, length) of coronary arteries was also measured. Results Intravessel symmetry sign and cork-screw sign were more common in arteries of FMD patients than in controls (5.4% vs 1.3%, p&amp;lt;0.001; 1.2% vs 0%, p=0.03, respectively). Number of arterial curves and TI of LAD, LCx and RCA were higher in FMD patients than in controls (arterial curves: p&amp;lt;0.01 for all arteries – TI: 14.9 vs 8.5 for LAD, 9.8 vs 6.6 for LCX, and 11.2 vs 6.6 for RCA; p&amp;lt;0.001 for all arteries). According to the segmental analysis, AUC under the ROC curve indicated the highest value for prediction of FMD for the number of curves (&amp;gt;30 degrees) (0.777; 95% CI:0.713 to 0.833) or TI (0.794; 95% CI: 0.731 to 0.848), both in distal LAD. For the distal LAD indices, the best sensitivity and specificity values were for ≥4 curves (sens. 74%, spec. 72%) or for a TI &amp;gt;5.6 (sens. 55%, spec. 94%), respectively. These predictive values were confirmed after correction for potential confounders. Interestingly, neither proximal LAD or proximal LCx number of curves or TI were related to FMD. No difference was found in terms of areas of coronary arteries between FMD patients and controls, with the only exception of LM (20.8±6.6 vs 17.9±5.8, p=0.002). Mean coronary artery lenght was significantly different between controls and patients with FMD (respectively LM 8.4±4.2 vs 9.8±4.5, p=0.022; LAD 117.8±28.6 vs 109.1±30.2, p=0.042; LCx 85.2±43.9 vs 62.9±31.4, p&amp;lt;0.001; RCA 104.2±24.4 vs 115.7±25.8, p=0.002). No coronary aneurysms, ectasias or dissections were found in the examined cohort. Conclusions Distal and not proximal coronary arterial tortuosity is related to FMD. Coronary tortuosity defined as 4 distal LAD curves or a TI &amp;gt;5.6 are highly specific for FMD. Funding Acknowledgement Type of funding source: None