P76 Management of Giant Coronary Artery Aneurysms as a Coronary Sequelae of Kawasaki Disease – Surgical or Conservative Management?

Abstract Introduction Kawasaki Disease (KD) is the commonest childhood acquired heart disease in developed countries, predominantly affecting children younger than 5 years’ of age. These patients are at high risks of developing coronary arterial aneurysms (CAA) and CAA with absolute internal luminal diameters measuring ≥8mm or Z-score ≥10 are classified as giant coronary aneurysms (GCA). Case Report A 19-year-old girl with previous history of Kawasaki disease was diagnosed with giant coronary aneurysms on echocardiograms. Her subsequent CT coronary angiography showed large aneurysms in the right coronary artery (29mm x 25mm) and left anterior descending artery (10mm x 10mm) with signs of calcification. However, surprisingly, despite the evidence of large coronary aneurysms, she was completely asymptomatic and was physically active, participating in competitive sports. Her cardiac MRI scan 17 years after KD onset showed right coronary perfusion deficit in the distal right coronary territory but with no evidence of myocardial infarction. A repeated cardiac CT was performed and showed delayed filling of the distal right coronary artery. However, it was uncertain at this point if the aneurysm was causing true ischaemia or simply delayed perfusion. An ECG stress test was subsequently performed for further investigation which showed no significant abnormality. After having a detailed discussion in the MDT and with colleagues from San Diego, USA, a conservative approach was decided to be in the patient’s best interest. She has been advised to avoid competitive or strenuous exercise due to the associated risks. She is fit-and-well, and is currently on Warfarin variable dose and Aspirin 75mg OD, with annual cardiac MRI surveillance. Discussion 20% of KD patients develop CAA and approximately 5% of KD patients have evidence of GCA. Unlike small and medium CAA, GCA regress in a much slower and more constant rate, and they never achieve complete resolution. A Japanese retrospective study showed the 10-year coronary event-free survival rates in small, medium and giant CAA as 100%, 96% and 61% respectively (p<.001). Patients with GCA are at significantly increased risks of coronary complications and major cardiac events (48%). Despite maximal medical treatment, patients with GCA have high risks of aneurysm rupture which is associated with high mortality rates. These patients are also at lifelong risks of atherosclerosis and stenosis which may lead to myocardial ischaemia and even death. Therefore, it is absolutely essential to control for cardiovascular risk factors and adhere to strict treatment regime. These patients also require lifelong, uninterrupted cardiology follow-up in a specialist KD clinic. Catheter and surgical interventions may be indicated in some patients. There have been recent attempts in combining CABG with downsizing reconstruction for GCA in an attempt to decrease GCA diameter and improve the coronary flow rate to prevent thrombosis which has shown quite promising results. However, the decisions whether to undergo a surgical intervention should be tailored to each individual, taking into consideration all aspects of a patient, including their disease status and social life. Conclusion With an increasing prevalence of KD in UK, it is essential to always consider the diagnosis of KD in a febrile child with raised inflammatory markers, especially in patients presenting with prolonged fever (longer than 4-5 days) as a delay in commencing treatment poses a significantly increased risk of developing coronary complications which are associated with high morbidity and mortality rates.

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276 Management of Giant Coronary Artery Aneurysms as a Coronary Sequelae of Kawasaki Disease – Surgical or Conservative Management?

British Journal of Surgery ◽

10.1093/bjs/znab134.252 ◽

2021 ◽

Vol 108 (Supplement_2) ◽

Author(s):

F F Quek ◽

R Tulloh

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Cardiac Mri ◽

Previous History ◽

Developed Countries ◽

Strenuous Exercise ◽

Coronary Perfusion ◽

Best Interest ◽

Coronary Aneurysms ◽

The Right

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3,4-Methylenedioxymethamphetamine (MDMA, Ecstasy) Intoxication in a Young Adult with Giant Coronary Aneurysms from Kawasaki Disease

Academic Forensic Pathology ◽

10.1177/1925362118797751 ◽

2018 ◽

Vol 8 (3) ◽

pp. 752-762 ◽

Cited By ~ 1

Author(s):

Renee Eigsti ◽

Dennis J. Firchau ◽

Marcus B. Nashelsky

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Left Ventricle ◽

Coronary Arteries ◽

Coronary Artery Spasm ◽

Artery Aneurysm ◽

Unknown Etiology ◽

Stress Tests ◽

Coronary Aneurysms ◽

Increased Risk

Kawasaki disease is an acute vasculitis of unknown etiology that usually occurs in children less than five years old. Coronary artery aneurysm due to arteritis is the most common cardiac complication. Chronic aneurysms are associated with an increased risk of developing luminal thrombosis and ischemic myocardial injury. We present a case of an 18-year-old male with a history of Kawasaki disease complicated by chronic giant aneurysms of the right and left coronary arteries. Serial echocardiographic studies and treadmill electrocardiogram stress tests as recent as 1.5 years before death revealed excellent cardiac function by clinical criteria. The decedent sustained a witnessed collapse after ingesting 3,4-methylenedioxymethamphetamine (MDMA) and ethanol. He was pronounced dead in the emergency department after unsuccessful resuscitative efforts. Autopsy revealed large aneurysms of the three main epicardial coronary arteries with extensive foci of severe stenosis by intimal fibrosis and organizing thrombus. Microscopic examination revealed multifocal severe myocyte hypertrophy. There were remote microinfarcts in the anterior and posterior aspects of the left ventricle and a recent, healing microinfarct in the posterior aspect of the left ventricle. Toxicology examination of postmortem femoral blood revealed MDMA, ethanol, and amiodarone. This case illustrates the residual, lasting effects of cardiac disease due to Kawasaki disease and a potential complication in the setting of use of an illicit stimulant, MDMA, an amphetamine derivative that produces sympathetic activation and cardiovascular effects including tachycardia, vasoconstriction, dysrhythmias, and coronary artery spasm. Kawasaki disease-related abnormalities of the heart likely resulted in a lower threshold for developing a fatal cardiac dysrhythmia under the circumstance of stimulant use.

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Recurrent Kawasaki Disease: A Case Report of Three Separate Episodes at >4-Year Intervals

Children ◽

10.3390/children5110155 ◽

2018 ◽

Vol 5 (11) ◽

pp. 155 ◽

Cited By ~ 1

Author(s):

Nikita Goswami ◽

Katherine Marzan ◽

Elizabeth De Oliveira ◽

Sharon Wagner-Lees ◽

Jacqueline Szmuszkovicz

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Systemic Vasculitis ◽

High Dose ◽

Z Score ◽

Coronary Aneurysms ◽

Increased Risk ◽

The Right ◽

Work Up ◽

High Dose Aspirin

Kawasaki disease (KD) is a self-limited systemic vasculitis, most often occurring in children 1–5 years old. It has a 2% recurrence rate and is associated with coronary aneurysms (CA), which can develop within two weeks of onset. A 25% increased risk is noted in patients who are recalcitrant to treatment. We describe a patient with recurrence of KD three times, approximately four years apart. A 10-year-old female with two previous episodes of KD, at 11 months and five years of age), in which she met five out of five criteria for KD and had no coronary involvement, presented with 15 days of fever, conjunctivitis and mucocutaneous changes. Infectious work-up was negative, and she was diagnosed with incomplete KD meeting three out of five criteria. An echocardiogram (ECHO) on day 12 revealed dilation of the right coronary artery (RCA) and left coronary artery (LCA). Treatment with intravenous immunoglobulin (IVIG) and high-dose aspirin was started at an outside hospital. After transfer, serial ECHOs showed evolving coronary aneurysms, left anterior descending (LAD) z-score + 8.2 and RCA z-score + 4.0. She received 10 mg/kg infliximab (day 18) and began clopidogrel. A cardiac MRI (day 20) demonstrated progression of the LAD aneurysm, with a z-score + 13, and warfarin was started. To our knowledge, this is the first report of recurrent KD occurring three times at ~5 year intervals.

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477 Three cases of Kawasaki disease developed at young age and with a long term follow-up

European Heart Journal Supplements ◽

10.1093/eurheartj/suab148.007 ◽

2021 ◽

Vol 23 (Supplement_G) ◽

Author(s):

Giorgia Zagarese ◽

Alessandro Varriale ◽

Gabriele Pesarini ◽

Alessandra Cristofaletti ◽

Camilla Sandrini ◽

...

Keyword(s):

Myocardial Infarction ◽

Coronary Artery ◽

Kawasaki Disease ◽

Acute Phase ◽

Right Coronary Artery ◽

Case Reports ◽

Vascular Complications ◽

Medical Attention ◽

First Case ◽

Coronary Aneurysms

Abstract Kawasaki disease is a relatively new disease and it took years since its first discovery to find and establish precise diagnostic and therapeutic indications. We report three cases of Kawasaki disease diagnosed between 1976 and 1993, which developed vascular complications and which were followed up for more than 20 years. The first case involved a 45-year-old woman who was first diagnosed with Kawasaki disease at age 3 years old. Her condition was complicated in the acute phase by a myocardial infarction and by the development of an apical aneurysm. The second case involved a 30-years-old woman who was first diagnosed with Kawasaki disease at age 6 months. The disease was complicated by coronary aneurysms and by right coronary artery occlusion. The third case involved a 29-year-old male who received a diagnosis of Kawasaki disease at age 1 year old. Echocardiography in the acute phase showed coronary aneurysms. One year later a new echocardiography revealed a thrombosis of the right coronary artery without myocardial infarction. The coronarography confirmed the coronary aneurysms and the thrombosis. All three cases were treated with a medical therapy and were followed up with periodic imaging exams, stress tests, 24-h Holter monitorings and cardiological check-ups. With these three case reports we have highlighted the possibility of finding a population of adult patients who may come to medical attention for acute events, such as myocardial infarction, presenting complicated, and disastrous angiographic pictures: it is plausible that such a population, in addition to cardiovascular risk factors, may have developed a KD in the years of lacking diagnostic and therapeutic options.

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472 Coronary disease in ADPKD patient: a giant coronary aneurysm

European Heart Journal Supplements ◽

10.1093/eurheartj/suab140.021 ◽

2021 ◽

Vol 23 (Supplement_G) ◽

Author(s):

Francesco Della Mora ◽

Simone Fezzi ◽

Marta Dal Porto ◽

Michele Pighi ◽

Flavio Ribichini

Keyword(s):

Coronary Artery ◽

Right Coronary Artery ◽

Invasive Coronary Angiography ◽

Previous History ◽

Giant Aneurysm ◽

Atherosclerotic Disease ◽

Circumflex Artery ◽

Coronary Aneurysms ◽

Increased Risk ◽

The Right

Abstract Aims Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic disorder driven by mutation of one of two genes: PKD1, which codifies polycystin-1, and PKD2, which codifies polycystin-2. The mutated proteins determine the formation of multiple renal cysts with a consequent decline in kidney function eventually leading to end-stage renal disease (ESRD). In the last decades the cardiovascular complications of ADPKD are emerging as the leading cause of death, but coronary artery disease (CAD) remains to be an uncommon complication. Methods and results A 60-year-old male patient affected by ADPKD, in dialysis treatment for ESRD, was admitted in 2020 to our hospital for invasive coronary angiography (ICA), checking eligibility for kidney transplantation. He had a previous history of hypertension and chronic ischaemic cardiomyopathy. ICA performed in 2017 for unstable angina assessed ectasiant coronary arteries with diffuse atherosclerotic disease (Figure 1), determining significant stenosis of the proximal left anterior descending artery and proximal circumflex artery, treated with percutaneous coronary intervention (PCI). In 2020 was so repeated ICA, that evidenced a good result of the previous PCI, but pointed out a severe progression of ectasiant disease, which led to formation of giant aneurysm of the proximal tract of the right coronary artery, assessed at 3.8 cm × 2.5 cm (Figure 2), fistulizing to the right atrium and determining significant flow limitation in the following part of the right coronary artery. The absence of any symptoms and the lack of evidence of ongoing heart dysfunction, led our team to indicate conservative management and angiography follow-up. Conclusions Cardiovascular disease is a major cause of morbidity and death in ADPKD, underlying a tendency towards accelerated atherosclerosis, but wide data about coronary involvement are still lacking. ADPKD patients seem to have an increased risk of developing coronary aneurisms, but either due to the expression of mutated proteins in arterial smooth cells, to the accelerated atherosclerotic disease or to the combination of both, is still controversial. Consequently, it is difficult to differentiate the underlying pathophysiology of aneurysm formation in an individual patient and to speculate whether ADPKD patients have an increased risk of developing coronary aneurysms independent of their accelerated atherosclerotic process.

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Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review

Cardiology in the Young ◽

10.1017/s1047951117000658 ◽

2017 ◽

Vol 27 (8) ◽

pp. 1529-1537 ◽

Cited By ~ 3

Author(s):

Michelle Keir ◽

Catriona Bhagra ◽

Debra Vatenmakher ◽

Francisca Arancibia-Galilea ◽

Katrijn Jansen ◽

...

Keyword(s):

Myocardial Infarction ◽

Cardiac Arrest ◽

Coronary Artery ◽

Cardiovascular Events ◽

Coronary Anomalies ◽

Coronary Artery Anomalies ◽

Major Cardiovascular Events ◽

Coronary Aneurysms ◽

Increased Risk ◽

In Pregnancy

AbstractObjectivesIndividuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population.MethodsWe performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death.ResultsA total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease.ConclusionsWomen with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

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Abstract 34: Genome-wide Association Study Identified New Susceptibility Loci To Coronary Artery Aneurysm-related In Kawasaki Disease

Circulation ◽

10.1161/circ.131.suppl_2.34 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Kyu Yeun Kim ◽

Mo Kyung Jung ◽

Yoon-Sun Bae ◽

Woohyuk Ji ◽

Dongjik Shin ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Association Study ◽

Genome Wide Association Study ◽

Coronary Artery Aneurysm ◽

Artery Aneurysm ◽

Genome Wide Association ◽

Korean Children ◽

Genome Wide ◽

Increased Risk

Kawasaki disease (KD) is an acute systemic vasculitis syndrome that predominantly affects children younger than 5 years of age, and may causes serious, sometimes life-threatening, cardiac sequela associated with coronary artery aneurysm (CAA). To identify genetic variants that confers a highly increased risk of coronary artery aneurysm-related in Kawasaki disease. In this study, we carried out genome-wide association study (GWAS) in a Korean children population including 102 CAA-related KD cases and 126 controls. Fifteen genetic loci were found to be significantly correlated with KD risk (P<1.0X10(-7)). Our case-control study revealed that rs4236089 C allele in chloride intracellular channel 5 (CLIC5) gene at 6p21.1 was significantly associated with KD patients with CAA (odds ratio (OR)=4.6, P=7.53X10(-7)). These findings suggest that the CLIC5 gene may play a crucial role in CAA development pathway of KD.

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Incidental Diagnosis of Anomalous Right Coronary Artery Originating from the Opposite Sinus, in a Kawasaki Disease Patient

The Kitakanto Medical Journal ◽

10.2974/kmj.71.199 ◽

2021 ◽

Vol 71 (3) ◽

pp. 199-201

Author(s):

Yuiko Suzuki ◽

Tetsuro Tamai ◽

Shuhei Takahashi ◽

Akiho Ueda ◽

Rena Okada ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Right Coronary Artery ◽

Disease Patient ◽

Kawasaki Disease Patient ◽

Incidental Diagnosis

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Anomalous origin of the right coronary artery with interarterial course: a mid-term follow-up of 28 cases

Scientific Reports ◽

10.1038/s41598-021-97917-w ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Francisco Albuquerque ◽

Pedro de Araújo Gonçalves ◽

Hugo Marques ◽

António Ferreira ◽

Pedro Freitas ◽

...

Keyword(s):

Coronary Artery ◽

High Risk ◽

Right Coronary Artery ◽

Cardiac Death ◽

Anomalous Origin ◽

Cardiac Symptoms ◽

Asymptomatic Patients ◽

Increased Risk ◽

The Right

AbstractAnomalous origin of the right coronary artery from the opposite sinus (right-ACAOS) with interarterial course (IAC) has been associated with increased risk of sudden cardiac death (SCD). Widespread use of coronary computed tomography angiography (CCTA) has led to increased recognition of this condition, even among healthy individuals. Our study sought to examine the prevalence, anatomical characteristics, and outcomes of right-ACAOS with IAC in patients undergoing CCTA for suspected coronary artery disease (CAD). We conducted a retrospective analysis of consecutive patients referred for CCTA at one tertiary hospital from January 2012 to December 2020. Patients exhibiting right-ACAOS with IAC were analyzed for cardiac symptoms and mid-term occurrence of first MACE (cardiac death, SCD, non-fatal myocardial infarction (MI) or revascularization of the anomalous vessel). CCTAs were reviewed for anatomical high-risk features and concomitant CAD. Among 10,928 patients referred for CCTA, 28 patients with right-ACAOS with IAC were identified. Mean age was 55 ± 17 years, 64% were male and 11 (39.3%) presented stable cardiac symptoms. Most patients had at least one high risk anatomical feature. During follow-up, there were no cardiac deaths or aborted SCD episodes and only 1 patient underwent surgical revascularization of the anomalous vessel. Right-ACAOS with IAC is an uncommon finding (prevalence of 0.26%). In a contemporary population of predominantly asymptomatic patients who survived this condition well into adulthood, most patients were managed conservatively with a low event rate. Additional studies are needed to support medical follow-up as the preferred option in this setting.

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Abstract 219: Long - Term Prognosis Of Patients With Kawasaki Disease Complicated By Significant Coronary Aneurysm (diameter ≥ 6 mm)

Circulation ◽

10.1161/circ.131.suppl_2.219 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

Ji Seok Bang ◽

Gi Beom Kim ◽

Bo Sang Kwon ◽

Eun Jung Bae ◽

Chung Il Noh ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Coronary Artery Bypass ◽

Artery Bypass ◽

Coronary Aneurysm ◽

Coronary Intervention ◽

Surgical Interventions ◽

Coronary Aneurysms ◽

The Mean

Background: Some patients with Kawasaki disease (KD) develop large coronary aneurysms (diameter ≥ 6mm) and coronary stenosis, leading to ischemic heart disease. This study determined the long-term outcome for patients with Kawasaki disease complicated by significant coronary aneurysm. Methods and Results: From the database in Seoul National University Children’s Hospital between December 1986 and December 2013, medical records of 83 patients (61 men and 22 women) with large coronary aneurysms (diameter ≥ 6mm) were retrospectively reviewed. Information on patient demographics, catheter or surgical interventions, and most recent status was collected. From these data, we calculated the survival rate, cumulative coronary intervention rate, coronary artery bypass graft surgery rate. The mean age at onset was 4.28 ± 2.62 years, and the mean observational period was 13.2 ± 6.5 years. The maximum coronary artery internal diameter ranged from 6.1 to 25mm (median 9mm). Giant coronary aneurysm was 57 patients (68.7%) and large coronary aneurysm (6-8mm) was 26 patients (31.3%). Coronary aneurysms had progressed to coronary artery stenosis and/or complete occlusion in 42 patients (50.6%). The overall freedom from coronary intervention, coronary artery bypass surgery, or coronary thrombi was respectively 85.5%, 85.5%, and 90.3%. Catheter and surgical coronary interventions (median 1 intervention; range 1 to 5 interventions) were performed in 20 patients (24.1%) at 9 month to 18 years after onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8mm vs ≥8mm). Myocardial infarction occurred in 8 (8.98 %) patients. During this study period, 1 patient died and 98% survival rates was seen in our patients group. Conclusion: The long-term survival of patients with Kawasaki disease complicated by large coronary aneurysm is good even though a fourth of patients underwent multiple catheter or surgical interventions. Further research should focus on the indications for and effectiveness of percutaneous and surgical coronary interventions in large coronary aneurysm (≥ 6mm).

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