Giant Coronary Aneurysm Research Articles

Comprehensive Evaluation of Kawasaki Disease With Electrocardiogram-gated Cardiac Multidetector Computed Tomography

We present a case of a young woman with Kawasaki disease who was comprehensively evaluated by electrocardiograph-gated cardiac multidetector computed tomography (MDCT). MDCT disclosed 3-vessel giant coronary arterial aneurysms and associated focal apical septal myocardial pathology. This was characterized by an early enhancement defect, myocardial thinning, and hypokinesia. These findings are likely due to focal ischemic insult resulting from distal embolization of thrombus material from the giant proximal right coronary arterial aneurysms. This case illustrates the full capabilities of MDCT in the comprehensive evaluation of Kawasaki disease.

608 The Study of Late Arterial Lesion in Children with Coronary Aneurysm Due to Kawasaki Disease

Objectives: Concerns have been raised regarding the existence of endothelial damage and the possibility of a predisposition to premature atherosclerosis in young adulthood with a history of Kawasaki disease (KD). This study was designed to evaluate the endothelial function and carotid intima-media thickness in children with coronary aneurysms. Methods: Thirty-one children with coronary aneurysms (9 with medium and 22 with giant aneurysms) due to KD and 21 healthy controls were enrolled. The duration from the onset of disease was 1-12.5 years (median 2.53 years) in KD group. Brachial artery flow-mediated dilation, non- flowmediated dilation, carotid artery stiffness index and intima-media thickness were determined by highfrequency ultrasound. Results: One KD patient had angina pectris, another had heart failure and abnormal Q waves. Endothelial dependent flow-mediated dilation (FMD) and Non flow-mediated dilation was lower in patients with coronary aneurysms due to KD as compared with normal controls (P< 0.05). Arterial stiffness index was raised in KD group than in normal controls (P< 0.05). There was no difference between the two groups in carotid intima-media thickness. The FMD was lower in KD patients with myocardial ischemia or ECG abnormalities than in patients without (P< 0.05), while no difference was found in FMD between patients with giant aneurysms and those with medium aneurysms. Conclusions: The endothelial function in patients with coronary aneurysms due to Kawasaki disease is damaged, especially in patients with giant coronary aneurysm and myocardial ischemia.

Giant Congenital Coronary Aneurysm of the Left Anterior Descending Artery

Coronary artery aneurysms are uncommon, especially in young patients. Most aneurysms in young patients are congenital, traumatic or due to Kawasaki disease. Giant coronary aneurysms warrant surgical management due to their propensity for complications. Coronary artery bypass grafting (CABG) with suture closure of the involved coronary artery seems to be an appropriate management strategy. We describe a giant coronary artery aneurysm of the left descending coronary artery, 15 x 18 cm in size, occurring in a 27-year-old male patient. This report underscores the need for further investigation to find the primary pathological defect causing giant coronary aneurysms, which often eludes us. Our experience suggests that this entity should be considered in the differential diagnosis of any young patient with chest pain.

Six patients with Kawasaki disease showing retropharyngeal low‐density areas on computed tomography

Six patients with Kawasaki disease showing retropharyngeal low‐density areas on computed tomography

Biopsy-proven myocardial sequels in Kawasaki disease with giant coronary aneurysms

Although most Kawasaki disease with giant coronary aneurysms is asymptomatic, conventional investigations might not identify previous lesions, or all Kawasaki disease with giant aneurysms at risk of future myocardial lesions. We evaluated the long-term histopathology of the myocardium, especially of intramural small vessels in asymptomatic Kawasaki disease with giant aneurysms. The initial study comprised 16 consecutive Kawasaki patients - male-to-female ratio was 12:4 - aged from 2 to 12 years, and in the subsequent study, the same patients were aged from 4.9 to 16 years. Endomyocardial biopsies were histopathologically evaluated. Microangiopathies, mitochondrial abnormalities, and loss or disarray of myofibrils were compared by electron microscopy. The incidence of histopathological abnormalities such as degeneration, hypertrophy, and inflammatory cell infiltration was quite high in the initial study, and inflammatory cell infiltration, interstitial fibrosis, and disarray were very noticeable at follow-up biopsies. The area of fibrous tissue was significantly higher in patients administered with intravenous immunoglobulin at follow-up biopsies. Electron microscopy showed microangiopathies including microthrombi within intramural small vessels in some patients at follow-up biopsies. The sites of the coronary aneurysms did not seem to have an impact on the biopsy findings, suggesting that the underlying pathophysiology is related to the original disease process. Whether the abnormalities were due to direct myocardial injury, chronic ischaemia, repeated small-vessel thrombosis, or other problems associated only with biopsies, is difficult to determine. However, this subgroup had residual abnormal lesions in the myocardium. Follow-up should be more aggressive in this group of patients to identify myocardial damage that could be asymptomatic.

Case–control study of giant coronary aneurysms due to Kawasaki disease: The 19th nationwide survey

The risk factors for recently reported cases of giant coronary aneurysms due to Kawasaki disease have not been elaborated. Fifty-three patients with giant coronary aneurysms, diagnosed as Kawasaki disease in 2005 and 2006, were selected from the 19th nationwide survey of the disease in Japan. With all the other patients recorded at the same hospitals as a control group, OR and their 95%CI were calculated to delineate the risk factors. In multivariate analyses, patients aged younger than 1 year (OR compared with 1-2-year-olds = 6.57) and those older than 5 years (OR compared with 1-2-year-olds = 4.24), those who received additional intravenous immunoglobulin (IVIG) without the use of steroid (OR = 8.38) and those who received steroid administration with or without the additional use of IVIG (OR = 220.51 and 83.83, respectively), showed significantly higher OR for giant coronary aneurysms. As for IVIG therapy, the additional use of IVIG (OR = 14.84), total dosage of IVIG exceeding 2500 mg/kg (OR compared with 1500-2499 mg/kg = 12.26) and the duration of IVIG administration for more than 3 days (OR = 30.12), were found to significantly increase the risk of developing giant aneurysms in univariate analyses that were adjusted for sex and age. The observation of 53 patients with giant coronary aneurysms due to Kawasaki disease among those included in the nationwide survey presented some risk factors, together with considerations about the associated aneurysms.

Giant right coronary artery aneurysm complicated by acute myocardial infarction

A coronary artery aneurysm is uncommon and frequently asymptomatic. This report presents a surgical case of a giant coronary artery aneurysm complicated by acute myocardial infarction. A 26-year-old man with sudden chest pain was referred to our hospital. Myocardial infarction was suspected, and emergency coronary angiography was performed. A giant coronary aneurysm was found in the mid-portion of the right coronary artery. The aneurysm, which was thrombosis-occluded, was successfully resected, and the right coronary artery was anastomosed in an end-to-end fashion. Although the strategy for treating a coronary artery aneurysm without myocardial ischemia remains controversial, surgical intervention should be considered in cases with a giant coronary artery aneurysm, even if asymptomatic, provided the surgical risk is low.

Giant coronary aneurysms developed in a child of Kawasaki disease with only 3 days of fever

Prolonged fever, 5 days or more, is the cardinal feature of Kawasaki disease. We described a 5-year-old boy with initial presentations of fever, conjunctivitis, and strawberry tongue. The fever only lasted for 3 days. However, giant coronary aneurysms developed later. This patient reminds us that coronary complications could happen even the fever is less than 5 days. Criteria of fever duration might need reconsideration.

Giant Coronary and Systemic Aneurysms of Kawasaki Disease in an Infant

Kawasaki disease (KD) was diagnosed in a 4-month-old male infant after 4 days of fever, rash, and bulbar conjunctivitis. He received two doses of intravenous immunoglobulin (IVIG), high-dose oral acetylsalicylic acid, and three doses of methylprednisolone within in the first week of illness for persistent fever. During the third week of his illness, echocardiogram showed giant aneurysms at the origin of the right coronary artery (17 mm) and at the bifurcation of the left coronary artery (9 mm). Anticoagulant therapy was started. At 9-month follow-up, the child was clinically well, and there were no ST-T wave changes on electrocardiogram. Aortography showed a giant aneurysm (19 9 19 mm) at the origin of the right coronary artery and another aneurysm (9 9 17 mm) distally (Figs. 1 and 2). The left coronary artery showed a large aneurysm (10 9 6 mm) at the bifurcation (Fig. 1). There was no evidence of stenosis in the right or left coronary artery. Subclavian artery injections showed large aneurysms in both the right (8 9 12 mm) and left (12 9 24 mm) brachial arteries (Fig. 3). Abdominal and carotid angiography were normal. At present, the infant is being maintained on low-dose aspirin and anticoagulant therapy. The international normalized ratio (INR) has been maintained between 2 and 2.5 (Fig. 4). KD is an acute multisystem vasculitis of unknown etiology that predominantly affects smalland medium-sized arteries. Fifteen percent of KD occurs in patients from 6 months to 4 years of age and often presents as the incomplete form [1]. Giant aneurysms (diameter [ 5 mm) are considered a high risk factor for the long-term development of stenotic coronary lesions [2]. Administration of IVIG within 10 days of illness decreases the incidence of giant aneurysms [3]. The risk of coronary artery aneurysms is higher for patients who do not respond to the first dose of IVIG, as happened in our case [3]. Systemic artery aneurysms have been reported to occur in 2% of patients with KD, and they are often associated with coronary aneurysms [4]. Fig. 1 Aortography showing a large aneurysm at the origin of the right coronary artery (white arrow) and a large aneurysm at the bifurcation of the left main coronary artery (red arrow). (Color figure online)

Giant Coronary Aneurysms Caused by Kawasaki Disease

An 8-year-old boy who presented to our hospital had all of the signs consistent with Kawasaki disease (KD; i.e., all criteria were met). He was treated with intravenous gamma globulins and oral acetylsalicylic acid. Transthoracic echocardiography showed a dilated left coronary artery (LCA) that was 15 mm in diameter. His platelet count was increased at 1.2 million/mm. The patient was well when he was discharged to home. There was no change in his echocardiogram on discharge. During the 6-month followup period, the child had no cardiac symptoms. A routine follow-up coronary angiography showed an 18 mm–wide coronary artery aneurysm (CAA) in the LCA. A few smaller aneurysms in the distal part of the left circumflex artery were also noticed (Fig. 1). The right coronary artery (RCA) was completely blocked (Fig. 2). Contrast medium, however, was visible distally in the RCA due to collateral flow. Discussion

Grown-up Kawasaki disease patients who have giant coronary aneurysms

Many Kawasaki disease (KD) patients have reached adulthood in Japan. The current status of adult patients who have giant coronary aneurysms with KD is not well understood. Medical records of 48 KD patients (33 males and 15 females) with giant coronary aneurysms (maximum coronary artery internal diameter >8 mm) aged 20 years or over were retrospectively reviewed. The age of the patients at the diagnosis of KD ranged from 0.3 to 12.8 years (median 2.9 years) and the age of the patients in this review ranged from 20.0 to 33.1 years (median 25.2 years). During the follow-up period, the maximum coronary artery internal diameter ranged from 8.2 to 30.0 mm (median 10 mm). Giant coronary aneurysms progressed to coronary artery stenosis and/or complete occlusion in 34 (74%) of 46 patients. Coronary artery bypass graft surgery was performed in 9 (19%) of 48 patients. Myocardial infarction occurred in 14 (31%) of 45 patients. Other complications or problems occurred in 5 patients with angina pectoris, low left ventricular ejection fraction, ventricular tachycardia, hemorrhagic cerebral infarction, or thyroid carcinoma respectively. In the patients followed up, 4 dropped out. In addition, 1 patient succeeded in pregnancy and delivered a baby. Close attention should be paid to ventricular tachycardia in adult KD patients with giant coronary aneurysms, especially for those who have low left ventricular ejection fraction. To reduce the number of dropped out patients, it is important that the patients should be referred to a new doctor when they change their place of residence.

Pathogenesis and management of Kawasaki disease

Kawasaki disease (KD) is an acute systemic inflammatory illness of young children that can result in coronary artery aneurysms, myocardial infarction and sudden death in previously healthy children. Clinical and epidemiologic features support an infectious cause, but the etiology remains unknown four decades after KD was first identified by Tomisaku Kawasaki. Finding the cause of KD is a pediatric research priority. We review the unique immunopathology of KD and describe the current treatment. New research has led to identification of viral-like cytoplasmic inclusion bodies in acute KD tissues; this finding could lead to identification of the elusive etiologic agent and result in significant advances in KD diagnosis and treatment. Current management of acute KD is based upon prospective, multicenter treatment trials of intravenous immunoglobulin (IVIG) with high-dose aspirin. Optimal therapy is 2 g/kg IVIG with high-dose aspirin as soon as possible after diagnosis during the acute febrile phase of illness, followed by low-dose aspirin until follow-up echocardiograms indicate a lack of coronary abnormalities. The addition of one dose of intravenous pulse steroid has not been shown to be beneficial. For the 10–15% of patients with refractory KD, few controlled data are available. Options include repeat IVIG (our preference), a 3-day course of intravenous pulse methylprednisolone, or infliximab (Remicade®). Patients with mild-to-moderate coronary abnormalities should receive an antiplatelet agent such as low-dose aspirin (3–5 mg/kg/day) or clopidogrel (1 mg/kg/day up to 75 mg), and those with giant (∼8 mm diameter) or multiple coronary aneurysms should receive an antiplatelet agent with an anticoagulant such as warfarin or low-molecular-weight heparin. Acute coronary obstruction requires acute thrombolytic therapy with a surgical or percutaneous interventional procedure.

Giant post-traumatic coronary aneurysm as an infrequent cause of inferior ST elevation myocardial infarction

A 64-year-old woman with diabetes as the only known cardiovascular risk factor was admitted to the emergency department with ischaemic chest pain and inferior ST elevation on the electrocardiogram and complete atrioventricular block. Primary coronary angiography was immediately performed, 2.5 h after the beginning of the pain, showing a calcified round mass ( Panel A ) together with total occlusion of the proximal right …

Giant Right Coronary Aneurysm and Syncope

Giant Right Coronary Aneurysm and Syncope

Six principal symptoms and coronary artery sequelae in Kawasaki disease

The relationship between coronary artery sequelae due to Kawasaki disease and the six principal symptoms associated with the latter are still unknown. Among the 16,952 patients reported in the 17th nationwide survey on Kawasaki disease, 14,068 were found to have definite and typical cases with five or six principal symptoms. The proportions of coronary artery aneurysms including dilatation of the artery were compared between the patient group with six symptoms and that with five. Percentages for those with all six symptoms (7223) were 4.7% for coronary dilatations (i.e. small aneurysms), 1.7% for coronary aneurysms (i.e. middle-sized aneurysms), and 0.42% for giant coronary aneurysms (i.e. large aneurysms) 1 month after onset, whereas for those with five symptoms (6,845), the corresponding percentages were 4.1%, 1.4%, and 0.22%, respectively. For overall observation, the odds ratios were 1.44, 1.28, and 1.70 for the three types of aneurysm, respectively. For male patients, all the odds ratios were >1.0, and some were significant. In contrast, odds ratios <1.0 were observed among those with six symptoms in some subgroups among female patients despite the lack of significance. Because odds ratios were elevated - especially for male patients with Kawasaki disease - with all six principal symptoms, patients with the six symptoms are more problematic for coronary sequelae among the definite cases, although there was not a specific symptom for the sequelae.

Progressive Left Coronary Stenosis After Rotablator Ablation Appreciated by Doppler Echocardiography

We describe a patient with a history of Kawasaki disease who showed coronary jet flow in the giant aneurysm on color-Doppler echocardiography caused by progressive coronary stenosis. A 6-year-old boy who had bilateral giant coronary aneurysms (14.9 mm in diameter) of the left main trunk (Fig. 1a) as sequelae of Kawasaki disease presented to us with anginal chest pain. Two years ago he had undergone coronary artery bypass graft surgery using the bilateral internal mammary artery grafted to the left anterior descending and left circumflex artery. Coronary angiography this time showed obstruction of both right and left internal mammary arteries. A color-Doppler echocardiography showed an aliased jet originating from the coronary ostium and flowing into the left giant coronary aneurysm with an early peak diastolic flow of 1.6 m/s (normal: 0.18– 0.3) with acute deceleration, being much faster than peak systolic flow velocity of 1.0 m/s (Fig. 1b). Because it was difficult to repeat the bypass graft surgery, he underwent successful rotablator ablation of the left coronary stenosis using 1.5-mm burr and the left coronary stenosis was significantly alleviated with a decreased peak diastolic flow velocity of 1.0 m/s (Fig. 1c) and temporary resolution of anginal chest pain. However, 2 months later, he complained of refractory chest pain again, with accelerated diastolic left coronary flow velocity of 2.0 m/s and underwent another attempt at rotablator ablation, but, unfortunately, he fell into shock during the procedure and could not be saved with multiple resuscitations. This case illustrates that color-Doppler echocardiography is a useful tool for evaluating coronary artery stenosis with a giant coronary aneurysm caused by Kawasaki disease and also emphasizes the difficulty of treating these small children with coronary stenosis [1, 2].

Giant coronary aneurysm in adult Kawasaki disease: angiographic, 64-slice coronary MDCT and cardiac MRI appearances

Kawasaki disease is a small-to-medium-vessel vasculitis that preferentially affects infants and young children. This condition is rare in adults, and therefore the diagnosis can easily be missed in a patient presenting to a primary care clinic. We report an unusual case of a patient who presented with ventricular fibrillation on a background of adult Kawasaki disease. To identify the advantages of using coronary multidetector computed tomography (MDCT) and cardiac magnetic resonance imaging (MRI) in diagnosing adult Kawasaki disease. We studied a 52-year-old patient with Kawasaki disease using coronary angiography, cardiac MDCT and MRI. Invasive coronary angiography demonstrated an occluded right coronary artery (RCA) and appearances suspicious for a calcified giant RCA aneurysm. The full extent of the aneurismal RCA was depicted with MDCT. Cardiac MRI revealed a chronic inferior segment myocardial infarction representing an arrhythmia substrate. Our case highlights the increasing utility of contrast-enhanced cardiac MRI and MDCT in the diagnosis of this rare condition in adults.

P-282 Mid- to Long-Term Follow-Up of 44 Children with Kawasaki Disease Complicated with Medium Or Giant Coronary Aneurysm

P-282 Mid- to Long-Term Follow-Up of 44 Children with Kawasaki Disease Complicated with Medium Or Giant Coronary Aneurysm

Concomitant giant coronary artery and coronary sinus aneurysms

consecutive reoperations were needed because of recurrent prosthetic dehiscence and mitral valve dysfunction. It was considered that a mitral homograft could be a valid alternative, but functional results were disappointing because of limited mobility and deficient coaptation of the anterior leaflet. Implantation of an annuloplasty ring and repair of the valve commissure were unsuccessful, and instead of explanting the homograft it was decided to remove the annuloplasty ring and insert a bioprosthesis in a transvalvular position, with the homograft acting as a native valve. At 14 months of follow-up, the patient is free of symptoms, and functional results are satisfactory. Hypersensitivity to the suture ring of the valve prosthesis is an appealing explanation for our findings. In this respect, the interposed homograft avoided the potential risk of intolerance to the suture ring and also prevented direct contact between the infected tissue and the prosthesis.

Multicenter and Retrospective Case Study of Warfarin and Aspirin Combination Therapy in Patients With Giant Coronary Aneurysms Caused by Kawasaki Disease

To determine the prognosis of patients with giant coronary aneurysms (GA) caused by Kawasaki disease (KD) treated with combined oral warfarin and aspirin. A multicenter follow-up study of 83 patients (65 males, 18 females) with GA who had been treated for > or =3 months with warfarin. Most patients were placed on the combination therapy as soon as the GA was detected and remained on it for 6.0 +/-5.3 years, giving a total of 482 patient-years. Target international normalized ratio of prothrombin time ranged from 1.5 to > or =2.5. During this observational period, 5 patients suffered from 8 episodes of acute myocardial infarction and 1 died. Coronary thrombus formation enforced 6 courses of intracoronary thrombolysis in 3 patients (1-4 times). Consequently, freedom of cardiac events was 92.5% at 1 year and 91% at 10 years and the linearized cardiac event rate was 2.9% patient-year. Hemorrhagic complications occurred on 8 occasions (1 subdural hematoma) in 5 patients, giving 1.7% patient-year. The combination of warfarin and aspirin has an acceptably high cardiac-event-free survival in patients with GA caused by KD, though it has a certain risk of hemorrhagic complications.