Abstract O.72: The Spectrum Of Cardiovascular Lesions Requiring Intervention In Young Adults After Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
John B Gordon ◽  
Lori B Daniels ◽  
Andrew M Kahn ◽  
Matthew Vejar ◽  
Chisato Shimizu ◽  
...  
Keyword(s):  
Young Adults ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Vascular Inflammation ◽  
Cardiovascular Complications ◽  
Coronary Artery Aneurysms ◽  
History Of ◽  
Proximal Coronary Artery ◽  
Cardiothoracic Surgeon ◽  
Cardiovascular Interventions

Background: Coronary artery aneurysms resulting from vascular inflammation associated with Kawasaki disease (KD) in childhood may remain clinically silent until adulthood. Young adults presenting with large aneurysms, unstable angina, or myocardial infarction (MI) following KD in childhood present unique challenges to the interventional cardiologist and cardiothoracic surgeon. We present a range of management issues raised by this patient population. Methods: Participants who underwent cardiovascular interventions were identified from an observational cohort of 154 individuals with a history of KD enrolled in the San Diego Adult KD Collaborative. Of these 154 participants, 63 (40.9%) were originally diagnosed with KD and followed by one of the co-authors (JCB) and were designated as Cohort 1. The remaining 91 participants (Cohort 2) were referred by their physician or self-referred for participation in the study. Results: Of the 154 participants, 20 (12.9%; 2 from Cohort 1 and 18 from Cohort 2) underwent cardiovascular interventions: 9 had percutaneous interventions and 11 had surgery. Twelve participants had been diagnosed with KD in childhood, 7 had a history of a KD-compatible illness in childhood, and 1 had proximal coronary artery aneurysms compatible with KD. Fourteen participants were asymptomatic until experiencing a major cardiovascular event: 8 presented with an acute MI, 3 presented with angina, 1 presented with end-stage congestive heart failure requiring cardiac transplantation, and 2 presented with extremity claudication. Conclusions: Cardiovascular complications in individuals with a history of KD illustrate the following points: 1) Even small to moderate-sized aneurysms that “normalize” by echocardiography in childhood can lead to stenosis and thrombosis decades after the acute illness; 2) Coronary interventions without intravascular ultrasound may result in underestimation of vessel lumen diameter; 3) Failure to assess the extent of calcification may lead to suboptimal procedural outcomes, and 4) Patients with symptomatic peripheral aneurysms may benefit from endarterectomy or resection. Interventional cardiologists should be aware of the complications encountered in this growing population of young adults.

scholarly journals Management of sequelae of Kawasaki disease in adults

2018 ◽  
Vol 2017 (3) ◽  
Author(s):  
John B Gordon ◽  
Jane C Burns
Keyword(s):  
Young Adults ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Management Strategies ◽  
Collaborative Study ◽  
Calcium Score ◽  
Calcium Deposition ◽  
Coronary Artery Aneurysms ◽  
Study Results ◽  
Growing Population

Background: A growing population of young adults is presenting to cardiologists with late manifestations of Kawasaki disease (KD) that include cardiomyopathy, ischemia, and infarction. The management of these conditions differs in important ways from atherosclerotic heart disease, and yet there is little awareness in the adult cardiology community regarding the special challenges posed by the cardiovascular sequelae of KD. Methods: Observations were made on a population of 140 adult KD patients enrolled in the San Diego Adult KD Collaborative Study. Results: Coronary artery aneurysms resulting from KD in childhood are associated with a high risk of thrombosis and stenosis at the inlet or outlet of the aneurysm. These aneurysms are often highly calcified and may contain a large thrombus burden that may obscure the true size of the aneurysm. Pitfalls in the management of these patients stem largely from failure to recognize the nature of the lesions, which leads to attempts to dilate highly calcified stenotic segments and undersizing of stents. Intravascular ultrasound is helpful in appreciating the true dimensions of the aneurysm, which may be filled with thrombus. Thrombolysis and use of anti-platelet agents followed by systemic anti-coagulation are appropriate management strategies for patients presenting with acute infarction. Bypass grafting with the internal thoracic arteries can be a successful strategy, but care must be taken to avoid competitive flow through the native vessel leading to graft failure. In contrast to the individuals who developed coronary artery aneurysms, young adults who had documented normal echocardiograms associated with their acute KD in childhood and who have no evidence of calcium deposition in the arterial wall as assessed by computed tomography (CT) calcium score appear to have no increased cardiovascular risk in the medium term. Long-term outcomes for adults post-KD in childhood are still being defined. Conclusions: KD poses special management challenges for the adult cardiologist who must recognize the unique features of the cardiovascular lesions in this growing population of patients. 

Onset of Kawasaki disease immediately after birth

2018 ◽  
Vol 7 (2) ◽  
Author(s):  
Cristina Medeiros Ribeiro de Magalhães ◽  
Riccardo Pratesi ◽  
Lenora Gandolfi ◽  
Rosa Harume Uenishi ◽  
Claudia B. Pratesi
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
Rare Event ◽  
Cardiovascular Complications ◽  
Postnatal Period ◽  
Older Children ◽  
Coronary Artery Aneurysms ◽  
Common Causes ◽  
Therapeutic Measures

Abstract Kawasaki disease (KD) is one of the most common causes of childhood systemic vasculitis, with the potential to cause significant cardiovascular complications, especially if undiagnosed. An investigation of coronary artery abnormalities with the application of appropriate therapeutic measures can improve the prognosis in these infants, decreasing the risk of coronary artery aneurysms. Neonatal KD is a rare event, often characterized only by few of the clinical features observed in older children. In this case report, we describe a newborn male who presented an incomplete clinical presentation of KD, where symptoms of the disease erupted immediately after birth. The objective of this report is to draw the attention of pediatricians and neonatologists to the possible presence of KD in an asymptomatic newborn during the immediate postnatal period, even in the absence of the classic features of KD, such as fever.

scholarly journals Cyclophosphamide use in treatment of refractory Kawasaki disease with coronary artery aneurysms

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Olha Halyabar ◽  
Kevin G. Friedman ◽  
Robert P. Sundel ◽  
Annette L. Baker ◽  
Margaret H. Chang ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Retrospective Chart Review ◽  
Severe Neutropenia ◽  
Rapid Progression ◽  
Coronary Artery Aneurysms ◽  
Multicenter Studies ◽  
Primary Indication ◽  
History Of ◽  
Anti Inflammatory

Abstract Background Despite timely administration of IVIG, some patients with Kawasaki disease (KD) develop rapidly progressive or giant coronary artery aneurysms (CAA). Case presentation We describe our experience using cyclophosphamide (CYC) for the treatment of such cases as well as a review of the literature on the use of CYC in KD. Through a retrospective chart review of our KD population, we identified ten children treated for KD with intravenous CYC (10 mg/kg/dose) for one or two doses. Seven patients were male, the median age was 2.0 years (range 4 months − 5 years). All patients received initial IVIG between day 4–10 of illness. Other anti-inflammatory treatments administered before CYC included second IVIG (n = 9), corticosteroids (n = 10), infliximab (n = 4), cyclosporine (n = 2), and anakinra (n = 1). Median illness day at administration of the first CYC dose was 22.5 days (range:10–36 days). The primary indication for treatment with CYC for all patients was large or giant CAA and/or rapid progression of CAA. Three patients received a second dose of CYC (10 mg/kg) for progressively enlarging CAA. CAA did not progress after final CYC treatment. One patient with a history of neutropenia in infancy developed severe neutropenia 9 days after treatment with CYC, which recovered without intervention or complications. No patient developed infections or other serious toxicity from CYC. Conclusion In KD patients with severe and progressive enlargement of CAA despite anti-inflammatory therapy, CYC seemed to arrest further dilation and was well-tolerated. Future multicenter studies are needed to confirm our findings in this subgroup of KD patients.

scholarly journals Cyclophosphamide Use in Treatment of Refractory Kawasaki Disease With Coronary Artery Aneurysms

2020 ◽  
Author(s):  
Olha Halyabar ◽  
Kevin G Friedman ◽  
Robert P Sundel ◽  
Annette L Baker ◽  
Margaret H Chang ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Retrospective Chart Review ◽  
Severe Neutropenia ◽  
Rapid Progression ◽  
Coronary Artery Aneurysms ◽  
Multicenter Studies ◽  
Primary Indication ◽  
History Of ◽  
Anti Inflammatory

Abstract Background. Despite timely administration of IVIG, some patients with Kawasaki disease (KD) develop rapidly progressive or giant coronary artery aneurysms (CAA). Case presentation. We describe our experience using cyclophosphamide (CYC) for the treatment of such cases as well as a review of the literature on the use of CYC in KD. Through a retrospective chart review of our KD population, we identified ten children treated for KD with intravenous CYC (10 mg/kg/dose) for one or two doses. Seven patients were male, the median age was 2.0 years (range 4 months -5 years). All patients received initial IVIG between day 4-10 of illness. Other anti-inflammatory treatments administered before CYC included second IVIG (n=9), corticosteroids (n=10), infliximab (n=4), cyclosporine (n=2), and anakinra (n=1). Median illness day at administration of the first CYC dose was 22.5 days (range:10-36 days). The primary indication for treatment with CYC for all patients was large or giant CAA and/or rapid progression of CAA. Three patients received a second dose of CYC (10mg/kg) for progressively enlarging CAA. CAA did not progress after final CYC treatment. One patient with a history of neutropenia in infancy developed severe neutropenia 9 days after treatment with CYC, which recovered without intervention or complications. No patient developed infections or other serious toxicity from CYC. Conclusion. In KD patients with severe and progressive enlargement of CAA despite anti-inflammatory therapy, CYC seemed to arrest further dilation and was well-tolerated. Future multicenter studies are needed to confirm our findings in this subgroup of KD patients.

scholarly journals Kawasaki Disease- Management Strategies Given Symptoms Overlap to COVID-19: A Review

2021 ◽  
Vol 59 (236) ◽  
Author(s):  
Linna Wang ◽  
Sheng Zhang ◽  
Ji Ma ◽  
Jing Ni ◽  
Juyan Wang ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Inflammatory Disease ◽  
Early Treatment ◽  
Respiratory Infections ◽  
Management Strategies ◽  
Artery Aneurysm ◽  
Hemodynamic Instability ◽  
Cardiovascular Complications ◽  
Coronary Artery Aneurysms

Kawasaki disease is an acute, self-limiting vasculitis in children. Early treatment is necessary to prevent cardiovascular complications. The acute phase of Kawasaki disease may present with hemodynamic instability. An association between viral respiratory infections and Kawasaki disease has been reported. Studies have shown that Kawasaki and Kawasaki-like disease may be associated with and have symptoms overlapping COVID-19. Children with COVID-19 may present as Kawasaki-like disease with pediatric inflammatory multisystem syndrome, or macrophage activation syndrome. Clinicians need to be aware of the early diagnosis and management of Kawasaki disease to prevent the development of coronary artery aneurysms. The symptoms overlap of multisystem inflammatory disease seen in COVID-19 adds to the difficulties in timely diagnosis and treatment. Children with Kawasaki disease require regular follow-up plans for coronary artery aneurysms. This adds to the difficulties during the changed environment of COVID-19 for control and prevention. Missed diagnosis and early treatment of Kawasaki disease with immunoglobulin and aspirin results in the development of coronary artery aneurysm in up to 25% of cases, with grave consequences. Here, we briefly review the management of typical and atypical Kawasaki disease which has symptoms overlapping with the multisystem inflammatory disease as seen in COVID-19.

Abstract 187: Endothelial Cell Function in Young Adults with History of Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Lori B Daniels ◽  
Matthew Vejar ◽  
Andrew M Kahn ◽  
John B Gordon ◽  
Jane C Burns
Keyword(s):  
Young Adults ◽  
Endothelial Cell ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Cell Function ◽  
Reactive Hyperemia ◽  
Single Layer ◽  
Artery Occlusion ◽  
Endothelial Cell Function ◽  
History Of

Background: The endothelium is a single layer of epithelial cells that lines the blood vessels and is a major component in maintaining vascular homeostasis. Impaired endothelial cell (EC) function can contribute to cardiovascular disease states, but whether there is an association between endothelial dysfunction and the presence of coronary artery aneurysms after Kawasaki disease (KD) is unknown. Methods: We enrolled 110 teens and young adults, ages 16 to 30 years, with a history of KD (61% male), as well as 30 healthy controls (HC, 53% male). EC function (reactive hyperemic response after a 5 minutes brachial artery occlusion) was measured using the EndoPAT 2000. Reactive hyperemia index (RHI) and PAT ratios were determined. KD subjects were categorized based on their worst coronary artery status (normal, transiently dilated/resolved aneurysm, aneurysm). Results: The mean age of KD subjects and HC at the time of EndoPAT was 25 ± 9 and 26 ± 7 years, respectively (p=0.42). Mean time since onset of KD was 23 ± 19 years. Subjects with a history of transiently dilated/resolved aneurysms had a higher mean lnRHI compared to other KD subjects (0.91 vs 0.74, p=0.01) and HC (0.72, p=0.02). When PAT ratio was evaluated as a function of time after occlusion release, those with transiently dilated/resolved aneurysms had a trend toward a more favorable profile while other groups appeared similar (Figure). Conclusion: Most young adults with a history of KD have normal EC function. Individuals with a history of transiently dilated coronary arteries had a paradoxical trend toward better EC function, which may indicate healthier vasculature. The significance of this finding merits further study.

Giant right coronary artery aneurysm

2020 ◽  
Vol 28 ◽  
pp. 1-3
Author(s):  
Alexandre Bonfim ◽  
Ronald Souza ◽  
Sérgio Beraldo ◽  
Frederico Nunes ◽  
Daniel Beraldo
Keyword(s):  
Coronary Artery ◽  
Right Coronary Artery ◽  
Coronary Artery Aneurysm ◽  
Clinical Course ◽  
Artery Aneurysm ◽  
Giant Aneurysm ◽  
Coronary Artery Aneurysms ◽  
History Of ◽  
Artery Disease ◽  
The Right

Right coronary artery aneurysms are rare and may result from severe coronary disease, with few cases described in the literature. Mortality is high, and therapy is still controversial. We report the case of a 72-year-old woman with arterial hypertension, and a family history of coronary artery disease, who evolved for 2 months with episodes of palpitations and dyspnea on moderate exertion. During the evaluation, a giant aneurysm was found in the proximal third of the right coronary artery. The patient underwent surgical treatment with grafting of the radial artery to the right coronary artery and ligation of the aneurysmal sac, with good clinical course.

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