Atypical coronary artery aneurysms due to Kawasaki disease in Noonan syndrome with a novel PTPN11 mutation

2018 ◽  
Vol 29 (2) ◽  
pp. 228-230
Author(s):  
Shiori Takai ◽  
Kei Takasawa ◽  
Shozaburo Doi
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Acute Phase ◽  
Therapeutic Intervention ◽  
Noonan Syndrome ◽  
Coronary Artery Aneurysms ◽  
Ptpn11 Mutation

AbstractWe report a 3-year-old boy with giant and atypical coronary artery aneurysms in the acute phase of Kawasaki disease, despite appropriate therapeutic intervention, in Noonan syndrome with a novel heterozygous PTPN11 mutation, c. 907 G>A (p.Asp303Asn). We hypothesised that this PTPN11 mutation might affect both hyperinflammation caused by Kawasaki disease and vascular fragility in the coronary artery, resulting in coronary artery aneurysms.

Abstract O.71: Thrombosis and Thromboprophylaxis for Patients with Giant Coronary Artery Aneurysms after Kawasaki Disease: A Study from the North American Kawasaki Disease Registry

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Cedric Manlhiot ◽  
William T Mahle ◽  
Kevin D Hill ◽  
Jennifer S Li ◽  
Dawn Tucker ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Acute Phase ◽  
North American ◽  
Event Rate ◽  
Disease Registry ◽  
Coronary Artery Aneurysms ◽  
The North ◽  
Thrombotic Complications

Background: Children with giant coronary artery aneurysms (CAA) after Kawasaki disease (KD) are at substantial risk of thrombosis. There are currently no evidence-based guidelines for optimal thromboprophylactic therapy in these children. Methods: The North American Kawasaki Disease Registry was queried to identify all patients with giant CAA (maximum coronary artery z-score >10) and their antithrombotic therapy. Freedom from thrombosis was modelled using the Kaplan-Meier method; thrombotic complication rate was calculated per patient-year/month of follow-up. Results: n=202 patients with giant CAA were included, of whom 28 (14%) experienced either coronary artery thrombosis with or without myocardial infarction. Freedom from thrombotic complications was 92%, 85% and 79% at 3 months, 5 and 10 years after diagnosis, respectively. Non-pharmacological factors associated with increased risk of thrombotic complications included higher maximum coronary artery z-scores (HR: 1.7/+10 SD, p<0.001), higher number of coronary artery branches with giant CAA (HR: 2.6/branch, p<0.001), higher number of discrete CAA (HR: 1.4/aneurysm, p=0.001) and presence of complex CAA (involving the bifurcation or non-discrete; HR: 3.0, p=0.05). A total of 982 patient-years of follow-up were available for analysis (11% low molecular weight heparin (LMWH), 32% warfarin, 57% antiplatelet alone). All patients were maintained on ASA, with 47 patients (23%) also receiving clopidogrel. Patients while on LMWH had the highest event rate, at 1 event per 13 patient-years, compared to 1 per 39 on warfarin and 1 per 33 on no anticoagulant. However, LMWH was predominantly prescribed immediately after the acute phase, which is also the highest risk phase for thrombosis. When limiting analysis to events within 3 months of the acute phase, patients on LMWH had the lowest event rate at 1 per 46 patient-months, compared to 1 per 27 on warfarin and 1 per 33 on no anticoagulant (p=NS). Conclusions: Current thromboprophylaxis strategies in patients with giant CAA have suboptimal efficacy, and residual thrombosis risk persists. New anticoagulants/antiplatelet agents should be assessed in this population to determine if they provide better, safer and more tolerable thromboprophylaxis.

Rare case of Kawasaki disease with cardiac tamponade and giant coronary artery aneurysms

2021 ◽  
pp. 1-2
Author(s):  
Andrea Vergara ◽  
Emanuele Monda ◽  
Cinzia Mautone ◽  
Francesca Renon ◽  
Antonio Di Masi ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Cardiac Tamponade ◽  
Acute Phase ◽  
Circumflex Coronary Artery ◽  
Coronary Artery Aneurysms ◽  
Fever Onset ◽  
Cardiovascular Involvement ◽  
Peculiar Case ◽  
Proximal Right Coronary Artery

Abstract Kawasaki disease is an acute systemic vascular disease, generally self-limited and typically affecting children <5 years old, which leads to coronary artery aneurysms in about 25% of untreated cases. Cardiovascular involvement is characterised by transient pancarditis, in acute phase, while coronary illness, ranging from mild dilation to giant CAAs occurs late, rarely before the 10th day since fever onset. Here, we describe a peculiar case of KD, which occurred in a 4-month-old infant and presented with exudate cardiac tamponade and early giant aneurism of both the proximal right coronary artery) and the left circumflex coronary artery, in acute phase of the disease.

scholarly journals 0328: Evaluation of coronary artery wall echogenicity in Kawasaki disease acute phase

2015 ◽  
Vol 7 (1) ◽  
pp. 102
Author(s):  
Fanny Riou ◽  
Aurélie Chalard ◽  
Aurélie Chausset ◽  
Jean René Lusson ◽  
Etienne Merlin ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Acute Phase ◽  
Artery Wall ◽  
Coronary Artery Wall

scholarly journals Evaluation of coronary artery wall echogenicity in Kawasaki disease acute phase

2014 ◽  
Vol 107 (8-9) ◽  
pp. 485
Author(s):  
F. Riou ◽  
A. Chalard ◽  
A. Chausset ◽  
J.R. Lusson ◽  
E. Merlin ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Acute Phase ◽  
Artery Wall ◽  
Coronary Artery Wall

Abstract 221: Use of Statins in Patients with Coronary Artery Aneurysms: A Pilot Study from the North American Kawasaki Disease Registry

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Michael Khoury ◽  
Michael A Portman ◽  
Cedric Manlhiot ◽  
Anne Fournier ◽  
Rejane F Dillenburg ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Pilot Study ◽  
Kawasaki Disease ◽  
Linear Regression ◽  
North American ◽  
Z Score ◽  
Disease Registry ◽  
Coronary Artery Aneurysms ◽  
The North ◽  
Significant Difference

Background: Statins have been considered as therapy for children with coronary artery aneurysms (CAA) after Kawasaki disease (KD), due to potential beneficial pleiotropic effects which might influence chronic vascular processes and inflammation. Methods: The North American Kawasaki Disease Registry was queried to identify patients who have received statins in the first 6 months following the convalescent phase of KD. Each identified patient was matched by age, gender and CAA z score to 3 patients who were statin-naïve (controls). Linear regression models adjusted for repeated measures and maximum coronary involvement were used to determine an association of statin use with longitudinal changes in coronary artery diameter z-score. Kaplan-Meier analysis was used to compare freedom from angiographically-confirmed stenosis or interventions. Results: Of 29 patients with KD and CAA (maximum coronary artery z-score >10) who received statins at any time (of n=621, 5%), 10 (9 males) patients were started within 6 months of the acute KD episode. The mean age at KD was 6.3±3.4 years (5.4±3.5 for controls, p=0.57). Mean maximum CAA z-score was 36±14 (vs. 29±16, p=0.20); 90% of statin patients and 87% of matched controls had CAAs in 3 or more branches. Linear regression analysis of 442 serial echocardiograms showed that maximum CAA z-score decreased by -1.5 (95%CI: -2.7; -0.4) SD/year (p=0.008) for control patients compared to -2.9 (95%CI: -4.4; -1.4) SD/year (p<0.001) for statin treated patients. The difference between the rate of change of CAA z-score for statin vs. control patients did not reach statistical significance (controls vs. statins: +1.4 SD/year, 95%CI: -0.6; +3.4, p=0.18). n=7 patients (3 on statin, 4 controls) developed stenosis or had revascularization, with no significant difference between groups (HR for statin group: 2.2 (0.4-11.4), p=0.41). Conclusions: This underpowered pilot study suggests that equipoise likely exists with regards to statin therapy in children with KD and CAA, and that a formal registry-nested trial might be considered.

scholarly journals Genetic variants of glutamate receptor gene family in Taiwanese Kawasaki disease children with coronary artery aneurysms

2014 ◽  
Vol 4 (1) ◽  
pp. 67 ◽  
Author(s):  
Ying-Ju Lin ◽  
Jeng-Sheng Chang ◽  
Xiang Liu ◽  
Hsinyi Tsang ◽  
Ting-Hsu Lin ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Gene Family ◽  
Glutamate Receptor ◽  
Genetic Variants ◽  
Receptor Gene ◽  
Coronary Artery Aneurysms ◽  
Receptor Gene Family

Aerobic exercise function of patients with persistent coronary artery aneurysms secondary to Kawasaki disease

1996 ◽  
Vol 17 (4) ◽  
pp. 226-230 ◽  
Author(s):  
J. Rhodes ◽  
Z. M. Hijazi ◽  
G. R. Marx ◽  
D. R. Fulton
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Aerobic Exercise ◽  
Coronary Artery Aneurysms
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