Coronary Artery Changes in Patients with Multisystem Inflammatory Syndrome in Children: Los Angeles Experience.

We compared cardiac findings in patients with multisystem inflammatory syndrome in children and Kawasaki disease in the first 6 months of the 2020 coronavirus disease pandemic to patients with Kawasaki disease during 2016-2019. We saw a high rate of coronary aneurysms in 2020, with a similar rate of coronary involvement but greater volume and incidence of cardiac dysfunction compared with previous years.

Macrophage activation syndrome in children with Kawasaki disease: an experience from a tertiary care hospital in northwest India.

OBJECTIVES: To carry out a review of clinical characteristics, laboratory profiles, management and outcomes of patients with Kawasaki disease (KD) and macrophage activation syndrome (MAS). METHODS: Medical records of patients treated for KD and MAS between January 1994 and December 2019 were reviewed. Patient demographics, clinical signs, laboratory values, coronary artery abnormalities, treatments and outcomes of patients with KD and MAS were recorded. We also performed a review published studies on the subject. RESULTS: Of the 950 cases with KD, 12 (1.3%; 10 boys, 2 girls) were diagnosed with MAS. The median age at diagnosis was 4 years (range 9 months-7.5 years). The median interval between onset of fever and diagnosis of KD was 11 days (range 6-30). Thrombocytopenia was seen in 11 patients. The median pro-brain natriuretic peptide value was 2101 pg/ml (range 164-75 911). Coronary artery abnormalities were seen in 5 (41.7%) patients; 2 had dilatation of the left main coronary artery (LMCA), 1 had dilatation of both the LMCA and right coronary artery (RCA), 1 had dilatation of the RCA and 1 had bright coronary arteries. All patients received IVIG as first-line therapy for KD. MAS was treated with i.v. methylprednisolone pulses followed by tapering doses of oral prednisolone. Additional therapy included i.v. infliximab (n = 4), second-dose IVIG (n = 1) and oral ciclosporin (n = 1). CONCLUSION: MAS is an unusual and underrecognized complication of KD. In our cohort of 950 patients with KD, 1.3% had developed MAS. KD with MAS is associated with an increased propensity towards development of coronary artery abnormalities.

Outcomes of Kawasaki disease with giant coronary aneurysms: a single-centre study in southwest China.

BACKGROUND: Giant coronary aneurysms are the most severe complications of Kawasaki disease. There are few reports of outcomes from China. Most previous studies were based only on absolute aneurysmal dimensions. The aim of the present study was to catalog the outcomes of Kawasaki disease with giant coronary aneurysms in southwest China based on absolute dimensions and the z-score adjusted for body surface area. METHODS AND RESULTS: All patients diagnosed with giant coronary aneurysms (z-score ≥ 10 or absolute dimension ≥ 8 mm) between December, 2002 and December, 2018 were included. We retrospectively analysed patient characteristics and clinical data from 38 patients with giant coronary aneurysms. Over a median follow-up period of 30.5 months (range from 1.7 months to 22.3 years), including patients in chronic phase who had been diagnosed prior to 2002, eight patients had myocardial infarction, including two deaths and one patient with coronary artery bypass grafting. The 1-, 2-, and 5-year event-free rates were 0.63, 0.63, and 0.53 for thrombosis, respectively, and 0.86, 0.81, and 0.81 for major adverse cardiac events, respectively. The 1-, 2-, and 5-year regression-free rates were 0.94, 0.85, and 0.67, respectively. A total of 73.7% of patients remained active. CONCLUSION: In the early stages of Kawasaki disease, patients with giant coronary aneurysms often experience major cardiovascular events; however, they are also likely to have normalisation of the coronary internal luminal diameter. With long-term anticoagulation, close cardiologic monitoring, and prompt thrombolytic therapy, most patients can achieve disease-free periods.

Fifteen-minute consultation: Kawasaki disease: how to distinguish from other febrile illnesses: tricks and tips.

Kawasaki disease (KD) is challenging to diagnose because there is no specific laboratory test and the presentation is often similar to common childhood infections. We highlight some of those KD diagnostic challenges. KD, a self-limiting vasculitis, can cause coronary artery aneurysms. The aim is to optimise management during the acute febrile illness to try and prevent these because a giant coronary artery aneurysm is devastating enough without thinking that it might have been prevented. The conundrum for acute paediatricians is which clinical features best distinguish the febrile child with possible KD, needing intravenous immunoglobulin, from the many other children with febrile illnesses.

Utilization and outcomes of polytetrafluoroethylene covered stents in patients with coronary artery perforation and coronary artery aneurysm: Single center 15-year experience.

OBJECTIVES: Determine the outcomes of polytetrafluoroethylene (PTFE) covered stents for coronary artery perforation (CAP) and coronary artery aneurysm (CAA). BACKGROUND: PTFE covered stents have been used for treatment of potentially life-threatening CAP and CAA. The short and long-term outcomes of the PTFE covered stent for CAP and CAA have not been well studied. METHODS: We performed a retrospective study of PTFE covered stents that were placed in the patients from 2003 to 2017. Short term outcomes included in-hospital mortality, pericardial effusion, cardiac tamponade, and length of stay. Long-term outcomes included target lesion revascularization (TLR), in-stent restenosis (ISR), and long-term mortality. RESULTS: Fifty-three PTFE covered stents were placed in 32 patients of which there were 24 patients with a CAP with a mean age of 75 ± 8 years. Two patients died in-hospital, with no additional deaths at 30 days. The rate of ISR was 25%, with estimated rates of TLR of 2.6% (3 years) and 17.8% (5 years). The median survival was 55.6 months, with survival at 10 years estimated to be 30.9%. Eight patients received a PTFE covered stent for CAA with a mean age of 59 ± 15 years with no in-hospital or 30-day mortality. Median follow-up of 49 months showed no evidence of TLR. The all-cause mortality was 12% at 1 year and 38% at 3 years. CONCLUSIONS: PTFE covered stents is an effective option in patients with CAP and CAA. The long-term outcomes may be related to the pathology of the disease rather than the stent itself.

Prognostic significance, angiographic characteristics and impact of antithrombotic and anticoagulant therapy on outcomes in high versus low grade coronary artery ectasia: A long-term follow-up study.

OBJECTIVES: To assess the prognostic significance of high vs. low grade coronary artery ectasia (CAE) and the impact of antithrombotic or anticoagulant therapy on adverse cardiac outcomes. BACKGROUND: There is paucity of knowledge on the impact of angiographic characteristics in CAE or that of antithrombotic or anticoagulant therapy on outcomes. METHODS AND RESULTS: In this retrospective study, we reviewed angiograms and medical records of all cases of confirmed CAE (2001-2011). Extent of CAE was categorized using the Markis classification. Types 1 and 2 were categorized as high-grade and types 3 and 4 as low-grade CAE. Angiographic flow was recorded as normal or sluggish (

Assessment of the impact of isolated coronary artery ectasia on left ventricular functions with 3D speckle-tracking echocardiography.

BACKGROUND: Coronary artery ectasia (CAE) is an angiographic definition of coronary artery pathology in which the diameter of the ectatic segment measures more than 1.5 times the diameter of an adjacent healthy reference segment. No previous study has reported on the use of 3D-STE for assessing the left ventricular (LV) functions in patients with isolated CAE. As a result of this, we aimed to evaluate the effects of isolated CAE on LV functions using 3D-STE in the present study. METHODS: Ninety-one patients with isolated CAE and 90 controls who proved to have normal coronary angiograms were enrolled to the study. 3D-STE was performed and GLS, GCS, GAS, and GRS were obtained for every subject after coronary angiography. RESULTS: The mean age of the patients was 61.75 ± 10.02 years, and 71.8% were male. GLS, GCS, GAS, and GRS were significantly depressed in the isolated CAE group than in the control group (P < .001; P < .001; P = .001; and P = .001, respectively). ROC analyses were performed to find out the ideal strain cut off values to predict the presence of isolated CAE. A GLS value of >-16 has 92.1 % sensitivity, 88.5 % specificity; and a GCS value of >-20 has 86.7 % sensitivity, 89.2 % specificity to detect the presence of isolated CAE. CONCLUSION: Isolated CAE has a considerable negative effect on LV functions as evaluated by 3D-strain parameters, and 3D-STE could be an effective method to detect early stage myocardial impairment in patients with isolated CAE.

Clinical features and mid-term follow-up in infants younger than 3 months with Kawasaki disease in a Chinese population.

AIM: To explore the clinical features and mid-term follow-up of Kawasaki disease (KD) in infants younger than 3 months of age in a Chinese population. METHODS: We performed a retrospective analysis of clinical signs, laboratory data, echocardiography results and outcomes for patients with KD diagnosed at our hospital from January 2009 to December 2013. A total of 1150 children were diagnosed with KD, and 200 KD patients were enrolled in this study. Group 1 included 40 children younger than 3 months of age. We randomly selected a control group as Group 2 included 160 children older than 3 months of age who fulfilled diagnostic criteria for KD and maintained follow-up for more than 1 year. RESULTS: There was a significant difference in clinical manifestations between the two groups, except respiratory infection. Group 1 was more likely to have incomplete presentation (P < 0.001). There were no significant differences in laboratory data except for white blood cell counts between the two groups. Coronary artery abnormalities were significantly different between the two groups (P < 0.001). At a mean follow-up of 18 months (range 12-48 months), all patients with coronary artery abnormalities, except for giant coronary aneurysms, returned to normal in terms of diameter as assessed by echocardiography. CONCLUSIONS: Infants younger than 3 months of age with KD often present with incomplete criteria, and diagnosis may be delayed. In addition, there may be a higher risk of developing coronary artery abnormalities. All patients except those with giant coronary aneurysms recovered well without complications at mid-term follow-up.

Heart Rate, Responsiveness to Intravenous Immunoglobulin, and Coronary Artery Aneurysms in Kawasaki Disease.

OBJECTIVE: To evaluate whether heart rate (HR) was associated with intravenous immunoglobulin (IVIG) responsiveness or development of coronary artery lesions (CALs) in patients with Kawasaki disease. STUDY DESIGN: We conducted a retrospective cohort study using data from in patients with Kawasaki disease who were hospitalized in our institution from 2006 to 2016. The patients were divided into 5 groups according to the age- and temperature-adjusted HR z score (HRZage/temp) just before IVIG administration. The ORs of outcomes were estimated by using logistic regression models, with the middle group set as the reference. RESULTS: Of the 322 patients, a total of 98 patients (30%) were refractory to initial IVIG treatment. The patients whose HRZage/temp belonged to the lowest group were at the highest risk of being refractory to the initial IVIG treatment (OR 2.10 [95% CI 1.01-4.37]). Multivariable analyses showed the same trend, though this was not statistically significant. The patients with the highest HRZage/temp were most likely to develop CALs (OR 2.61 [95%CI 0.86-7.92]). CONCLUSIONS: In patients with Kawasaki disease , HRs has a different relationship with IVIG responsiveness and CALs. Low HRZage/temp might be associated with high risk of being refractory to the initial IVIG treatment, while the risk of developing CALs increased among those whose HRs were high. Further studies are necessary to investigate the mechanisms regarding HR and these outcomes in Kawasaki disease.

Coronary Artery Ectasia Predicts Future Cardiac Events in Patients With Acute Myocardial Infarction.

OBJECTIVE: Coronary artery ectasia (CAE) is an infrequently observed vascular phenotype characterized by abnormal vessel dilatation and disturbed coronary flow, which potentially promote thrombogenicity and inflammatory reactions. However, whether or not CAE influences cardiovascular outcomes remains unknown. APPROACH AND RESULTS: We investigated major adverse cardiac events (MACE; defined as cardiac death and nonfatal myocardial infarction [MI]) in 1698 patients with acute MI. The occurrence of MACE was compared in patients with and without CAE. CAE was identified in 3.0% of study subjects. During the 49-month observation period, CAE was associated with 3.25-, 2.71-, and 4.92-fold greater likelihoods of experiencing MACE (95% confidence interval [CI], 1.88-5.66; P<0.001), cardiac death (95% CI, 1.37-5.37; P=0.004), and nonfatal MI (95% CI, 2.20-11.0; P<0.001), respectively. These cardiac risks of CAE were consistently observed in a multivariate Cox proportional hazards model (MACE: hazard ratio, 4.94; 95% CI, 2.36-10.4; P<0.001) and in a propensity score-matched cohort (MACE: hazard ratio, 8.98; 95% CI, 1.14-71.0; P=0.03). Despite having a higher risk of CAE-related cardiac events, patients with CAE receiving anticoagulation therapy who achieved an optimal percent time in target therapeutic range, defined as ≥60%, did not experience the occurrence of MACE (P=0.03 versus patients with percent time in target therapeutic range <60% or without anticoagulation therapy). CONCLUSIONS: The presence of CAE predicted future cardiac events in patients with acute MI. Our findings suggest that acute MI patients with CAE are a high-risk subset who might benefit from a pharmacological approach to controlling the coagulation cascade.

Endovascular Embolization of Coronary Artery-Pulmonary Artery Fistulas with Double Coronary Aneurysms.

A 75-year-old woman with chest discomfort and a continuous murmur was admitted to our hospital. During noninvasive examination, computed tomography angiography showed a coronary artery-pulmonary artery fistula with double giant coronary aneurysms (one was 42 mm× 32 mm× 32 mm, and the other was 25 mm× 20 mm× 17 mm) arising from the proximal part of the left anterior descending (LAD) artery. Stress myocardial scintigraphy showed ischemia at the LAD area. Given her frailty, the heart team, including cardiac surgeons, judged that surgical treatment would be difficult. Thus, endovascular embolization for the abnormal vessels was selected. After coronary angiography, two coronary aneurysms were embolized by 53 coils, and the feeding artery was embolized by two coils and one Amplatzer Vascular Plug 4™. A small pulmonary artery fistula remained after the procedures; thus, additional embolization was performed 3 months after the index procedure. Thereafter, angiography showed no flow into the aneurysms, and her symptoms improved.Endovascular embolization might be an effective treatment to achieve aneurysm occlusion in patients at high risk for surgical treatment. Although the present case had double coronary aneurysms with a large feeder vessel, the combination procedure of coils and vascular plug was able to embolize this abnormal vessel.

Coexistence of a permanent form of a junctional reciprocating tachycardia and coronary sinus aneurysm: A case report.

A 58-year-old man with a long R-P' narrow QRS tachycardia underwent an electrophysiological study. The tachycardia was diagnosed as a permanent form of junctional reciprocating tachycardia (PJRT), and the earliest atrial activation site during tachycardia was coronary sinus (CS) ostium. Radiofrequency ablation at the site was initially not successful because the tip impedance and temperature were unstable. After changing of the ablation catheter to that with contact force sensor, the accessory pathway was immediately ablated and the PJRT was no longer induced. A retrograde CS angiogram revealed a fusiform aneurysm, which was located at the earliest activation site during the tachycardia.

Adding fuel to the fire: Coronary artery dissection complicating blunt chest trauma.

A 21year-old male presented to the emergency department with 6 h of atypical chest pain after suffering blunt chest trauma. His electrocardiogram revealed 1-1.5mm ST segment elevation in leads V1-V3 with reciprocal depressions in II, III, and aVF. Mid-anterior wall akinesis was observed on echocardiography associated with an estimated left ventricular ejection fraction of 40%. A left main coronary artery dissection was diagnosed and treated surgically with a bypass graft. Although rare, coronary dissections can be a catastrophic complication of chest trauma.

Regression and Complications of z-score-Based Giant Aneurysms in a Dutch Cohort of Kawasaki Disease Patients.

Kawasaki disease (KD) is a pediatric vasculitis. Its main complication is the development of coronary artery aneurysms (CAA), with giant CAA at the end of the spectrum. We evaluated regression and event-free rates in a non-Asian cohort of patients with giant CAA using the current z-scores adjusted for body surface area instead of absolute diameters. KD patients with giant CAA (z-score ≥10) visiting our outpatient clinic between January 1999 and September 2015 were included. Patient characteristics and clinical details were extracted from medical records. Regression was defined as all coronary arteries having a z-score of ≤3. A major adverse event was defined as cardiac death, myocardial infarction, cardiogenic shock, or any coronary intervention. Regression-free and event-free rates were calculated using the Kaplan-Meier method. We included 52 patients with giant CAA of which 45 had been monitored since the acute phase. The 1-, 2-, and 5-year regression-free rates were 0.86, 0.78, and 0.65, respectively. The 5-year, 10-year, and 15-year event-free rates were 0.79, 0.75, and 0.65, respectively. Four children, whose CAA would not have been classified as 'giant' based on absolute diameters instead of z-scores, had experienced an event during follow-up. CONCLUSION: We found a high percentage of children in whom the lumen of giant CAA completely normalized. Four children not classified as 'giant' based on absolute diameters with z-scores of ≥10 experienced a cardiac event. Hence, the use of z-scores seems to be justified.

Importance of anatomical dominance in the evaluation of coronary dilatation in Kawasaki disease.

Introduction In Kawasaki disease, although coronary dilatation is attributed to vasculitis, the effect of myocardial inflammation is underestimated. Coronary dilatations are determined by Z-scores, which do not take into account dominance. The aim of the present study was to describe the impact of coronary dominance on dilatation in Kawasaki disease. METHODS: We performed a retrospective analysis of coronary dilatations according to angiography categorisation of dominance. RESULTS: Of 28 patients (2.6 [0.2-10.1] years), right dominance was present in 15 patients and left in 13. Early dilatation was present in all patients, of whom 11 were ipsilateral to the dominant segment and 17 contralateral. Ipsilateral dilatations were present at diagnosis (9/11 versus 6/17, p=0.02) compared with contralateral dilatations, which developed 2 weeks after diagnosis (9/11 versus 16/17, p=0.29). Coronary artery Z-scores of patients with contralateral dilatation increased at 2 weeks, before returning to baseline values (2.0±2.2 at diagnosis, 4.1±1.8 at 2 weeks, 1.8±1.2 at 3-6 months, p=0.001), compared with patients with ipsilateral dilatation in whom Z-scores were maximal at diagnosis and remained stable (3.0±0.9, 2.7±1.1 and 2.6±1.5, respectively, p=0.13). Dominant coronary artery Z-scores were higher compared with non-dominant segments at diagnosis (3.0±0.9 versus 1.0±0.8, p<0.001) and at late follow-up (2.6±1.5 versus 0.4±1.4, p=0.002) in patients with ipsilateral dilatation. CONCLUSION: Progression of coronary dilatation after diagnosis may be a sign of dilatation secondary to vasculitis, as opposed to regression of Z-scores in ipsilateral dilatations, probably related to physiological vasodilatation in response to carditis. This needs to be validated in larger studies against vasculitic and myocardial inflammatory markers.

Percutaneous exclusion of a rapidly enlarging left main coronary artery aneurysm using coils and an Amplatzer™ septal occluder.

Coronary artery aneurysms (CAAs) are often an incidental finding amongst patients undergoing coronary angiography. Most CAAs are managed conservatively; rarely a larger CAA can lead to intramural thrombus formation and coronary artery embolization/obstruction even without the presence of significant stenosis. Despite these clinical implications, therapeutic options are limited to case reports and no clearly defined guidelines have been established for treatment. In this article, we present a unique case of a rapidly enlarging left main CAA, with no identifiable etiology that was treated with percutaneous exclusion via coil embolization and an Amplatzer™ septal occluder device. We also discuss existing literature, pathophysiology, and management options for CAAs. © 2016 Wiley Periodicals, Inc.

Indications and immediate and long-term results of a novel pericardium covered stent graft: Consecutive 5 year single center experience.

BACKGROUND: The use of covered stent grafts during percutaneous coronary intervention (PCI) is a life saving solution to seal acute iatrogenic vessel rupture. However, the presence of an impenetrable mechanical barrier is also appealing during treatment of friable coronary plaques but the synthetic PTFE-membrane that might trigger excessive neointimal proliferation has limited its elective-use. Pericardium tissue may offer an appealing "natural" alternative. Aim of our study is to report the consecutive 5-year single center experience with the use of pericardium-covered stents (PCS) (ITGI-Medical, Israel) in a variety of emergency and elective applications. METHODS: Nineteen consecutive patients undergoing implantation of PCS at the Royal Brompton in the last 5-years. Reasons for PCS implantation included treatment of degenerated vein grafts, large coronary aneurysms, and acute iatrogenic vessel rupture. RESULTS: Angiographic success, defined as the ability of the device to be deployed in the indexed lesion with no contrast extravasation with residual angiographic stenosis <30% and a final thrombolysis in myocardial infarction (TIMI)-3 flow was achieved in all cases. Procedural success, defined as the achievement of angiographic success without any major adverse cardiovascular event (MACE) was achieved in 94.7% of patients. In-stent restenosis (ISR) was observed in 26.3% and all patients underwent successful target vessel revascularization with DES (mean time to restenosis 9.0 ± 4.0 months). At a mean follow-up of 32.5 ± 23.3 months no acute or late stent thrombosis was observed. CONCLUSION: PCSs were effective in the treatment of friable embolization-prone coronary plaques, sealing of acute iatrogenic vessel rupture and exclusion of large aneurysms with no thrombosis but high target lesion revascularization.

Cardiovascular Response to Exercise Testing in Children and Adolescents Late After Kawasaki Disease According to Coronary Condition Upon Onset.

Multiple cardiovascular sequelae have been reported late after Kawasaki disease (KD), especially in patients with coronary artery lesions. In this perspective, we hypothesized that exercise response was altered after KD in patients with coronary aneurysms (CAA-KD) compared to those without history of coronary aneurysms (NS-KD). This study is a post hoc analysis of exercise data from an international multicenter trial. A group of 133 CAA-KD subjects was compared to a group of 117 NS-KD subjects. Subjects underwent a Bruce treadmill test followed to maximal exertion. Heart rate (HR), systolic blood pressure (SBP), and diastolic blood pressure (DBP) were assessed at each stage of the test including recovery. Myocardial perfusion was evaluated by stress and rest Tc-99m sestamibi SPECT imaging. Endurance time was similar between NS-KD and CAA-KD (11.3 ± 2.6 vs. 11.0 ± 2.6 min; p = 0.343). HR, SBP, and DBP responses to exercise were similar between groups (p = 0.075-0.942). Myocardial perfusion defects were present in 16.5 % CAA-KD versus 22.2 % NS-KD (p = 0.255). Analysis based on myocardial perfusion status identified a lower heart rate at 1 min into recovery as well as lower DBP at 1 and 5 min into recovery in patients with abnormal SPECT imaging (p = 0.017-0.042). Compared to patients without CA involvement, the presence of coronary aneurysms at the subacute phase of KD does not induce a differential effect on exercise parameters. In contrast, exercise-induced myocardial perfusion defect late after the onset of KD correlates with abnormal recovery parameters.