Evaluation of Coronary Circulation by 13N-Ammonia Myocardial Perfusion Positron Emission Tomography in Patients with Right Coronary Artery Occlusion Due to Kawasaki Disease.

BACKGROUND: Although occlusion of the right coronary artery (RCA) is common in the remote stages of Kawasaki disease, revascularization of the RCA is challenging in children and is usually managed by observation without intervention. METHODS: Using adenosine-stress 13N-ammonia myocardial perfusion positron emission tomography, we evaluated coronary circulation in 14 patients (12 males) with RCA occlusion to identify ischemia (myocardial flow ratio < 2.0) in the RCA region and examined hemodynamics, cardiac function, and coronary aneurysm diameter. These variables were also compared in patients with/without RCA segmental stenosis (SS). RESULTS: There were five cases of ischemia in the RCA region. RCA myocardial blood flow (MBF) at rest was higher in patients with ischemia than in those without ischemia, but the difference was not significant (1.27 ± 0.21 vs. 0.82 ± 0.16 mL/min/g, p = 0.2053). Nine patients presented with RCA SS, and age at onset of Kawasaki disease tended to be lower in those with SS. The maximum aneurysm diameter of RCA was significantly smaller in patients with SS (10.0 ± 2.8 vs. 14.7 ± 1.6, p = 0.0239). No significant differences in other variables were observed between patients with/without ischemia and SS. CONCLUSIONS: At rest, MBF in the RCA region was relatively well preserved, even in patients with RCA occlusion, and there was no progressive deterioration in cardiac function. Adenosine stress showed microcirculatory disturbances in only half of the patients, indicating that it is reversible in children with Kawasaki disease.

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.

Assessment of myocardial function by two-dimensional speckle tracking echocardiography in patients with Kawasaki disease: a mid-term follow-up study.

BACKGROUND: Myocardial impairment proved by histological studies persists in late convalescent phase Kawasaki disease patients. Whether Kawasaki disease-induced myocardial lesions can be detected in an earlier time is not well explored. In this study, we aimed to evaluate left ventricular (LV) myocardial function by two dimensional speckle tracking echocardiography (2DSTE) in late convalescent phase Kawasaki disease patients. METHODS: A total of 68 Kawasaki disease patients during mid-term phase including 47 with no coronary artery aneurysm (NCAA) and 21 with coronary artery aneurysm (CAA), and 60 controls with age matched were consecutively enrolled. RESULTS: No significant differences on conventional echocardiographic LV systolic function indices were found among group comparison. Compared with controls, Kawasaki disease patients had lower global longitudinal stain (GLS) and global circumferential stain (GCS). In subgroup analysis, both those with CAA and without CAA had lower GLS, lower GCS, higher amino-terminal propeptide of type III procollagen (PIIINP) and higher carboxyterminal propeptide of procollagen type I (PIPC) than in controls. GLS had significantly negative correlations with PIIINP (r = -0.69, P = 0.002) and PIPC (r = -0.82, P = 0.000). CONCLUSION: Subclinical myocardial dysfunction in mid-term follow-up Kawasaki disease patients existed regardless of coronary artery status despite normal measurements of LV systolic function by routine echocardiography, and myocardial fibrosis may play a contributed role in this subclinical myocardial function impairment. 2DSTE is a valuable imaging modality for detecting regional and global myocardial dysfunction in Kawasaki disease patients in an early time.

Role of Occlusion Position in Coronary Artery Fistulas with Terminal Aneurysms: A Hemodynamic Perspective.

PURPOSE: Thrombosis within an occluded coronary arterial fistula (CAF) may cause angina and myocardial infarction. This study aims to estimate how the occlusion position of CAFs with terminal aneurysm affects the risk stratification of thrombosis in the fistula in terms of hemodynamics. METHODS: Twelve CAF models were reconstructed based on patient-specific computed tomography angiogram (CTA) images. They were classified into three groups: preserved group (untreated fistula), aneurysm-reserved group (occluded at the fistula terminal: distal occlusion) and aneurysm-removed group (occluded before the aneurysm: proximal occlusion). Hemodynamics results were analyzed and compared with the clinical follow-up results. RESULTS: The results showed that: (1) Hemodynamic patterns within the fistula before and after treatment were significantly different among patients. (2) Aneurysm-removed occlusions showed better improvements with respect to the CAF blood-stealing phenomena. (3) Irrespective of whether aneurysms were removed or not, a disturbed flow pattern was observed. Areas having high OSI and low TAWSS were present in the post-occluded CAFs. The removal of the aneurysm, however, would alleviate the flow disturbance, and decrease the proportion of the area of OSI > 0.3. (4) The thrombosis region spotted in the follow-up patient CTAs was consistent with the computed high OSI area. CONCLUSIONS: A proximal occlusion, namely, removing the aneurysm of the CAF, may help in reducing the risk of thrombosis after surgery. However, follow-up studies with a larger cohort should be carried out to test and verify this speculation in the future.

Left ventricular systolic dyssynchrony in patients with Kawasaki disease: a real-time three-dimensional echocardiography study.

The left ventricular (LV) systolic dyssynchrony index (SDI) is an important prognostic indicator for many cardiovascular diseases; however, the characteristics of the SDI in patients with Kawasaki disease (KD) are unknown. In this study, we aimed to identify and quantify the SDI using real-time three-dimensional echocardiography (RT3DE) in KD patients during different phases. In addition, we intended to explore whether the SDI is associated with systolic dysfunction. Seventy consecutive KD patients and seventy age- and sex-matched controls were enrolled. The SDIs (percent of cardiac cycle) of 16 segments (16-SDI%) and 12 segments (12-SDI%) were calculated based on the defined standard deviation of each segment time from end diastole to the minimal systolic volume according to the 17-segment model (apex excluded). In the acute phase, the 16-SDI% and 12-SDI% were significantly higher in KD patients than in controls (4.40 ± 0.14 vs. 1.98 ± 0.12, P = 0.000; 3.55 ± 1.21 vs. 1.67 ± 0.93, P = 0.009, respectively), and patients with coronary artery aneurysm (CAA) exhibited higher 16-SDI% (P = 0.021) and 12-SDI% (P = 0.034) than patients without CAA. In the convalescent phase, patients with CAA still had higher 16-SDI% (P = 0.002) and 12-SDI% (P = 0.031) than controls, while the SDI in patients without CAA recovered to normal. The 16-SDI% was negatively correlated with the LV ejection fraction obtained from RT3DE (r = - 0.845, P = 0.000). Mechanical dyssynchrony is prevalent in KD patients during the acute phase and transient in patients without CAA, while patients with CAA still have impaired synchrony even in the convalescent phase. LV systolic dysfunction is associated with increased dyssynchrony. RT3DE is a valuable modality for identifying and quantifying dyssynchrony in KD patients.

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.

A plethora of manifestations following a Mycoplasma pneumoniae infection: a case report.

Mycoplasma pneumoniae infection can present with a plethora of symptoms and result in a systemic vasculitis by activating a cascade of autoimmune reactions. In this case report, a young man without relevant past medical history was admitted to the hospital with diarrhea, abdominal pain and spiking fever. A CT-scan showed terminal ileitis. A 5-day broad spectrum antibiotic treatment (ciprofloxacin/clindamycin) did not result in any clinical improvement. On the contrary, the patient developed a cholestatic hepatitis, bilateral anterior uveitis and a dry cough. Extensive serological testing finally led to the diagnosis of a M. pneumoniae infection by paired serology (≥4-fold rise in IgG titer). In the diagnostic work-up, a PET-CT was performed and showed increased tracer uptake in the carotids and vertebral arteries, suggesting the diagnosis of vasculitis. After start of azithromycin and low-dose corticosteroids (0.5 mg/kg/day), a gradual clinical and biochemical improvement was observed. But subsequently, the patients relapsed and presented with an acute coronary syndrome. Coronary angiography revealed aneurysmatic deformation of the three coronary arteries, leading to the assumption of coronary vasculitis. Clinical improvement was achieved with high-dose corticosteroids (1 mg/kg/day). This case shows that M. pneumoniae is not merely a pulmonary infection, but that its primary symptoms can be diverse and misleading. All clinicians should be aware of its extrapulmonary manifestations.

Giant coronary aneurysms, from diagnosis to treatment: A literature review.

Coronary aneurysms are classically defined as a segment of the artery in which dilation exceeds the diameter of an adjacent portion (considered as a reference point) by more than 1.5times. In rare instances, coronary artery aneurysms are large enough to be called giant coronary artery aneurysms, which have been reported as occurring with an incidence of 0.02%. However, there is no clear consensus on how giant coronary artery aneurysms should be defined, and their aetiology is not entirely clear; many causes have been suggested, with atherosclerosis being the most common among adults, accounting for up to 50% of cases, and paediatric diseases, such as Kawasaki disease and Takayasu arteritis, being the other main aetiology. Although giant coronary artery aneurysms are often incidental findings, many complications, such as local thrombosis, distal embolization, rupture and vasospasm, associated with ischaemia, heart failure and arrhythmias, have been reported. The optimal medical, interventional or surgical management, still needs to be clarified. This literature review aims to summarize current knowledge on giant coronary artery aneurysms.

The incidence of arrhythmias during exercise stress tests among children with Kawasaki disease: A single-center case series.

OBJECTIVE: Based on 2017 guidelines, participation in competitive sports with prior history of Kawasaki Disease (KD) requires those with coronary artery aneurysms (CAA) z score ≥ 5 to undergo evaluation for evidence of inducible ischemia or arrhythmias. The use of exercise stress testing (EST) to evaluate arrhythmias among KD patients has never been reported. This retrospective single-center case series study sought to describe the presence of inducible arrhythmias during EST in KD patients with or without CAA. METHODS: Single-center retrospective review of medical records of patients diagnosed with KD between 1989-2015 at Texas Children's Hospital, Houston, Texas who underwent EST were included. RESULTS: Among 1007 patients diagnosed with KD, 95 (9%) underwent 165 ESTs at a median time of 9.6 years (IQR 5.8-11.3 years) from diagnosis. Of these 95 patients, 37 had normal coronaries, 21 dilated (z score 2 to <2.5), 10 small (5 >z ≥2.5), 12 medium (10>z ≥ 5 absolute dimension <8 mm), 10 large (z ≥10 or absolute dimension ≥8 mm), 5 severe (myocardial infarct or bypass graft). Supraventricular tachycardia was not seen. Ventricular arrhythmias during EST were uncommon and seen only among patients with CAA z ≥5. Ventricular tachycardia occurred in a single patient with a large CAA, known VT and ICD. High-grade ventricular ectopy was seen in one patient who had severe CAA and underwent bypass grafting. CONCLUSIONS: Arrhythmias on EST were noted only among patients with CAA z ≥5. The current guidelines are a reasonable approach to increasing healthy activity among KD patients. Clarification regarding which inducible arrhythmias meet criteria for activity restriction may be helpful to guide sport participation.

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.

Pharmacologic stress cardiovascular magnetic resonance in the pediatric population: A review of the literature, proposed protocol, and two examples in patients with Kawasaki disease.

Pharmacologic stress cardiovascular magnetic resonance (PSCMR) is a well-established and reliable diagnostic tool for evaluation of coronary artery disease in the adult population. Stress imaging overall and PSCMR in particular is less utilized in the pediatric population with limited reported data. In this review, we highlight the potential use of PSCMR in specific pediatric cohorts with congenital and acquired heart disease, and we review the reported experience. A suggested protocol is presented in addition to two case examples of patients with Kawasaki disease where PSCMR aided decision making.

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.

Descifrando la fisiopatología de la disección coronaria espontánea / Unravelling the pathophysiology of spontaneous coronary artery dissections

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