Coronary Abnormalities Research Articles

Previous or coincident infections with suspected Kawasaki disease. Should we change our approach?

IntroductionKawasaki disease (KD) is a multisystem vasculitis associated with coronary artery abnormalities. Infections could be a trigger of the inflammation. The main aim of this study was to describe the presence of infections in children with KD, and to analyse the clinical characteristics and the presence of coronary abnormalities in these cases. Patients and methodsA retrospective study was performed within the Kawasaki Disease network (KAWA-RACE (2011-2016). An analysis was performed that included patients with positive microbiological findings (PMF) during the acute phase, as well as those with a previous recent infection (PRI) during the 4 weeks preceding KD diagnosis. ResultsThe study included a total of 621 children with KD, with PMF being found in 101 (16.3%) patients, and a PRI in 107 (17.2%). Significantly less echocardiographic abnormalities were found in the group with a PRI, when compared to those without a PRI (23 vs. 35%, P=.01) and also a lower proportion of overall coronary artery lesions (16 vs. 25%, P=.054). No significant differences were found in the proportion of aneurysms in either of these groups (PRI or PMF) when compared to those without infection. ConclusionsIn the present study, no differences were found in the incidence of coronary aneurysms in either of the groups, with or without PRI or PMF. Therefore, if KD is suspected, appropriate treatment should be started despite having a confirmed infection.

Evaluating 3D-printed models of coronary anomalies: a survey among clinicians and researchers at a university hospital in the UK

ObjectiveTo evaluate the feasibility of three-dimensional (3D) printing models of coronary artery anomalies based on cardiac CT data and explore their potential for clinical applications.DesignCardiac CT datasets of patients with...

Epidemiology of coronary abnormalities of discharge and branching and their clinical significance

Purpose.Until the mid-20th century they could be discovered only during autopsy, it means after death. With the introduction of coronary angiography it become possible to find them in a living person. Later on, new modalities such as computed tomography (CT) and magnetic resonance imaging (MRI) enhanced futher our abilities. It is very important to discover coronary anomalies in a living person, because some of them could lead to sudden cardiac death (SCD). In fact, coronary artery anomalies are the second main cause of the SCD in young athletes. Another importance is driven by the fact, that some of them could lead to lifethreatening complications during cardiac surgery when unknown before the operation.Methods.We prospectively reviewed all coronary angiography films from 2011 to 2016 in our center. Coronary anomalies were reviewed and classified by two independent experienced operators. Patients with congenital heart disease and coronary fistulas were excluded.Results.Out of 5055 patients 148 (2.9%) had coronary artery anomalies of origin and distribution. Those were 120 men (81.1%) and 28 women (18.9%) with an age range between 29 to 88 years. The three most common anomalies were myocardial bridge (48.7%), separate origin of the conus branch (13.5%) and separate origin of the LAD and LCX (8.1%).Conclusion.In our study we found more or less the same types and incidence rates of coronary artery anomalies as in the world literature. We had apparently higher rates of myocardial bridges, compared to average number on angiography studies, but very close to authopsy study rates.

Evaluation of regional myocardial perfusion in methamphetamine abusers using real-time myocardial contrast echocardiography.

To evaluate the feasibility of assessing regional myocardial perfusion using real-time myocardial contrast echocardiography(MCE) at rest for detecting coronary microcirculation abnormalities in methamphetamine abusers.Materialand methods: Twenty-two male methamphetamine abusers (11 without chest pain, 11 with chest pain), free of ascertainedcoronary artery disease, were enrolled in this study. A control group of 22 age-matched male healthy participants was studiedfor comparison. Standard 2D, flow and tissue Doppler echo with measurements of cardiac morphologic and functional indicators,MCE with measurements of regional myocardial perfusion were performed, respectively. Compared to healthyparticipants, methamphetamine abusers had higher blood pressure, greater left ventricular mass index and more impaireddiastolic function, with preserved cardiac sizes and systolic function. Methamphetamine abusers with chest pain had a fasterheart rate than those without chest pain and healthy participants. MCE in methamphetamine abusers, especially with chestpain, had significant longer contrast agent arrival times, less functional capillary blood volumes, slower microvascular flowvelocities and less myocardial perfusion than healthy participants (p<0.05). Moreover, along with the increases of dosage andduration of use (from group A to group C, group A: 1-2 g/day, <2 years; group B: 2-3 g/day, 2-5 years; group C: >3 g/day, >5years) the reductions in the myocardial perfusion indices were more significant (p<0.01). The cutoff value with 5.1 dB2/s ofthe myocardial perfusion at the left ventricular apex had a sensitivity of 87.5%, specificity of 75.2% and accuracy of 81.9% fordifferentiating methamphetamine abusers from normal subjects. Real-time MCE can effectively detect coronarymicrocirculation abnormalities in methamphetamine abusers at rest and myocardial perfusion is significantly reduced in methamphetamineabusers. This finding may be involved in the occurrence and development of cardiac damage.

Extreme ST-segment elevations in seemingly no significant angiographic coronary artery abnormalities: a case report

BackgroundObstructive coronary artery disease is found in approximately 97% of patients presenting with ST-elevation myocardial infarction and 92% of patients with non ST-elevation myocardial infarction (Bainey KR, Welsh RC, Alemayehu W, Westerhout CM, Traboulsi D, Anderson T, et al. Int J Cardiol 264: 12–17, 2018). Recent studies showed that myocardial infarction without obstructive coronary atherosclerosis (MINOCA) is also associated with a long-term risk of adverse events (Bainey KR, Welsh RC, Alemayehu W, Westerhout CM, Traboulsi D, Anderson T, et al. Int J Cardiol 264: 12–17, 2018).. The following case illustrates that MINOCA may also be associated with short term adverse events (depending on the underlying mechanism).Case presentationA 49-year old Caucasian male with no significant medical history was referred to our cardiac emergency department with acute chest pain. The ambulance ECG showed extreme ST-segment elevation anterolateral (‘tombstone sign’), which had resolved completely at arrival in the hospital. Coronary angiography showed no obstructive coronary artery disease. Conservative (medical) therapy was started and patient was discharged. Two days later he presented with recurrent cardiac ischemia with ventricular fibrillation. Coronary angiography showed no changes compared with earlier presentation. During admission to the ICU his clinical condition gradually deteriorated, eventually leading to his death. Post-mortem studies showed no significant atherosclerotic lesions. Massive myocardial infarction was found, probably caused by temporary occlusion of the left main coronary artery.ConclusionsSeveral pathophysiological mechanisms are recognized in MINOCA, of which vasospasm is the most probable one in this case. MINOCA is associated with increased over-all mortality and risk of ventricular arrhythmias. Therefore, additional testing should be considered when there is no explanation for the mismatch between ST-elevations (STEMI) and (no significant) coronary abnormalities.

Echocardiographic Study of the Coronary Sinus in the Indeterminate Form of Chagas Disease.

We investigated the occurrence of coronary sinus abnormalities in the indeterminate form of Chagas disease (CD). Differences between the maximum and minimum diameters of the coronary sinus (∆%) on echocardiography were evaluated in individuals with the indeterminate form of CD (n=14) and those without (n=16) CD. The association of the difference with abnormalities detected by echocardiography and myocardial scintigraphy was assessed. The mean Δ% values did not differ significantly between the groups. There was no correlation of the measurements with echocardiographic and myocardial scintigraphy findings. The coronary sinus evaluation revealed no differences between the groups.

Coronary Artery Emergencies in Children

Coronary artery issues are rare in children, leading to challenges for the pediatric emergency medicine practitioner. The differential diagnosis can be grouped into congenital or acquired coronary abnormalities. Congenital coronary anomalies leading to myocardial ischemia or aborted sudden cardiac death are most often due to an anomalous origin of a coronary artery. Acquired coronary artery disease is most commonly related to the sequelae of Kawasaki disease. Lastly, rare, specific patient populations are at higher risk for early coronary disease. This review discusses the most common pediatric causes of coronary artery events, providing an overview of the evaluation and management in an emergency department setting.

Uso de inibidores da glicoproteína IIb/IIIa na intervenção coronária percutânea primária: experiência de 10 anos no Brasil

ABSTRACT Background Individualized use of glycoprotein IIb/IIIa inhibitors during primary percutaneous coronary intervention in scenarios with high thrombotic load or occurrence of coronary flow abnormalities may be considered, despite scarce scientific evidence. Our objective was to compare patients undergoing primary percutaneous coronary intervention treated or not with glycoprotein IIb/IIIa inhibitors during the procedure. Methods A national, multicenter, retrospective study that included consecutive patients undergoing primary percutaneous coronary intervention, treated or not with glycoprotein IIb/IIIa inhibitors, between June 2006 and March [...]

SPECTRUM OF IMMUNODEFICIENCIES IN JACOBSEN SYNDROME

Introduction Jacobsen syndrome (JS) is a rare contiguous gene deletion syndrome associated with a distal deletion of chromosome 11q. Immune deficiency in JS has not been well-established but usually involves antibody defects. Here we report 2 cases of Jacobsen syndrome with combined T and B cell defects presenting at different ages. Case Description The first case is now a 22-month-old girl with JS who was initially seen at 2 months of age. She has corneal opacities, IUGR, thrombocytopenia (Paris-Trousseau syndrome), and intracranial hemorrhages. Newborn screening (NBS) for severe combined immunodeficiency (SCID) was negative. At 2 months of age, she had slightly low T-cell, moderately low B-cell, and normal NK-cell counts. At 6 months of age, she developed progressive T-cell cytopenia and intermittently low NK cell counts. Immunoglobulin levels and IgG response to vaccines were normal. She has no history of recurrent or severe/opportunistic infections. The second case, a 22-day-old girl, was referred because of positive NBS for SCID. She has left cystic renal dysplasia, hypoplastic right hand, trigonocephaly, and coronary abnormalities (ASD, small coronary fistula). Laboratory evaluation revealed thrombocytopenia, lymphopenia, normal IgM and IgG, low IgA, and moderately low T and B cell counts and normal NK cell counts. Genetic evaluation confirmed the diagnosis of JS. Findings in these cases suggest that immune deficiency in JS can start at any age and may be progressive. Discussion Jacobsen syndrome is one of the syndromic primary immunodeficiencies where type and severity of immune deficiency vary and can be progressive.

VD05 BENTALL OPERATION IN BICUSPID VALVE AND ANOMALOUS LEFT CIRCUMFLEX CORONARY ARTERY: ANATOMICAL AND TECHNICAL IMPLICATIONS

Abstract Bicuspid aortic valve (BAV) is often associated with coronary abnormalities. The finding of BAV associated with an anomalous circumflex coronary artery (ACCA) originating from the right Valsalva sinus (VS) and surrounding the non-coronary VS is however a rather rare occurrence. We report a case of successful Bentall operation with a composite button technique in a 56-year-old man with aortic root dilatation, BAV insufficiency and coexisting ACCA. In this case a 25 SJM composite graft was implanted in supraannular fashion rather than intraannular to avoid: 1) ACCA injury or obstruction of the proximal course near the annulus by suture ligation, 2) compression by prosthetic ring, 3) distortion of ACCA during ostia reimplantation. We demonstrated that attention to the anatomic relationship of ACCA to the aorta is crucial and prosthesis supraannular implantation technique allows a safe aortic root replacement.

Accuracy of non-invasive stress testing in women and men with angina in the absence of obstructive coronary artery disease

ObjectiveWhile >20% of patients presenting to the cardiac catheterization laboratory with angina have no obstructive coronary artery disease (CAD), a majority (77%) have an occult coronary abnormality (endothelial dysfunction, microvascular dysfunction (MVD), and/or a myocardial bridge (MB)). There are little data regarding the ability of noninvasive stress testing to identify these occult abnormalities in patients with angina in the absence of obstructive CAD. MethodsWe retrospectively evaluated 155 patients (76.7% women) with angina and no obstructive CAD who underwent stress echocardiography and/or electrocardiography before angiography. We evaluated Duke treadmill score, heart rate recovery (HRR), metabolic equivalents, and blood pressure response. During angiography, patients underwent invasive testing for endothelial dysfunction (decrease in epicardial coronary artery diameter >20% after intracoronary acetylcholine), MVD (index of microcirculatory resistance ≥25), and intravascular ultrasound for the presence of an MB. ResultsStress echocardiography and electrocardiography were positive in 58 (43.6%) and 57 (36.7%) patients, respectively. Endothelial dysfunction was present in 96 (64%), MVD in 32 (20.6%), and an MB in 83 (53.9%). On multivariable logistic regression, stress echo was not associated with any abnormality, while stress ECG was associated with endothelial dysfunction. An abnormal HRR was associated with endothelial dysfunction and MVD, but not an MB. ConclusionConventional stress testing is insufficient for identifying occult coronary abnormalities that are frequently present in patients with angina in the absence of obstructive CAD. A normal stress test does not rule out a non-obstructive coronary etiology of angina, nor does it negate the need for comprehensive invasive testing.

Coronary Arcade Visualized in 256 Sliced Multi- Detector Cardiac Computed Tomography

A 52-year-old male patient presented to our Department of Internal Medicine with severe sustained chest pain for at least 18 hours, for ruling out acute myocardial infarction. We performed a cardiac 256 multi-sliced computed tomography to document a coronary arcade as the coronary abnormality.

Utilization of Coronary Artery to Aorta for the Early Detection of Kawasaki Disease

Timely diagnosis of coronary involvement is paramount in Kawasaki disease (KD) as it can be associated with long-term morbidity. However, echocardiographic measurements of coronary artery dilation in KD are inconsistent and not proficient for all abnormal arteries. The purpose of this study was to investigate more valuable indices and determine their sensitivity and specificity for early diagnosis of coronary involvement in KD. We performed this retrospective study in 218 children. All patients underwent laboratory and echocardiographic evaluations upon admission. We measured the size of the left main coronary artery (LMA), left anterior descending coronary artery (LAD), right coronary artery (RCA), and aorta (Ao), and calculated the LMA/Ao, LAD/Ao, and RCA/Ao ratios. We also calculated the cut-off values of each index using receiver operating characteristic curves. LMA, LAD, and RCA measurements did not correlate with white blood cell count, platelet count, erythrocyte sedimentation rate, C-reactive protein level, or brain natriuretic peptide level. The LMA measurement was associated with hemoglobin, hematocrit, and iron saturation. LAD/Ao was correlated with white blood cell and platelet counts (P < 0.05), whereas RCA/Ao was correlated with ferritin level (P < 0.05). The cut-off value of LMA/Ao was 0.2, with a sensitivity of 85% and specificity of 70%. Individual coronary artery/Ao ratios might provide helpful insight for detection of coronary abnormality in KD in the acute phase. Further investigation is essential to clarify prompt early diagnosis of coronary involvement in KD.

Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease.

Kawasaki disease shock syndrome (KDSS) is an uncommon presentation of Kawasaki disease (KD). KDSS has been associated with more severe markers of inflammation, coronary abnormalities and i.v. immunoglobulin (IVIG) resistance. A retrospective, descriptive study of children with KDSS in two hospitals was performed. Relevant articles about KD and shock were collected, and demographic data, clinical presentation, laboratory variables, echocardiogram findings, treatment and special features were analyzed when available. Twelve patients diagnosed with KDSS were retrospectively reviewed from two centers in Mexico, along with 91 additional cases from the literature. Seventy-two patients presented with complete KD (69.9%), and 30.1% (31/103) had unusual KD manifestations. The most frequent diagnosis at the time of admission was toxic shock syndrome (TSS; n = 20). Sixteen of the 20 had coronary artery abnormalities. Overall, abnormalities in the coronary arteries were documented in 65% of the patients. The mortality rate was 6.8%. The presence of coronary aneurysms was significantly and positively correlated with male gender, IVIG resistance, inotrope treatment, cardiac failure, abdominal pain and neurological symptoms. IVIG-resistant patients had higher neutrophil : lymphocyte ratio. Abdominal symptoms, hypoalbuminemia and elevated C-reactive protein were present in almost all of the patients. Multisystem involvement with atypical presentation in KDSS is frequent. An important differential diagnosis is TSS. Mechanical ventilation, gastrointestinal and neurological symptoms were associated with IVIG resistance and the presence of coronary aneurysms. The first line of treatment includes IVIG and pulse corticosteroids; in severe cases, infliximab, anakinra, cyclosporine or plasmapheresis are alternative treatment options.

Hiroaki Shimokawa.

Hiroaki Shimokawa.

Interobserver variability in the classification of congenital coronary abnormalities: A substudy of the ANOCOR registry

Objectives We studied the observer variability in the description and classification of anomalous connections of the coronary arteries (ANOCOR) between a non-expert group of physicians (ANOCOR investigators) and a group of expert physicians, using the ANOCOR cohort. Background The diagnosis of ANOCOR requires an appropriate identification for the management of the patients involved. A misdiagnosis may have serious repercussions for the management to come. For example a subpulmonic course is sometimes interpreted as an interarterial course ( Fig. 1 ). Methods Consecutive patients identified by 71 referring cardiologists (ANOCOR investigators) were included in the ANOCOR cohort. ANOCOR was diagnosed by invasive and/or computed tomography (CT) coronary angiography. Angiographic images were reviewed by an angiographic committee with experience in the ANOCOR field. Both ANOCOR investigators and angiographic committee filled out a questionnaire to classify each ANOCOR with the type of coronary artery involved, the site of anomalous connection, and the initial course. Observer variability between ANOCOR investigators and angiographic committee was assessed by κ statistics. ANOCOR with an interarterial course were defined as at-risk. Results In total, 496 ANOCOR were identified among 472 patients of the ANOCOR cohort and an interarterial course was present in 31% of the abnormalities. The agreement for the type of artery was excellent (κ = 0.92, 95% CI: 0.86–0.98, P Table 1 ). Conclusions Observer variability in the assessment of ANOCOR between non-experienced and experienced physicians can be significant. We found that expert physicians provide a more robust classification of ANOCOR in comparison with non-expert physicians. Therefore, referral to physicians with a relevant experience should be considered, especially if an ANOCOR at-risk is suspected.

Kawasaki disease: abnormal initial echocardiogram is associated with resistance to IV Ig and development of coronary artery lesions

BackgroundKawasaki disease (KD) is an acute febrile systemic vasculitis that affects small and medium blood vessels. Intensified treatments for the most severely affected patients have been proposed recently, and the early identification of KD patients at high risk for coronary artery aneurysms (CAA) is crucial. However, the risk scoring systems developed in Japan have not been validated in European populations, and little data is available concerning the link between initial echocardiogram findings other than high z-scores and cardiac prognosis.MethodsIn order to investigate whether the presence of any abnormalities, other than high z-scores in first echocardiogram, are associated with resistance to IV immunoglobulins and/or subsequent development of CAA, we retrospectively analyzed data from children diagnosed with KD between 2006 and 2016 at a tertiary Hospital in Paris, France.ResultsA total of 157 children were included. The initial echocardiogram was performed after a median of 7 days of fever and was abnormal in 48 cases (31%). The initial presence of any echocardiographic abnormality (coronary artery dilatation, CAA, pericardial effusion, perivascular brightness of the coronary arteries, left-ventricular dysfunction and mitral insufficiency) was strongly associated with resistance to intravenous immunoglobulin (p = 0.005) and development of coronary artery lesions within the first 6 weeks of disease (p = 0.01). All patients (n = 7) with persistent coronary abnormalities at 1 year already had an abnormal initial echocardiogram. Severity scoring systems from Japan had low sensitivity (0–33%) and low specificity (71–82%) for predicting immunoglobulin resistance or cardiac involvement.ConclusionsIn European populations with mixed ethnic backgrounds, the presence of any abnormalities at the initial echocardiogram may contribute to early identification of patients with severe disease.

Effects of Dose Reduction on Diagnostic Image Quality of Coronary Computed Tomography Angiography in Children Using a Third-Generation Dual-Source Computed Tomography Scanner

Performing coronary computed tomography angiography (CCTA) using third-generation dual source computed tomography (3G-DSCT) scanners results in radiation dose reduction without sacrificing image quality in adults. The largest dose reductions have been reported with prospectively gated, high-pitch imaging. However, there are limited data to determine if these benefits extend to pediatric patients. We evaluated image quality and radiation dose range of CCTA performed in children using a 3G-DSCT scanner. A retrospective review of 44 children (median age 10years, range 0.6 to 17) who underwent imaging to evaluate coronary artery origins (n = 27), Kawasaki disease (n = 12) or other coronary abnormalities (n = 5) were performed. General anesthesia was used in 9/44 (20%) patients and a β blocker was administered in 19/44 (43%). Prospectively gated high-pitch scanning was most frequently used (n = 24). Other techniques used included prospectively-gated "step and shoot" (n = 14), retrospectively gated (n = 2) and nongated high-pitch scan (n = 4). Median effective radiation doses were lowest for prospectively gated high-pitch scans (0.5mSv, range 0.4 to 0.7). Overall coronary artery image quality grade (1-excellent and 4-nondiagnostic) was acceptable for all electrocardiography-gated techniques, with no significant differences between high-pitch and "step-and-shoot" scan types (median 1, range 1 to 3 vs median 1, range 1 to 4, p = 0.22). Image quality grade was diagnostic (1 to 3) for all proximal coronary segments but rare distal segments were nondiagnostic (0.8% segments for gated high-pitch scan). In conclusion, CCTA can be performed in children using 3G-DSCT scanners withacceptable image quality. Prospectively gated high-pitch scans deliver the lowest radiation dose without reduction in image quality compared with conventional scan techniques.

Systemic Ventricular Dysfunction Between Stage One and Stage Two Palliation.

Infants with a single ventricle can develop systemic ventricular dysfunction (SVD) after stage 1 operation, but available information is sparse. We reviewed our patients having Norwood, Sano, or hybrid procedures to better understand this problem. We conducted a retrospective, case-controlled cohort study of 267 patients having stage1 operation, examining outcomes between stages 1 and 2 (survival and subsequent cardiac surgeries), predictor variables, and histology of hearts explanted at transplantation. SVD developed in 32 (12%) patients and resolved in 13 (41%); mean age of onset was 3.0 ± 1.63 months; median = 2.79. SVD was not associated with cardiac anatomy, type of stage 1 procedure, weight, coronary abnormality, or atrioventricular valve regurgitation. The mean age of resolution = 12.1 ± 9.6 months; median = 6.3, and resolution may have been more likely with a systemic LV than RV (p = 0.067). Outcomes for the entire SVD group were less favorable than for those without, but patients with resolution of SVD had outcomes at least as good those without SVD. Myocardial histology (n = 4) suggested chronic ischemia. The risk of SVD after stage 1, while low, may be a fundamental feature of this patient population. SVD occurs with either a systemic RV or LV, although patients with a systemic LV may be more likely to have resolution than those with an RV. We identified no predictor variables, but histologic findings suggest chronic ischemia may be involved. Given the low incidence of SVD, multi-center studies will be required to better define predictors of onset and resolution.

To prevent SCD in junior soccer players: screening for coronary abnormalities by echocardiography

To prevent SCD in junior soccer players: screening for coronary abnormalities by echocardiography