Coronary Abnormalities Research Articles

An Atherogenic Diet Exacerbates Vascular Injury in an Experimental Model of Systemic lupus Erythematosus

Patients with systemic autoimmune diseases such as systemic lupus erythematosus (SLE) have an increased risk of developing premature atherosclerotic cardiovascular disease (CVD), which is a major contributor to morbidity and mortality in this patient population. Specifically, patients with SLE have evidence of vascular disease including endothelial dysfunction, arterial stiffness, and coronary perfusion abnormalities, all of which predispose them to the development of atherosclerotic lesions. Our laboratory has shown that an established female mouse model of SLE (NZBWF1) develops impaired endothelial dependent relaxation compared to age matched healthy controls (female NZW mice). In the present study, we hypothesized that SLE mice fed an atherogenic diet will have accelerated vascular injury indicative of premature atherosclerotic disease. In order to test this hypothesis, female control (NZW, n=20) and SLE (NZBWF1, n=20) mice were fed normal chow or a high fat diet supplemented with 0.5% cholate (HFD+cholate) for 14 weeks. Body weight and body composition were monitored by Echo MRI biweekly over the course of the study. At the conclusion of the study both carotid arteries and aortas were isolated from control and SLE mice. Carotid rings were suspended in organ chamber baths where endothelium‐dependent and endothelium‐independent relaxation were the concentration responses (10−8 to10−4 M) to acetylcholine (ACh) and sodium nitroprusside (SNP) were assessed in vessels pre‐contracted with the thromboxane mimetic U46619 (0.4 μg/mL). HFD+cholate did not alter maximal ACh (10−4 M) mediated relaxation (70.1±3.4% vs. 75.2±2.1% p=0.33) in control mice. However, SLE mice fed HFD+cholate had an impaired relaxation response to ACh (10−4 M) as compared to SLE mice fed normal chow (65.6±2.3% vs. 43.6±7.2%, p=0.02). SNP‐mediated relaxation was not different between the mice fed different diets in either control or SLE animals, indicating that the impaired ACh response in SLE mice is endothelial dependent. In order to examine the impact of HFD+cholate on atherosclerotic lesion formation, isolated aortas were subjected to en face staining using Sudan IV. Preliminary data suggest that control mice fed HFD+cholate did not develop atherosclerotic plaques, but SLE fed HFD+cholate showed evidence of plaques in the aortic arch. Taken together, these data suggest that the immune system dysfunction present in SLE may accelerate the progression of diet‐induced atherosclerosis.Support or Funding InformationE.B.T. was supported by an individual NIH National Research Service Award (F32HL137393). This work was supported by Veteran's Administration Merit award (BX002604‐01A2) to M.J.R. and NIH NHLBI awards PO1HL051971, P20GM104357 to UMMC‐Department of Physiology and Biophysics.This abstract is from the Experimental Biology 2018 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Fusion of Three-Dimensional Echocardiographic Regional Myocardial Strain with Cardiac Computed Tomography for Noninvasive Evaluation of the Hemodynamic Impact of Coronary Stenosis in Patients with Chest Pain

Combined evaluation of coronary stenosis and the extent of ischemia is essential in patients with chest pain. Intermediate-grade stenosis on computed tomographic coronary angiography (CTCA) frequently triggers downstream nuclear stress testing. Alternative approaches without stress and/or radiation may have important implications. Myocardial strain measured from echocardiographic images can be used to detect subclinical dysfunction. The authors recently tested the feasibility of fusion of three-dimensional (3D) echocardiography-derived regional resting longitudinal strain with coronary arteries from CTCA to determine the hemodynamic significance of stenosis. The aim of the present study was to validate this approach against accepted reference techniques. Seventy-eight patients with chest pain referred for CTCA who also underwent 3D echocardiography and regadenoson stress computed tomography were prospectively studied. Left ventricular longitudinal strain data (TomTec) were used to generate fused 3D displays and detect resting strain abnormalities (RSAs) in each coronary territory. Computed tomographic coronary angiographic images were interpreted for the presence and severity of stenosis. Fused 3D displays of subendocardial x-ray attenuation were created to detect stress perfusion defects (SPDs). In patients with stenosis >25% in at least one artery, fractional flow reserve was quantified (HeartFlow). RSA as a marker of significant stenosis was validated against two different combined references: stenosis >50% on CTCA and SPDs seen in the same territory (reference standard A) and fractional flow reserve<0.80 and SPDs in the same territory (reference standard B). Of the 99 arteries with no stenosis >50% and no SPDs, considered as normal, 19 (19%) had RSAs. Conversely, with stenosis >50% and SPDs, RSAs were considerably more frequent (17 of 24 [71%]). The sensitivity, specificity, and accuracy of RSA were 0.71, 0.81, and 0.79, respectively, against reference standard A and 0.83, 0.81, and 0.82 against reference standard B. Fusion of CTCA and 3D echocardiography-derived resting myocardial strain provides combined displays, which may be useful in determination of the hemodynamic or functional impact of coronary abnormalities, without additional ionizing radiation or stress testing.

Coronary artery abnormalities: Current clinical issues

Introduction and ObjectivePatients with anomalous coronary arteries arising from the opposite sinus of Valsalva (ACAOS), the left coronary artery (LCA) arising from the right sinus or the right coronary artery (RCA) from the left sinus with an interarterial course, may present from complete absence of symptoms to sudden cardiac death. Although there are guidelines on indications for surgery, controversy remains. MethodsA retrospective review was performed of all adult patients diagnosed with ACAOS in our hospital between 2007 and 2016. Demographic, clinical, perioperative and follow-up data were collected from clinical records and summarized. A review of the published literature was performed with special emphasis on clinical presentation, surgical indications and results. ResultsSeven symptomatic patients underwent surgery (mean age 57.1±8.9 years, two male, five female); they recovered without complications and to date have had no recurrence of myocardial ischemia. One asymptomatic patient with an anomalous RCA has been medically followed without evidence of myocardial ischemia. A 75-year-old woman, diagnosed in 2008 with an anomalous LCA, was not referred for surgery and died suddenly six months after diagnosis. ConclusionsSurgery for coronary abnormalities is performed with low risk and all published series report full operative survival. The indications for surgery are well established for patients with interarterial anomalous LCA and symptomatic patients with interarterial anomalous RCA. However, there is some uncertainty concerning asymptomatic patients, particularly those with an anomalous interarterial RCA, for whom we propose a more assertive approach, if young or engaged in strenuous activities.

EFFECT OF RANOLAZINE ON SYMPTOMS AND QUALITY OF LIFE IN PATIENTS WITH ANGINA IN THE ABSENCE OF OBSTRUCTIVE CORONARY ARTERY DISEASE: A RETROSPECTIVE COHORT STUDY

More than 20% of patients presenting to the cath lab have no significant obstructive coronary artery disease (CAD) despite having angina. Several occult coronary abnormalities, including endothelial dysfunction, microvascular dysfunction (MVD), and/or a myocardial bridge (MB), may explain their

Paediatric overdiagnosis modelled by coronary abnormality trends in Kawasaki disease

ObjectiveCompare trends in coronary artery (CA) abnormality diagnoses to trends in adverse cardiac outcomes among American children with Kawasaki disease (KD) to assess the fit of detection of CA abnormalities...

Congenital Extra-Cardiac Vascular Anomalies as Detected on Multislice Computed Tomography Angiography

Background: Accurate evaluation of extra cardiac and intra cardiac anomalies in congenital heart diseases (CHD) is vital for diagnosis and treatment. Trans-thoracic echocardiography (TTE) is the classical choice for children suspected to have CHD because TTE is safe, readily available and its capacity to perform Doppler flow studies therefore can measure hemodynamic parameters. Plus its ability to evaluate intra cardiac abnormalities like atrial septal defect and ventricular septal defect. Therefore, owing to its lower spatial resolution and limited acoustic window; it is not precise in evaluation of extra cardiac abnormalities. Aim of the Work: to evaluate the advantage of recent advances of ECG gated MDCT in diagnosis of congenital heart disease in pediatrics and assessment of associated extra cardiac abnormalities within the great vessels in comparison with echocardiography findings. Patients and Methods: The current study was carried out on 36 patients in Radio-diagnosis Department, Faculty of medicine, Ain Shams University and specialized private radiology centers. The patients were referred from pediatric hospital, cardiology clinic with their echocardiographic reports to radio-diagnosis department, cardiac imaging unit with a view to execute ECG- gated multi-slice CT of the heart & extra-cardiac great vessels. Results: In this study aortic anomalies represent the most common extra-cardiac anomalies accounting for 36.5% of our cases while comprising: Supravalvular aortic stenosis; 4.3%, Aortic CoA; 26.1%, arch anomalies with vascular rings; 52.2% and finally anomalous coronary arteries; 17.4%. By Using MDCT we were able to delineate eight cases of right aortic arch (RAA).Two of them with aberrant left subclavian artery (ALSCA) adding to two cases of left aortic arch with an aberrant right subclavian artery (ARSCA). On the other hand ,TTE failed to identify 6 cases of RAA with a sensitivity 75%, specificity 100%, NPV 96.61% and one case of ALSCA with a sensitivity 50.0%, specificity 100%, NPV 98.41% .Regarding coronary anomalies , Cardiac CTA succeeded in characterization of origin and course of all coronary abnormalities encountered in our study encompassing 4 cases specifically anomalous LAD from right coronary sinus running a prepulmonic course anterior to RVOT , RCA from LAD and single coronary artery adding to LCX- RV coronary fistula. While TTE failed to depict two cases of the coronary anomalies with a sensitivity of 50%, specificity 100%, PPV 100% and NPV 96.83%. Conclusion: CTA can provide the confident detection and exclusion of extra- cardiac vascular abnormalities with superb anatomical description which was feasible with a sensitivity 98.41%, specificity 99.76%, PPV 96.88% and NPV 99.88%.

Coronary microvascular dysfunction may be related to IGF-1 in acromegalic patients and can be restored by therapy

Background and aimsAcromegaly increases the risk of cardiovascular mortality. Data on the cardiovascular risk in asymptomatic acromegaly are limited. In particular, data on coronary microvascular abnormalities are lacking. We assessed coronary flow reserve (CFR) as a marker of coronary microvascular function in asymptomatic acromegaly. MethodsWe studied 40 acromegalic patients (23 male, age 52 ± 11 years) without clinical evidence of cardiovascular disease, and 40 control subjects matched for age and sex. Coronary flow velocity in the left anterior descending coronary artery was detected by transthoracic Doppler echocardiography, at rest, and during adenosine infusion. CFR was the ratio of hyperaemic to resting diastolic flow velocity. ResultsCFR was lower in patients than in controls (2.9 ± 0.8 vs. 3.7 ± 0.6, p < 0.0001) and was abnormal (≤2.5) in 13 patients (32.5%) compared with any control subjects (0%) (p < 0.0001). CFR was inversely related to insulin-like growth factor 1 (IGF-1) levels (r = −0.5, p < 0.004). In patients with CFR≤2.5, IGF-1 was higher (756 [381–898] μg/l versus 246 [186–484] μg/l, p < 0.007) whereas growth hormone (GH) levels were similar (6.3 [2.8–13.7] μg/l versus 5 [2.8–8.9] μg/l, p = 0.8). In multivariable linear regression analysis, IGF-1 was independently associated with CFR (p < 0.0001). In multiple logistic regression analysis, IGF-1 independently increased the probability of CFR≤2.5 (p = 0.009). In four patients with active disease (all with CFR<2.5), treatment with somatostatin analogues normalized CFR. However the other four patients with active disease were not responder. ConclusionsAcromegalic patients have coronary microvascular dysfunction that may be restored by therapy with somatostatin analogues. IGF-1 independently correlates with the coronary microvascular impairment, suggesting the pivotal role of this hormone in explaining the increased cardiovascular risk in acromegaly.

Patients with acute myocardial infarction and non-obstructive coronary arteries: safety and prognostic relevance of invasive coronary provocative tests.

Functional alterations of epicardial coronary arteries or coronary microcirculation represent a frequent cause of myocardial infarction and non-obstructive coronary arteries (MINOCA). We aimed at assessing the prognostic value of intracoronary provocative tests in patients presenting with MINOCA and in which other causes of MINOCA have been excluded. We prospectively evaluated patients with a diagnosis of MINOCA, excluding patients with aetiologies other than suspected coronary vasomotor abnormalities. Immediately after coronary angiography, an invasive provocative test using acetylcholine or ergonovine was performed. The incidence of death from any cause, cardiac death, and recurrence of acute coronary syndrome (ACS) was assessed at follow-up. We also assessed angina status using Seattle Angina Questionnaires (SAQ). We enrolled 80 consecutive patients [mean age 63.0 ± 10.7 years, 40 (50%) male]. Provocative test was positive in 37 (46.2%) patients without any complication. Among patients with a positive test, epicardial spasm was detected in 24 (64.9%) patients and microvascular spasm in 13 (35.1%) patients. After a median follow-up of 36.0 (range 12.0-60.0) months, patients with a positive test had a significantly higher occurrence of death from any cause [12 (32.4%) vs. 2 (4.7%); P = 0.002], cardiac death [7 (18.9%) vs. 0 (0.0%); P = 0.005], and readmission for ACS [10 (27.0%) vs. 3 (7.0%); P = 0.015] as well as a worse angina status as assessed by SAQ [Seattle score: 88.0 (33.0-100.0) vs. 100.0 (44.0-100.0); P = 0.001] when compared with patients with a negative test. We demonstrate that in patients presenting with MINOCA and suspected coronary vasomotor abnormalities, a positive provocative test for spasm is safe and identifies a high-risk subset of patients.

Assessment of transposition of the great arteries associated with multiple malformations using dual-source computed tomography.

PurposeTo determine the value of dual-source computed tomography (DSCT) in depicting the morphological characteristics and diagnosing the associated malformations for patients with transposition of the great arteries (TGA) before surgery.Materials and methodsTwenty-five patients with TGA who underwent DSCT and transthoracic echocardiography (TTE) examination were retrospectively reviewed. The morphological types of TGA, the spatial relationship between the pulmonary artery and the aorta, as well as coronary artery-associated abnormalities were assessed by DSCT. In contrast to TTE, the diagnostic accuracy of associated malformations on DSCT were analyzed and calculated with reference to surgical or digital subtraction angiography (DSA) findings. Effective doses (EDs) were also calculated.ResultsAmong the 25 patients, 12 (48%) had ventricular septal defects and left ventricular outflow tract stenosis. Sixteen patients (16/25, 64%) had great arteries with an oblique spatial relationship on DSCT. In addition, we found seven patients (7/25, 28%) with coronary artery malformation, including five with an abnormal coronary origin and two with signs of a myocardial bridge. According to DSA or surgical findings, DSCT was superior to TTE in demonstrating extracardiac anomalies (sensitivity, anomalies of great vessels: 100% vs. 93.33%, other anomalies: 100% vs. 46.15%). The mean estimated ED for those aged <10 years was <2 mSv (1.59 ± 0.95 mSv).ConclusionsDSCT can achieve an overall assessment of patients with TGA, including any associated malformations as well as the identification of the spatial relationship of the great arteries. DSCT can therefore be considered as an alternative imaging modality for surgical decision making.

Surgical unroofing of intramural anomalous aortic origin of a coronary artery in pediatric patients: Single-center perspective

BackgroundIntramural anomalous aortic origin of a coronary artery (AAOCA) is associated with an increased risk of sudden cardiac death. This is amenable to surgical coronary unroofing, but outcomes studies are lacking. ObjectiveTo perform a comprehensive review of our institutional experience with pediatric patients with AAOCA who underwent surgical repair with unroofing of the intramural segment, focusing on preoperative and postoperative course and testing as well as intraoperative findings. MethodsA retrospective cohort study was conducted to evaluate patients with AAOCA status post–coronary unroofing at Children's Hospital of Wisconsin. Data extraction included symptoms, preoperative and postoperative imaging and testing, surgical findings, and postoperative clinical course. ResultsFrom January 1999 to December 12, 2015, 63 patients underwent unroofing at a median age of 13 years (0.5-18 years). The majority underwent unroofing of an intramural right coronary (79%); 21% had an intramural left AAOCA. Symptoms suggestive of possible ischemia were present in about 50%. Additional structural cardiac anomalies were present in 33%. Transthoracic echocardiography was diagnostic in 60 of 63 (95%) and correlated with surgical findings in all cases. There was no surgical mortality associated with the unroofing, and no additional coronary reinterventions were performed. The median duration of postoperative follow-up was 3.1 years (7 days to 13.6 years). Symptoms either persisted or developed in 46% postoperatively. Postoperative exercise stress testing, stress echocardiography, and cardiac magnetic resonance imaging were performed in 76%, 8%, and 20%, respectively, of the cohort. None identified findings consistent with reversible coronary ischemia. Three patients had sudden cardiac arrest (1 death) after surgery without an identified residual coronary abnormality. ConclusionsTransthoracic echocardiography, with carefully designed coronary imaging protocols, can be diagnostic in accurately identifying intramural AAOCA in pediatric patients. Unroofing can be performed safely with no early morbidity, but symptoms can persist (including rare life-threatening events) without evidence of ischemia by postoperative provocative testing.

Usefulness of Routine Coronary CT Angiography in Patients with Transposition of the Great Arteries After an Arterial Switch Operation

Coronary complications in patients with transposition of the great arteries (TGA) after an arterial switch operation (ASO) are relatively rare, but of all the possible postoperative adverse events, they are potentially the most dangerous. The fate of the coronary arteries, which are transplanted during the neonatal ASO, remains uncertain. There is also no consensus regarding their postoperative evaluation, especially in asymptomatic patients. The aim of this study was to present the early results of routinely performed coronary computed tomography angiography (CCTA) in asymptomatic adolescents and young adults with TGA after an ASO. An initial series of 50 CCTAs performed in asymptomatic patients with TGA after an ASO were evaluated. In each case, a detailed examination of the coronary anatomy, its relationship to the surrounding structures, its exact position in the neoaortic sinus, and the presence of significant coronary abnormalities was performed. The CT scans revealed significant coronary abnormalities in 12 asymptomatic patients: three had acute proximal angulation and stenosis, four had an intra-arterial course, seven had a muscular bridge, one had a left anterior descending artery with an intramuscular course, and one had coronary fistulas to the pulmonary arteries. Additionally, in 25 patients, proximal acute angulation of at least one coronary artery was detected, and four of them had a high ellipticity index. Most of the potentially severe anatomical features were related to the left coronary artery or the left anterior descending artery. CCTA routinely performed on asymptomatic patients with TGA after an ASO provides accurate and useful information for postoperative management. The frequency of coronary anomalies and potentially dangerous anatomical features in this group of patients is high, and their impact on postoperative follow-up remains unknown.

Non-muscle myosin IIB (Myh10) is required for epicardial function and coronary vessel formation during mammalian development.

The coronary vasculature is an essential vessel network providing the blood supply to the heart. Disruptions in coronary blood flow contribute to cardiac disease, a major cause of premature death worldwide. The generation of treatments for cardiovascular disease will be aided by a deeper understanding of the developmental processes that underpin coronary vessel formation. From an ENU mutagenesis screen, we have isolated a mouse mutant displaying embryonic hydrocephalus and cardiac defects (EHC). Positional cloning and candidate gene analysis revealed that the EHC phenotype results from a point mutation in a splice donor site of the Myh10 gene, which encodes NMHC IIB. Complementation testing confirmed that the Myh10 mutation causes the EHC phenotype. Characterisation of the EHC cardiac defects revealed abnormalities in myocardial development, consistent with observations from previously generated NMHC IIB null mouse lines. Analysis of the EHC mutant hearts also identified defects in the formation of the coronary vasculature. We attribute the coronary vessel abnormalities to defective epicardial cell function, as the EHC epicardium displays an abnormal cell morphology, reduced capacity to undergo epithelial-mesenchymal transition (EMT), and impaired migration of epicardial-derived cells (EPDCs) into the myocardium. Our studies on the EHC mutant demonstrate a requirement for NMHC IIB in epicardial function and coronary vessel formation, highlighting the importance of this protein in cardiac development and ultimately, embryonic survival.

Late gadolinium enhancement on cardiac magnetic resonance imaging is associated with coronary endothelial dysfunction in patients with dilated cardiomyopathy.

Myocardial fibrosis and coronary endothelial dysfunction are important determinants of outcome in patients with heart failure. However, the relationship of these factors in patients with dilated cardiomyopathy (DCM) is not fully understood. This study aimed to investigate the relationship between endothelium-dependent coronary vasomotor abnormality and late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) in patients with DCM. We examined 38 consecutive patients with DCM. All patients underwent CMR and the acetylcholine (ACh) provocation test using cardiac catheterization. During the ACh provocation test, we sampled blood simultaneously from the coronary sinus and aortic root to compare lactate concentrations, and quantified coronary blood flow volume using an intracoronary Doppler-tipped guidewire. LGE was detected in 17 (44.7%) patients. The lactate extraction ratio (LER) in the ACh provocation test was significantly decreased in the LGE-positive group (before vs after ACh, 18.6±13.6 vs -13.3±24.8%; p<0.001) and in the LGE-negative group (before vs after ACh, 14.2±19.5 vs 3.3±16.2%; p=0.02). The rate of patients with an LER<0% (indicating myocardial lactate production due to myocardial ischemia) was significantly higher in the LGE-positive group than in the LGE-negative group [12 (70.6%) vs 7 (33.3%); p=0.02]. Multivariable logistic regression analysis showed that a post-ACh LER<0% was a significant predictor of LGE positivity (odds ratio 7.75; 95% confidence interval 1.37-43.68; p=0.02). In conclusion, ACh-provoked coronary vasomotor abnormality is associated with myocardial fibrosis in patients with DCM. These results suggest that coronary endothelial dysfunction is involved in myocardial fibrosis and worsening heart failure concomitant with DCM.

Prediction of regional coronary perfusion abnormalities using regional two-dimensional strain in non-ST elevation acute coronary syndrome

Prediction of regional coronary perfusion abnormalities using regional two-dimensional strain in non-ST elevation acute coronary syndrome

Retrospective study of the impact of unrecognised Kawasaki disease, coronary aneurysm and ectasia

BackgroundCoronary artery aneurysms resulting from unrecognised or untreated Kawasaki Disease (KD) have thrombotic and stenotic potential leading to myocardial infarction. We aimed to characterise the prevalence and outcomes of patients presenting to a UK-based adult tertiary cardiology service with angiographic evidence of aneurysm or ectasia. MethodsRetrospective review was undertaken of reports and original coronary angiograms in all patients under 50years old undergoing coronary angiography between 2011 and 2015. Aneurysm/ectasia were defined by calibre discrepancy >1.5× and the number of segments involved. Aneurysms were categorised as being probable, or unlikely to represent unrecognised KD. ResultsAneurysm or ectasia was observed in 49 of 1578 patients (3.7%). Eleven patients had angiographic findings consistent with probable antecedent KD (0.8%), a further 18 patients had evidence of coronary artery aneurysm (1.4%) and ectasia was observed in 20 patients (1.5%). The commonest mode of presentation was ST elevation myocardial infarction, observed in 71.4% of patients. Review of angiogram written reports demonstrated a spurious application of the terms aneurysm and ectasia, with a sensitivity of 10.3% & 55.0%, and a positive predictive value of reporting of 50.0% & 52.4%, respectively. ConclusionsAs the first UK study to characterise the angiographic prevalence of Kawasaki Disease, both aneurysm and ectasia were observed with a relatively low frequency. However, their presence is associated with high-risk acute ischaemic presentations. Furthermore, we demonstrated a poor level of reporting of coronary abnormalities and advocate an increased awareness of Kawasaki disease and coronary aneurysm/ectasia amongst adult cardiologists.

Kawasaki Disease Complicated With Macrophage Activation Syndrome: A Systematic Review.

Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is a rare and potentially fatal complication of Kawasaki disease (KD). We report 2 cases, performed a literature search, and analyze the characteristics of MAS associated with KD. A total of 69 patients were evaluated, 34 reported the date of the diagnosis of MAS and KD, 6% had a diagnosis of MAS before KD, 21% had a simultaneous presentation, and 73% had the diagnosis of MAS after KD. Different treatment approaches were observed with corticosteroids administered in 87%, cyclosporine in 49%, etoposide (VP-16) in 39%, and monoclonal anti-TNF in 6% of cases. Coronary abnormalities were especially high in this group of patients (46%) and 9 patients died (13%). The persistence of fever with splenomegaly, hyperferritinemia, thrombocytopenia, and elevated aspartate aminotransferase (AST) should prompt the consideration of MAS complicating KD.

Acute chest pain in an anxious woman

A 63 years old woman with past medical history of hypertension, osteoporosis, dyslipidaemia and anxiety, was admitted in the emergency room with acute chest pain after a family discussion, she presented oppressive retrosternal pain 7/10 of intensity, that irradiated to the left shoulder and the left arm, she was diaphoretic, her vital signs were normal and the physical examination was unremarkable, there were no abnormalities in the electrocardiogram and the blood tests showed normal haemogram as well the renal, liver and thyroid function, the troponin I was 6.9 μg/ml (normal value 0,04 μg/ml), a diagnosis of Non-ST Segment Elevation Myocardial Infarction (NSTEMI) was admitted, and an angiography was scheduled. In the meanwhile the chest pain relieved with nitroglycerine administration and anti-ischaemic treatment and the patient stayed haemodynamically stable. What is the diagnosis? Fig. 1 shows the ventriculography pattern of this patient that is typically known as Tako tsubo disease (TTD) or apical ballooning with a static apex and compensatory hipercontractility of the basal segments, without coronary abnormalities. The emotional stress has been related to this entity, causing a coronary vasospasm and the subsequent myocardial ischemia, due to these physiopathological facts, the clinical symptoms and laboratory findings of this illness look like the acute coronary syndromes. Some author had proposed the association between neoplasic diseases and TTD, mainly in patients that presented this condition without any stressing stimuli, they suggest that the catecholamines liberation by some tumours may be involved in the pathogenesis of those cases [[1]Sattler K. El-Battrawy I. Lang S. Zhou X. Schramm K. Tülümen E. et al.Prevalence of cancer in Takotsubo cardiomyopathy: short and long-term outcome.Int. J. Cardiol. 2017; https://doi.org/10.1016/j.ijcard.2017.02.093Abstract Full Text Full Text PDF Scopus (36) Google Scholar]. The prognosis of this condition is generally benign, although it may be complicated with malignant arrhythmias, cardiac rupture and cardiac tamponade [[1]Sattler K. El-Battrawy I. Lang S. Zhou X. Schramm K. Tülümen E. et al.Prevalence of cancer in Takotsubo cardiomyopathy: short and long-term outcome.Int. J. Cardiol. 2017; https://doi.org/10.1016/j.ijcard.2017.02.093Abstract Full Text Full Text PDF Scopus (36) Google Scholar]. Although the clinical and radiologic findings are typical, this illness only can be confirmed with after the myocardial function normalization in the follow up [[2]Giusca Sorin Eisele Tom Nunninger Peter Benedikt Münz G.K. Aborted sudden cardiac death in a female patient presenting with Takotsubo-like cardiomyopathy due to epicardial coronary vasospasm.Case Reports Cardiol. 2017; https://doi.org/10.1155/2017/7875240Crossref PubMed Google Scholar]. In this case the patient had completely recovered the cardiac function after 3 months of follow up under medical treatment with beta-blockers, ACE inhibitors as suggested in the literature [[2]Giusca Sorin Eisele Tom Nunninger Peter Benedikt Münz G.K. Aborted sudden cardiac death in a female patient presenting with Takotsubo-like cardiomyopathy due to epicardial coronary vasospasm.Case Reports Cardiol. 2017; https://doi.org/10.1155/2017/7875240Crossref PubMed Google Scholar]. Despite the available evidence many doubts remain about the appropriated follow up and prognosis of these patients [[3]Drakopoulou M.I. Gatzoulis M.A. Is Takotsubo syndrome a benign condition?.Hell. J. Cardiol. 2017; https://doi.org/10.1016/j.hjc.2017.03.009Crossref PubMed Scopus (2) Google Scholar]. The authors want to highlight this so called “benign” entity that can lead to fatal complications.

Interobserver variability in the classification of congenital coronary abnormalities: A substudy of the anomalous connections of the coronary arteries registry.

The diagnosis of anomalous connections of the coronary arteries (ANOCOR) requires an appropriate identification for the management of the patients involved. We studied the observer variability in the description and classification of ANOCOR between a nonexpert group of physicians and a group of expert physicians, using the ANOCOR cohort. Consecutive patients identified by 71 referring cardiologists were included in the ANOCOR cohort. Anomalous connection was diagnosed by invasive and/or computed tomography coronary angiography. Angiographic images were reviewed by an angiographic committee with experience in this field. Both investigators and angiographic committee filled out a questionnaire to classify each anomaly with the type of coronary artery involved, the site of anomalous connection, and the initial course. Observer variability between investigators and angiographic committee was assessed by κ statistics. Anomalous connection with a preaortic course was defined as at-risk. Among 472 patients of the ANOCOR cohort, 496 abnormalities were identified with a preaortic course present in 31%. The agreement for the type of artery was excellent (κ = 0.92, 95% CI = 0.86-0.98, P < .05), while the agreement for the site of anomalous connection was moderate (κ = 0.50, 95% CI = 0.42-0.58, P < .05), and the agreement for the initial course was only fair (κ = 0.32, 95% CI = 0.28-0.37, P < .05). Observer agreement for the identification of at-risk forms was moderate (κ = 0.497, 95% CI = 0.40-0.59, P < .05). Observer variability in the assessment of anomalous connection of the coronary arteries between nonexperienced and experienced physicians can be significant. We found that expert physicians provide a more robust classification in comparison with nonexpert physicians. Therefore, referral to physicians with a relevant experience should be considered, especially if an anomaly at-risk is suspected.

Echocardiographic Evaluation of Coronary Abnormalities and Cardiac Function in a Murine Model of Kawasaki Disease Using High-frequency Ultrasound.

Background:Murine model of coronary arterial inflammation has been widely accepted as an animal model of and used in Kawasaki disease (KD). This study sought to evaluate the developmental changes of coronary arteries and cardiac function in a murine model of KD with a high-frequency ultrasound system and to provide evidence for the preparation of the model of KD.Methods:Lactobacillus casei cell wall extract was prepared and injected into C57BL/6 mice intraperitoneally (i.p.) to induce KD. A total of 120 mice were grouped into three groups. The intravenous immunoglobulin (IVIG) treatment group was i.p. injected with IVIG (2 g/kg), while the KD model and normal control groups were i.p. injected with 0.5 ml of phosphate buffered solution on day 5. All high-resolution echocardiography detection of mouse heart was performed by the same senior technician. Animal echocardiography was performed by measuring the coronary artery dimensions and cardiac function on days 0, 7, 14, 28, and 56 (high-resolution small animal ultrasound [Vevo770 pattern; VisualSonic, Canada] with broadband probe [RMVTM707B; frequency, 30 mHz; depth of focus, 1.2 cm]) which were measured and analyzed with Vevo770 software.Results:Pathological studies revealed focal inflammatory infiltrate asymmetrically distributed around the coronary artery trunk in the KD model group. Echocardiographic study including coronary dimension and cardiac function measurements was successfully performed in all subjects. The KD model and IVIG treatment groups showed left coronary artery dilation on days 7, 14, 28, and 56. The diameter of left coronary artery in the KD model group (0.53 ± 0.09 mm; 0.36 ± 0.07 mm; 0.34 ± 0.05 mm; 0.34 ± 0.04 mm) was significantly larger than those of IVIG treatment group (0.22 ± 0.02 mm; 0.28 ± 0.03 mm; 0.26 ± 0.03 mm; 0.27 ± 0.05 mm; 0.26 ± 0.03 mm; all P < 0.01) and the normal control group (0.21 ± 0.02 mm; 0.22 ± 0.03 mm; 0.22 ± 0.02 mm; 0.23 ± 0.02 mm; 0.27 ± 0.04 mm; all P < 0.01) on days 7, 14, 28, and 56. No significant differences were observed in the measurements of cardiac function among the groups on days 0, 7, 14, 28, and 56 (all P > 0.05).Conclusions:Echocardiography could identify the consecutive changes of coronary artery in KD mice. Echocardiography is more convenient and direct in evaluating the coronary abnormalities in this animal model.

Stress echocardiography: An overview for use in pediatric and congenital cardiology.

Currently, the role of stress echocardiography primarily resides in diagnosing acquired coronary artery disease (CAD) in adults. Besides an increasing concern for traditional CAD in young patients due to obesity and other chronic pediatric diseases, there is also a growing population of adolescents and young adults with "at risk" coronary arteries due to: reimplanted coronaries in congenital heart disease, anomalous origin of the native coronary arteries, coronary abnormalities in Kawasaki's disease, and posttransplant coronary vasculopathy. Stress echocardiography is well suited for routine screening and monitoring in these patients. Also, due to the ability of stress echocardiography to provide real-time cardiac imaging, it is useful in areas beyond coronary ischemia. Utilizing Doppler derived velocities and pressure gradients, one is able to further evaluate and risk-stratify patients with valvular heart disease. In addition, stress echocardiography is useful in evaluating other areas including ventricular and coronary reserve. The benefits of stress echocardiography are it is: readily available, portable, and relatively cheap. It can be performed without sedation or radiation exposure which becomes very important in younger patients that require periodic monitoring. Stress echocardiography can also evaluate functional abnormalities instead of relative perfusion defects. Overall, stress echocardiography is currently an underutilized imaging modality that has a wide, and expanding, range of application in the practice of pediatric and congenital cardiology.