A Rare Case of Cardiac Myxoma With Multiple Feeding Vessels From the Right Coronary Artery and the Left Circumflex Artery

To the Editor, Congenital coronary artery anomalies are infrequently seen during coronary angiographic study, reportedly occurring in 0.64% to 1.3% of patients [1]. Most patients with a congenital coronary artery anomaly are asymptomatic; in rare cases, they may present with chest pain and have myocardial ischemia or other life-threatening conditions. We present a patient with the right coronary artery (RCA) originating from the terminal branch of the left circumflex (LCx) artery who presented with chest pain and was found to have a moderate stenotic lesion in the distal LCx artery. Fractional flow reserve (FFR) allowed us to evaluate this moderate stenotic lesion in a single coronary artery, and to treat with medical therapy only. A 63-year-old man was admitted to the cardiology unit with atypical chest pain. He had hypertension as coronary heart disease risk factor. His vital signs were stable, and physical examination showed no abnormalities. A resting electrocardiogram demonstrated a convex-shaped ST-segment elevation in the precordial lead without reciprocal change. Laboratory findings including cardiac markers showed no abnormalities. Echocardiography revealed normal left ventricular morphology, no regional wall motion abnormality, and a diastolic dysfunction of relaxation abnormality. The patient underwent coronary computed tomographic (CT) angiography. It demonstrated the presence of a single coronary artery with the LCx artery continuing in the course of the RCA and minimal possibility of chronic total occlusion (CTO) of the RCA ostium with a collateral supply from the LCx artery (Fig. 1). It also showed 60% diameter stenosis in the distal LCx artery (Fig. 1). Figure 1 Coronary computed tomography (CT) angiography findings. (A, B) Volume rendering CT imaging and (C) multiplanar reformation image showed a single coronary artery in which the right coronary artery (RCA) originated from a branch of the left circumflex (LCx) ... To differentiate single coronary artery from CTO of the RCA ostium and confirm the degree of stenosis in the distal LCx artery, we performed coronary angiography. Selective left coronary angiography displayed normal origin and course of the left main, LCx, and left anterior descending arteries. The LCx artery did not terminate after reaching the crux; giving rise to the posterior descending branch, it coursed in the right atrioventricular groove as if it were the RCA and ended when it reached the right sinus of Valsalva (Fig. 2A). There was a stenosis of 60% diameter in the distal part of the LCx proper where the RCA arose (Fig. 2B). Attempts to engage the right coronary catheter into the RCA ostium were futile, and aortography obtained in the left anterior-oblique projection displayed the absence of the ostium of the RCA in the right sinus of Valsalva (Fig. 2C). To decide the functional significance of the stenosis in the distal LCx artery, we performed an FFR study with a pressure wire and found that there was no critical functional stenosis (FFR, 0.9) (Fig. 2D). The patient was discharged with medications of aspirin, statin, nitrate, and β blocker and free from chest pain. Figure 2 (A) The single coronary artery visualized in the caudal angulation. (B) The left anterior oblique caudal view of selective left coronary angiography showed a stenosis of 60% in the distal left circumflex (LCx) proper artery (arrow). (C) Aortogram showed ... A single coronary artery is an extremely rare congenital anomaly characterized by a single coronary artery ostium from an aortic sinus, and is seen in only 0.024% to 0.066% of patients who undergo conventional coronary angiography [1]. A single coronary artery is usually asymptomatic and has a benign course, and these patients have a normal life expectancy. The presence of a single coronary artery may bring about myocardial ischemia because of the inability to adequately sustain normal coronary circulation [2]. FFR is a physiologic parameter that can be readily measured during the invasive procedure and can evaluate the functional significance of coronary stenosis. In particular, FFR plays an important role when making a decision for percutaneous coronary intervention (PCI) of angiographically moderate stenosis and assessing accurately the functional consequences of a given coronary stenosis with unclear hemodynamic significance [3]. In addition, the clinical outcome of patients in whom PCI is deferred because FFR indicated no hemodynamically significant stenosis was very favorable [4]. In this case, FFR was a good assessment modality because the lesion of interest in the LCx artery was a part of a single coronary artery that supplied both the LCx artery and RCA territories. With FFR, we could make a decision for deferred PCI and choose medical treatment, even though coronary CT angiography and coronary angiography showed moderate stenosis in the LCx artery. In conclusion, FFR could successfully evaluate the functional significance of a moderate stenotic lesion in a single coronary artery where the RCA originated from the terminal portion of the LCx artery.

Immunoglobulin G4 (IgG4)-related disease is rare. It is characterized by marked elevation in serum IgG4 concentration and infiltration of IgG4-positive plasma cells into a variety of tissues,1 particularly the adventitia surrounding great arteries. Here, we report the case of a patient who suffered an acute myocardial infarction and eventually died of a ruptured thoracic aorta as a result of IgG4-related disease. An 84-year-old man was admitted to our hospital complaining of new-onset substernal compressing pain. The diagnosis of acute posterolateral myocardial infarction was made by means of ECG, ultrasound imaging, and the presence of elevated serum creatine phosphokinase and troponin-I levels. Subsequently, urgent coronary angiography showed a huge coronary aneurysm (21 mm at its maximal diameter) accompanied by thrombus in the left circumflex artery (Figure 1 and online-only Data Supplement Movie I). Insignificant stenosis could be seen proximally in several branching arteries, and the right and left anterior descending arteries appeared patent (Figure 2A and online-only Data Supplement Movie II). Thrombus was successfully aspirated from the left circumflex artery, and a stent was subsequently implanted. Figure 1. Left circumflex coronary artery (LCX) images obtained by invasive coronary angiography (right anterior oblique caudal view). A huge coronary aneurysm (21 mm at its maximal diameter) is evident (dotted arrows), with thrombus at the distal end (arrow). See online-only Data Supplement Movie I. Figure 2. Right coronary artery (RCA) images obtained by invasive coronary angiography, coronary computed tomography angiography (CCTA), and intravascular ultrasound (IVUS) imaging. A , RCA image (left anterior oblique view). There was no significant stenosis. See online-only Data Supplement Movie II. B , Curved multiplanar reconstruction image …

Atherothrombosis is a systemic disease of the vessel wall that causes distinct clinical manifestations, depending on the affected circulatory bed and the characteristics of the individual lesions.1 These lesions may be quite heterogeneous.1 Thus, the clinical manifestations of atherothrombosis of the coronary arteries, of the arteries supplying the central nervous system, of the aorta, and of the peripheral circulation can be significantly different. Disruption-prone plaques in the coronary arteries, the so-called “vulnerable plaques,” tend to have a thin fibrous cap (cap thickness ≈65 to 150 μm) and a large lipid core (American Heart Association [AHA] plaque type IV-Va). Acute coronary syndromes often result from disruption of a modestly stenotic vulnerable plaque, not visible by x-ray angiography, which results in a thrombotic complication (AHA plaque type VI). During its evolution, a type Va plaque may also become fibrotic (AHA plaque type Vc) or calcified (AHA plaque type Vb).2,3⇓ In contrast to coronary artery vulnerable plaques characterized by high lipid content and a thin fibrous cap, high-risk plaques of the carotid arteries tend to be fibrotic and severely stenotic.3 ### Imaging of Atherothrombotic Disease Because there is striking heterogeneity in the composition of human atherothrombotic plaques, even within the same individual, reliable noninvasive imaging tools that can detect early atherothrombotic disease in the various regions and characterize the composition of the plaques are clinically desirable.3 Such imaging tools would improve our understanding of the pathophysiological mechanisms underlying atherothrombotic processes and allow us to better risk-stratify the disease. Additionally, such tools may permit optimal tailoring of treatment and allow direct monitoring of the vascular response. Presently, a number of imaging modalities are employed to study atherosclerosis; most identify luminal diameter or stenosis, wall thickness, and plaque volume.3 Two noninvasive imaging modalities, computed tomography and MRI, have been introduced to the study …

IntroductionAtrial myxoma is the most common benign primary cardiac tumour with 75% of myxoma originates from left atrium. It comprises of 50–85% of all primary cardiac tumour and incidence of...

Cardiac computed tomography (CT) and positron emission tomography (PET) are emerging as powerful noninvasive imaging tools for the evaluation of atherosclerosis in patients with known or suspected coronary artery disease (CAD). Unlike invasive coronary angiography, CT coronary angiography (CTA) not only assesses disease within the coronary lumen but can also provide direct qualitative and quantitative information about nonobstructive atherosclerotic plaque burden within the vessel wall. Thus, it is possible that CTA-based patient evaluation may provide more clinically relevant information on which to base risk assessments compared with conventional “lumenography.” On the other hand, PET is rapidly growing as a powerful and efficient alternative to conventional single-photon emission CT (SPECT) imaging to evaluate regional myocardial perfusion and metabolism in patients with CAD. In addition, PET scanners are now being converted to hybrid PET/CT devices, which, in the setting of CAD, offer the potential for a comprehensive noninvasive cardiac evaluation of anatomy and function. This review will discuss current and potential future applications of cardiovascular CT, PET, and hybrid PET/CT, with a particular focus on ischemic heart disease. The information provided by noninvasive imaging generally falls into 1 of 3 categories: myocardial perfusion, left ventricular (LV) function, or coronary artery anatomy. The clinical utility, value, and role of a noninvasive modality are based on 2 test characteristics: What type of information is provided (eg, stress perfusion, stress and/or rest LV function, coronary anatomy), and what is the accuracy of the information provided. For example, SPECT and PET both provide stress and rest perfusion information, but the latter will be a superior clinical tool if the imaging data better represent the actual defect size and are subject to less artifact. The advantage of PET over SPECT will be further enhanced, as will be discussed later, if it provides additional clinically relevant information not …

Cardiac allograft vasculopathy (CAV) is common, with a prevalence of 52% at 10 years after transplantation, and represents a leading cause of death beyond the first year, responsible for approximately 15% of deaths annually.1 It is characterized by diffuse and concentric intimal proliferation, typically involving the intramural as well as epicardial coronary arteries. Its diagnosis is difficult to establish clinically because of denervation of the transplanted heart. Consequently, it presents late with silent myocardial infarction, progressive heart failure, or arrhythmic sudden death.2 Screening is therefore required for its early detection. Although coronary intravascular ultrasound (IVUS) is considered the gold-standard technique for detecting the anatomic features of CAV (Table 1), its broad clinical use in this context is limited by cost and lack of widespread expertise, and its evaluation is limited to epicardial vessels.3 Coronary angiography, performed annually or biannually, remains the most common clinical screening method.4 However, because of the diffuse nature of CAV with a lack of normal reference segments and the relatively late occurring luminal narrowing, the sensitivity of angiography is as low as 30% when compared with IVUS (Figure 1).5 As a result, complications frequently occur before disease is evident angiographically.6 Furthermore, angiography is associated with significant albeit uncommon complications (overall complication rate, 7.4/1000 procedures, including rates of 0.65/1000, 1.6/1000, and 0.72/1000 for cerebrovascular accidents, vascular complications, and death, respectively), is disliked by transplant recipients, is costly, and repeated studies are associated with an important cumulative radiation dose.7 View this table: Table 1. Stanford Classification of CAV Severity on IVUS Figure 1. Invasive assessment of cardiac allograft vasculopathy (CAV) in a patient with severe disease, highlighting the limited sensitivity of conventional coronary angiography. Although no left anterior descending (LAD) flow-limiting stenoses are seen on angiography ( A ), intravascular ultrasound ( B ) shows significant intimal thickening, measuring up to …

Prior studies demonstrating shorter length of stay (LOS) from coronary computed tomography angiography (CCTA) relative to stress testing in emergency department (ED) patients have not considered time of patient presentation. The objectives of this study were to determine whether low-risk chest pain patients receiving stress testing or CCTA have differences in ED plus observation unit (OU) LOS and if there are disparities in testing modality use, based on the time of patient presentation to the ED. The authors examined a cohort of low-risk chest pain patients evaluated in an ED-based OU using prospective and retrospective OU registry data. During the study period, stress testing and CCTA were both available from 08:00 to 17:00 hours. CCTA was not available on weekends, and therefore only subjects presenting on weekdays were included. Cox regression analysis was used to model the effect of testing modality (stress testing vs. CCTA) on OU LOS. Separate models were fit based on time of patient presentation to the ED using 4-hour blocks beginning at midnight. The primary independent variable was testing modality: stress testing or CCTA. Age, sex, and race were included as covariates. Logistic regression was used to model testing modality choice by time period adjusted for age, sex, and race. Over the study period, 841 subjects presented Monday through Friday. Median LOS was 18.0 hours (interquartile range [IQR] = 11.7 to 22.9 hours). Objective cardiac testing was completed in 788 of 841 (94%) patients, with 496 (63%) receiving stress testing and 292 (37%) receiving CCTA. After age, race, and sex were adjusted for, patients presenting between 08:00 and 11:59 hours not only had a shorter LOS associated with CCTA (p < 0.0001), but also had a greater likelihood of being tested by CCTA (p = 0.001). None of the other time periods had significant differences in LOS or testing modality choice for CCTA relative to stress testing. In an OU setting with weekday and standard business hours CCTA availability, CCTA testing was associated with shorter LOS among low-risk chest pain patients only in patients presenting to the ED between 08:00 and 11:59 hours. That time period was also associated with a greater likelihood of being tested by CCTA, suggesting that ED providers may have intuited the inability of CCTA to shorten LOS during other times.

Coronary stenosis with lumen diameter reduction greater than 50% is recognized as coronary artery disease (CAD) [1–4]. Fractional flow reserve (FFR) is an epicardial lesion-specific parameter to determine the functional coronary stenosis, which is determined by pressure difference and resistance [5–8]. Previous studies have demonstrated that FFR guided percutaneous coronary intervention (PCI) could improve outcomes compared with anatomical invasive coronary angiography (ICA) guided PCI [6–10]. This study aims to overcome the deficiencies of invasive FFR and create a novel noninvasive FFR (FFRni). A 70-year-old female patient, diagnosed with CAD, stable angina pectoris, cardiac function III, essential hypertension III (very high-risk group), with blood pressure of 140/85 mm Hg, was enrolled in as a pilot study. Coronary computed tomography angiography (CCTA) was performed using multi-detector computed tomography scanners (Lightspeed 16 Pro). Original images were spilt into thin layers and directly exported into imaging control software MIMICS and processed to form an image sequence. Three-dimensional (3D) geometric models of the narrow coronary arteries including the right coronary artery (RCA), left anterior descending coronary (LAD) and left circumflex artery (LCX) were reconstructed and exported. Then, the geometric model was meshed with 3D Flotran elements in ANSYS software. Finite element analysis (FEA) was applied to analyze the velocity and pressure distribution of selected coronary arteries. Given boundary conditions including average velocity measured by transthoracic Doppler echocardiography with an ultrasound system (Sequoia C256) were applied in an inlet of 3D model. By setting the proper iteration time, the calculation went smoothly. ICA, as the “gold standard”, was performed with standard techniques. Invasive coronary angiography indicated that there was around 80% stenosis in the proximal RCA (Figure 1A). Mild (about 30%) stenosis in the proximal LAD and a diffuse lesion of the LCX (the narrowest was 80%) could also be quantified (Figure 2A). Additionally, the coronal section of CCTA images demonstrated moderate (around 60%) obstructive stenosis in the proximal RCA (Figure 1B). The cross-sectional images indicated 30% stenosis in the proximal and first branch segment of the LAD and diffuse patchy calcified plaques in the LCX leading to intermediate (50%) stenosis (Figure 2B). The diagnostic performance of FFRni was generally consistent with the results of ICA and CCTA. The values of proximal RCA, LAD and distal LCX were 0.73, 0.76 and 0.64, respectively, which suggested the severity of lesion-specific functional ischemia in distal myocardium with 0.75 as the cutoff value (Figures 1C and ​and2C2C). Figure 1 Anatomically obstructive stenosis of right coronary artery (RCA) with a lesion causing ischemia. A – Invasive coronary angiography indicates that stenosis is about 80% in the proximal RCA (black arrow). B – Multi-planar reformat of coronary ... Figure 2 Anatomically obstructive stenosis of left anterior descending coronary (LAD) and left circumflex artery (LCX) with/without functional ischemia. A – Invasive coronary angiography indicates that there exist 30% stenosis in proximal LAD (black arrow) ... Recently, FFR computed from CCTA (FFRCT) was reported. A good correlation between FFRCT and invasive FFR was certified through a randomized clinical controlled trial on 159 vessels in 103 patients [11]. The differences in the calculation process of FFRCT and FFRni in our study mainly reflect the following factors. During FFRCT calculation, coronary flow and pressure are unknown. A method to couple lumped parameter models of the microcirculation to the outflow boundaries of the 3D model was adopted. As a result of the cumbersome workload, it takes approximately 5 h/exam. We utilized FEA of the Flotran CFD module to solve the hemodynamic calculation under given boundary conditions. Therefore, it can greatly reduce the computation time to 3 h/exam. The more relaxed equipment requirements and faster inspection time guarantee potential clinical application of FFRni. Fractional flow reserve is a well-evaluated functional index to assess the ischemic significance of coronary lesions, helping making the decision of revascularization [12–14]. Nevertheless, the invasiveness and costliness are two major reasons restricting its further application. Finite element analysis and CFD over digital 3D modeling were applied in this pilot study and created a novel method to evaluate functional coronary stenosis by FFRni, which showed good consistency with ICA and CCTA. The superiority of no invasiveness and cost-effectiveness establishes the foundation of FFRni for its further applications in clinical practice. However, a large randomized clinical controlled trial assessed by FFRni compared with invasive FFR is urgently needed.

Cardiac Imaging 2040.

Coronary artery visibility on coronary CT angiography has rarely been investigated in young children with Kawasaki disease. This retrospective study was performed to quantitatively evaluate and compare coronary artery visibility with sufficient quality to measure it on coronary CT angiography among younger and older children with Kawasaki disease. Seventy-eight consecutive children with Kawasaki disease who underwent coronary CT angiography were divided into two groups: group 1 (age ≤ 6years; n = 37) and group 2 (age > 6years and < 18years; n = 41). The visibility of the right coronary artery, left anterior descending artery, and left circumflex artery was quantitatively evaluated by dividing the length of the assessable coronary artery by the length of the corresponding groove, and compared between the two groups. The coronary artery visibility in group 1 was significantly lower than that in group 2 for the right coronary artery (77.8 ± 26.3% vs. 94.2 ± 13.6%, p < 0.002) and left anterior descending artery (54.8 ± 19.5% vs. 69.6 ± 21.3%, p < 0.003, but the difference was not significant for the left circumflex artery (43.7 ± 23.1% vs. 43.9 ± 26.7%, p > 0.9). In both groups, the visibility of the right coronary artery was the highest, followed by those of the left anterior descending artery and left circumflex artery. Compared with older children with Kawasaki disease, younger children with Kawasaki disease demonstrate significantly lower visibility of the right coronary artery and left anterior descending artery on coronary CT angiography. In contrast, the visibility of the left circumflex artery showed no significant difference between younger and older children with Kawasaki disease.

See also the editorial by Schoepf and Decker in this issue.

To compare the diagnostic value of coronary CT angiography (CCTA) with use of 2 image postprocessing methods (CCTA_S) and (CCTA_OS) and original data (CCTA_O) for the assessment of heavily calcified plaques.Fifty patients (41 men, 9 women; mean age 61.9 years ± 9.1) with suspected coronary artery disease who underwent CCTA and invasive coronary angiography (ICA) examinations were included in the study. Image data were postprocessed with “sharpen” and smooth reconstruction algorithms in comparison with the original data without undergoing any image postprocessing to determine the effects on suppressing blooming artifacts due to heavy calcification in the coronary arteries. Minimal lumen diameter and degree of stenosis were measured and compared between CCTA_S, CCTA_OS, and CCTA_O with ICA as the reference method. The area under the curve (AUC) by receiver-operating characteristic curve analysis (ROC) was also compared among these 3 CCTA techniques.On a per-vessel assessment, the sensitivity, specificity, positive predictive value and negative predictive value, and 95% confidence interval (CI) were 100% (95% CI: 89%, 100%), 33% (95% CI: 22%, 45%), 41% (95% CI: 30%, 53%), 100% (95% CI: 85%, 100%) for CCTA_O, 94% (95% CI: 79%, 99%), 66% (95% CI: 54%, 77%), 57% (95% CI: 43%, 70%), and 95% (95% CI: 85%, 99%) for CCTA_S, 94% (95% CI: 79%, 99%), 44% (95% CI: 32%, 57%), 44% (95% CI: 32%, 57%), and 97% (95% CI: 79%, 99%) for CCTA_OS, respectively. The AUC by ROC curve analysis for CCTA_S showed significant improvement for detection of >50% coronary stenosis in left anterior descending coronary artery compared to that of CCTA_OS and CCTA_O methods (P < 0.05), with no significance differences for detection of coronary stenosis in the left circumflex and right coronary arteries (P > 0.05).CCTA with “sharpen” reconstruction reduces blooming artifacts from heavy calcification, thus, leading to significant improvement of specificity and positive predictive value of CCTA in patients with heavily calcified plaques. However, specificity is still moderate and additional functional imaging may be needed.

Purpose: To assess the coronary bypass grafts patency and the repeat revascularization rate, six months after coronary artery bypass grafting (CABG). Methods: We prospectively enrolled 145 consecutive patients undergoing isolated CABG between June 2014 and June 2016. We performed at 6 months of follow up a coronary computed tomography angiography (CTA) in patients whose stress tests were negative and an invasive coronary angiography (ICA) in the opposite case. Results: A total of 134 CTA and 11 ICA were performed, allowing the analysis of 321 grafts, including 143 left internal thoracic arteries (LITA), 89 right internal thoracic arteries (RITA) and 89 saphenous veins grafts (SVG). The average graft patency was 95.1% for LITA, 84.3% for RITA and 64% for SVG. The best patencies were obtained when these grafts were anastomosed to the left anterior descending artery (LAD): 96.3% for LITA, and 87.5% for RITA. SVG patency was homogeneous whether between the main right coronary artery and its branches (63.4% versus 65% respectively. p = 1), or between circumflex and RCA (72.7% versus. 63.9% respectively. p=0.6). On the right and circumflex coronary arteries, the patency of the SVG was significantly lower than that of RITA (66.26% versus 83.95% respectively, p = 0.011). At 6 months of follow up, the repeat revascularization rate was 2.07% (n=3/145). Conclusions: 6 months after CABG, RITA and LITA had good patencies especially on LAD, while SVG was occluded in almost a third of cases. On the circumflex and right coronary arteries, SVG patency was significantly lower than that of RITA. Keywords: Coronary Artery Bypass; Exercise Testing; Coronary Angiography; Computed Tomography Angiograph

Major advances in the field of pediatric cardiology and cardiac surgery over the last several decades have led to a dramatic improvement in survival rates for most forms of congenital heart disease (CHD). For example, hypoplastic left heart syndrome, a previously lethal defect, now has early survival rates up to 90% at major centers.1 These improved outcomes have produced a growing population of survivors with complex CHD who are now reaching adulthood (Figure 1). During this period, improvements in surgical and medical treatments have been accompanied by developments in diagnostic modalities. Echocardiography has replaced catheterization as the primary diagnostic modality, and it is now uncommon for newborn infants to undergo catheterization for purely diagnostic purposes. Although echocardiography remains the bedrock of noninvasive cardiac imaging, the array of diagnostic modalities and techniques available continue to grow and this has spawned the specialty of “noninvasive cardiac imaging” and the need for the “cardiac imager” to be adept in all the different modalities. Figure 1. Percentage of patients under the age of 1 year (grey bars) and over the age of 18 years (black bars) undergoing echocardiography at Children’s Hospital Boston from 1983 through 2006. Note the reverse trends of these age groups reflecting the steady increase in the proportion of adult patients with congenital heart disease. Although the absolute number of infants undergoing echocardiography during this time period has increased, their proportion has steadily declined. Echocardiography, cardiac magnetic resonance (CMR), and cardiac computed tomography (CCT) are the primary modalities used for noninvasive cardiac imaging in patients with CHD. Nuclear scintigraphy is used in selected circumstances. The Table summarizes the strengths and weaknesses of each modality. Figure 2 shows temporal trends in utilization for the various noninvasive cardiac imaging techniques at our center. It is clear that echocardiography is the most frequently …

Non-Invasive Imaging in Coronary Syndromes: Recommendations of The European Association of Cardiovascular Imaging and the American Society of Echocardiography, in Collaboration with The American Society of Nuclear Cardiology, Society of Cardiovascular Computed Tomography, and Society for Cardiovascular Magnetic Resonance