Coronary Artery Anatomy Research Articles

Identification of hemodynamically significant coronary artery disease with stress myocardial blood flow ratio by dynamic CT perfusion studies

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Coronary computed tomographic angiography (CTA) is an accepted noninvasive tool with high diagnostic accuracy for the evaluation of coronary artery disease (CAD). However, coronary CTA is limited in that it does not provide information on the hemodynamic relevance of coronary artery stenosis. Therefore, to determine the potential benefit of future coronary revascularization, a functional assessment is often required. A protocol combining coronary computed tomographic angiography (CTA) and CTP can provide a simultaneous assessment of both coronary artery ischemia and anatomy. Purpose This study sought to evaluate the diagnostic accuracy of stress myocardial blood flow ratio (SFR), a novel parameter derived from stress dynamic computed tomographic perfusion (CTP), for the detection of hemodynamically significant coronary stenosis. Methods A total of 98 patients (mean age 54.5 ± 10 years) with 128 vessels with either 1- or 2-vessel disease, scheduled for invasive angiography were included in this study. Stress dynamic CTP was performed followed by coronary CTA using a dual-source computed tomographic system. Fractional flow reserve was performed at subsequent invasive angiography to identify hemodynamically significant stenosis. Stress myocardial blood flow ratio (SFR) was defined as the ratio of hyperemic myocardial blood flow (MBF) in an artery with stenosis to hyperemic MBF in a non-diseased artery. The diagnostic accuracy of the SFR index was determined against the reference standard of invasive fractional flow reserve ≤0.80. Results 58 (45.3%) vessels were deemed hemodynamically significant by fractional flow reserve (FFR) measurement. Hyperemic MBF and SFR were lower for vessels with hemodynamically significant lesions (95 ± 32.2 ml/100 ml/min vs. 140.5 ± 30.4 ml/100 ml/min; p < 0.01 for both). When compared with ≥50% stenosis by CTA, the specificity for detecting ischemia by SFR increased from 44% to 92%, while the sensitivity decreased from 93% to 58%. The combination of stenosis ≥50% by CTA and SFR resulted in an area under the curve of 0.9, which was significantly higher compared with hyperemic MBF, area under the curve = 0.79 and p < 0.01. Conclusion Our study concludes that the calculation of SFR by dynamic CTP provides a pioneering and accurate method to identify flow-limiting coronary stenosis. Abstract Figure.

Prevalence of coronary anomalies detected by computed tomography at the Instituto Nacional Cardiovascular- INCOR

: To determine the prevalence of coronary anomalies (CA) in patients evaluated by 64-detector computed tomography (CT) at the Instituto Nacional Cardiovascular in Peru between 2016 and 2020. Retrospective observational study, coronary artery CT scans of 1486 patients were performed on a 64-detector row CT scanner and reviewed in search for coronary anomalies. The prevalence of CA detected by CT was 4.71% (70 cases) of which 64.3% were male. Abnormalities of origin were the most frequent, of which the origin of a coronary artery from the opposite coronary sinus was the most common (48.6%), with the right coronary being the main anomalous artery (31%), and the main path was interarterial (31%). Anomalous origin of the left main coronary from the pulmonary artery was found in 5 patients. Among the anomalies of the intrinsic coronary arterial anatomy the most frequent was the double left anterior descending artery (10%). Coronary fistulas accounted for 11.4% of cases. The prevalence of CA detected by 64-detector CT in a Peruvian institute was 4.71%. The most frequent coronary anomaly was the origin of the right coronary artery from the left coronary sinus with interarterial trajectory.

Chronic Total Occlusion Anatomy and Characteristics of Coronary Collaterals and Angiographic Features Predicting the Success of Chronic Total Occlusion Intervention

Background and Objective: Angiographic assessment of chronic total occlusion (CTO) anatomy and collateral characteristics of coronary arteries are necessary for CTO intervention. Materials and Methods: This was a hospital-based observational study of 100 coronary angiograms (CAG) with CTOs. CTO anatomy and collateral characteristics of coronary arteries were studied for predicting anterograde and retrograde CTO intervention. Results: Right coronary artery (RCA) CTO was the most common (62%), followed by left anterior descending (LAD). More than two-thirds of RCA and LAD CTO lesions were >20 mm and half were in mid-segment. Left circumflex artery (LCX) and RCA lesions were more frequently calcified. LAD CTOs often had blunt stump; LCX CTOs frequently had bending >45°. The mean J-CTO score was lowest in RCA CTOs (2.0 ± 1.19). There were 10 different types of collaterals in RCA CTOs, 8 in LAD CTOs, and only 4 in LCX CTOs. The most common RCA CTOs collateral was LAD septal to the right posterior descending artery (RPDA) (69.4%) and in LAD CTOs, the most common was septal collaterals from the RPDA to LAD (40.9%). RCA CTOs had a higher percentage of septal collaterals, less tortuosity, and favorable entry and exit angle when compared with other two arteries. Conclusion: RCA CTOs were the most common. Angiographic features in CTO lesions vary among three major coronary arteries. The RCA CTOs had lesser mean J-CTO score, more number of septal collaterals, less tortuous collaterals, and favorable entry and exit angle. RCA CTOs were better accessible for anterograde and retrograde intervention.

Evaluation of coronary arteries in congenital heart disease in children: diagnostic comparison of electrocardiogram-gated and non-electro-cardiogram-gated computed tomography cardiac angiography.

To compare the visualization and anatomy of coronary arteries in children (≤ 2 years) with congenital heart disease (CHD) on non-electrocardiogram (ECG)-gated and ECG-gated computed tomography angiography (CTA). In this retrospective study, approved by the Ethics Committee of our institute, evaluation of coronary arteries in CHD was performed in 40 children on non-ECG-gated CTA and in 42 children on ECG-gated CTA. The origin and course of the right coronary artery (RCA), left main coronary artery (LMCA), left anterior descending (LAD) artery, and left circumflex (LCX) artery were evaluated by 2 paediatric radiologists independently. ECG-gated CT scans yielded increased (additional) visualization of all the coronary arteries, when compared to non-ECG-gated CT scans. The RCA, LMCA, LAD artery, and LCX artery were visualized in 47.5%, 62.5%, 55%, and 32.5% of children, respectively, on non-ECG-gated studies, while they were visualized in 64.3%, 92.8%, 80.9%, and 62% children, respectively, on ECG-gated studies. The coronary artery anatomical variations were also supplementarily detected more in the ECG-gated group (23.8%) than in the non-ECG gated group (2.5%). ECG-gated CT cardiac angiography studies yield enhanced diagnostic outcomes for the evaluation of the coronary arteries in comparison to non-ECG-gated studies.

Prevalence and patterns of coronary artery anomalies in 28,800 adult patients undergoing angiography in a large tertiary care centre in India.

Coronary artery anomalies (CAAs) are a diverse group of disorders with varied clinical presentation and pathophysiological mechanisms. A majority of these anomalies are asymptomatic and often an incidental finding on coronary angiogram or autopsy. This retrospective study included 28,800 patients who underwent coronary angiography from 2016 to 2020. The coronary angiograms were reviewed by two independent reviewers and CAAs were documented. CAAs were classified into i) anomalies of coronary artery connection, ii) anomalies of intrinsic coronary arterial anatomy and iii) anomalies of myocardial/coronary artery interaction as proposed by the European Society of Cardiology. Of the 28,800 coronary angiograms, CAAs were present in 4.12% with anomalies in the left coronary artery (LCA) being most common. Anomalies of coronary artery connection were most common (48.48%) followed by anomalies of myocardial/coronary artery interaction (34.49%) and anomalies of intrinsic coronary artery anatomy (17.03%). Among anomalies of coronary artery connection, absent left main trunk or split LCA with separate origins of left anterior descending coronary artery and left circumflex coronary artery from the left coronary sinus of Valsalva (22.59%) was most common. An intramural course or "myocardial bridge" had an incidence of 1.16% while incidence of coronary artery fistulae (CAF) was 0.115%.

770 Small heart and single coronary artery in a young patient with chronic fatigue syndrome: a case report

Abstract Aims Chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) is a clinically defined condition reported mostly in adults, characterized by fatigue and unexplained aspecifical symptoms. Patients with CFS/ME frequently have reduced stroke volume with an inverse relation between cardiac output and post-exertional malaise severity. We describe a rare case of a young man affected by CFS/ME, small heart, and anomalous anatomy of left main coronary artery (LMCA). Methods and results A 19-year-old Caucasian male referred to our clinic complaining weakness, lack of concentration, and sleepiness. He suffered by CFS/ME, D hypovitaminosis, inflammatory bowel disease, and exocrine pancreas insufficiency. Blood tests revealed no pathological findings. Faecal exams documented intestinal dysbiosis and low pancreatic elastase. He was treated with oxygen-ozone rectal insufflations and probiotics. Physical examination was unremarkable. Electrocardiogram showed normal sinus rhythm. Echocardiogram revealed reduced diameters of the left ventricle (LV), normal aortic root dimensions and, in a five-chamber apical view, a binary structure that seemed to cross the aorta perpendicularly. Cardiac magnetic resonance (CMR) found reduced LV stroke volume (34 ml/m2) and end-diastolic volume (57 ml/m2) together with reduced end-diastolic wall mass (51 g/m2). Right ventricle volumes were reduced too. In addition, the exam confirmed the anomalous origin of LMCA stemming from the proximal segment of right coronary artery and following a retro-aortic course. Mechanism of CFS/ME remains unknown, although various factors have been implicated, including immune activation, chronic viral infection, and emotional disorders. A considerable number of patients affected by CFS has an anatomically small heart. Small heart syndrome, in fact, may contribute to the onset of CFS/ME. Previous studies hypothesized that clinical manifestations of CMS/ME were caused by reduced venous return, cardiac output, and heart mass, together with decreased arterial oxygen saturation. Single coronary artery is an uncommon congenital anatomic abnormality identified by a single coronary ostium giving rise to all arteries supplying the heart. Thus, we reported a rare case of a very young man affected by chronic fatigue syndrome and small heart, investigated not only with echocardiogram but also with CMR, not often used in this clinical setting. More, we found an anomalous origin of LMCA. From literature, it’s not reported any cases of a patient including these three rare conditions (CFS/ME, small heart, and single coronary artery). Conclusions This case highlights that CFS/ME together with small heart is a condition possible also in young people. More studies and reports could be necessary to better define the association between cardiac congenital anomalies and CFS/ME. 770 Figure

462 Accidental detection of an anomalous origin of the coronary arteries in a patient with STEMI: a case report

Abstract Coronary artery anomalies and variants are relatively uncommon congenital disorders of the coronary artery anatomy and constitute the second most common cause of sudden cardiac death in young competitive athletes. Normally there are two main coronary arteries, which stem from the sinuses of Valsalva and descend towards the cardiac apex. The most frequent anomalous origin of the coronary arteries associated with sudden cardiac death is the anomalous origin of a coronary artery from the contralateral sinus, particularly if the anomalous coronary artery has a course between the aorta and the pulmonary artery. The diagnosis of a coronary artery anomaly is insidious since patients are usually asymptomatic. Indeed, in most of the cases, coronary anomalies are discovered incidentally during coronary angiography or on autopsy following sudden cardiac death. However, in some cases, symptoms like angina, syncope, heart failure, and myocardial infarction may occur. We want to describe the case of a 54 years old man who went to another hospital of our district with chest pain. The EKG showed an infero-lateral STEMI. The patient underwent systemic thrombolysis complicated by arrhythmic storm, treated with effective DC shock, then he was urgently transferred to our Hospital to perform a PCI rescue. The coronary angiography showed a critical stenosis of the right coronary artery, treated with the implantation of a zotarolimus-eluting coronary stent system. Surprisingly, an abnormal origin of the anterior interventricular artery and circumflex artery from seperated hosts in the right coronary sinus was detected. A computed tomography showed the separated origin of the right coronary artery and the left main from the right coronary sinus; moreover, the left main presented a retroaortic course between the bulb and the left atrium; the left anterior intraventricular artery run anterior to the aortic root. During hospitalization, a diagnosis of diabetes was made with consequent setting of adequate hypoglycemic therapy. Serial echocardiograms showed an improval in the ejection fraction (from 35–40% to 50%). The patient was discharged in well clinical conditions and 1 month later at the follow-up, during an outpatient visit, he was fully asymptomatic. The dynamic EKG according to Holter showed rare isolated ventricular beats; laboratory tests were normal. The anomalous origin of a coronary ostium from the contralateral sinus is the most frequently anomaly which is associated with sudden cardiac death, in particular when the left coronary artery origins from the right sinus. The rapid advancement of the imaging techniques, including CT, CMR, intravascular ultrasound, and optical coherence tomography, have provided us with a wealth of new information on this subject. Coronary artery CT offers the best performance in terms of spatial resolution, acquisition time, and image contrast but the use is limited due the dose of ionizing radiation and the use of contrast agents, in particular taking into account that most patients are young. CMR is capable of visualizing the origin of the coronary arteries non-invasively, without use of ionizing radiation and contrast agents. Currently however, due to spatial resolution, the capability of CMR to visualize smaller coronary branches is still a limiting factor preventing full assessment of coronary arteries using this modality. Developments and more widespread access to advanced cardiac imaging will undoubtedly lead to earlier diagnoses. The taxonomies of the anomalous origin of coronary arteries are inconsistent and complex and so we want to make our contribution to the register of cases of abnormal origin of coronary arteries for the risk of sudden cardiac death and ischaemic events related to.

C68. Patient with Zero Coronary Calcium Score and Significant Stenosis in Left Anterior Descending Coronary Artery: a case report

Abstract Background Coronary heart disease is pathological process characterized by the accumulation of atherosclerotic plaque in the epicardial artery, either obstructive or non-obstructive. Coronary calcium score (CCS) detects and calculates coronary artery calcification (CAC) and CT coronary angiography (CTCA) allows evaluation of the coronary arteries anatomy for luminal stenosis due to atherosclerotic plaque with or without calcification. Case Description A 70 years-old man, admitted to the emergency department with a chief complain of chest pain. Physical examination was within normal limits. His treadmill stress testing was positive ischemic response one month prior. Then patient undergoing CTCA, the result was zero calcium score with 90-99% stenosis at proximal left anterior descending (LAD) due to soft plaque. Invasive coronary angiography (ICA) confirmed the CT findings and showed total occlusion with grade V thrombus in osteal LAD. Discussion A study showed that 1-2% of symptomatic patients with zero CAC potentially had obstructive CAD. It is supported with this report that the patient had typical chest pain with zero calcium score. CCTA has high sensitivity and negative predictive value for the detection of CAD but has a relatively low positive predictive value, especially for moderate severity coronary. In this report CTCA showed 90% -99% stenosis at the proximal LAD and correlate with ICA findings.

C68. Patient with Zero Coronary Calcium Score and Significant Stenosis in Left Anterior Descending Coronary Artery: a case report

Abstract Background Coronary heart disease is pathological process characterized by the accumulation of atherosclerotic plaque in the epicardial artery, either obstructive or non-obstructive. Coronary calcium score (CCS) detects and calculates coronary artery calcification (CAC) and CT coronary angiography (CTCA) allows evaluation of the coronary arteries anatomy for luminal stenosis due to atherosclerotic plaque with or without calcification. Case Description A 70 years-old man, admitted to the emergency department with a chief complain of chest pain. Physical examination was within normal limits. His treadmill stress testing was positive ischemic response one month prior. Then patient undergoing CTCA, the result was zero calcium score with 90-99% stenosis at proximal left anterior descending (LAD) due to soft plaque. Invasive coronary angiography (ICA) confirmed the CT findings and showed total occlusion with grade V thrombus in osteal LAD. Discussion A study showed that 1-2% of symptomatic patients with zero CAC potentially had obstructive CAD. It is supported with this report that the patient had typical chest pain with zero calcium score. CCTA has high sensitivity and negative predictive value for the detection of CAD but has a relatively low positive predictive value, especially for moderate severity coronary. In this report CTCA showed 90% -99% stenosis at the proximal LAD and correlate with ICA findings.

Myocardial Infarction by Percutaneous Embolization Coil Deployment in a Swine Model

Myocardial infarction (MI) is the leading cause of mortality worldwide. Despite the use of evidence-based treatments, including coronary revascularization and cardiovascular drugs, a significant proportion of patients develop pathological left-ventricular remodeling and progressive heart failure following MI. Therefore, new therapeutic options, such as cellular and gene therapies, among others, have been developed to repair and regenerate injured myocardium. In this context, animal models of MI are crucial in exploring the safety and efficacy of these experimental therapies before clinical translation. Large animal models such as swine are preferred over smaller ones due to the high similarity of swine and human hearts in terms of coronary artery anatomy, cardiac kinetics, and the post-MI healing process. Here, we aimed to describe an MI model in pig by permanent coil deployment. Briefly, it comprises a percutaneous selective coronary artery cannulation through retrograde femoral access. Following coronary angiography, the coil is deployed at the target branch under fluoroscopic guidance. Finally, complete occlusion is confirmed by repeated coronary angiography. This approach is feasible, highly reproducible, and emulates the pathogenesis of human non-revascularized MI, avoiding the traditional open-chest surgery and the subsequent postoperative inflammation. Depending on the time of follow-up, the technique is suitable for acute, sub-acute, or chronic MI models.

PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM: NOT CLEARED FOR TAKEOFF

TOPIC: Pulmonary Vascular Disease TYPE: Fellow Case Reports INTRODUCTION: In patients with underlying lung disease, pulmonary evaluation is required prior to air travel due to hypoxic conditions in higher altitudes. We present a case of a young man aspiring to become a pilot, found to have a congenital cardiopulmonary defect of pulmonary atresia. CASE PRESENTATION: An 18 year old male with right pulmonary atresia and intact ventricular septum (PA-IVS) presented to the pulmonary clinic seeking medical clearance for flight training school, endorsing fatigue and dyspnea on exertion. Vitals signs and physical examination were benign without cardiac murmurs, peripheral edema, or cyanosis. Chest CT revealed absent right PA (pulmonary artery) with contrast reflux. A subsequent angiogram revealed blood flow from the right internal mammary artery to the right upper PA and from the celiac and inferior diaphragmatic artery to the right lower PA. Left and right cardiac catheterization showed normal pressures in the right atrium, right ventricle, and PA. Mean PA pressure was 14 mmHg, and wedge pressure was 6 mmHg. Left ventricular end diastolic pressure was 6 mmHg, with normal coronary artery and vein anatomy. Oxygen saturations were: SVC 86%, RA 82%, RV 81%, left PA 81%, and femoral artery 96%. Treadmill stress test was inconclusive due to early termination as the patient did not achieve 85% of maximum heart rate. Occupational medicine recommended HAST (high altitude simulation testing) in concordance with FAA regulations. Before HAST was performed, the patient opted out of his dream career of becoming a pilot. DISCUSSION: PA-IVS is a rare congenital heart disease accounting for < 1 % of total heart defects [2]. It is characterized by atresia of the RV outflow tract. The precise cause is unclear, but thought to be related to an insult during embryological development. PA-IVS can be classified into membranous and muscular forms with the former harboring a better long term prognosis. The muscular form is associated with abnormal connections between the RV and coronary artery. In our patient, left heart catheterization did not reveal abnormal coronary anatomy to suggest muscular PA-IVS.HAST is utilized in patients with cardiopulmonary defects to predict physiological response to hypobaric conditions at higher altitudes. The patient is asked to breathe a mixture of gases with an oxygen saturation of 15.1%, and simulates cabin pressures up to 2400 meters. The test is considered abnormal if there is evidence of hypoxemia (pAO2 < 50 mmHg), symptoms, and/or arrhythmias. We believe that our patient would have had an appropriate response to HAST due to a favorable form of PA-IVS and normal cardiopulmonary pressures and saturations. CONCLUSIONS: In adolescents with congenital cardiopulmonary defects a thorough workup including HAST is recommended prior to allowing exposure to a suboptimal environment. REFERENCE #1: Daubeney PE, Sharland GH, Keeton BR, et al. Pulmonary atresia with intact ventricular septum. Circulation 1998 (8(6): 562-566. REFERENCE #2: Ferencz C, Rubin JD, McCarter RJ, Brenner JI, Neill CA, Perry LW, Hepner SI, Downing JW. Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study. Am J Epidemiol. 1985 Jan;121(1):31-6. DISCLOSURES: No relevant relationships by Moises Cossio, source=Web Response No relevant relationships by Domingo Franco-Palacios, source=Web Response No relevant relationships by Arjun Mahendra, source=Web Response No relevant relationships by Toribiong Uchel, source=Web Response

Dynamic Myocardial Perfusion CT for the Detection of Hemodynamically Significant Coronary Artery Disease

ObjectivesIn this international, multicenter study, using third-generation dual-source computed tomography (CT), we investigated the diagnostic performance of dynamic stress CT myocardial perfusion imaging (CT-MPI) in addition to coronary CT angiography (CTA) compared to invasive coronary angiography (ICA) and invasive fractional flow reserve (FFR). BackgroundCT-MPI combined with coronary CTA integrates coronary artery anatomy with inducible myocardial ischemia, showing promising results for the diagnosis of hemodynamically significant coronary artery disease in single-center studies. MethodsAt 9 centers in Europe, Japan, and the United States, 132 patients scheduled for ICA were enrolled; 114 patients successfully completed coronary CTA, adenosine-stress dynamic CT-MPI, and ICA. Invasive FFR was performed in vessels with 25% to 90% stenosis. Data were analyzed by independent core laboratories. For the primary analysis, for each coronary artery the presence of hemodynamically significant obstruction was interpreted by coronary CTA with CT-MPI compared to coronary CTA alone, using an FFR of ≤0.80 and angiographic severity as reference. Territorial absolute myocardial blood flow (MBF) and relative MBF were compared using C-statistics. ResultsICA and FFR identified hemodynamically significant stenoses in 74 of 289 coronary vessels (26%). Coronary CTA with ≥50% stenosis demonstrated a per-vessel sensitivity, specificity, and accuracy for the detection of hemodynamically significant stenosis of 96% (95% CI: 91%-100%), 72% (95% CI: 66%-78%), and 78% (95% CI: 73%-83%), respectively. Coronary CTA with CT-MPI showed a lower sensitivity (84%; 95% CI: 75%-92%) but higher specificity (89%; 95% CI: 85%-93%) and accuracy (88%; 95% CI: 84%-92%). The areas under the receiver-operating characteristic curve of absolute MBF and relative MBF were 0.79 (95% CI: 0.71-0.86) and 0.82 (95% CI: 0.74-0.88), respectively. The median dose-length product of CT-MPI and coronary CTA were 313 mGy·cm and 138 mGy·cm, respectively. ConclusionsDynamic CT-MPI offers incremental diagnostic value over coronary CTA alone for the identification of hemodynamically significant coronary artery disease. Generalized results from this multicenter study encourage broader consideration of dynamic CT-MPI in clinical practice. (Dynamic Stress Perfusion CT for Detection of Inducible Myocardial Ischemia [SPECIFIC]; NCT02810795)

The Potential Role of Cardiac CT in the Evaluation of Patients With Known or Suspected Cardiomyopathy: From Traditional Indications to Novel Clinical Applications.

After 15 years from its advent in the clinical field, coronary computed tomography (CCTA) is now widely considered as the best first-step test in patients with low-to-moderate pre-test probability of coronary artery disease. Technological innovation was of pivotal importance for the extensive clinical and scientific interest in CCTA. Recently, the advent of last generation wide-coverage CT scans paved the way for new clinical applications of this technique beyond coronary arteries anatomy evaluation. More precisely, both biventricular volume and systolic function quantification and myocardial fibrosis identification appeared to be feasible with last generation CT. In the present review we would focus on potential applications of cardiac computed tomography (CCT), beyond CCTA, for a comprehensive assessment patients with newly diagnosed cardiomyopathy, from technical requirements to novel clinical applications.

Lessons learned from ECMO support in pediatric patients with D-transposition of the great arteries: preoperative, intraoperative and postoperative

BackgroundExtracorporeal membrane oxygenation (ECMO) support on D-transposition of the great arteries (D-TGA) carries formidable challenges.MethodsA retrospective study was performed on pediatric patients with D-TGA supported by ECMO from July 2007...

Contrast Echocardiography for the Detection of a Giant Right Coronary Artery Aneurysm: A Useful Diagnostic Tool

Coronary artery aneurysm is an uncommon condition defined as abnormal dilatation of the coronary artery. We report the case of a man admitted to the Emergency Department for suspected intestinal obstruction. At abdominal/thoracic Computed Tomography (CT) it was reported a suspected pericardial cyst. Contrast echocardiography showed a clear pattern of blood flow inside the mass with a probable origin at a very limited region where it was also possible to sample an ECG-synchronized system-diastolic Pulse-Wave (PW)-Doppler pattern, typical for a coronary artery flow. Our suspicion of a giant coronary artery aneurysm was then confirmed by coronary CT and coronary angiography. The patient was transferred to a Cardiac Surgery center where he underwent surgical treatment for the aneurysm. Most coronary aneurysms are clinically silent and are often incidentally detected. Coronary angiography is the ‘‘gold standard’’ for diagnosis and evaluation of coronary artery aneurysms. Among non-invasive diagnostic techniques, Computed Tomography Coronary Angiography is the best method for coronary artery anatomy and coronary abnormalities. However, echocardiography with the use of a contrast agent, a cost-effective non-radiation nature, and a widespread use method, maybe a reliable method to detect and characterize the masses, allowing a differential diagnosis. Learning objective 1. Recognition and evaluation of cardiac masses require first-line imaging methods such as echocardiography. 2. The use of additional non-invasive (cardiac CT or MRI) and/or invasive imaging methods are often required. 3. Contrast echocardiography may be helpful, as second-line imaging methods, to better characterize the mass and approach the correct diagnosis.

Anomalous aortic origin of a coronary artery: learning from the past to make advances in the future.

To review anomalous aortic origin of a coronary artery (AAOCA) anatomy, prevalence, mechanism and risk of ischemia, presentation, evaluation, management, and future directions. Although most anatomic variants of AAOCA are benign, a small number are associated with increased risk of sudden death. A complete evaluation, including the use of advanced noninvasive imaging and provocative testing should be performed on nearly every patient with AAOCA. On the basis of recent studies, the ischemic risk appears to be greatest with a left anomalous coronary artery but an anomalous right coronary artery is not benign. Other risk factors include: a left anomalous coronary with an intramural course, high take-off, or slit-like orifice, and a right anomalous coronary with a longer intramural course. Exercise restriction is rarely recommended. Management primarily consists of nonoperative care, or surgical repair in those who are symptomatic or who have high-risk variants. Surgery itself continues to evolve; however, it is not benign, with a higher than expected chance of morbidity. Advances have been made over the past decade regarding management of patients with AAOCA; however, the mechanism of ischemia and ability to predict risk is still incompletely understood. Management decisions should be based on anatomy, results of investigations, and shared decision-making with patients and their families. Surgery may be recommended for those at higher risk and should be done at centers experienced in AAOCA surgery. Future research should be collaborative in order to share experiences and insights to help advance our understanding of risk and ultimately to improve patient management.

Value of echocardiography to diagnose coronary arteries patterns in transposition of great arteries

Abnormal coronary pattern may complicate coronary transfer during arterial switch operation. The objective of this study was to evaluate the accuracy of echocardiographic to assess coronary arteries anatomy in transposition of the great arteries and determine impact on outcomes. Retrospective analysis of data in neonates with transposition of the great arteries. Preoperative echocardiographic coronary artery pattern and surgical intraoperative reports were compared. Mismatch between transthoracic echocardiography and surgical intraoperative reports, and its impact on perioperative outcome were assessed. Coronary patterns were classified in 4 groups: I = normal, II = coronary with intramural course, III = CA loop. One hundred and eight neonates who underwent arterial switch operation were included: 68 ranged in group I, 7 in group II, 32 in group III and 1 in group II + III. Intraoperative complications occurred in 10 cases. Five patients died, from coronary cause in 3 ( Table 1 ). Survival rates were 96% at 1-month. Transthoracic echocardiography and surgical intraoperative reports differed in 17.6%. Death rates were 15.8% in case of inappropriate diagnosis and 2.2% if the diagnosis was appropriate ( P = 0.0108) ( Fig. 1 ). Mortality in group II increased up to 50% in case of inappropriate diagnosis versus 0% if concordance. By multivariate analysis, an inappropriate preoperative transthoracic echocardiography diagnosis of coronary pattern was the only significant risk factor for mortality ( P = 0.04). Echocardiography can assess coronary artery anatomy in neonates with transposition of the great arteries. Intramural coronary course is often misdiagnosed. Missed diagnosis of coronary artery anomaly preoperatively may impact on perioperative mortality.

Applicability of the Leiden Convention and the Lipton Classification in Patients with a Single Coronary Artery in the Setting of Congenital Heart Disease

: BackgroundIn single coronary artery (SCA) anatomy, all coronary tributaries arise from a single ostium, providing perfusion to the entire myocardium. Coronary classification systems can facilitate the description of SCA anatomy. Aim: Evaluation of the applicability of Lipton classification and the Leiden Convention coronary coding system in SCA. Methods: All patients (n = 6209) who underwent computed tomography (CT) scanning between 2014 and 2018 were retrospectively examined for the presence of SCA and classified, according to Lipton classification and the Leiden Convention coronary coding system. Results: The prevalence of SCA was 0.51% (32/6209). Twenty-eight patients (87.5%) had coexisting congenital heart disease (CHD), most frequently pulmonary atresia (9/32, 28.1%). Ten patients (10/32, 31.25%) could not be classified with either the Leiden Convention or Lipton classification (pulmonary atresia n = 9, common arterial trunk (CAT) n = 1). In one case with CAT, Lipton classification, but not the Leiden Convention, could be applied. In two cases with the transposition of the great arteries and in two cases of double outlet right ventricle, the Leiden Convention, but not the Lipton classification, could be applied. Conclusions: Both classifications are useful to detail information about SCA. As Lipton classification was not developed for structural heart disease cases, in complex CHD with abnormal position of the great arteries, the Leiden Convention is better applicable. The use of both systems is limited in pulmonary atresia. In this scenario, it is better to provide a precise description of the coronary origin and associated characteristics that might affect treatment and prognosis.

Reducing radiation dose from myocardial perfusion imaging in subjects with complex congenital heart disease

IntroductionThe prevalence of defects and effective radiation dose from various myocardial perfusion imaging (MPI) strategies in congenital heart disease (CHD) is unknown. MethodsWe studied 75 subjects with complex CHD (ages 5 to 80 years) referred for MPI between 2002 and 2015. A rest and exercise or pharmacologic stress MPI was performed using 99mTechnetium sestamibi, 82rubidium or 13N-ammonia, and Sodium iodide SPECT (single-photon emission computed tomography), SPECT/CT or Cadmium zinc telluride (CZT) SPECT or PET (positron emission tomography)/CT scanners. Deidentified images were interpreted semi-quantitatively in three batches: stress only MPI, stress/rest MPI, and stress/rest MPI with taking into account a history of ventricular septal defect repair. Effective radiation dose was estimated for stress/rest MPI and predicted for 1-day stress-first (normal stress scans), and for 2-day stress/rest MPI (abnormal stress scans). ResultsThe median age was 18.6 years. The most common type of CHD was transposition of the great arteries (63%). Rest/stress MPI was abnormal in 43% of subjects and 25% of the abnormal scans demonstrated reversible defects. Of the subjects with abnormal MPI, 33% had significant underlying anatomic coronary artery obstruction. Estimated mean effective radiation dose ranged from 2.1 ± 0.6 mSv for 13N-ammonia PET/CT to 12.5 ± 0.9 mSv for SPECT/CT. Predicted effective radiation dose was significantly lower for stress-first MPI and for 2-day stress/rest protocols. ConclusionsDue to the relatively high prevalence of abnormal stress MPI, tailored protocols with a stress-first MPI as well as the use of 2-day protocols and advanced imaging technologies including CZT SPECT, novel image reconstruction software, and PET MPI could substantially reduce radiation dose in complex CHD.

Coronary Artery Dominance and Cardiovascular Pathologies in Patients with Bicuspid Aortic Valve

BackgroundBicuspid aortic valve (BAV) is the most common congenital heart defect and is associated with a number of cardiac pathologies including coarctation of the aorta (CoA), aortic dilation, aortic stenosis, and premature coronary artery disease (CAD). Furthermore, BAV has been associated with left dominant coronary artery anatomy but there is still much debate regarding this association. To date, no study has investigated the association between coronary artery dominance and cardiac pathologies in patients with BAV. MethodsA total of 45 patients with BAV who underwent coronary computed tomography angiography (CTA) and/or cardiac catheterization at a single center were included. Coronary artery dominance, presence of CAD, CoA, and aortic dilation was determined from coronary CTA and/or cardiac catheterization reports. Incidence of aortic valve stenosis and aortic valve regurgitation was determined from echo reports. ResultsOf the 45 patients with BAV, 80% (36) had right dominant coronary artery circulations. Patients with BAV with left dominant circulations had a significantly (p = 0.04) higher incidence of CoA compared to patients with right dominant circulations (44% vs. 14%). There was no significant difference when comparing the incidences of aortic dilation, aortic valve stenosis, aortic valve regurgitation and CAD in right and left dominant patients. ConclusionsOur study found a predominance of right dominant coronary artery circulation in patients with BAV, similar to the general population. Interestingly, left dominance was significantly associated with CoA in patients with BAV. This observation highlights the importance of further studies regarding how coronary artery dominance can be associated with other cardiovascular pathologies in patients with BAV.