The role of coronary CT angiography in athletes.

The sudden death of a young or high-level athlete or adolescent during recreational sports is one of the events with the greatest impact on public opinion in modern society. Sudden cardiac death (SCD) is the principal medical cause of death in athletes and can be the first and last clinical presentation of underlying disease. To prevent such episodes, pre-participation screening has been introduced in many countries to guarantee cardiovascular safety during sports and has become a common target among medical sports/governing organizations. Different cardiac conditions may cause SCD, with incidence depending on definition, evaluation methods, and studied populations, and a prevalence and etiology changing according to the age of athletes, with CAD most frequent in master athletes, while coronary anomalies and non-ischemic causes prevalent in young. To detect silent underlying causes early would be of considerable clinical value. This review summarizes the pre-participation screening in athletes, the specialist agonistic suitability visit performed in Italy, the anatomical characteristics of malignant coronary anomalies, and finally, the role of coronary CT angiography in such arena. In particular, the anatomical conditions suggesting potential disqualification from sport, the post-treatment follow-up to reintegrate young athletes, the diagnostic workflow to rule-out CAD in master athletes, and their clinical management are analyzed.

Transcatheter aortic valve implantation in patients with anomalous origin of a coronary artery.

Transcatheter aortic valve implantation (TAVI) has become a well-established treatment option for elderly patients with symptomatic severe aortic stenosis. Coronary artery anomalies are an infrequent finding and there have only been few anecdotal reports of patients with coronary anomalies treated with TAVI. We here present a comprehensive overview of existing reports in addition to an own case series to facilitate better understanding of this potentially challenging clinical scenario.

A Survey on the Management of Anomalous Aortic Origins of the Coronary Arteries.

The management of patients with an anomalous aortic origin of a coronary artery (AAOCA) remains controversial despite the publication of the 2017 American Association for Thoracic Surgery (AATS) expert guidelines. We surveyed the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and the Pediheart.net online community regarding their care of patients with anomalous origins of the right or left coronary from the opposite cusp with inter-arterial courses and compared them to the AATS guidelines. We received 111 complete responses. Four notable variations from the AATS recommendations were identified. Respondents were more likely to use ECG exercise testing than the stress imaging recommended in the AATS guidelines. For a 16-year-old with AAOCA, recommendations for surgery generally followed the AATS guidelines. However, for asymptomatic left AAOCA without signs of ischemia on stress imaging, only 69.4% felt surgery was appropriate or somewhat appropriate. In the setting of a 16-year-old with right AAOCA free from signs or symptoms of ischemia, respondents were more likely to recommend surgery if the patient was a competitive athlete, a topic not directly addressed in the AATS guidelines. After surgical treatment of AAOCA, only 24% of respondents recommended lifelong antiplatelet therapy despite recommendations for this in the AATS guidelines. Respondents recommendations were generally consistent with the 2017 AATS guidelines but with important variations in the use of stress imaging, indications for surgery in asymptomatic left AAOCA, the impact of identification as a competitive athlete and duration of postoperative antiplatelet therapy.

Anomalous arising of left coronary artery from the pulmonary artery (ALCAPA).

Computed tomography 3D reconstruction of a patient with Bland-White-Garland syndrome showing a dilated right coronary artery and thin left coronary artery.

Aortopulmonary window and coronary anatomy - still a pre-operative diagnostic trouble!

We present a case of aortopulmonary window in which the diagnosis of anomalous left coronary artery originating from pulmonary artery was made intra-operatively even if the coronary arteries anatomy was correctly studied pre-operatively with echocardiography. No evidence of coronary anomalies or indirect sings of coronary anomalies has been noted. Should we improve our pre-operative diagnostic accuracy and how?

Can ectopic right coronary from the left sinus have a different course than intramural? A case of ectopic right with retroaortic course.

Retrocardiac course of an ectopic right coronary artery is newly described. The alternative (usual) course of an anomalous right coronary from the left sinus of Valsalva is pre-aortic and intramural, with stenosis. In the present exceptional case, there was no stenosis.

Surgical procedures for coronary arteries in pediatric cardiac surgery: Risk factors and outcomes.

BACKGROUND: Limited data exist regarding the coronary revascularization procedures needed during the repair of several congenital and pediatric cardiac malformations. We aimed to determine risk factors for in-hospital mortality and long-term outcomes of various pediatric coronary revascularization procedures. METHODS: We retrospectively reviewed the records of 32 consecutive pediatric patients who underwent coronary revascularization procedures at our institution between May 1995 and June 2020. In-hospital mortality, risk factors, surgical indications, revascularization patency, and mid- and long-term follow-up data were investigated. Patients were categorized into the coronary artery bypass grafting (n = 11) and other coronary artery procedure (n = 21) groups. RESULTS: The median age and weight of patients at the time of surgery were 9 months and 4.8 kg, respectively. There were five in-hospital deaths (5/32, 15.6%). The mortality rates were 27.2% (3/11) in the coronary artery bypass grafting group and 9.5% (2/21) in the other coronary artery procedure group (p = .206; 95% confidence interval: 0.496-25.563). The mortality rates for planned and rescue procedures were 8.3% (2/24) and 37.5% (3/8) (p = .06), respectively. The median follow-up time was 12.5 years. Control imaging studies for coronary patency were performed in 70.3% (19/27) of surviving patients. The overall coronary patency rate was 94.7% (18/19). CONCLUSIONS: Pediatric coronary revascularization procedures with elective-planned indications can be performed with good outcomes. Young age and rescue and emergency procedures may carry an increased risk of in-hospital mortality, although not found to be statistically significant. Surviving patients require lifelong follow-up regarding the patency of reperfused coronary arteries.

Total anomalous origin of the coronary arteries from the pulmonary artery: a systematic review.

BACKGROUND: Although rare, coronary artery anomalies can have significant clinical implications. Total anomalous origin of the coronary arteries from the pulmonary artery (TCAPA) represents a rare subtype of coronary artery anomaly for which little is known. The aim of this review was to characterise the presentation, utilised diagnostic modalities, associated cardiac lesions, and treatment strategies in patients with TCAPA. METHODS: A systematic review was performed for cases of TCAPA using PubMed, Embase, and Web of Science. Keywords searched included "total anomalous origin of the coronary arteries from the pulmonary artery," "single ostium anomalous coronary artery from the pulmonary artery," and "anomalous origin of both coronary arteries from the pulmonary artery." RESULTS: Fifty-seven cases of TCAPA were identified in 50 manuscripts. Fifty-eight per cent of patients were male and the median age at presentation was 10 days (mean 1.71 ± 6.6 years, range 0 days-39 years). Most patients were symptomatic at the time of presentation; cyanosis (n = 22) and respiratory distress (n = 14) were the most common symptoms. Cases were most commonly diagnosed at autopsy (n = 26, 45.6%), but operative intervention was pursued in 22 cases (45.6%); aortic re-implantation (n = 14) and a Takeuchi-type repair (n = 7) were the most common routes of repair. CONCLUSIONS: The clinical presentation of patients with TCAPA was found to be variable, likely related to the presence of associated cardiac lesions. TCAPA should be considered in patients with suspected anomalous origin of the left coronary artery from the pulmonary artery for the serious consequences that can occur if not promptly corrected.

Congenital coronary collateral.

Intercoronary communications are a very rare congenital coronary artery anomalies. We report a case of a man who underwent elective coronary angiography that showed a bidirectional direct intercoronary communication between right coronary and left circumflex arteries.

Occlusion of left main coronary artery presenting with ventricular fibrillation in teenagers: an unrecognised cause of sudden death?

In young patients with unexplained ventricular fibrillation, coronary occlusion may be missed by echocardiogram and misinterpreted by CT. We report two patients presenting with ventricular fibrillation and initially negative workup, later identified to have occlusion of left main coronary artery. We demonstrate the importance of angiography to rule out coronary occlusion in patients with unexplained ventricular fibrillation.

Prevalence and morphologic features of dual left anterior descending artery subtypes in coronary CT angiography.

OBJECTIVES: The purpose of this study was to identify the prevalence of dual left anterior descending artery and define the morphological features using coronary computed tomography angiography in order to raise awareness of dual left anterior descending artery among radiologists. MATERIALS AND METHODS: The radiologic reports of 1912 patients who underwent coronary computed tomography angiography at our institution were searched retrospectively for the presence of dual left anterior descending artery. Computed tomography images of patients with dual left anterior descending artery were then reviewed, and the morphological features of dual left anterior descending artery were evaluated. RESULTS: Dual left anterior descending artery was identified in 1.3% of the patients in this study population. Type 1 dual left anterior descending artery was the most common subtype (76%). Four additional cases that were not classified before were also detected. CONCLUSION: Dual left anterior descending artery has a variety of subtypes reported mostly as odd cases, and gaining diagnostic awareness of dual left anterior descending artery is more critical, rather than listing and counting the subtypes. With the widespread use of coronary computed tomography angiography, it has become essential for radiologists to have knowledge about dual left anterior descending artery, as it is vital, especially for surgical planning.

Dual-source computed tomography coronary artery imaging in children.

Computed tomography (CT) has a well-established diagnostic role in the assessment of coronary arteries in adults. However, its application in a pediatric setting is still limited and often impaired by several technical issues, such as high heart rates, poor patient cooperation, and radiation dose exposure. Nonetheless, CT is becoming crucial in the noninvasive approach of children affected by coronary abnormalities and congenital heart disease. In some circumstances, CT might be preferred to other noninvasive techniques such as echocardiography and MRI for its lack of acoustic window influence, shorter acquisition time, and high spatial resolution. The introduction of dual-source CT has expanded the role of CT in the evaluation of pediatric cardiovascular anatomy and pathology. Furthermore, technical advances in the optimization of low-dose protocols represent an attractive innovation. Dual-source CT can play a key role in several clinical settings in children, namely in the evaluation of children with suspected congenital coronary artery anomalies, both isolated and in association with congenital heart disease. Moreover, it can be used to assess acquired coronary artery abnormalities, as in children with Kawasaki disease and after surgical manipulation, especially in case of transposition of the great arteries treated with arterial switch operation and in case of coronary re-implantation.

Imaging Approaches for Coronary Artery Anomalies: Purpose and Techniques.

PURPOSE OF REVIEW: Comprehensive discussion of alternative techniques in imaging coronary artery anomalies (CAAs) in different diagnostic scenarios. RECENT FINDINGS: At primary screening, intramural course essentially correlates with stenosis and clinical repercussion in some types of CAAs. Potential clinical aims in imaging patients with CAAs may be primary screening, severity evaluation, preoperative planning, and postoperative follow-up. Appropriate techniques are echocardiography, magnetic resonance imaging, computed tomography angiography, and intravascular ultrasound (IVUS). Rarely, IVUS may be needed in patients with potentially serious CAAs and has unique advantages and limitations. IVUS data are essential for in-depth knowledge of mechanisms of coronary dysfunction (exercise-related dynamic stenosis of variable severity) and for indicating interventional treatment. In adolescents or adults, intramural course of an anomalous coronary artery is the only important feature accompanied by stenosis of potential severity and is especially relevant in patients with a high-risk status (elite sport athletes, military recruits).

Congenital absence of coronary ostia in a single/common coronary system.

Coronary ostial atresia seen with pulmonary atresia and coronary-cameral fistulae or, more rarely, in isolation manifested as left main coronary artery atresia, is well described. We describe the clinical course and post-mortem findings in a neonate who suffered a fatal cardiac arrest and was found to have congenital absence of both coronary ostia in a single/common coronary system.

Does an Absent Left Main Lead to a Main Event?

This is a case report of a 29-year-old, male applicant for life insurance who was discovered to have an absence of the left main coronary artery (LMCA), with the left anterior descending coronary artery (LAD) and left circumflex coronary artery (LCX) each arising directly from the left sinus of Valsalva. A brief review of several types of coronary artery anatomic variants will be presented.

Atrioventricular depolarization differences identify coronary artery anomalies in Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is the leading cause of acquired heart disease in children. Signal average electrocardiogram changes in patients during the acute phase of KD with coronary artery anomalies (CAA) include depolarization changes. We set out to determine if 12-lead-derived atrioventricular depolarization differences can identify CAA in patients with KD. METHODS: A blinded, retrospective case-control study of patients with KD was performed. Deep Q waves, corrected QT-intervals (QTc), spatial QRS-T angles, T-wave vector magnitudes (RMS-T), and a novel parameter for assessment of atrioventricular depolarization difference (the spatial PR angle) and a two dimensional PR angle were assessed. Comparisons between groups were performed to test for significant differences. RESULTS: One hundred one patients with KD were evaluated, with 68 having CAA (67.3%, mean age 3.6 ± 3.0 years, 82.6% male), and 32 without CAA (31.7%, mean age 2.7 ± 3.2 years, 70.4% male). The spatial PR angle significantly discriminated KD patients with CAA from those without, 59.7° ± 31.1° versus 41.6° ± 11.5° (p < .001). A spatial PR angle cutoff value of 56.9° gave positive/negative predictive values and odds ratios of 93.8%, 43.5%, and 11.5% (95% confidence interval (CI) 2.6-52.2). The two dimensional PR angle either below 7° or above 92° gave positive/negative predictive values and odds ratios of 100.0%, 38.8%, and 21.1% (95% CI 1.2-362.8). No other parameters significantly differentiated the groups. CONCLUSION: Atrioventricular depolarization differences, measured by the spatial or two dimensional PR angle differentiate KD patients with CAA versus those without.

Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest.

OBJECTIVES: To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R). STUDY DESIGN: This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014. RESULTS: A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 years, 25th-75th percentiles]) were enrolled. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT). CONCLUSION: Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest.

MDCT of congenital coronary artery fistulas.

OBJECTIVE: The purpose of this article is to describe the evaluation of congenital coronary artery fistulas (CAFs) with MDCT angiography with ECG gating (MDCTA), including the clinical manifestations, scanning techniques, differential diagnosis, and other imaging methods that may be used. CONCLUSION: Congenital CAFs are rare coronary artery anomalies of termination. MDCTA is a first-line modality for pretreatment planning, and imaging findings should be recognized because CAFs may be detected incidentally.