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Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Anomalous aortic origin of the left coronary artery (AAOCLA) with an extended transseptal course behind the right ventricular outflow tract (RVOT) is a rare variant that poses challenges not addressed by current surgical techniques. We utilized a novel transconal approach in 7 consecutive patients., Methods: A retrospective review was made of a prospectively collected database for consecutive patients undergoing transconal unroofing of transseptal AAOLCA. Surgical repair entails transection of the RVOT, unroofing the septal course of the AAOLCA, followed by RVOT extension with a rectangular strip of autologous pericardium. Preoperative characteristics, operative details, and postoperative course were abstracted., Results: All 7 patients identified were symptomatic. Median age was 48 years (range, 12 to 62). The AAOLCA with transseptal course was confirmed by computed tomography angiography. Three patients had provocative testing demonstrating anterolateral ischemia. Four patients underwent cardiac catheterization with intravascular ultrasound and indexed fractional flow reserve, confirming flow-limiting lesion. Importantly, 3 patients had negative provocative noninvasive testing for ischemia. Median postoperative hospital length of stay was 6 days (range, 4 to 12). No mortality or major complications occurred during a median follow-up of 0.75 years. Postoperative evaluation demonstrated anatomically patent unroofed AAOLCA with improved indexed fractional flow reserve compared with preoperative (0.59 ± 0.16 vs 0.90 ± 0.03, P = .05)., Conclusions: Complete unroofing of AAOLCA with transseptal course repaired with posterior extension of RVOT is an effective technique with excellent early outcome. Multimodality provocative testing is critical to evaluate these lesions as individual studies may be misleading. Intravascular ultrasound with indexed fractional flow reserve is clinically useful to confirm the hemodynamic significance of specific lesions., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Coronary sinus (CS) ostial atresia/abnormalities prevent access to the CS from the right atrium (RA) for left ventricular (LV) lead implantation. Some patients with CS ostial abnormalities also have a small persistent left superior vena cava (sPLSVC)., Objective: The purpose of this study was to describe CS ostial abnormalities and sPLSVC as an opportunity for LV lead implantation and unrecognized source of stroke., Methods: Twenty patients with CS ostial abnormalities and sPLSVC were identified. Clinical information, imaging methods, LV lead implantation techniques, and complications were summarized., Results: Forty percent had at least 1 previously unsuccessful LV lead placement. In 70%, sPLSVC was identified by catheter manipulation and contrast injection in the left brachiocephalic vein, and in 30% by levophase CS venography. In 30%, sPLSVC was associated with drainage from the CS into the left atrium (LA). When associated with CS ostial abnormalities, the sPLSVC diameter averaged 5.6 ± 3 mm. sPLSVC was used for successful LV lead implantation in 90% of cases. In 80%, the LV lead was implanted down sPLSVC, and in 20%, sPLSVC was used to access the CS from the RA. Presumably because of unrecognized drainage from the CS to the LA, 1 patient had a stroke during implantation via sPLSVC., Conclusion: When CS ostial abnormalities prevent access to the CS from the RA, sPLSVC can be used to successfully implant LV leads. In some, the CS partially drains into the LA and stroke can occur spontaneously or during lead intervention. It is important to distinguish sPLSVC associated with CS ostial abnormalities from isolated PLSVC., (Copyright © 2021 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Coronary venous anatomy can be divided into the greater cardiac venous system and the lesser cardiac venous system. With protocol optimization, including appropriate contrast bolus timing, coronary veins can be depicted with excellent detail on CT. Knowledge of variant coronary venous anatomy can sometimes play a role in pre-procedural planning. Analysis of the coronary venous anatomy on CT can detect coronary venous anomalies that cause right to left shunts with risk of stroke, left to right shunts, and arrhythmias., (Published by Elsevier Inc.)

Aims: The left coronary anomalous origin from the opposite sinus (L- ACAOS) constitutes the most clinically relevant arterial abnormality among the wide spectrum of coronary artery anomalies. We investigated the physiology of L-ACAOS with and without intramural course (IM) in athletes, using the computational fluid dynamic (CFD) analysis., Methods and Results: The coronary artery circulation with L-ACAOS with and without IM has been segmented and then reconstructed, after reviewing both the angiographic and computed tomography findings of 13 consecutive athletes (10 males, mean age 45.1 ± 8.2 years) with L-ACAOS collected in our institution between 1st January 2003 and 1st January 2018. Vorticity magnitude, static pressure and wall shear stress (WSS) have been analysed in a model of L-ACAOS with no IM course and in L-ACAOS-IM at rest and during exercise. The mean vorticity magnitude and WSS significantly increased from rest to exercise in both models, in right coronary artery, left anterior descending and left circumflex coronary arteries. The mean static pressure significantly increased with exercise in IM (1.118e + 004 vs 1.164e + 004 Pa, p < 0.001) as well as the mean vorticity magnitude and the mean WSS (7012.78 1/s vs 9019.56 1/s, p < 0.001, Δ = 2006.78 1/s and 3.02 Pa vs 2.11 Pa, p < 0.001, Δ = 0.91 Pa). This net increment was transmitted to the entire left coronary system in L-ACAOS-IM but not in L-ACAOS with no IM., Conclusions: In L-ACAOS, different hemodynamic parameters observed in the intramural segment seem to confirm that IM is compressed during exercise. These rheological properties might propagated along the left coronary system, potentially predisposing, if confirmed in vivo, distal coronary segments to a higher risk of spasm and thrombosis in athletes., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Coronary artery fistula is a rare congenital cardiac anomaly. We report a 34-year-old woman who presented with a recurrent large pericardial effusion during pregnancy. She was found to have a right coronary artery to coronary sinus fistula. The coronary sinus was severely dilated due to coronary sinus ostial stenosis. Primary surgical closure of coronary artery fistula was performed with resection of coronary sinus ostial stenosis., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) is a rare malformation traditionally considered "malignant" in cases of interarterial course. Recently, a protective effect of the low interarterial subtype (between the aorta and the right ventricle outflow tract) has been described. We present an IVUS-guided percutaneous intervention in a patient with anomalous origin of the left coronary artery from the right coronary sinus presenting with anterior ischemia. In patients with ACAOS, the integration of complementary image techniques is recommended, defining the anomalous course of the vessel and providing an accurate assessment of the individual risk for each patient. The use of IVUS may be advisable, emerging as a really useful tool to complete the study and guide the treatment., (Copyright © 2018. Published by Elsevier Inc.)

Aims: Although anomalous origin of left (AOLCA) and right coronary artery (AORCA) from the wrong sinus may cause sudden death (SD) in athletes, early diagnosis and management of these anomalies are still challenging. We analysed clinical/instrumental profiles of athletes identified with AOLCA/AORCA focusing our attention on diagnosis, management and follow-up., Methods and Results: We report 23 athletes (17 males, mean age 27±17yrs.), 6 with AOLCA and 17 with AORCA. Diagnosis was made by trans-thoracic echocardiography (TTE) in 21/23(91%). Symptoms were present only in 10(41%). Only 3 had an abnormal rest-ECG and 9(39%) an abnormal stress test ECG (3 ST-depression, 4 ventricular arrhythmias, 1 supraventricular arrhythmias, 1 rate-dependent left-bundle-branch-block). Anatomy of the anomalous coronary artery showed no significant correlation with clinical presentation, except for a tendency to higher occurrence of proximal hypoplasia in symptomatic athletes (83% vs 40%, p=0.09). All athletes were disqualified from competitive-sports and advised to avoid strenuous effort. Surgery was recommended to all athletes with AOLCA and 6 with AORCA, but only 6 underwent surgery. No major cardiac events or ischemic symptoms/signs occurred during a mean follow-up of 65±70months., Conclusions: Early diagnosis of AOLCA/AORCA in athletes is feasible by TTE. Typical symptoms/signs of myocardial ischemia are present only in one third of cases thus underlying the need of a high index of clinical suspicion to achieve the diagnosis. After exercise restriction, none had major cardiac events or ischemia symptoms/signs recurrence. There was no correlation between anatomical characteristics and clinical presentation with the possible exception of coronary hypoplasia., (Copyright © 2017 Elsevier B.V. All rights reserved.)

The diagnosis of anomalous origin of the left main coronary artery from the right coronary sinus with an interarterial course in children and adolescents is considered life-threatening and clinical guidelines recommend surgical correction. The prognostic implications of this diagnosis in adults are not clear. This anomaly may present in adults as sudden cardiac death or may be diagnosed incidentally. Treatment of this anomaly in adults should be tailored individually taking into account the clinical presentation and patient characteristics., (Copyright © 2017 Elsevier B.V. All rights reserved.)

We describe the unusual case of a patient with an antenatal aorto-left ventricular tunnel (ALVT) diagnosis and severe left ventricular (LV) dysfunction who underwent successful repair immediately after birth. To the best of our knowledge, no such case has been reported in the English literature. Our case demonstrated that neonates at the worst end of the ALVT spectrum can survive and achieve normalization of LV function through a timely and multidisciplinary approach., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Cardiac resynchronization therapy (CRT) is an important therapeutic strategy in heart failure. However, there is a high incidence of lead implantation failure and suboptimal response, particularly in ischemic cardiomyopathy. This failure rate may partly be secondary to lack of suitable coronary sinus branches for lead implantation. We sought to assess the presence of coronary sinus (CS) tributaries in patients with ischemic and non-ischemic cardiomyopathy., Materials and Methods: Multidetector computed tomography (MDCT) was performed in 100 patients: 25 coronary artery bypass graft (CABG) patients with impaired left ventricular ejection fraction (LVEF), 25 CABG patients with preserved LVEF, 25 patients with non-ischemic cardiomyopathy, and 25 controls. The presence of the CS and its tributaries, including the posterior interventricular vein (PIV), posterolateral vein (PLV), left marginal vein (LMV), and the anterior interventricular vein (AIV) was assessed., Results: The CS, PIV, and AIV were demonstrated in all patients, whereas presence of a PLV and LMV was identified in 68% and 48% of CABG patients with impaired LVEF, 96% and 68% of CABG patients with preserved LVEF, 92% and 80% of patients with non-ischemic cardiomyopathy, and 100% and 80% of controls (p = 0.001 and 0.046 for PLV and LMV, respectively)., Conclusions: This is the first report to demonstrate that the posterolateral vein and left middle vein, branches of the coronary sinus, are detectable in a significantly smaller number of CABG patients with impaired LVEF compared to controls, CABG with preserved LVEF, and non-ischemic cardiomyopathy. The absence of CS tributary veins in ischemic cardiomyopathy potentially hinders proper lead implantation and results in suboptimal CRT response., (Copyright © 2016 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Anomalous aortic origin of the left coronary artery from the right coronary sinus is a rare congenital anomaly that may cause sudden death. Direct translocation of the left coronary artery could be difficult due to its intramural course and proximity to the aortic valve. We described the surgical management of a child after sudden hemodynamic collapse and the coronary translocation technique that prevented distortion of the aortic root and allowed successful translocation of the abnormal coronary artery despite its intramural course immediately behind the aortic valve commissure., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We herein present a new surgical technique in the treatment of an anomalous left coronary artery from the right coronary sinus in an 18-year-old patient with an intramural course of the left main coronary artery., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We present a case of atresia of the right atrial ostium of the coronary sinus. Recognizing this anomaly is important during catheter ablation or cardiac surgery, which could be noninvasively evaluated by multidetector CT., (Copyright © 2015 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease. Among previous cases, anomalous connection of the right pulmonary vein (RPV) to the coronary sinus (CS) was seldom reported. Here, we report an 8-month-old girl, initially presenting with congestive heart failure, who had this rare anomaly with moderate-sized secundum atrial septal defect (ASD). After confirmation by multi-detector row computed tomography (MDCT) and cardiac catheterization, the infant underwent a successful surgical repair. To our knowledge, this is the first reported infant with this kind of rare anomaly., (Copyright © 2013. Published by Elsevier B.V.)

Among the wide spectrum of congenital abnormalities of coronary arteries, a single coronary artery is often confused with an ectopic coronary artery connected with the contralateral coronary artery. Both abnormalities are characterized by a single coronary ostium, but they differ by the lack or not of an initial ectopic course. The prognosis of anomalous connections of coronary arteries depends mainly on the type of the initial course in relation to other cardiac structures. Therefore, the distinction between a single coronary artery and an ectopic coronary artery connected with the contralateral artery is of importance., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Persistence of a left-sided superior vena cava (PLSVC) with absent right superior vena cava (isolated PLSVC) is a very rare venous malformation and commonly associated with congenital heart disease or alterations of the cardiac situs. We describe an unusual case of a young boy presenting with persistent atrial tachycardia and congestive heart failure. He was detected to have unexplained grossly dilated right atrium, right ventricle with systolic dysfunction and a giant coronary sinus (CS). The dilated CS closely mimicked a pseudo cor-triatriatum on echocardiography. Contrast echocardiography from both arms revealed opacification of the CS before the right atrium. Bilateral upper limb venography confirmed the presence of absent right SVC and isolated persistent left SVC draining into the giant coronary sinus., (Copyright © 2013 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

A 75-year-old man, 8 years after CABG, with ischemic cardiomyopathy underwent cardiac resynchronization therapy (CRT) for refractory heart failure. Retrograde occlusion venography revealed absence of lateral vein. A functionally occluded middle cardiac vein with branch to anterolateral vein was used for left ventricular lead implantation. Using a collateral route for left ventricular lead implantation is a new technique. Lead position was stable with excellent threshold. Follow-up at 6 months reveals continued stable lead position., (Copyright © 2013 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

A single left coronary artery is an extremely rare congenital anomaly that is commonly associated with other congenital cardiac malformations. We present a case involving a single left coronary artery and coronary sinus anomaly demonstrated on coronary angiography and multidetector computed-tomography angiography. To the best of our knowledge, this rare combination has not been reported previously., (Copyright © 2012 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

A patient with combined aortic insufficiency and stenosis underwent aortic valve replacement. After weaning off cardiopulmonary bypass, a new shunt into the left atrium was noted that had not been apparent on multiple previous echocardiographs. Using an agitated saline test, we confirmed a shunt between the coronary sinus and left atrium. The assumption was made that there had been an iatrogenic fistula created with placement of the retrograde catheter; however, upon opening the left atrium it was realized that the patient had a previously undiagnosed, congenitally unroofed coronary sinus. It was repaired primarily and the patient had an uneventful recovery., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Anomalous origin of coronary artery (AOCA) has been described by coronary angiography or autopsy. However the actual prevalence of such abnormalities is unknown. Multi-detector computed tomography (MDCT) offers the possibility to visualize AOCA non-invasively. The purpose of this study was to report the prevalence AOCA by MDCT., Methods and Results: In 5869 consecutive subjects who underwent coronary MDCT (Aquilion 64, Toshiba Medical Systems Corporation, Otowara, Japan) at one center, the prevalence of AOCA was 89 (1.52%) patients. The most common abnormality (33 cases, 0.56%) was the origin of the coronary artery or branch from the opposite or non-coronary sinus. The right coronary arteries (RCA) arising from the left coronary artery sinus (LCS) was observed in 27 cases (0.46%). Vascular cross-sectional area of such RCA arising from LCS was significantly smaller in 11 patients with angina than in 10 patients without symptoms [3.02 (1.68-7.67) mm² vs 5.93 (2.54-12.04) mm² p < 0.05]. The left coronary artery arising from the non-coronary sinus was observed in 2 cases (0.03%), and the left anterior descending coronary artery (LAD) or left circumflex coronary artery (LCX) arising from the right coronary sinus was observed in 4 cases (0.07%). Single coronary arteries were found in 5 cases (0.09%). Fifteen patients (0.26%) presented multiple coronary ostia, all of 15 (0.26%) had the LAD and LCX separately arising from the left coronary sinus. High take off was found in 36 cases (0.60%)., Conclusion: MDCT can accurately detect and characterize the type of AOCA., (Copyright © 2011 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

The anomalous origin of the entire coronary system by three separate ostia within the right coronary sinus is a very rare anomaly with only 34 cases reported in the literature to date. A patient with this rare anomaly who developed coronary artery disease, requiring revascularization, is presented. His coronary computed tomography angiography and coronary angiographic findings are discussed. The present case demonstrates the complimentary roles of coronary computed tomography angiography and conventional cardiac catheterization in managing a patient with anomalous coronary arteries and coronary artery disease.

Coronary artery fistulae are very rare congenital anomalies which constitute 0.2-0.4% of all congenital heart diseases. The right chambers of the heart are the most frequent communication site of the coronary fistulae and may cause hemodynamic impairment in the coronary circulation. The fistulae arise frequently from right coronary and left anterior descending arteries. Fistulae from left circumflex artery are very rare. We report a case in which transcatheter embolization was performed with 3-mm Guglielmi detachable coils in a young adult with a rare type of congenital fistula originating from the circumflex artery and draining into the coronary sinus.

We diagnosed unroofed coronary sinus preoperatively by the 3D volume rendering and endocardial views with 64-slice MDCT, and those clearly showed the defect of the roof of the coronary sinus and the relationship with the surrounding structure. MDCT is a good non-invasive tool for the evaluation of unroofed coronary sinus, and the reconstructed endocardial view is useful for preoperative surgical planning.

The present report describes the technical aspects of the atrial switch operation in the setting of dextrocardia, bilateral superior vena cavae, left atrial isomerism, and unroofed coronary sinus. Augmentation of the right atrial wall using bovine pericardium and in situ pericardial technique for construction of the pulmonary venous baffle ensured unobstructed systemic and pulmonary venous pathways.

Left persistent superior vena cava connected to coronary sinus may coexist with right superior vena cava connected to left atrium. Surgical correction is not mandatory if there is no cyanosis due to a big interjugular bridging vein.

Total anomalous pulmonary venous connection (TAPVC) to the coronary sinus is a well-known entity but variations in connection sites are known to occur, the commonest among them is mixed connection. Here we describe two rare variants of TAPVC to coronary sinus. Group I (3 cases) in which there were dual sites of connection and group II (2 cases), TAPVC to coronary sinus was associated with a persistent left superior vena cava (LSVC). This was seen in total number of 45 cases of isolated TAPVC and 8 cases of TAPVC to coronary sinus between 2000 and December 2005 in our institute. All patients underwent surgical correction. In both of these groups, surgical correction may pose a challenge, which is discussed.