Primary endovascular embolisation of intracranial arteriovenous malformations (AVM)-UK single centre experience.

PURPOSE: Intracranial arteriovenous malformations (AVMs) treated at our institution with modern techniques of endovascular intervention were analysed for the rate of complete occlusion, associated morbidity, and mortality. To our knowledge, this is the first series from the UK evaluating the effectiveness of endovascular embolisation as a primary treatment for selected cases. METHODS: All newly referred AVMs between January 2017 and June 2022 were reviewed and those treated with primary endovascular intervention were identified. Details of the endovascular procedures were retrospectively reviewed. RESULTS: In 5½ years, 41.1% of AVMs referred to our institution have been triaged for primary endovascular intervention. Sixty-eight AVMs were embolised and followed-up: 44 ruptured and 24 unruptured. Spetzler-Martin grading varied from I to III, and a single AVM was grade IV. The approach was arterial in 73.5%, solely venous in 7.4%, and combined in 19.1%. The mean follow-up was 18 months for imaging and 26 months for clinical assessment. Complete obliteration was achieved in 95.6%. Ruptured AVM cohort: The rate of functional deterioration was 13.6%. Unruptured AVM cohort: The rate of functional deterioration secondary to complications from embolisation was 4.2%. CONCLUSIONS: Endovascular embolisation may be a favourable option for primary AVM treatment in carefully selected patients. However, selection criteria need to be better delineated for more specialists to consider this as a primary therapy.

Multi-modality treatment approach for paediatric AVMs with quality-of-life outcome measures.

PURPOSE: Despite the potentially devastating and permanently disabling effects of paediatric arteriovenous malformations (pAVMs), there is a paucity of studies reporting long-term quality-of-life (QoL) outcomes in AVM patients. We aim to evaluate the management strategies for paediatric intracranial pAVMs in the UK and long-term QoL outcomes using a validated paediatric quality-of-life outcome measure. METHODS: In this single-centre case-series, we retrospectively reviewed a prospectively maintained database of all paediatric patients (i.e. 0-18 years old) with intracranial AVMs, who were managed at Alder Hey Children's Hospital from July 2007 to December 2021. We also collected the PedsQL 4.0 score for these patients as a measure of QoL. RESULTS: Fifty-two AVMs were included in our analysis. Forty (80%) were ruptured, 8 (16%) required emergency intervention, 17 (35%) required elective surgery, 15 (30%) underwent endovascular embolisation, and 15 (30%) patients underwent stereotactic radiosurgery. There was an 88% overall obliteration rate. Two (4%) pAVMs rebled, and there were no mortalities. Overall, the mean time from diagnosis to definitive treatment was 144 days (median 119; range 0-586). QoL outcomes were collected for 26 (51%) patients. Ruptured pAVM presentation was associated with worse QoL (p = 0.0008). Location impacted psychosocial scores significantly (71.4, 56.9, and 46.6 for right supratentorial, left supratentorial, and infratentorial, respectively; p = 0.04). CONCLUSION: This study shows a staged multi-modality treatment approach to pAVMs is safe and effective, with superior obliteration rates with surgery alone. QoL scores are impacted by AVM presentation and location regardless of treatment modality.

Lodewyk H.S. van Mierop (March 31, 1927-October 17, 2021): a true giant.

We honour a great man and a true giant. Lodewyk H.S. van Mierop (March 31, 1927 - October 17, 2021), known as Bob, was not only a Paediatric Cardiologist but also a dedicated Scientist. He made many significant and ground-breaking contributions to the fields of cardiac anatomy and embryology. He was devoted as a teacher, spending many hours with medical students, Residents, and Fellows, all of whom appreciated his regularly scheduled educational sessions. Those of us who were fortunate to know and spend time with him will always remember his great mind, his willingness to share his knowledge, and his ability to encourage spirited and fruitful discussions. His life was most productive, and he will long be remembered by many through his awesome and exemplary scientific contributions.His legacy continues to influence the current and future generations of surgeons and all providers of paediatric and congenital cardiac care through the invaluable archive he established at University of Florida in Gainesville: The University of Florida van Mierop Heart Archive. Undoubtedly, with these extraordinary contributions to the fields of cardiac anatomy and embryology, which were way ahead of his time, Professor van Mierop was a true giant in Paediatric Cardiology. The invaluable archive he established at University of Florida in Gainesville, The University of Florida van Mierop Heart Archive, has been instrumental in teaching medical students, Residents, Medical Fellows, and Surgical Fellows. Only a handful of similar archives exist across the globe, and these archives are the true legacy of giants such as Dr. van Mierop. We have an important obligation to leave no stone unturned to continue to preserve these archives for the future generations of surgeons, physicians, all providers of paediatric and congenital cardiac care, and, most importantly, our patients.

Retro-aortic brachiocephalic vein with azygous vein continuation: a rare vascular variant.

We present an infant with incidentally found retro-aortic left brachiocephalic vein draining directly into azygous vein. This rare vascular variant is asymptomatic but does have clinical implications with surgical and interventional procedures.

Takayasu's arteritis presenting as acute myocardial infarction: case series and review of literature.

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu's arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu's arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. The purpose of this report is to describe the challenges in the clinical diagnosis and management of Takayasu's arteritis with myocardial infarction. We also seek to enhance awareness about unique presentations of Takayasu's arteritis within the paediatric community.

Screening for cerebrovascular disorder on the basis of family history in asymptomatic children.

Background: Cerebrovascular disorders represent a group of uncommon, heterogeneous, and complex conditions in children. We reviewed the screening practice for the detection of cerebrovascular disorder in asymptomatic children referred to our neurovascular service on the basis of a positive family history and parental and/or treating physician concern.Methods: Retrospective case-note review of referrals to our neurovascular service (July 2008-April 2018). Patients were included if the referral was made for screening, on the basis of a positive family history of cerebrovascular disorder. Symptomatic children, those with previous cranial imaging, or children under the care of a clinical geneticist (i.e. due to the child or their relative having HHT or mutations in KRIT1) were not eligible for inclusion.Results: Forty-one children were reviewed, 22 males (Median age 10.7 years, range 0.6-15.6 years). This represented 22% of the total number of referrals over a 10-year period. Twenty-nine children had an MRI/MRA brain. Twenty-eight children were referred due to a family history of intracranial aneurysm and/or subarachnoid haemorrhage, but only two had two first-degree relatives affected. Ten children were referred due to a family history of arteriovenous malformation. Three children were referred due to a family history of stroke. No cerebrovascular disease was detected during the study period (n = 29).Conclusions: Parental and/or physician concern generated a substantial number of referrals but no pathology was detected after screening. Whilst general screening guidance exists for the detection of intracranial aneurysms, consensus guidelines for the screening of children with a positive family history do not, but are required both to guide clinical practice and to assuage parental and/or physician concerns.

Optimized Computed Tomography Angiography Protocol for the Evaluation of Thrombus in Patients with Fontan Anatomy.

The Fontan procedure is the final stage in the palliative surgical approach to patients with single-ventricle physiology. These patients have an increased risk for thromboembolic disease in the Fontan circuit, which can be evaluated by chest computed tomography angiography (CTA) in acute settings. However, false-positive results are common secondary to unusual streaming patterns in the Fontan circuit. A biphasic CTA protocol was evaluated for the capability to clearly identify structures of the Fontan circuit that are critical for the evaluation of thromboembolic disease. The study was a retrospective chart review of Fontan patients with a chest CTA scan obtained between 2011 and 2017. Two pediatric cardiologists with additional training in cardiac CT imaging independently reviewed each CTA and awarded one point for each of 5 Fontan circuit structures clearly identified resulting in a score range of 0-5. A score of 0-2 considered not capable, 3-4 partially capable, and 5 capable to clearly identify critical structures of the Fontan circuit. During the study period, 46 CTA scans were performed on 21 patients. Of the CTA scans using a biphasic protocol, 62.5% (10/16) were considered capable to clearly identify all 5 critical structures of the Fontan circuit vs 27% (8/30) of the CTA scans using a monophasic protocol (p = 0.027). Overall our results suggest that the single-site biphasic CTA protocol has greater diagnostic capability to detect the presence of Fontan thromboembolic disease when compared to the more traditional monophasic CTA protocol. Future prospective studies are needed to confirm these findings.

Safety and efficacy of intracranial aneurysm embolization using the "combined remodeling technique": low-profile stents delivered through double lumen balloons: a multicenter experience.

PURPOSE: Since appearance of the balloon-remodeling technique and stent-assisted coiling, complex aneurysms have been treated successfully by endovascular means worldwide. Although these two techniques have been widely proven, the combination of both traditionally made the procedures more complicated technically. The aim of our study was to determine the technical success, safety, and efficacy of the low-profile stents delivered through double lumen balloons. METHODS: Clinical, procedural, and angiographic data were analyzed. RESULTS: Eighty-four patients (55 women; age range 20-81 years) harboring 86 aneurysms were included in this study. Aneurysm maximal diameter ranged from 2 to 26 mm, with mean 7.5 mm. There were 62 unruptured, 15 recanalized, and 9 acutely ruptured aneurysms. Aneurysm locations were ACoA (31), MCA (36), supraclinoid ICA (4), carotid bifurcation (2), basilar (7), PCA (3), PICA (2), and VA (1). Ninety-three devices were implanted (63 LVIS jr, 15 LEO Baby, 14 ACCLINO Flex and 1 Neuroform Atlas) through the double lumen balloons (Scepter C or XC and Eclipse 2 L). We found 2 minor clinical events (2.4%) and 1 major event (1.2%). Total intra-procedural technical complication rate was 11.6%. Follow-up was available for 71 patients with an average follow-up of 7 months. Complete and near complete occlusion was 90.1%. Residual aneurysms were seen in 9.9%. CONCLUSION: The "combined remodeling technique" with low-profile stents delivered through double-lumen balloons is technically feasible, safe, and effective for the treatment of intracranial aneurysms. This technique allows the operator to avoid extra maneuvers.

Prophylactic Platelet Transfusions for Critically Ill Patients With Thrombocytopenia: A Single-Institution Propensity-Matched Cohort Study.

BACKGROUND: Thrombocytopenia is frequently encountered in critically ill patients, often resulting in prophylactic transfusion of platelets for the prevention of bleeding complications. However, the efficacy of this practice remains unclear. The objective of this study was to determine the relationship between prophylactic platelet transfusion and bleeding complications in critically ill patients. METHODS: This is a retrospective cohort study of adults admitted to surgical, medical, or combined medical-surgical intensive care units (ICUs) at a single academic institution between January 1, 2009, and December 31, 2013. Inclusion criteria included age ≥18 years and a platelet count measured during ICU admission. Propensity-matched analyses were used to evaluate associations between prophylactic platelet transfusions and the outcomes of interest with a primary outcome of red blood cell transfusion in the ensuing 24 hours and secondary outcomes of ICU and hospital-free days and changes in sequential organ failure assessment scores. RESULTS: A total of 40,693 patients were included in the investigation with 3227 (7.9%) receiving a platelet transfusion and 1065 (33.0%) for which platelet transfusion was prophylactic in nature. In propensity-matched analyses, 994 patients with prophylactic platelet transfusion were matched to those without a transfusion. Patients receiving prophylactic platelets had significantly higher red blood cell transfusion rates (odds ratio 7.5 [5.9-9.5]; P < .001), fewer ICU-free days (mean [standard deviation] 20.8 [9.1] vs 22.7 [8.3] days; P = .004), fewer hospital-free days (13.0 [9.7] vs 15.8 [9.4] days; P < .001), and less improvement in sequential organ failure assessment scores (mean decrease of 0.2 [3.6] vs 1.8 [3.3]; P < .001) in the subsequent 24 hours. These findings appeared robust, persisting in multiple predefined sensitivity analyses. CONCLUSIONS: Prophylactic administration of platelets in the critically ill was not associated with improved clinical outcomes, though residual confounding may exist. Further investigation of platelet transfusion strategies in this population is warranted.

A single centre experience with an evolving approach for the repair of coarctation of the aorta.

BACKGROUND: Isolated coarctation of the aorta can be repaired by either lateral thoracotomy or sternotomy approach with end-to-end anastomosis. Most commonly, neonates with coarctation of the aorta also have hypoplasia of the arch, requiring median sternotomy and extended end-to-side anastomosis with arch augmentation. The aim of this study was to describe our experience as the institution adopted the median sternotomy approach for repair, by reviewing complications, mortality, and reintervention. METHODS: Retrospective chart review of 66 patients aged 0-1 year who had arch repair performed by a single surgeon over an 8-year period was performed. Median age at surgery was 22 days (4-232) and median weight was 3.08 kg (1.25-8.0). Forty-one (62%) patients underwent median sternotomy. RESULTS: There was 1 death from a noncardiac cause. Eighteen per cent of our patients were ≤2.5 kg. Vocal cord paresis occurred in 16% of patients under 2.5 kg and 9.5% of patients 2.5 kg or above at the time of surgery. Hypertension at 6-month follow-up was greater in patients under 2.5 kg (44%) than patients 2.5 kg or above (15%). Total surgical reintervention rate was 6%. For patients above 2.5 kg, the surgical reintervention rate was 5.4% and for patients below 2.5 kg, the surgical reintervention rate was 8.3%. CONCLUSION: We concluded that for neonates with coarctation of the aorta and hypoplastic arch, median sternotomy is a safe surgical approach with low morbidity and mortality with the possible advantage of reduced surgical re-intervention and mortality in the population below 2.5 kg.

Idiopathic intracranial hypertension VISION (venous intervention versus shunting in IIH for optic nerve disc swelling) trial: patient perspective questionnaire.

INTRODUCTION: Headaches, visual problems and tinnitus are symptoms of Idiopathic Intracranial Hypertension (IIH) which resolve with reduction of CSF pressure. Impaired cranial venous outflow has been implicated in the pathogenesis and there is evidence of good treatment results in IIH using venous sinus stenting. We are currently initiating a multi-centre randomised controlled trial, the VISION study (Venous Intervention versus Shunting in IIH for Optic Disc Swelling) comparing radiological (venous sinus stenting) to surgical intervention (CSF shunting). As part of the preparations for VISION we made a basic questionnaire available to members of the website IIH UK ( www.iih.org.uk ). METHODS: 10-point questionnaire pertaining to IIH diagnosis, symptoms and management using www.surveymonkey.com . RESULTS: 250 questionnaires were returned. 95.6% of respondents were female, mostly ≤40 years of age. 70% were diagnosed in the last 5 years, but only 35% were diagnosed less than a year after onset of symptoms. 59.4% of patients had not undergone any radiological/surgical intervention, 34.9% had had CSF diversion, 3.6% venous stenting and 2.0% had stent plus shunt. 16.8% indicated their lives were most affected by tinnitus and 18.1% by visual problems, but 49.6% said they were most affected by their headaches. 81% of patients indicated they would be happy to participate in a randomised trial comparing the two treatment options of venous stenting and CSF shunting. CONCLUSION: IIH patients want to be actively involved in their treatment and are favourably disposed towards clinical research. Variation exists in treatment modalities offered. There are individual differences regarding impact of symptoms.

Intracranial dural arteriovenous fistula mistaken as cervical transverse myelitis.

We describe a man with an intracranial dural arteriovenous fistula that presented as a subacute longitudinally extensive cervical myelopathy. The uncommon location of the fistula and the absence of specific radiological signs resulted in initial misdiagnosis as longitudinally extensive transverse myelitis. Neurologists should have a high index of suspicion for dural arteriovenous fistula in older men, especially those with subacute or chronic symptoms, acellular cerebrospinal fluid and, particularly, if there is neurological deterioration soon after corticosteroid treatment. Patients need early angiography to identify this treatable cause of myelopathy.

Prophylactic Plasma Transfusion Is Not Associated With Decreased Red Blood Cell Requirements in Critically Ill Patients.

BACKGROUND: Critically ill patients frequently receive plasma transfusion under the assumptions that abnormal coagulation test results confer increased risk of bleeding and that plasma transfusion will decrease this risk. However, the effect of prophylactic plasma transfusion remains poorly understood. The objective of this study was to determine the relationship between prophylactic plasma transfusion and bleeding complications in critically ill patients. METHODS: This is a retrospective cohort study of adults admitted to the intensive care unit (ICU) at a single academic institution between January 1, 2009 and December 31, 2013. Inclusion criteria included age ≥18 years and an international normalized ratio measured during ICU admission. Multivariable propensity-matched analyses were used to evaluate associations between prophylactic plasma transfusion and outcomes of interest with a primary outcome of red blood cell transfusion in the ensuing 24 hours and secondary outcomes of hospital- and ICU-free days and mortality within 30 days of ICU discharge. RESULTS: A total of 27,561 patients were included in the investigation with 2472 (9.0%) receiving plasma therapy and 1105 (44.7%) for which plasma transfusion was prophylactic in nature. In multivariable propensity-matched analyses, patients receiving plasma had higher rates of red blood cell transfusion (odds ratio: 4.3 [95% confidence interval: 3.3-5.7], P < .001) and fewer hospital-free days (estimated % increase: -11.0% [95% confidence interval: -11.4, -10.6%], P < .001). There were no significant differences in ICU-free days or mortality. These findings appeared robust, persisting in multiple predefined sensitivity analyses. CONCLUSIONS: Prophylactic administration of plasma in the critically ill was not associated with improved clinical outcomes. Further investigation examining the utility of plasma transfusion in this population is warranted.

The role of rheumatoid arthritis (RA) flare and cumulative burden of RA severity in the risk of cardiovascular disease.

OBJECTIVE: To examine the role of rheumatoid arthritis (RA) flare, remission and RA severity burden in cardiovascular disease (CVD). METHODS: In a population-based cohort of patients with RA without CVD (age ≥30 years; 1987 American College of Rheumatology criteria met in 1988-2007), we performed medical record review at each clinical visit to estimate flare/remission status. The previously validated RA medical Records-Based Index of Severity (RARBIS) and Claims-Based Index of RA Severity (CIRAS) were applied. Age- and sex-matched non-RA subjects without CVD comprised the comparison cohort. Cox models were used to assess the association of RA activity/severity with CVD, adjusting for age, sex, calendar year of RA, CVD risk factors and antirheumatic medications. RESULTS: Study included 525 patients with RA and 524 non-RA subjects. There was a significant increase in CVD risk in RA per time spent in each acute flare versus remission (HR 1.07 per 6-week flare, 95% CI 1.01 to 1.15). The CVD risk for patients with RA in remission was similar to the non-RA subjects (HR 0.90, 95% CI 0.51 to 1.59). Increased cumulative moving average of daily RARBIS (HR 1.16, 95% CI 1.03 to 1.30) and CIRAS (HR 1.38, 95% CI 1.12 to 1.70) was associated with CVD. CVD risk was higher in patients with RA who spent more time in medium (HR 1.08, 95% CI 0.98 to 1.20) and high CIRAS tertiles (HR 1.18, 95% CI 1.06 to 1.31) versus lower tertile. CONCLUSIONS: Our findings show substantial detrimental role of exposure to RA flare and cumulative burden of RA disease severity in CVD risk in RA, suggesting important cardiovascular benefits associated with tight inflammation control and improved flare management in patients with RA.

Case Report: Traumatic Carotid-Cavernous Fistula.

Carotid-cavernous fistulae (CCFs) are a rare complication of head trauma, with potentially serious consequences. We report the case of a 45-year-old male patient who presented with posttraumatic CCF 2 months after sustaining a head injury. Appropriate imaging in the form of computed tomography of the head, magnetic resonance imaging of the head, and digital subtraction angiography of the intracranial vessels was performed, and the CCF was successfully coil embolized. This resulted in good resolution of the patient's symptoms. We discuss the cause, presentation, diagnosis, and treatment of CCFs. Carotid-cavernous fistulae are a rare sequela of craniofacial trauma; therefore, a high index of suspicion must be maintained to establish a diagnosis and prevent serious consequences.

Cerebral CT Venography Using a 320-MDCT Scanner With a Time-Density Curve Technique and Low Volume of Contrast Agent: Comparison With Fixed Time-Delay Technique.

OBJECTIVE: The purpose of this study was to compare a cerebral CT venography (CTV) technique performed on a 320-MDCT scanner with the use of a time-density curve (TDC) and a small volume of IV contrast medium (35 mL, with 15 mL used for the test bolus) with CTV performed using an established reference technique in which scanning is performed at a fixed time delay of 30 seconds with the use of a larger volume of contrast medium (100 mL). MATERIALS AND METHODS: The time of peak enhancement was determined from the TDC generated from a scan in which a test bolus dose was used. CTV was performed at the time of peak enhancement. The diagnostic quality of 31 CTV venograms acquired using this technique was compared retrospectively with the diagnostic quality of 29 CTV venograms obtained at a fixed time delay of 30 seconds. The densities in the major venous sinuses and the degree of arterial contamination on the scans acquired using the two techniques were compared using objective and semiobjective methods. The semiobjective assessments were made independently by two neuroradiologists. RESULTS: Attenuation was higher in the venous structures seen on CTV images acquired using the TDC technique. Of the scans obtained using the TDC technique, the proportion deemed to be of good quality, on the basis of a grading scale, was statistically significantly higher (p < 0.05). Also, the degree of arterial contamination was statistically significantly lower (p < 0.05). The interrater agreement for semiobjective assessments ranged from good to very good. CONCLUSION: We describe a CTV technique performed using a low volume of IV contrast medium and a TDC on a 320-MDCT scanner. This technique provides better venous opacification and lower arterial contamination compared with use of the fixed time-delay technique.

Pocket echocardiography system for detection of patent ductus arteriosus in neonates.

BACKGROUND: Neonates are commonly referred for a cardiology consult and an echocardiogram to rule out patent ductus arteriosus (PDA). OBJECTIVES: Evaluate the usefulness of current pocket echocardiography system (PES) in PDA detection compared to traditional full-featured echo system (FFES). HYPOTHESIS: The determination of the presence of a PDA in neonates can be done using PES. METHODS: Fifty newborns with orders for echo evaluation were included in this study. A 5-minute PES scan was performed first. Then a full echo study was performed on a traditional FFES. Images were evaluated by three pediatric cardiologists blinded to the patients and the FFES results. RESULTS: The overall accuracy of reviewers rating PES versus FFES to rule in PDA had low false-positive rates 9.5% (95% CI: 1.2-30%), 11.8% (95% CI: 1.5-36%), 11.1% (95% CI: 1.4-35%) and the false-negative rate to rule out PDA was 0% (95% CI: 0-18%), 5.5% (95% CI: 0.14-27%), 0% (95% CI: 0-26%) for each reviewer 1, 2, and 3, respectively. Upon further analysis, PES scan was shown not to be accurate on infants with body weight below 1000 g with encouraging results for infants above 1000 g and those >37 weeks gestational age. CONCLUSIONS: Our results suggest that the current PES could potentially be used in larger and near-term infants but has less than acceptable performance in low birth weight and premature infants in determining who should undergo a FFES study for persistent PDA diagnosis. Improvements in the technology along with developing limited training protocols for noncardiology personnel should make it possible for PES scan to be used as a screening tool and as an extension of physical examination especially in limited resource settings.

Simultaneous bilateral application of the Scepter mini balloon microcatheter for occlusion of ethmoidal dural arteriovenous fistulas.

Ethmoidal dural arteriovenous fistulas are a rare entity accounting for 10 % of all dAVFs.3-6 Haemorrhage occurs in up to 91 % of cases, which is a particularly high risk and warrants therapeutic intervention.8-9 Endovascular treatment for these fistulas using the conventional detachable microcatheter technique is associated with certain limitations and risks; 8.3 % rate of incomplete obliteration and an 8.3 % rate of complications. Complications include reflux of liquid embolic agent, posterior ischaemic optic neuropathy, acute visual loss, and small subdural haematoma secondary to a micro-perforation.8,10-12 We present our recent experience with the Scepter Mini Balloon Microcatheter for the endovascular treatment of ethmoidal dural arteriovenous fistulas in 3 patients, involving bilateral simultaneous inflation of the balloon. It demonstrates a novel application of this technology with good outcomes. It supports the use of this microcatheter in treating ethmoidal dural arteriovenous fistulas endovascularly, either as a first-line option or as an adjunct to surgery.

Case Report: Multimodality evaluation and clinical management of a single coronary artery.

A 14-year-old male with no significant medical history presented with intermittent palpitations for 2-3 months that occurred at rest and were associated with light-headedness. Electrocardiogram in clinic showed sinus arrhythmia with early repolarization and no ischemic changes. The echocardiogram showed normal cardiac structure and function, however, there was a concern for possible anomalous origin of the left coronary artery. Contrast-enhanced CT coronary artery angiogram confirmed a single coronary origin from the right coronary sinus. The single main coronary artery gave rise to the right coronary artery (RCA) and the left coronary artery (LCA). The LCA demonstrated a trans-septal course before it gave rise to the left anterior descending and left circumflex artery. There were intraarterial and intramural portions of the LCA, and the sinoatrial node artery arose from the LCA. The RCA demonstrated a normal course to the right atrioventricular groove, and the posterior descending artery arose from the RCA. Treadmill exercise stress test showed excellent functional capacity without exercise-induced chest pain or ischemic ECG changes. Invasive coronary angiography ruled out luminal narrowing or dynamic compression. Given the absence of physiologic or anatomic evidence of coronary flow restriction, no intervention was pursued and the palpitations were deemed to be likely unrelated to the coronary anomaly and eventually subsided spontaneously on 6 month follow-up.