Utility of positron emission tomography myocardial perfusion imaging for identifying ischemia and guiding treatment in patients with anomalous coronary arteries.

BACKGROUND: The assessment of anomalous coronary arteries (AAOCA) remains controversial without an optimal stress modality for ischemia. We evaluated the value of PET-CT myocardial perfusion imaging in these patients and subsequent management. METHODS AND RESULTS: AAOCA patients (n = 82) undergoing PET-CT from 2015 to 2021 were retrospectively chart reviewed. Multivariable analyses performed to assess relevant clinical and imaging factors associated with ischemia on PET and AAOCA surgery. Key characteristics include mean age 45 ± 20 years, 30 (37%) female, 45 (55%) with chest pain, 19 (23%) anomalous left main coronary artery, 58 (71%) anomalous right coronary artery, 26 (32%) with objective ischemia on PET-CT, and 37 (45%) who underwent AAOCA surgery. Adverse outcomes over mean follow-up of 2.2 ± 1.8 years included one death and two myocardial infarctions. Anomalous left main was independently associated with ischemia on PET-CT, odds ratio (95% confidence intervals) 4.15 (1.31-13.1), P = .006. Chest pain and ischemia on PET-CT were independently associated with and provided incremental prognostic value for surgery, odds ratio 9.73 (2.78-34.0), P < .001 and 6.79 (1.99-23.2), P = .002, respectively. CONCLUSION: Ischemia on PET-CT occurred in a third of our cohort, identifying patients who may benefit from surgery. Larger studies are needed to evaluate the interplay between AAOCA, ischemia by PET and surgery.

A novel echocardiographic approach for assessing coronary artery origins.

INTRODUCTION: Anomalous aortic origin of a coronary artery (AAOCA) is a potential etiology of sudden cardiac death (SCD) in physically active individuals. Identification of coronary artery origins is an essential part of comprehensive pre-participation athletic screening. Although echocardiography is an established method for identifying AAOCA, current imaging protocols are time intensive and readers frequently have low confidence in coronary artery identification. METHODS: Echocardiographic images from a sample of 110 patients from a database of competitive athletes ages 13-22 years from the Kansas City metropolitan area were reviewed by six echocardiographers of varying experience. Coronary artery images were provided to the readers in the conventional single plane for all the patients; then biplane images of the same patients were presented to the readers. While reviewing the images, readers recorded perceived confidence level of identifying the coronary artery from 1 (least confident) to 5 (most confident). Ratings and differences between ratings were summarized descriptively by means and standard deviations across all readings as well as by individual reader. RESULTS: The mean confidence level of echocardiogram readers in identifying coronary artery origins increased by 0.4 points (P = .05) on a five-point confidence scale when using biplane imaging rather than single plane imaging. When assessing the variability of confidence of readers on the same patient, the between-reader variability improved from 25.9% to 10.3%. CONCLUSIONS: Biplane echocardiographic imaging increases the confidence of readers in identifying coronary artery origins.

Echocardiographic diagnosis and follow-up for ALCAPA syndrome treated with the Takeuchi procedure.

We present the case of an 18-year-old man with aborted sudden cardiac death. His initial echocardiogram suggested an anomalous origin of the left coronary artery from the pulmonary artery. Diagnosis was confirmed with coronary angiography. He underwent Takeuchi procedure and fully recovered. A two-year follow-up echocardiogram showed a moderate supravalvular pulmonary stenosis related to the transpulmonary baffle. The presence of extensive collateral circulation should raise suspicion of ALCAPA. Postoperative surveillance in this group of patients needs to be oriented in finding complications such as supravalvular pulmonary stenosis, aortic and pulmonary valve insufficiency, and baffle obstruction and leaks.

Stress cardiomyopathy in dextrocardia with situs inversus and anomalous coronary arteries.

We present a 53-year-old male with the rare constellation of stress cardiomyopathy, dextrocardia with situs inversus and anomalous coronary anatomy. This case highlights the difficulties faced when managing patients with uncommon disorders and demonstrates a rare overlap of acquired and CHD.

Adult-type ALCAPA syndrome: A rare coronary artery anomaly.

Anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA or Bland-White-Garland syndrome) is a rare but serious congenital coronary artery anomaly, with a poor prognosis without surgical repair. There are two types of ALCAPA syndrome: infant type and adult type. We present a rare case of a 63-year-old female patient, with isolated left anterior descending artery origin from the pulmonary artery. Coronary computed tomography angiography revealed giant and tortuous coronary arteries with many collaterals between the left and right coronary system. The patient refused any surgical treatment.

Comprehensive intraoperative transesophageal echocardiography of anomalous left coronary artery from pulmonary artery: What to look for and where to look?

Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) accounts for 0.4% of the congenital heart diseases. Comprehensive 2D and 3D transesophageal echocardiographic imaging of a well-collateralized subset of ALCAPA is described. A nonstandard short-axis view of both aorta and pulmonary arteries showed the origin of left coronary artery from the posterior sinus of the pulmonary artery and right coronary artery in its usual position. Pulse-wave interrogation of the coronary arteries showed the direction of flow in opposite directions. Using the real time-3D, the en-face views of the origins of both coronaries were also demonstrated.

Hybrid CCTA/SPECT myocardial perfusion imaging findings in patients with anomalous origin of coronary arteries from the opposite sinus and suspected concomitant coronary artery disease.

BACKGROUND: Anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS) are associated with adverse cardiac events. Discrimination between ACAOS and coronary artery disease (CAD)-related perfusion defects may be difficult. The aim of the present study was to investigate the value of hybrid coronary computed tomography angiography (CCTA)/SPECT-MPI in patients with ACAOS and possible concomitant CAD. METHODS: We retrospectively identified 46 patients (mean age 56 ± 12 years) with ACAOS revealed by CCTA who underwent additional SPECT-MPI. ACAOS with an interarterial course were classified as malignant, whereas all other variants were considered benign. CCTA/SPECT-MPI hybrid imaging findings (ischemia or scar) were analyzed according to the territory subtended by an anomalous vessel or a stenotic coronary artery. RESULTS: Twenty-six (57%) patients presented with malignant ACAOS. Myocardial ischemia or scar was found only in patients who had concomitant obstructive CAD in the vessel matching the perfusion defect as evidenced by hybrid CCTA/SPECT imaging. CONCLUSION: Hybrid CCTA/SPECT-MPI represents a valuable non-invasive tool to discriminate the impact of ACAOS from concomitant CAD on myocardial ischemia. Our results suggest that in a middle-aged population myocardial ischemia due to ACAOS per se may be exceedingly rare and is more likely attributable to concomitant CAD.

An interesting and unusual coronary artery anomaly.

Cardiac CT angiography utilized in the emergency room for triage of low-to-intermediate risk chest pain on occasion identifies unexpected abnormalities. In this case, multiple coronary anomalies existed in the same patient. Images and clinical decision making are discussed in this presentation. Coronary anomalies are being recognized with increased frequency due to increasing use of this modality in the evaluation of chest pain. The clinical implications of these findings are not always clear.

Stress test to STEMI: Utility of coronary CTA in the diagnosis and management of anomalous right coronary artery from the left coronary cusp.

A 42-year-old woman presented to the emergency department with chest pain. Acute coronary syndrome was ruled out. During dobutamine stress echocardiography (DSE), she developed chest pain and inferior ST elevation. Emergent coronary angiography revealed no culprit lesions but did show an anomalous right coronary artery (RCA). Coronary CT angiography (CCTA) confirmed an anomalous RCA arising from the left coronary cusp with a slit-like ostium and interarterial course (ARCA-LCC-IA). Herein, we review the extant literature on ARCA-LCC-IA, its clinical presentation, the vital role of CTA and MRI in its diagnosis, as well as challenges and controversies surrounding management.

Single right coronary artery with absence of the left main coronary artery, left anterior descending artery, and circumflex artery.

Single coronary artery is an extremely rare congenital coronary anomaly in which the entire coronary arterial system arises from a solitary ostium. The Lipton's classification is used for single coronary artery anomalies. Herein, we present a 72-year-old woman with single coronary artery anomaly admitted with atypical chest pain. Coronary angiography and multidetector computed tomography coronary angiography findings were shared.

Exercise restriction is not associated with increasing body mass index over time in patients with anomalous aortic origin of the coronary arteries.

Anomalous aortic origin of the coronary arteries is associated with exercise-induced ischaemia, leading some physicians to restrict exercise in patients with this condition. We sought to determine whether exercise restriction was associated with increasing body mass index over time. From 1998 to 2015, 440 patients ⩽30 years old were enrolled into an inception cohort. Exercise-restriction status was documented in 143 patients. Using linear mixed model repeated-measures regression, factors associated with increasing body mass index z-score over time, including exercise restriction and surgical intervention as time-varying covariates, were investigated. The 143 patients attended 558 clinic visits for which exercise-restriction status was recorded. The mean number of clinic visits per patient was 4, and the median duration of follow-up was 1.7 years (interquartile range (IQR) 0.5-4.4). The median age at first clinic visit was 10.3 years (IQR 7.1-13.9), and 71% (101/143) were males. All patients were alive at their most recent follow-up. At the first clinic visit, 54% (78/143) were exercise restricted, and restriction status changed in 34% (48/143) during follow-up. The median baseline body mass index z-score was 0.2 (IQR 0.3-0.9). In repeated-measures analysis, neither time-related exercise restriction nor its interaction with time was associated with increasing body mass index z-score. Surgical intervention and its interaction with time were associated with decreasing body mass index z-score. Although exercise restriction was not associated with increasing body mass index over time, surgical intervention was associated with decreasing body mass index z-score over time in patients with anomalous aortic origin of the coronary arteries.

Bilateral congenital coronary ostial obstruction: Exertional syncope in an otherwise healthy 8-year-old.

Congenital stenosis/atresia of a coronary artery is an exquisitely rare anomaly (Congenit Heart Dis, 2, 2007, 347) with increased risk of sudden death. Bilateral coronary obstruction is even more unusual but has been reported in conjunction with aortic valve disease, syphilis, and Takayasu's arteritis. To the best of our knowledge, obstruction of both coronaries in a pediatric patient has only been reported once (Ann Thorac Surg, 55, 1993, 1564). We present a patient with an intramural, anomalous aortic origin of the right coronary artery from the contralateral sinus (AAORCA) with near atresia of the left main coronary ostium. The diagnosis was made by echocardiogram and confirmed by catheterization and magnetic resonance imaging.

Perioperative management of a patient with left ventricular dysfunction and anomalous coronary arteries.

Anesthetic management of patients with coronary artery disease undergoing noncardiac surgery is quite challenging. Such patients are at increased risk of perioperative cardiac complications and death. We report an illustrative case of a 62-year-old male with ischemic heart disease and anomalous coronary arteries for obstructed paraumbilical hernia repair.

A different kind of Christmas tree: anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA).

Anomalous right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital coronary anomaly that has an incidence of 0.002%. We report a case of a previously healthy female who presented to our hospital with pneumonia and was incidentally discovered to have ARCAPA. This was initially diagnosed on echocardiography by the unusual echocardiographic finding of multiple color flow Doppler signals around the right ventricular free wall and apex which were subsequently confirmed by angiography to be due to extensive collateral circulation between the left and right coronary arteries. This represents an unusual echocardiographic manifestation of this very rare condition.

A Re-do Repair of Tetralogy of Fallot With an Anomalous Coronary Artery Using a Valved Conduit for the Right Ventricular Outflow Tract Reconstruction.

Several techniques can be used for the repair of right ventricular outflow tract (RVOT) stenosis in patients with an anomalous coronary artery. Here, we report a case of conduit obstruction after re-operation following double-tract reconstruction and release of stenosis by main pulmonary artery transection and conduit replacement. The patient is a female child diagnosed with tetralogy of Fallot with an anomalous coronary artery (right coronary across right ventricle outflow) who underwent correction with a double-tract RVOT repair at the age of 10 months (weight: 8 kg). At the age of eight years (weight: 24 kg), a conduit re-implantation procedure was required because of an increase in body weight. Designing smooth conduits proved challenging due to the anomalous coronary artery and a short main pulmonary arterial trunk. RVOT stenosis was documented early postoperatively, and further intervention was deemed necessary. At 13 years of age (weight: 45 kg), the patient underwent implantation of an adult-size valved conduit. Transection of the main pulmonary artery and extensive mobilization of the bilateral pulmonary arteries were effective in creating a relative laminar blood flow. Postoperative evaluations confirmed that the conduit was well-shaped and had satisfactory valve functionality. This case highlights the potential difficulties involved with replacing an additional conduit after double-tract cardiac repair procedures, particularly due to anatomical constraints posed by a coronary artery and a short pulmonary arterial trunk. Main pulmonary artery transection and comprehensive mobilization of branch pulmonary arteries can be a solution to conduit design difficulties in RVOT reconstruction after double-tract cardiac repair procedures.

Unveiling the Rarity: An Anomaly of the Left Circumflex Artery Supplying Left Ventricular Apex and Presenting With Myocardial Infarction.

Coronary arteries are typically identified based on the myocardial territory they supply. In rare cases of coronary artery anomalies, the apex of the heart may be supplied by arteries other than the left anterior descending artery. While it is more common for the posterior descending artery from the right coronary artery to supply the apex, there are rare instances where the left circumflex (LCX) artery performs this function. This case report describes an unusual occurrence where the left ventricular apex is supplied by an obtuse marginal branch of the LCX artery. We present this case due to its rarity, unique presentation, and the challenges it poses for both medical and surgical management.

Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery: Advantages of Multimodality Imaging.

Coronary anomalies are one of the most surprising yet challenging pediatric cardiology diagnoses. The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is frequently underdiagnosed due to a lack of typical signs or symptoms. We present a case of ARCAPA in a healthy six-month-old girl during follow-up of a newly detected heart murmur. Echocardiography raised the suspicion of a coronary anomaly, but the diagnosis was unclear, so cardiac catheterization and computed tomography were performed, which posteriorly confirmed the diagnosis. The patient underwent surgical repair, and the short-term follow-up has been uneventful. Regular monitoring is essential due to the potential long-term complications of ARCAPA, including myocardial ischemia, heart failure, and sudden cardiac death, underscoring the importance of early diagnosis and continuous management.

A Cardiologist's Clinical Dilemma: An Incidental Finding of a Potentially High-Risk Anomalous Right Coronary Artery Origin.

Coronary artery anomalies may place patients at risk for various adverse events. We present a case of a 62-year-old male with a two-year history of intermittent chest pain. A computed tomography coronary angiogram revealed a rare finding of an anomalous right coronary artery (ARCA) originating from the left ascending aorta, with high-risk features. This case highlights the complexities in diagnosing and managing ARCA, underscoring the importance of individualized care and careful consideration of invasive intervention risks versus potential benefits.

Clinical Outcomes in Adult Patients With an Anomalous Right Coronary Artery from the Left Sinus of Valsalva.

An anomalous origin of the right coronary artery from the opposite sinus of Valsalva with an intramural course (R-ACAOS-IM) may cause sudden cardiac death in children and adolescents. However, the natural history and management of patients in whom this anomaly is detected later during adulthood remains uncertain. The goals of this study were to assess the impact of an R-ACAOS-IM on the clinical outcomes in an adult population and to determine if adult patients with this anomaly who do not have significant coronary artery disease (CAD) can be managed safely without surgical intervention. A database review identified patients aged >35 years with anomalous coronary arteries diagnosed by cardiac catheterization or coronary computed tomography angiography. The outcomes of patients with R-ACAOS-IM were compared with patients with anomalous left circumflex coronary arteries with retroaortic course (LCx-RA) (an anomaly not associated with ischemic events). The primary outcome was all-cause mortality. The study population consisted of 185 patients aged 59 ± 12 years. Clinical characteristics were similar in the R-ACAOS-IM (n = 88) and LCx-RA (n = 97) groups. At a follow-up of 6.6 ± 4.5 years, there was no difference in mortality (hazard ratio 0.64, 95% confidence interval 0.32 to 1.28, p = 0.20) when adjusted for gender, age, and CAD. A subgroup analysis of 88 patients with no obstructive CAD managed nonoperatively found no difference between the LCx and R-ACAOS-IM groups in mortality (hazard ratio 2.45, 95% confidence interval 0.45 to 13.40, p = 0.30). There was no significant difference between the 2 groups in the composite outcome of death, nonfatal myocardial infarction, or survived cardiac arrest. The outcome of adult patients who have anomalous R-ACAOS-IM are similar to patients who have anomalous LCx-RA with a known benign course. In conclusion, these results suggest that most patients who survive this anomaly into adulthood may be managed conservatively without intervention.