Anomalous Coronary Artery Research Articles

Unexplained near-drowning can reveal ALCAPA in children

An 11-year-old girl experienced an episode of near-drowning. She was immediately rescued and was defibrillated. Transthoracic echocardiography and coronary computed tomographic angiography confirmed the diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA). We report a rare description of this congenital coronary anomaly in a child, revealed after exercise-induced sudden cardiac arrest while swimming.

Hemodynamic Relevance of Anomalous Coronary Arteries Originating From the Opposite Sinus of Valsalva-In Search of the Evidence.

Coronary artery anomalies (CAA) represent a heterogeneous group of congenital disorders of the arterial coronary circulation, defined by an anomalous origin of the coronary ostium and/or vessel course. Of particular interest are anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS). The interarterial variants (with the anomalous vessel situated between the great arteries) are historically called “malignant,” based on an anticipated higher risk for myocardial ischemia and sudden cardiac death (SCD), especially affecting young patients during strenuous physical activity. However, the interarterial course itself may not be the predominant cause of ischemia, but rather represents a surrogate for other ischemia-associated anatomical high-risk features. As the exact pathophysiology of ACAOS is not well-understood, there is a lack of evidence-based guidelines addressing optimal diagnostic work-up, downstream testing, sports counseling, and therapeutic options in patients with ACAOS. Therefore, treating physicians are often left with uncertainty regarding the clinical management of affected patients. This review focuses on the pathophysiologic consequences of ACAOS on myocardial ischemia and discusses the concept of the interplay between fixed and dynamic coronary stenosis. Further, we discuss the advantages and limitations of the different diagnostic modalities and give an outlook by highlighting the gaps of knowledge in the assessment of such anomalies.

Current Strategies for the Management of Anomalous Origin of Coronary Arteries from the Pulmonary Artery.

The coronary arteries, which have to originate from the aorta, may sometimes rise from the pulmonary artery. This study evaluated clinical and diagnostic findings, treatment methods, and follow up of cases with anomalous coronary arteries arising from the pulmonary artery. Eight patients with the diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA) (N = 6) and anomalous right coronary artery from the pulmonary artery (ARCAPA) (N = 2), between January 2014 and January 2020 from a single center university hospital, were included in the study. Data from patients' demographic characteristics, electrocardiography, echocardiography, angiographic findings, operation, hospitalization, and follow up were evaluated. The study included eight patients (six females and two males) - six patients with ALCAPA and two with ARCAPA. The ages of the patients ranged between 3-135 (average: 53.25) months. The median body weight was calculated as 17.4 kg. Severe mitral valve insufficiency was detected in two patients and two other patients had a moderate degree of mitral insufficiency on echocardiography. Ejection fractions ranged between 16-74%. One patient had perimembranous malalignment large ventricular septal defect with pulmonary stenosis. Operative techniques were Takeuchi procedure (three patients), direct implantation (four patients), and left internal thoracic artery to left main coronary artery bypass (one patient). Mechanical cardiac support was not required in the postoperative period. Mortality did not occur. Mitral insufficiency and ejection fractions improved following correction of the coronary anatomy. It is important to diagnose the ALCAPA or ARCAPA, where the coronary artery originates from the pulmonary artery. Patients should be treated before congestive heart failure and fatal complications occur. Surgical correction should be planned regardless of symptom status, even though some of patients reach adulthood with an increased number of collaterals.

Anomalous aortic origin of a right coronary artery: two brothers with an identical pattern.

We report on the diagnosis of anomalous coronary artery in two brothers. Following the diagnosis of anomalous coronary artery in one sibling, we screened immediate family relatives and found the same anomaly in the older brother. Familiarity in this pathology is extremely rare. We analysed and compared clinical, echocardiographic and radiological findings in the two brothers.

Outcomes in anomalous aortic origin of a coronary artery after surgical reimplantation

ObjectiveAnomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We compared outcomes data of patients who underwent transection and reimplantation (TAR) and patients who underwent an unroofing. MethodsPatients who presented to the Coronary Artery Anomalies Program were evaluated and managed following a standardized approach. Anatomy was determined using computed tomography angiography, myocardial perfusion using advanced stress imaging, and surgical intervention according to anatomic features. ResultsSixty-one patients underwent surgical repair of AAOCA between 2012 and 2019: 16 (26%) patients underwent TAR of the anomalous coronary without an aortic button and 45 (74%) patients underwent coronary unroofing. Compared with patients who underwent an unroofing, patients who underwent TAR had similar intramural length (5 mm with interquartile range of 4-7.7 vs 6 mm with interquartile range of 5-7; P = .6). One patient with an anomalous right coronary underwent coronary artery bypass grafting after TAR because of persistent postoperative ischemic changes. One patient with unroofing of an anomalous left coronary artery presented with recurrent aborted sudden cardiac death and underwent subsequent TAR, without further events. At last follow-up, 15 of 16 patients (94%) who underwent TAR and 42 of 45 (93%) patients who underwent an unroofing were released to unrestricted exercise activities. ConclusionsCoronary artery TAR is a useful surgical alternative for AAOCA when there is a course below the commissure, when unroofing does not relocate the ostium to the appropriate sinus, or when unroofing results in compression by the intercoronary pillar.

Impact of Reverse Left Ventricular Remodeling on Outcomes of Patients with Anomalous Left Coronary Artery from the Pulmonary Artery after Surgical Correction.

The impact of reverse left ventricular remodeling (r-LVR) on clinical outcomes after surgical correction of anomalous left coronary artery from the pulmonary artery (ALCAPA) remains unclear. This study aims to examine the prognostic significance of r-LVR in patients with ALCAPA after surgery. We prospectively identified 61 patients undergoing surgical correction for ALCAPA; 54 patients had adequate echocardiographic image quality with quantitative biplane analysis performed both at baseline and at 30-day postoperative follow-up. Postoperative r-LVR was defined as a reduction of ≥ 10% in left ventricular end-diastolic volume index during follow-up. Cox proportional-hazards regression was used to investigate the independent association of r-LVR and all-cause mortality. Among 54 patients (age: 21.2 ± 7 months; 37% females), r-LVR occurred in 35 patients (64.8%) after surgery. Compared to patients with r-LVR, patients without r-LVR had significantly higher level of N-terminal pro B-type natriuretic peptide (NT-proBNP) [2176 (711, 4219) vs 998 (623, 2145) P < 0.001] and lower survival rate (47.3% vs 82.9%, HR = 5.72 [1.96 to 17.20], P < 0.001) at 1-year follow-up. NT-proBNP (OR = 2.27 [1.67 to 18.3], P = 0.02) was an independent predictor of r-LVR in multivariate analysis. Moreover, r-LVR was significantly associated with a lower rate of all-cause mortality (HR = 0.27 [0.08 to 0.98], P = 0.03) in multivariate analysis, even after adjustment for clinical and echocardiographic variables. R-LVR occurred in more than half of patients with ALCAPA undergoing surgical correction and it was associated with better clinical outcomes. NT-proBNP is an independent predictor of r-LVR.

ALCAPA: 3D MDCT presents an intuitive perspective

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is one of the rare congenital anomalies with exceptional presentation till adulthood. The complications include left ventricular dysfunction, severe mitral regurgitation and myocardial infarction. We report a case of a young female with dyspnea and murmur with left to right shunt which was finally diagnosed as ALCAPA by multi-detector cardiac computed tomography (MDCT). A 45-year-old female presented with dyspnoea on exertion New York Heart Association [(NYHA) II class] and chest heaviness for seven days. Physical examination reveals 2/6 systolic diffuse murmur over the left sided chest with predominance over the pulmonary area. Electrocardiogram (ECG) reveals sinus rhythm with infrequent premature ventricular contractions (PVCs) and echocardiography reveals left to right shunt flow with inconspicuous ends. Cardiac computed tomography (CT) study reveals very interesting and unusual findings. Only one coronary artery was found to originate from the aortic root (Figure 1A and Figure 1B). Right coronary artery (RCA) originates from the right coronary sinus as a large-sized and ecstatic artery with a highly tortuous course and abundant collaterals (Figure 1C). No coronary origin was detected from the left coronary cusp. Surprisingly, the left coronary equivalent artery was revealed, originating from the main pulmonary artery with course toward anterior interventricular groove and branching into left anterior descending and left circumflex coronary arteries with normal courses (Figure 1D and Figure 1E). Finally, cardiac CT unveiled the definitive diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA). Approximately 90% of the infants with ALCAPA die during the first year of their life and very few survive till adulthood [1]. The adult cohort of ALCAPA is increasing due to better advanced cardiac imaging techniques like multidetector computed tomography (MDCT). It is characterized by exuberance in collateral coronary circulation from RCA to left coronary artery (LCA); thus allowing survival up to adulthood, coronary “steal” phenomenon from RCA to LCA and pulmonary “steal” phenomenon as LCA to PA [2]. In the adult type of ALCAPA, re-establishment of dual coronary system by ligation of the LCA from the pulmonary artery with coronary artery bypass grafting and reimplantation of LCA coronary button to aorta with interposition graft are the preferred methods of surgical approach [3].

Anomalous right coronary artery from the pulmonary artery in a young infant with ventricular septal defect

We report a case of anomalous right coronary artery from pulmonary artery (ARCAPA), which is a very rare congenital coronary defect. It can be an isolated lesion or associated with congenital cardiac defects such as tetralogy of Fallot or shunt lesions in up to 40' of cases. The clinical presentation varies from asymptomatic to signs of myocardial ischemia, cardiac failure, and/or ventricular arrhythmia. The electrocardiogram can be normal in a significant fraction of the cases. The diagnosis of ARCAPA may be confirmed by cardiac catheterization, computed tomographic angiogram, and cardiac magnetic resonance angiogram. Reimplantation of anomalous coronary artery is the treatment of choice. In the current era, systematic preoperative screening by echo is recommended.

Tetralogy of Fallot with coronary crossing the right ventricular outflow tract: A tale of a bridge and the artery.

A coronary artery crossing the right ventricular outflow tract is a subset of a larger pathomorphological cohort known as an anomalous coronary artery (ACA) in the tetralogy of Fallot (TOF). The best possible outcome in a patient with TOF and ACA is decided by judicious selection of optimum preoperative investigative information, the timing of surgery, astute assessment of preoperative surgical findings, and appropriate surgical technique from a wide array of choices. In most instances, the choice of surgical technique is determined by the size of the pulmonary annulus and the anatomical relation of ACA to the pulmonary annulus. In the present era, complete, accurate preoperative diagnosis and primary repair is a routine procedure with strategies to avoid a right ventricle-to-pulmonary artery conduit.

Anomalous Right Coronary Artery Origin from Left Sinus (R- ACAOS) Coronary Intervention Case Series; “Our Approach” - A Single Center Experience

Anomalous origin of right coronary artery (RCA) from the left coronary sinus is very rare coronary anomaly. Various individual case reports and case series have been reported in the literature. Patients with anomalous right coronary artery may often be asymptomatic and detected on incidental CT coronary angiogram or at times may present with acute coronary syndrome, sudden cardiac death or malignant arrhythmias. Coronary intervention in an anomalous right coronary artery is often challenging and any interventionist should be familiar with the incidence of this anomaly and always choose an optimal, safe and a good co- axial guiding catheter support for a successful intervention. Here, we present three individual cases with anomalous RCA presenting with positive stress test to acute coronary syndrome. All three cases underwent successful intervention with Judkins left 3.0 (JL 3.0) guiding catheter without any perioperative complications.

P48 ALCAPA Presenting With Cardiac Arrest

Congenital coronary artery abnormalities are rare and have an embryogenesis that is not well understood. Anomalous left coronary artery from the pulmonary artery (ALCAPA) accounts for 0.4% of congenital cardiac abnormalities, often presenting with myocardial ischaemia due to coronary mal-perfusion [ [1] Cheezum M.K. Liberthson R.R. Shah N.R. Villines T.C. O’Gara P.T. Landzberg M.J. et al. Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva. J Am Coll Cardiol. 2017; 69: 1592-1608 Crossref PubMed Scopus (107) Google Scholar ].

Percutaneous Coronary Intervention in Anomalous Single Coronary Artery From the Anterior Right Sinus of Valsalva

An anomalous single coronary artery (SCA) is extremely rare with registry studies suggesting a prevalence of 0.014%-0.066%. SCA may be associated with symptomatic transient myocardial ischaemia, arrhythmias, and sudden cardiac death regardless of the presence of coronary atherosclerosis.

Anomalous Left Coronary Artery Arising from the Right Coronary Sinus Presenting with Acute Coronary Syndrome

Approximately 80% of the coronary artery anomalies are not malignant and asymptomatic. Abnormal origin of the coronary artery has a variable presentation in the young population such as atypical chest pain or unexpected cardiac death. It is necessary to identify these anomalies, as lethal outcomes can be prevented with appropriate treatment and management. Left coronary artery abnormally arising from the right coronary cusp is rare and can be life-threatening if it follows the track between the aorta and pulmonary artery. The second most common cause of sudden cardiac deaths in the young population especially in athletes is coronary artery anomalies following hypertrophic obstructive cardiomyopathy. The case presented here is about abnormally arising left coronary artery following the course between the right ventricle outflow tract and aorta. The patient presented with the non-ST segment elevation-acute coronary syndrome (NSTEACS) for the first time but after 15 months he again presented with ST segment elevation-acute coronary syndrome (STE -ACS) for which he underwent Primary percutaneous coronary intervention of Right coronary artery and for anomalous left coronary artery he was managed conservatively as per patient request.

M17 Surgical Management of Anomalous Right Coronary Artery: Royal Melbourne Hospital Experience

M17 Surgical Management of Anomalous Right Coronary Artery: Royal Melbourne Hospital Experience

Erratum: Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Case Report in a 49-Year-Old Woman

Erratum: Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Case Report in a 49-Year-Old Woman

Anomalous right coronary artery from the pulmonary artery (ARCAPA) in a 63-year-old male patient with a stable angina pectoris and positive stress test

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a very rare congenital cardiac anomaly. Herein, computed tomography and conventional coronary angiography imaging findings of the patient with ARCAPA are presented.

Echocardiographic Visualization of Retroaortic Anomalous Coronary Artery

Echocardiographic Visualization of Retroaortic Anomalous Coronary Artery

Retroaortic anomalous coronary artery visualization on transthoracic echocardiogram

Incidental findings are not uncommon in echocardiography. A transthoracic echocardiogram (TTE) of an adult woman with arterial hypertension showed a tubular structure with hyperechoic walls in the atrial side of the atrioventricular groove in apical views. Coronary computed tomography angiography correlated this finding with a retroaortic course of the anomalous circumflex artery (retroaortic anomalous coronary artery [RAC], benign coronary artery variant). Recently described as the RAC sign on TTE, practitioners should be aware of this finding to avoid mistaken it as artifacts, catheters/leads, or calcified mitral annulus.

Anomalous left coronary artery from the pulmonary artery repair outcomes: Preoperative mitral regurgitation persists in the follow-up.

This study aims to present the midterm outcomes of surgical correction of the anomalous left coronary artery from the pulmonary artery (ALCAPA) with a focus on mitral regurgitation (MR). Medical records of 36 ALCAPA patients who underwent surgery in a single center were retrospectively reviewed. There were one (2.7%) neonate, 19 (52.7%) infants, 15 (41.6%) children, and 1 (2.7%) adult patient operated. Coronary reimplantation, tube reconstruction, and coronary artery bypass surgery techniques were performed in 29 (80.5%), 6 (16.6%), and 1 (2.7%) patient, respectively. Sixteen (44.4%) patients had a low ejection fraction and 13 (36.1%) patients had at least moderate MR preoperatively. None of our patients underwent a concomitant mitral surgery during the initial repair. Three and two patients died in the early and late postoperative period, respectively. Two patients underwent reoperation due to MR and pulmonary stenosis, separately. Preoperative MR was the only factor associated with at least moderate MR at the final follow-up (p < .01) and the presence of preoperative moderate or over-moderate MR estimated at least moderate MR at the final follow-up with 100% sensitivity and 80% specificity. Although the mitral valve was not repaired in the first operation, the reoperation rate is low. However, a moderate or higher preoperative MR predicted MR at the last follow-up. Performing annuloplasty in such patients can be a strategy to be researched.

Ascending aorta replacement in patients with coronary ostia localized above the sinotubular junction.

The study objective was to describe the technique and outcomes of original coronary ostial slide plasty in patients with anomalous coronary artery origin (ACAO) localized in the aneurysmal ascending aorta (AA) being replaced because of its isolated pathology and otherwise non-pathological aortic root. A total of 23 patients (median age 52 years) with the ascending phenotype of proximal aorta aneurysm and ACAO of at least 1 coronary artery localized in the AA being replaced underwent ostial slide plasty to transpose the ACAO to the respective sinus of Valsalva and, consequently, to allow an AA replacement with placement of the proximal anastomosis at the level of the sinotubular junction (STJ). In 15 patients, the aortic valve was bicuspid, and all but 3 patients presented with a relevant valve defect. In addition to remodelling the STJs (all patients), valve-sparing repair or replacement was performed in 12 and 8 patients, respectively. No patient died during the entire follow-up (median 72, range 3-183 months). One patient required replacement of a recurrently insufficient valve that was repaired primarily using cusp patch plasty, but there were no further cardiac reoperations nor any re-interventions on the proximal aorta, aortic valve and/or coronary artery ostia. Two patients received peripheral coronary stents (8 and 7 years after surgery, respectively) due to coronary heart disease. Transposition of the ACAO from the replaced AA into the normal sinus of Valsalva using the ostial slide plasty offers a simple and safe surgical option enabling a recreation of a durable STJ at the level of the anastomosis between the root and the aortic graft.