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Silvana Molossi, Hitesh Agrawal
The clinical evaluation of patients with an anomalous aortic origin of a coronary artery (AAOCA), a congenital abnormality of the origin or course of a coronary artery that arises from the aorta, is challenging given its first presentation being sudden cardiac arrest in about half of the patients. Symptoms of chest pain, shortness of breath and syncope during exertion should be of concern in evaluating young athletes and nonathletes. The lack of abnormal signs on the physical exam and electrocardiogram further adds to the difficulty in establishing the diagnosis...
June 16, 2017: Congenital Heart Disease
Constantine Mavroudis
Coronary artery disease ranges from congenital in origin such as anomalous aortic origin of a coronary artery (AAOCA) to acquired diseases such as Kawasaki disease, and previously repaired conditions such as transposition of the great arteries. It is not uncommon, in the long run, for proximal coronary stenosis to develop following arterial switch, AAOCA repair, Ross procedure and Kawasaki disease leading to coronary artery bypass grafing (CABG). The objective of this report is to discuss the indications, challenges, and outcomes of CABG in infants, children, and young adults with acquired and congenital lesions...
June 9, 2017: Congenital Heart Disease
Carlos M Mery
Anomalous aortic origin of a coronary artery (AAOCA) is the second most common cause of sudden cardiac death (SCD) in young athletes in United States. The exact pathophysiological mechanisms of SCD are unknown. There is lack of long-term outcome data on repaired and unrepaired AAOCA and our current risk stratification scheme for these patients is suboptimal. These patients are evaluated in a nonuniform manner across institutions in United States, and even by different providers residing in the same institution...
June 9, 2017: Congenital Heart Disease
Hitesh Agrawal, Oriana K Wright, Kathleen E Carberry, S Kristen Sexson Tejtel, Carlos M Mery, Silvana Molossi
BACKGROUND: Long-term outcome data on patients with anomalous aortic origin of coronary arteries (AAOCA) is sparse and they are often managed in a nonuniform manner. There is subjective perception of anxiety and unmet needs in these patients and families. METHODS: An online survey of 13 questions was sent to 74 families of patients with AAOCA between May and October 2015. Descriptive statistics were performed. RESULTS: A total of 31 (47%) families responded...
May 11, 2017: Congenital Heart Disease
Michael K Cheezum, Richard R Liberthson, Nishant R Shah, Todd C Villines, Patrick T O'Gara, Michael J Landzberg, Ron Blankstein
Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva is increasingly recognized by cardiac imaging. Although most AAOCA subtypes are benign, autopsy studies report an associated risk of sudden death with interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA). Despite efforts to identify high-risk ALCA and ARCA patients who may benefit from surgical repair, debate remains regarding their classification, prevalence, risk stratification, and management...
March 28, 2017: Journal of the American College of Cardiology
Hitesh Agrawal, Silvana Molossi, Mahboob Alam, S Kristen Sexson-Tejtel, Carlos M Mery, E Dean McKenzie, Charles D Fraser, Athar M Qureshi
The evaluation of the vast majority of children with anomalous aortic origin of a coronary artery (AAOCA) and/or myocardial bridges is performed with non-invasive testing. However, a subset of these patients may benefit from invasive testing for risk stratification. All patients included in the Coronary Anomalies Program (CAP) at Texas Children's Hospital who underwent cardiac catheterization were included. Techniques included selective coronary angiograms (SCA), intravascular ultrasound (IVUS), and fractional flow reserve (FFR) measurements with provocative testing using adenosine and/or dobutamine infusions...
March 2017: Pediatric Cardiology
Matthew D Elias, James Meza, Brian W McCrindle, Julie A Brothers, Stephen Paridon, Meryl S Cohen
BACKGROUND: Management of young patients with anomalous aortic origin of a coronary artery (AAOCA) may involve exercise restriction. We sought to identify the association of exercise restriction with changes over time in body mass index (BMI) and exercise capacity in this cohort. METHODS: We performed a retrospective review of patients with AAOCA seen at The Children's Hospital of Philadelphia between January 1, 1998, and August 31, 2014. Linear mixed model repeated-measures analysis assessed changes in BMI and exercise capacity...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
Jeremy L Herrmann, Leah A Goldberg, Abigail M Khan, Sara L Partington, Julie A Brothers, Christopher E Mascio, Thomas L Spray, Yuli Y Kim, Stephanie Fuller
BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) presents in varying age-groups. Assuming management algorithms differ between pediatric and adult institutions, we compared the perioperative management of patients with AAOCA at two such centers. METHODS: A retrospective review was conducted at a pediatric and an adult institution of patients 14 years or older who underwent surgical repair of AAOCA between January 2000 and May 2014. RESULTS: Twenty patients from the pediatric center (median age: 16...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
Richard D Mainwaring, Daniel J Murphy, Ian S Rogers, Frandics P Chan, Edwin Petrossian, Michal Palmon, Frank L Hanley
OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) has been associated with myocardial ischemia and sudden death. The past decade has provided important insights into the natural history and typical patterns of presentation. However, there are also a number of unresolved controversies regarding the indications for surgery and the efficacy of that surgery. The purpose of this study was to review our surgical experience with AAOCA in 115 patients at a single institution. DESIGN: One hundred and fifteen patients have undergone surgical repair of AAOCA at our institution...
May 2016: World Journal for Pediatric & Congenital Heart Surgery
Simon Lee, Santosh C Uppu, Irene D Lytrivi, Javier Sanz, Justin Weigand, Miwa K Geiger, Rajesh U Shenoy, Kanwal Farooqi, Khanh H Nguyen, Ira A Parness, Shubhika Srivastava
BACKGROUND: Anomalous aortic origin of a coronary artery from the wrong Sinus of Valsalva (AAOCA) is a rare congenital anomaly and is associated with sudden cardiac death. Morphologic features considered to be "high risk" are significant luminal narrowing, acute coronary angulation at its origin, intramural course, and long interarterial course. A consistent approach for characterization of these features is lacking. METHODS: A retrospective single-center review of all patients diagnosed with AAOCA using echocardiogram and computed tomography (CT)/magnetic resonance imaging (MRI) studies was performed...
2016: World Journal for Pediatric & Congenital Heart Surgery
Ralph S Mosca, Colin K L Phoon
Anomalous aortic origin of the coronary artery (AAOCA) from the opposite sinus of Valsalva with an interarterial course has become a high-profile lesion as a result of its association with sudden cardiac death in otherwise young and healthy individuals. Despite our incomplete knowledge of its pathophysiology and natural history, surgical intervention is often recommended. Evidence now shows AAOCA to be relatively common, with lower than previously suspected rates of sudden cardiac death. Analysis of this information reveals that AAOCA is not always a surgical disease...
2016: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
José Amado, Mónica Carvalho, Wilson Ferreira, Paula Gago, Vasco Gama, Nuno Bettencourt
Anomalous aortic origin of coronary arteries (AAOCA) is one of the most frequent causes of cardiovascular sudden death among the young population. We aimed to determine the prevalence and anatomic characteristics of AAOCA in a population referred to computed tomography angiography (CTA) and to describe the clinical prognosis of these findings at middle term follow-up. From a total of 3539 CTA, 53 were found to have AAOCA. This population was compared to an age and gender matched control group (n = 106) from the same CTA list...
June 2016: International Journal of Cardiovascular Imaging
Tetsuma Kawaji, Hiroki Shiomi, Satoshi Shizuta, Takeshi Kimura
Anomalous aortic origin of coronary artery (AAOCA) is rare but critical for the association with sudden cardiac death. In detecting the origin of AAOCA, coronary CT angiography is more accurate than invasive coronary angiography, but only with anatomical evaluation. A novel technology FFRCT, which is noninvasively computed by coronary CT image, can evaluate functional ischemia of coronary artery even without atherosclerotic plaque and has potential for innovation in AAOCA.
March 2016: Journal of Cardiovascular Computed Tomography
Richard Lorber, Shubhika Srivastava, Travis J Wilder, Susan McIntyre, William M DeCampli, William G Williams, Peter C Frommelt, Ira A Parness, Eugene H Blackstone, Marshall L Jacobs, Luc Mertens, Julie A Brothers, J René Herlong
OBJECTIVES: This study sought to compare findings from institutional echocardiographic reports with imaging core laboratory (ICL) review of corresponding echocardiographic images and operative reports in 159 patients with anomalous aortic origin of a coronary artery (AAOCA). The study also sought to develop a "best practice" protocol for imaging and interpreting images in establishing the diagnosis of AAOCA. BACKGROUND: AAOCA is associated with sudden death in the young...
November 2015: JACC. Cardiovascular Imaging
Brian D Conway, Michael J Bates, Robert A Hanfland, Nicholas S Yerkes, Sonali S Patel, Domenico Calcaterra, Joseph W Turek
OBJECTIVE: Operative repair for anomalous aortic origin of a coronary artery (AAOCA) has been described using various innovative techniques. Common to each series is the use of a full sternotomy. As demand for minimally invasive approaches to adult cardiac surgery has increased, the upper hemisternotomy has emerged as a safe and effective technique for aortic valve and root replacement. This report reviews our results and describes the application of an upper hemisternotomy to an algorithm-based surgical approach for AAOCA...
March 2015: Innovations: Technology and Techniques in Cardiothoracic and Vascular Surgery
Carlos M Mery, Silvana M Lawrence, Rajesh Krishnamurthy, S Kristen Sexson-Tejtel, Kathleen E Carberry, E Dean McKenzie, Charles D Fraser
Anomalous aortic origin of a coronary artery (AAOCA) is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. It is the second most common cause of sudden cardiac death in young athletes. Its exact prevalence, the pathophysiological mechanisms that cause sudden cardiac death, the actual risk of death for the different types of AAOCA, the optimal way to evaluate these patients, and whether any treatment strategies decrease the risk of sudden cardiac death in patients diagnosed with AAOCA are unknown...
2014: Seminars in Thoracic and Cardiovascular Surgery
Jeffrey A Poynter, Igor Bondarenko, Erle H Austin, William M DeCampli, Jeffrey P Jacobs, Gerhard Ziemer, Paul M Kirshbom, Christo I Tchervenkov, Tara Karamlou, Eugene H Blackstone, Henry L Walters, J William Gaynor, Carlos M Mery, Jeffrey M Pearl, Julie A Brothers, Christopher A Caldarone, William G Williams, Marshall L Jacobs, Constantine Mavroudis
BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) encompasses a wide morphologic spectrum, which has impeded consensus regarding indications for the diverse repair strategies. We constructed a profile of current surgical techniques and explore their application to morphologic variants. METHODS: Patients<30 years old (n=113) with isolated AAOCA who underwent operations at 29 Congenital Heart Surgeons Society (CHSS) institutions from 1998 to 2012 were identified from the CHSS AAOCA Registry...
October 2014: World Journal for Pediatric & Congenital Heart Surgery
Kenta Izumi, Manuel Wilbring, Jürgen Stumpf, Klaus Matschke, Utz Kappert
Anomalous aortic origin of the coronary artery (AAOCA) is a rare cardiac anomaly associated with myocardial ischemia, infarction, and even sudden death. We report 2 patients presenting with AAOCA of the right coronary artery originating from the left coronary sinus with an initial intramural course. In both patients, we performed uncomplicated direct reimplantation of the right coronary artery into the right coronary sinus. For this purpose, the right coronary artery was dissected from the transmural emersion point out of the aortic wall, subsequently as proximal as possible transected and directly reimplanted into the right coronary sinus...
August 2014: Annals of Thoracic Surgery
Poonam P Thankavel, Matthew S Lemler, Claudio Ramaciotti
Anomalous aortic origin of coronaries from the contralateral sinus (AAOCA) is rare but an important cause of cardiac death in the otherwise healthy young athlete. This necessitates prompt, accurate identification; transthoracic echocardiography (TTE) remains the primary screening tool. Assessment of accuracy is difficult since the true prevalence of the disease is unknown, with estimates at 0.3-1.07%. The incidence by TTE remains much lower, between 0.09 and 0.17% even with sophisticated equipment and a high index of suspicion...
January 2015: Pediatric Cardiology
Vikas Sharma, Harold M Burkhart, Joseph A Dearani, Rakesh M Suri, Richard C Daly, Soon J Park, Justin M Horner, Sabrina D Phillips, Hartzell V Schaff
BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) has been associated with myocardial ischemia and sudden death. The optimal management of patients with AAOCA is controversial. We examined our experience with surgical unroofing of AAOCA to determine the midterm effect of surgical repair. METHODS: From October 1992 through December 2011, 75 patients with AAOCA underwent surgical unroofing. RESULTS: Mean age was 39.6 ± 19.6 years; 23 patients (32%) were aged younger than 30 years...
September 2014: Annals of Thoracic Surgery
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