Anomalous aortic origin of coronary artery with sudden death: Case report and review

doi:10.1016/0002-8703(70)90316-9

American Heart Journal

Volume 79, Issue 2, February 1970, Pages 254-257

https://doi.org/10.1016/0002-8703(70)90316-9 Get rights and content

Abstract

This case report describes an anomalous origin of the left coronary artery. Despite the origin of the vessel from the aorta, this anomaly results in sudden death from coronary insufficiency because of the unusual course of the vessel between the aorta and pulmonary artery. This coronary artery anomaly has been described four times before in case reports, but it is not generally recognized as an entity with fatal potential.

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Cited by (70)

Anomalous interarterial left coronary artery: An evidence based systematic overview
2008, International Journal of Cardiology
Citation Excerpt :
The ALMCA prevalence and management have been assessed in non-randomized (case reports, case series and review articles) studies only. A total of 264 cases were identified [3–77] and the results are shown (Fig. 1, Tables 1–4). Table 1 shows the demographics, clinical characteristics, diagnostic tools and ostial anatomy of the systematic review patients.
Isolated anomalous left main coronary artery (ALMCA) from the right aortic sinus of Valsalva (RASV) with an interarterial course between the pulmonary trunk and aorta is a rare congenital abnormality. We performed an evidence based systematic overview spanning 4 decades to assess the prevalence, clinical features and management of this anomaly.
A computerized search spanning 40 years was conducted to identify articles describing cases of ALMCA arising from the RASV with an interarterial course. The bibliographies of all relevant articles were also searched.
The search identified 264 cases. Age ranged from 3.5 months to 87 years. Male/female ratio was 2.9/1. Forty-nine percent of the cases were diagnosed postmortem. Cardiac catheterization was the most common diagnostic tool (41.7%) followed by echocardiography, magnetic resonance imaging (MRI) and computerized assisted tomography. Fifty-seven (21.6%) cases underwent surgical procedures with no mortality and low morbidity.
ALMCA from the RASV is associated with increased risk of sudden death, notably in young patients. Unfortunately the majority are diagnosed postmortem. More than a third present with sudden cardiac death. Echocardiography, computerized assisted tomography and cardiac MRI are valuable non-invasive diagnostic tools. Cardiac catheterization provides a definitive diagnosis in the majority. Surgical correction is the mainstay of treatment with low risk and good anatomic and functional results.
Surgical Therapy for Anomalous Aortic Origin of the Coronary Arteries
2008, Operative Techniques in Thoracic and Cardiovascular Surgery
The Athlete's Heart 2007: Diseases of the Coronary Circulation
2007, Cardiology Clinics
Citation Excerpt :
In both anatomic variants, the left main coronary artery has several possible routes to the left side: (1) anterior to the pulmonary artery, (2) posterior to the aorta, (3) within the intraventricular septum beneath the right ventricular infundibulum, and (4) between the great vessels. The first three are generally benign and have not been associated with exercise-related death, whereas passage of the left main coronary artery between the great vessels has been associated with exercise related death [15–18]. There are two possible mechanisms for the association between a right sinus left main coronary artery and sudden death in athletes [19,20].
The augmentation of myocardial function that accompanies vigorous exercise is dependent upon adequate coronary artery blood flow reserve. Coronary artery pathology may limit coronary blood flow and produce myocardial ischemia. The clinician charged with the care of athletes must have a high index of suspicion for underlying coronary artery pathology when faced with an individual with suggestive symptoms. This article discusses diseases of the coronary circulation relevant to athletes.
Surgical therapy for anomalous aortic origin of the coronary arteries
2005, Pediatric Cardiac Surgery Annual
Anomalous aortic origin of the coronary arteries is a rare defect and is associated with an increased risk of cardiac symptoms including sudden death. The most important subtypes are those in which the anomalous coronary artery arises from the wrong sinus as in anomalous left main coronary artery from the right sinus and anomalous right coronary artery from the left sinus. Ischemia can result when the flap-like orifice becomes obstructed, usually during exercise. Indications for surgical intervention are evolving. Currently, the favored approach for repair is an unroofing type of procedure that results in relocation of the coronary artery into the appropriate sinus of Valsalva.
Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery
2003, Annals of Thoracic Surgery
Citation Excerpt :
Anomalous aortic origin of a coronary artery is a rare and potentially lethal congenital anomaly. Increased risk of exertional sudden death is well described with both ALMCA and ARCA [2, 3, 12]. The combined incidences of these defects are reported to be between 0.1% and 0.3%.
Anomalous aortic origin of a coronary artery (AAOCA) from an incorrect sinus of Valsalva is a relatively rare congenital defect and is associated with sudden death. Several surgical techniques have been described to address this defect, but functional outcome has never been addressed. In this report we evaluate a consecutive series of patients treated with unroofing techniques using transthoracic echocardiography, graded exercise testing, and stress echocardiography to assess functional repair.
Nine consecutive patients (range 7 to 65 years) underwent surgical repair of AAOCA from 1995 to 2001. In all patients the intramural segment was at or below the level of the commissure. All patients underwent a modified unroofing procedure to move the coronary artery orifice to the appropriate sinus. In 2 patients, a new orifice was created without significant unroofing and disruption of the commissure. Patients were evaluated prospectively with exercise electrocardiography testing and by resting and stress echocardiography.
Of the 9 patients, 8 presented with symptoms suggestive of ischemia (chest pain, dyspnea on exertion, or syncope). Six patients had anomalous left main coronary artery arising from the right sinus of Valsalva, and 3 patients had anomalous right coronary artery from the left sinus of Valsalva. Transthoracic echocardiography and graded exercise testing was performed in all 9 patients (mean 29 months, range 4 to 85 months) after repair. Of the 9 patients, 8 also underwent stress echocardiography. In 8 of 9 patients the newly created coronary artery ostium was visualized by either two-dimensional echocardiography or color flow Doppler. All patients were symptom free at the time of follow-up. Exercise stress echocardiography was negative in all patients. Of the 8 patients, 7 had normal left ventricular shortening. No patients had regional wall motion abnormalities suggestive of ischemia. All patients were intervention free except 1 patient who developed severe aortic insufficiency and underwent a subsequent Ross procedure 44 months after his initial procedure.
Anomalous origin of a coronary artery from an incorrect sinus of Valsalva is known to be associated with increased risk of sudden death. Surgical correction can be carried out with minimal risk and good anatomic and functional results. Manipulation of the commissure can be avoided by creation of a neo-ostia without extensive unroofing of the intramural segment or manipulation of the intercoronary commissure. This may avoid aortic valve malfunction.
Sudden death from anomalous coronary artery in an 11-year-old child [7]
2001, American Journal of Emergency Medicine

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Case reportAnomalous aortic origin of coronary artery with sudden death: Case report and review

Abstract

Am. J. Cardiol.

Am. J. Med.

Am. Heart J.

Coronary artery anomalies

Circulation

Case report
Anomalous aortic origin of coronary artery with sudden death: Case report and review