Anomalous aortic origin of a coronary artery

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Anomalous Aortic Origin of a Coronary Artery (AAOCA)
Anomalous origin of the right coronary artery from the left coronary sinus, seen on MRI with an inter-arterial, potentially dangerous course.
Synonyms AAOCA
Pronounce N/A
Field Cardiology
Symptoms Often asymptomatic; can include chest pain, syncope, palpitations, dyspnea, or sudden cardiac death during exertion
Complications Myocardial ischemia, arrhythmia, sudden cardiac arrest
Onset Congenital (present at birth)
Duration Lifelong if untreated
Types AAOCA with interarterial, intramural, retroaortic, prepulmonic, or intraconal course
Causes Congenital heart defect affecting coronary artery anatomy
Risks Vigorous exercise, especially in young athletes
Diagnosis Echocardiography, CT angiography, MRI, coronary angiography
Differential diagnosis Hypertrophic cardiomyopathy, long QT syndrome, coronary artery disease
Prevention Screening in high-risk individuals, especially athletes
Treatment Surgical correction (e.g. unroofing, coronary reimplantation); conservative management in low-risk cases
Medication Beta-blockers in selected cases
Prognosis Excellent with early detection and appropriate management
Frequency 0.1%–0.3% of general population
Deaths Rare but significant cause of sudden cardiac death in youth


Anomalous Aortic Origin of a Coronary Artery (AAOCA) is a rare but potentially life-threatening congenital heart defect where one of the coronary arteries arises from the aorta abnormally, leading to increased risk of myocardial ischemia or sudden cardiac death, especially during exercise.

Overview

AAOCA is a structural anomaly in which a coronary artery originates from the wrong aortic sinus. The most common and clinically significant variants include:

These anomalous origins may follow a dangerous "interarterial" course, passing between the ascending aorta and the pulmonary artery, which can lead to compression of the artery during physical activity, resulting in restricted blood flow to the heart.

Epidemiology

AAOCA affects approximately 0.1% to 0.3% of the general population. It is the second leading cause of sudden cardiac death among children and young athletes in the United States, after hypertrophic cardiomyopathy.

Clinical Presentation

Most patients are asymptomatic and diagnosed incidentally. Symptomatic individuals may present with:

Diagnosis

The evaluation of AAOCA includes:

Pathophysiology

AAOCA with an interarterial or intramural course may cause the coronary artery to be compressed between the great vessels during exercise. This leads to impaired coronary perfusion, resulting in ischemia, arrhythmias, or sudden death. The risk is higher with LCA anomalies compared to RCA anomalies.

Classification

AAOCA can be subclassified based on the origin and path of the anomalous artery:

  • Interarterial course – Between the aorta and pulmonary artery (highest risk)
  • Intramural course – Within the wall of the aorta
  • Retroaortic course – Behind the aorta
  • Prepulmonic course – In front of the pulmonary artery
  • Septal (subpulmonic) course – Through the interventricular septum

Treatment

Treatment decisions are based on symptoms, type of anomaly, and risk factors.

Surgical Indications

  • Symptomatic patients (e.g., with chest pain or arrhythmia)
  • LCA originating from the right sinus with interarterial or intramural course
  • Evidence of myocardial ischemia or abnormal stress test

Surgical Procedures

  • Unroofing procedure – Eliminating the intramural segment
  • Coronary reimplantation – Reattaching the artery to the correct sinus
  • Pulmonary artery translocation – To decompress the interarterial course

Conservative Management

  • Close monitoring of asymptomatic patients with RCA from the left sinus
  • Lifestyle modifications and exercise restriction in high-risk patients
  • Beta-blockers in select cases

Research and Registry

In 2009, the Congenital Heart Surgeons' Society (CHSS) launched a multi-center North American Registry for AAOCA. The aim is to:

  • Study natural history and outcomes
  • Compare surgery versus observation
  • Develop evidence-based treatment guidelines

Eligible patients include those diagnosed at age ≤30 with otherwise structurally normal hearts. Over 140 patients were enrolled as of mid-2011, making it the largest AAOCA cohort globally.

Related Anomaly: ARCAPA

Anomalous Right Coronary Artery from the Pulmonary Artery (ARCAPA) is a rare variant of coronary artery anomaly. It results in a left-to-right shunt and chronic ischemia. Surgical correction aims to establish a dual coronary system.

Prognosis

With early detection and appropriate management, the prognosis is generally excellent. Surgical correction significantly reduces the risk of sudden cardiac death. Lifelong follow-up is essential.

See also

External links

Congenital heart defects
Heart septal defect
Valvular heart disease
Other




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Contributors: Prab R. Tumpati, MD