Single coronary artery: a fatal R-I type

Area of Science:

• Cardiology
• Pathology
• Genetics

Background:

• Coronary artery anomalies affect ~1.3% of adults undergoing coronary arteriography.
• Single coronary arteries are exceptionally rare, occurring in 0.024-0.066% of this population.
• The L/R-I type, with an anatomically correct course, is particularly uncommon.

Observation:

• A 6-year-old boy experienced sudden cardiac death during exercise.
• Autopsy revealed a single right coronary artery (R-I type) originating from the right sinus of Valsalva.
• Microscopic examination showed myocardial calcifications and scarring in the mitral valve papillary muscles.

Findings:

• The isolated coronary artery exhibited compensatory lumen widening.
• DNA analysis excluded common ion channel disorders.
• Sudden cardiac death was attributed to chronic ischemic heart disease secondary to the coronary anomaly.

Implications:

• This case underscores the potential for rare coronary artery anomalies to cause fatal ischemic heart disease in young individuals.
• Understanding the pathophysiology of single coronary arteries is crucial for diagnosing and managing at-risk patients.
• Autopsy findings provide valuable insights into the long-term effects of coronary artery anomalies on myocardial structure.