Characteristics and long-term outcome for congenital left main coronary artery atresia

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Congenital left main coronary artery atresia (LMC) is a rare condition.
• Diagnosis can be challenging, often presenting with symptoms like heart murmurs due to mitral regurgitation in infancy.
• Myocardial ischemia can worsen with age and exercise.

Purpose of the Study:

• To characterize the clinical presentation and long-term outcomes of children with congenital LMC atresia.
• To evaluate the effectiveness of surgical interventions for this condition.
• To highlight diagnostic challenges and emphasize the need for early differentiation.

Main Methods:

• Case series reporting on four pediatric patients with LMC atresia.
• Review of clinical characteristics, diagnostic methods (echocardiography, coronary angiography), and surgical interventions (coronary artery bypass grafting, angioplasty).
• Long-term follow-up to assess outcomes and cardiac events.

Main Results:

• Three patients presented with mitral regurgitation and worsening ischemia; one with suspected hypertrophic cardiomyopathy and Noonan syndrome.
• Two-dimensional echocardiography was insufficient for diagnosis, though small left coronary arteries were noted.
• Surgical interventions, including coronary artery bypass grafting and angioplasty, resulted in good long-term outcomes for most patients, with two remaining event-free for over 30 years.

Conclusions:

• Early differentiation of LMC atresia is crucial, especially in infants with small left coronary arteries and mitral regurgitation.
• Coronary artery revascularization and mitral annuloplasty are indicated.
• Both coronary artery bypass grafting and angioplasty demonstrate favorable long-term results, with mitral regurgitation severity influencing prognosis.