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Congenital Aortic Insufficiency From an Abnormal Left Aortic Cusp Results in Acute Coronary Syndrome

Tam T Doan1, Amna Qasim1, Debra Kearney2

  • 1The Lillie Frank Abercrombie Section of Cardiology, 3984Texas Children's Hospital, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA.

World Journal for Pediatric & Congenital Heart Surgery
|August 14, 2020
PubMed

Insights

Acute coronary syndrome (ACS) is rare in children, but a six-week-old infant presented with cardiac arrest due to severe aortic insufficiency (AI) from a congenital aortic cusp anomaly. The infant recovered after an emergent Ross operation.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Cardiovascular Surgery

Background:

  • Acute coronary syndrome (ACS) is an uncommon manifestation in pediatric patients.
  • Congenital aortic insufficiency (AI) is a rare cause of ACS in infants.
  • Early diagnosis and intervention are critical for managing pediatric cardiovascular emergencies.

Observation:

  • A previously healthy six-week-old infant experienced an out-of-hospital cardiac arrest.
  • Diagnostic evaluation revealed severe AI attributed to an anomalous left aortic cusp.
  • The severe AI was the underlying cause of the acute coronary syndrome.

Findings:

  • The infant's presentation of ACS secondary to congenital AI is a rare clinical scenario.
  • Successful emergent surgical repair was achieved using the Ross operation.
  • The Ross procedure effectively addressed the aortic insufficiency and restored cardiac function.

Implications:

  • This case highlights the importance of considering rare cardiac anomalies in pediatric ACS presentations.
  • Congenital AI should be investigated in infants with unexplained cardiac arrest and ischemic symptoms.
  • The Ross operation is a viable surgical option for severe congenital AI in infants, offering favorable outcomes.

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