Unusual cause of left ventricular dysfunction in a child

Maria Emanuel Amaral1, Pedro Epifânio1, Natália Noronha1

  • 1Serviço de Cardiologia Pediátrica - Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Insights

An unusual congenital heart defect, Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA), was diagnosed in an 8-year-old. Successful surgical repair created a dual coronary artery system, highlighting diagnostic challenges.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Cardiovascular Surgery

Background:

  • Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) is a rare congenital heart defect.
  • It often leads to myocardial ischemia in children and high mortality within the first year if undiagnosed.
  • Early diagnosis and intervention are critical for survival.

Observation:

  • An 8-year-old asymptomatic child presented with left ventricular dilatation and dysfunction.
  • Echocardiogram revealed turbulent flows at the interventricular septum, suggesting ALCAPA.
  • Previous diagnosis at 3 months was heart failure with dilated cardiomyopathy.

Findings:

  • Computerized angiotomography and cardiac catheterization confirmed ALCAPA.
  • The patient underwent successful surgical reimplantation of the left coronary artery to the aorta.
  • This created a dual coronary perfusion system, restoring normal blood flow.

Implications:

  • This case highlights an unusual long-term survival of undiagnosed ALCAPA.
  • It underscores the importance of high clinical suspicion and multimodality imaging in diagnosing rare pediatric cardiac conditions.
  • Successful surgical correction offers a favorable prognosis for ALCAPA patients.

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