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Related Experiment Videos

Life-threatening pulmonary hypertensive crises occurring preoperatively.

A Magnay1, C Bull

  • 1Institute of Child Health, Birmingham, UK.

International Journal of Cardiology
|August 1, 1992
PubMed
Summary

Infants with left atrial isomerism and atrioventricular septal defects can experience dangerous pulmonary hypertension crises. Surgical repair can resolve these episodes, but labile pulmonary vascular resistance poses a preoperative risk.

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Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Pediatric Critical Care

Background:

  • Left atrial isomerism is a complex congenital anomaly.
  • Primum atrioventricular septal defects are common in such conditions.
  • Pulmonary hypertension can be life-threatening in infants.

Observation:

  • A 10-week-old infant with left atrial isomerism and a primum atrioventricular septal defect presented with recurrent collapse.
  • These episodes were identified as paroxysmal pulmonary hypertension.
  • Pulmonary artery pressure was normal between hypertensive crises.

Findings:

  • Pulmonary hypertensive crises were persistent early in the infant's presentation.
  • Surgical correction led to the resolution of pulmonary hypertensive crises.

Related Experiment Videos

  • Labile pulmonary vascular resistance was identified as a potential preoperative concern, especially with an intact ventricular septum.

    • Implications:

    • Early recognition and management of paroxysmal pulmonary hypertension are crucial in infants with left atrial isomerism and atrioventricular septal defects.
    • Surgical intervention can be effective in resolving these crises.
    • Preoperative assessment of pulmonary vascular resistance is important for risk stratification and management planning.