Pediatric Pulmonary Atresia

Pediatric Pulmonary Atresia

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Summary

Pediatric pulmonary atresia is a birth defect where the heart valve that controls blood flow from the heart to the lungs does not form during development.

Expanded overview

Pulmonary atresia is a congenital (present at birth) heart defect that immediately affects a baby. The pulmonary valve that carries blood from the heart to the lungs doesn’t form during development and often requires medical attention soon after birth.

In a healthy heart, the right side of the heart pumps blood into the lungs through the pulmonary artery. Then, the oxygen-rich blood flows back into the heart from the lungs and out toward the rest of the body. Without the pulmonary valve, oxygenated blood flows from the lungs through other openings that traditionally close during development or shortly after birth.

Types

  • Pulmonary atresia with an intact ventricular septum – The wall, or septum, between the ventricles (lower chambers of the heart) remains complete and intact.
  • Pulmonary atresia with a ventricular septal defect  – A hole in the wall that separates the lower chambers lets blood flow into and out of the right-lower side.

Symptoms

Symptoms usually occur in the first few hours of life, but can appear within days. Symptoms include:

  • Cyanosis (bluish discoloration of the skin, fingernails and mouth due to lack of oxygen)
  • Fast breathing
  • Fatigue (tiredness)
  • Poor eating habits
  • Shortness of breath
  • Heart murmur

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