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Pulmonary Atresia

A condition in which the pulmonary valve is completely closed, with no direct blood flow from the right ventricle to the pulmonary artery.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Kardiyoloji department. Book Appointment →

What is Pulmonary Atresia?

In pulmonary atresia the pulmonary valve is completely closed, so there is no direct flow between the right ventricle and pulmonary artery. The right ventricle may be hypoplastic, and the defect may occur with an intact ventricular septum or together with a ventricular septal defect.

Pulmonary blood flow is supplied through a patent ductus arteriosus, major aortopulmonary collateral arteries (MAPCAs) or staged surgical shunts. Ductal closure causes severe hypoxia and acidosis; prostaglandin E1 infusion can be life-saving.

Diagnosis is made prenatally or in the neonatal period by echocardiography. Depending on the anatomy, treatment is staged biventricular or univentricular (Fontan) surgical repair.

Symptoms

Marked cyanosis from birth
Sudden deterioration when the ductus closes
Tachypnea and respiratory distress
Feeding difficulty
Metabolic acidosis
Cardiac systolic murmur
Single S2

Risk Factors

Family history of congenital heart disease
Maternal diabetes
Maternal rubella
Chromosomal anomalies (22q11 deletion)
Teratogenic drug exposure
Advanced maternal age
Smoking and alcohol during pregnancy

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Cyanosis and respiratory distress in a newborn
  • Anomaly detected on prenatal echocardiography
  • Sudden hypoxia and acidosis
  • Color change during feeding
  • Regular follow-up after staged surgery

Treatment Methods

01
Prostaglandin E1 to maintain ductal patency
02
Neonatal Blalock-Taussig shunt or RVOT reconstruction
03
MAPCA unifocalization surgery
04
Biventricular repair where anatomy permits
05
Glenn and Fontan operations (single-ventricle strategy)
06
Long-term congenital cardiology follow-up

Which Department to Visit?

You can visit our Kardiyoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.