In what heart defects is Prostaglandin E (PGE) given to prevent cyanosis?

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Last updated: June 24, 2025View editorial policy

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From the Guidelines

Prostaglandin E (PGE) is given to prevent cyanosis in congenital heart defects that are dependent on a patent ductus arteriosus (PDA) for adequate blood flow, including critical pulmonary stenosis, pulmonary atresia, severe tetralogy of Fallot, tricuspid atresia, and critical coarctation of the aorta. These defects are characterized by obstructed blood flow to the lungs or systemic circulation, and the use of PGE helps to maintain patency of the ductus arteriosus, allowing for adequate oxygenation and circulation. According to the study by 1, PGE1 is initiated in neonates with ductal-dependent critical right or left heart obstruction to prevent cyanosis and maintain systemic circulation.

The typical dosage of PGE is 0.01-0.1 mcg/kg/min administered as a continuous IV infusion until surgical intervention can be performed 1. PGE works by relaxing smooth muscle in the ductus arteriosus, keeping it open and allowing for mixing of oxygenated and deoxygenated blood or maintaining systemic circulation. This is crucial in these conditions because the ductus arteriosus normally closes within the first days of life, which would lead to profound cyanosis or circulatory collapse in affected infants.

Some key points to consider when administering PGE include:

  • Monitoring for side effects such as apnea, fever, hypotension, and seizures
  • Ensuring respiratory support is readily available during administration
  • Recognizing when PGE1 therapy may be detrimental, such as in cases of pulmonary venous obstruction 1
  • Continuously assessing the patient's cardiac function and overall clinical status to determine the need for ongoing PGE therapy or surgical intervention.

It's worth noting that the study by 1 discusses the management of adults with congenital heart disease, but does not provide specific guidance on the use of PGE in neonates with ductal-dependent lesions. Therefore, the recommendation to use PGE in these cases is based on the study by 1, which provides more relevant guidance on the management of neonates with critical congenital heart disease.

From the FDA Drug Label

Alprostadil injection, USP is indicated for palliative, not definitive, therapy to temporarily maintain the patency of the ductus arteriosus until corrective or palliative surgery can be performed in neonates who have congenital heart defects and who depend upon the patent ductus for survival Such congenital heart defects include pulmonary atresia, pulmonary stenosis, tricuspid atresia, tetralogy of Fallot, interruption of the aortic arch, coarctation of the aorta, or transposition of the great vessels with or without other defects

The heart defects where Prostaglandin E (PGE) is given to prevent cyanosis include:

  • Pulmonary atresia
  • Pulmonary stenosis
  • Tricuspid atresia
  • Tetralogy of Fallot
  • Interruption of the aortic arch
  • Coarctation of the aorta
  • Transposition of the great vessels with or without other defects 2

From the Research

Heart Defects Requiring PGE to Prevent Cyanosis

The following heart defects require Prostaglandin E (PGE) to prevent cyanosis:

  • Lesions with ductus dependent pulmonary blood flow, such as:
    • Pulmonary atresia with or without ventricular septal defect 3, 4, 5, 6, 7
    • Critical valvular pulmonic stenosis 3, 4, 6, 7
  • Lesions with ductus dependent systemic blood flow, such as:
    • Critical aortic stenosis 3, 5
    • Coarctation of aorta 3, 4, 5
    • Interruption of aortic arch 3, 5
  • Admixture lesions, such as:
    • Transposition of great arteries 3, 4, 5, 7

Administration of PGE

PGE is given as a continuous intravenous infusion, with an initial dose of 0.05 to 0.4 ug/kg/min 3, or a lower initial dose of 20 ng/kg/minute 6. The dose is decreased to 0.01 ug/kg/min 3 or 10 ng/kg/minute 6 for maintenance once the desired effect is achieved.

Efficacy and Side Effects of PGE

PGE has been shown to be effective in maintaining the patency of the ductus arteriosus and improving oxygenation in infants with ductus-dependent congenital heart disease 3, 4, 5, 6, 7. However, it can cause side effects such as apnea, hypotension, hyperthermia, and seizures 3, 5, 6, 7.

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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