Which congenital heart diseases respond to prostaglandin (Prostaglandin E1)?

From the Guidelines

Congenital heart diseases with systemic outflow obstruction respond to prostaglandin (Prostaglandin E1).

Relevant Conditions

• These conditions include:
  • Hypoplastic left heart syndrome
  • Interrupted aortic arch
  • Coarctation of the aorta
  • Congenital diaphragmatic hernia (CDH) with suprasystemic levels of pulmonary hypertension (PH) or right ventricular (RV) failure

Rationale

Prostaglandin E1 is used to dilate the ductus arteriosus, which is essential for maintaining cardiac output in these conditions 1.

Dosage and Administration

The exact dosage and administration of prostaglandin E1 may vary depending on the specific condition and patient response, but it is typically given as a continuous infusion 1.

Important Considerations

Oxygen administration should be withheld in these patients until an anatomic diagnosis can be made, as it can constrict the ductus arteriosus and worsen the condition 1.

Additional Therapies

Other therapies, such as inhaled nitric oxide (iNO) and pulmonary vasodilator drugs, may also be considered in certain cases, but their use should be cautious and individualized 1.

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 congenital heart diseases that respond to prostaglandin (Prostaglandin E1) 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
• These defects are characterized by restricted pulmonary or systemic blood flow, and patients who depend on a patent ductus arteriosus for adequate blood oxygenation and lower body perfusion may respond to prostaglandin (Prostaglandin E1) 2

From the Research

Congenital Heart Diseases Responding to Prostaglandin E1

The following congenital heart diseases have been found to respond to prostaglandin E1 (PGE1) therapy:

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

Efficacy of PGE1 Therapy

PGE1 therapy has been shown to be effective in maintaining the patency of the ductus arteriosus in neonates with ductus-dependent congenital heart disease 3, 5, 4. The efficacy of PGE1 therapy can be assessed by:

• Increase in pulmonary blood flow, as measured by a rise in PaO2 and SaO2% 3
• Increase in systemic blood flow, as measured by the appearance of lower limb pulses 3
• Improvement in mixing, as measured by echocardiography 3

Dosage and Administration of PGE1

The initial dose of PGE1 is typically 0.05-0.4 μg/kg/min, with a maintenance dose of 0.01 μg/kg/min 3, 4. However, some studies have suggested that a lower initial dose of 0.01 μg/kg/min may be effective and safe 6, 7. PGE1 is typically administered as a continuous intravenous infusion, and the dosage may need to be adjusted based on the individual patient's response to therapy.