Can a patient with pulmonary valve atresia (PVA) survive without a patent ductus arteriosus (PDA)?

From the Guidelines

No, a patient with pulmonary valve atresia (PVA) cannot survive without a patent ductus arteriosus (PDA) or an alternative source of pulmonary blood flow. In pulmonary valve atresia, there is complete obstruction of blood flow from the right ventricle to the pulmonary arteries, preventing deoxygenated blood from reaching the lungs for oxygenation. The PDA serves as a critical lifeline by allowing blood to flow from the aorta to the pulmonary arteries, providing the only source of pulmonary blood flow. Without this connection, profound hypoxemia would rapidly develop, leading to death.

Key Considerations

• The PDA is crucial for survival in patients with PVA, as it provides the only source of pulmonary blood flow.
• Prostaglandin E1 infusion is used to maintain PDA patency until surgical intervention can be performed 1.
• Surgical options, such as a systemic-to-pulmonary shunt or right ventricular outflow tract reconstruction, are necessary to establish reliable pulmonary blood flow.
• Ductal stenting may provide acceptable short-term palliation in cyanotic newborns who have another source of pulmonary blood flow, but it is not a reliable long-term solution due to the risk of early restenosis 1.

Clinical Implications

• The timing of intervention is urgent, typically within the first days of life, as closure of the PDA would be fatal in these patients.
• Careful follow-up is mandatory after ductal stenting, as early and unpredictable restenosis of the stented duct makes such palliation less reliable and potentially hazardous 1.
• The use of flexible premounted stents designed for coronary artery use and long-term antiplatelet therapy may help diminish in-stent restenosis 1.

From the Research

Patient Survival with Pulmonary Valve Atresia (PVA)

• Patients with pulmonary valve atresia (PVA) can survive with or without a patent ductus arteriosus (PDA) through various medical interventions 2, 3, 4.
• Studies have shown that percutaneous treatment of PVA, including perforation-dilatation of the pulmonary valve and stenting of the PDA, is feasible and can avoid early high-risk surgery 2.
• The use of Conquest Pro coronary guidewire for perforation of atretic pulmonary valve and subsequent balloon dilatation and PDA stenting has been reported as effective and safe in patients with PVA 3.
• Concomitant stenting of the PDA at the time of radiofrequency valvotomy in patients with PVA and intermediate right ventricle has been shown to be feasible and safe with encouraging medium-term outcomes 4.

Role of Patent Ductus Arteriosus (PDA)

• The presence of a PDA is crucial for the survival of patients with PVA, as it allows for blood flow to the lungs 5, 6.
• However, patients with PVA can survive without a PDA if they undergo surgical interventions, such as the Glenn or Fontan procedure, which can improve survival rates 6.
• The absence of a PDA in patients with PVA is rare and often associated with other congenital heart defects, such as tricuspid atresia or stenosis 6.

Medical Interventions and Outcomes

• Various medical interventions, including radiofrequency valvotomy, balloon dilatation, and PDA stenting, have been used to treat patients with PVA 2, 3, 4.
• The outcomes of these interventions vary depending on the severity of the anomaly and the presence of other congenital heart defects 2, 3, 4, 6.
• Long-term follow-up is essential to monitor for potential complications, such as coronary artery anomaly, ventricular arrhythmia, left-ventricular outflow tract obstruction, and aortic dilatation 6.