What is an example of a ductus arteriosus (DA)-dependent cardiac condition?

From the Guidelines

Hypoplastic left heart syndrome is a ductus arteriosus-dependent cardiac condition. This condition is characterized by the underdevelopment of the left side of the heart, including the left ventricle, aortic valve, and ascending aorta, making it reliant on the patent ductus arteriosus for survival 1. The patent ductus arteriosus allows blood to flow from the pulmonary artery to the aorta, providing systemic circulation. Without this connection, infants with hypoplastic left heart syndrome would not be able to deliver oxygenated blood to the body. Some key features of hypoplastic left heart syndrome include:

• Underdevelopment of the left ventricle, aortic valve, and ascending aorta
• Reliance on the patent ductus arteriosus for systemic circulation
• Need for surgical intervention, typically starting with the Norwood repair in the neonatal period, followed by a bidirectional cavopulmonary anastomosis and finally a Fontan procedure 1
• Potential long-term sequelae, including aortic obstruction, neoaortic dilation, and coronary ischemia Management of hypoplastic left heart syndrome typically involves administration of prostaglandin E1 to maintain ductal patency until surgical intervention can be performed. Close monitoring for side effects of prostaglandin E1, such as apnea, fever, and hypotension, is necessary. Other ductus arteriosus-dependent conditions include critical coarctation of the aorta, interrupted aortic arch, and critical aortic stenosis, which are typically diagnosed shortly after birth when the ductus begins to close, resulting in cardiovascular collapse 1.

From the Research

Ductus Arteriosus-Dependent Cardiac Conditions

• A ductus arteriosus-dependent cardiac condition is one that relies on the patency of the ductus arteriosus to maintain blood flow to the body or lungs.
• Examples of such conditions include:
  • Hypoplastic left heart syndrome (HLHS) 2, 3, 4, 5
  • Critical aortic stenosis 3
  • Coarctation of the aorta 3, 6
  • Interrupted aortic arch 3
  • Pulmonary atresia 6
  • Tricuspid atresia 6
  • Critical pulmonic stenosis 6
• In the case of HLHS, the condition is characterized by an underdeveloped left ventricle and associated structures, and treatment typically includes medical management to maintain patency of the ductus arteriosus, followed by palliative procedures 2, 3, 4, 5.
• The use of prostaglandin E1 (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates with ductus-dependent congenital heart disease, including HLHS 6.