Persistent right umbilical vein associated with complex congenital cardiac malformation

Area of Science:

• Neonatal Medicine
• Pediatric Cardiology
• Medical Imaging

Background:

• Umbilical venous catheterization is a common neonatal resuscitation technique.
• Distinguishing between umbilical arteries and veins during resuscitation can be challenging.
• Persistent right umbilical vein (PRUV) is a rare anomaly.

Observation:

• A preterm infant presented with severe cyanosis, necessitating umbilical catheterization.
• Chest X-ray suggested PRUV, and echocardiography revealed complex congenital heart disease (double outlet right ventricle with mitral atresia).
• The infant's condition was refractory to interventions, including balloon atrioseptostomy, and the infant died.

Findings:

• There is a significant association between PRUV and congenital cardiac malformations.
• PRUV can be detected prenatally with specific examination.
• PRUV may be the sole prenatal indicator of underlying congenital heart disease.

Implications:

• Prenatal diagnosis of PRUV warrants further cardiac evaluation.
• Postnatal identification of PRUV should prompt echocardiography and cardiologic assessment, even without clinical cyanosis.
• Recognizing PRUV aids in identifying neonates at risk for critical congenital heart defects.