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Fetal circulation is a unique system that facilitates the exchange of gases, nutrients, and waste products between the developing fetus and the mother. This intricate process takes place through a special organ called the placenta.
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The development of the vascular system in a fetus is a complex and intricate process that begins as early as 15 to 16 days post-conception. This process starts outside the embryo, specifically in the mesoderm of the yolk sac, chorion, and connecting stalk. Approximately two days later, the formation of blood vessels occurs within the embryo itself.
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Contemporary Outcomes in Fetuses Diagnosed with Vascular Rings.

Ian Milligan1,2, William Border3,4, Ritu Sachdeva3,4

  • 1Emory University, Atlanta, GA, USA. ian.l.milligan@gmail.com.

Pediatric Cardiology
|June 24, 2023
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Fetal echocardiography increasingly detects vascular rings. Many infants develop symptoms in early or late infancy, and genetic testing is often positive, indicating a need for increased screening.

Keywords:
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Area of Science:

  • Cardiology
  • Pediatric Surgery
  • Medical Imaging

Background:

  • Vascular rings are congenital anomalies increasingly diagnosed prenatally via fetal echocardiography.
  • These anomalies can lead to significant respiratory and gastroesophageal symptoms in infants.
  • Understanding clinical outcomes and diagnostic patterns is crucial for effective management.

Purpose of the Study:

  • To analyze clinical outcomes in fetuses diagnosed with vascular rings.
  • To evaluate patterns of diagnostic and genetic testing in this population.
  • To assess the timing and nature of symptom presentation and surgical intervention.

Main Methods:

  • Retrospective cohort study of 46 fetuses with postnatally confirmed vascular rings (2017-2022).
  • Analysis of clinical outcomes including symptoms, surgical timing, and freedom from intervention.
  • Assessment of diagnostic testing (imaging, bronchoscopy) and genetic testing frequencies and yields.

Main Results:

  • Prenatal diagnosis of vascular rings increased over time.
  • Vascular rings were isolated in 59% of cases, with others associated with cardiac or non-cardiac anomalies.
  • Symptoms developed in 24%, presenting bimodally before 100 days or after 400 days of life; 17% required surgery.
  • Genetic testing was positive in 33% (22q11 deletion, Trisomy 21), yet obtained in only 46% of patients.

Conclusions:

  • Prenatal diagnosis of vascular rings is rising, with symptom onset occurring in early or late infancy.
  • A significant proportion of patients have associated genetic abnormalities, suggesting genetic testing should be more frequent.
  • Optimizing diagnostic and genetic workups is essential for managing infants with prenatally diagnosed vascular rings.