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Interruption of the aortic arch in adults
A Rangel1, E Chávez, I Espinosa
1Departamento de Hemodinamia, Hospital de Especialidades, Centro Médico La Raza, IMSS, México, D. F.
Summary
Interruption of the aortic arch is a rare congenital heart defect. Survival to adulthood is uncommon, with associated defects impacting outcomes, especially in patients with ventricular septal defects.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Interruption of the aortic arch (IAA) is a rare congenital heart defect.
- Some patients with IAA may survive to adulthood without surgical intervention.
- Associated intracardiac malformations can significantly influence survival rates.
Observation:
- This study reports on three young adults (18-19 years) with IAA.
- Two patients had type C IAA; one had type B IAA with a subpulmonic ventricular septal defect and pulmonary valve insufficiency.
- A literature review of 106 IAA cases was conducted, categorized by isolated IAA and IAA with ventricular septal defect (VSD).
Findings:
- Fifty percent of IAA patients die within 15 days of life; only 5% survive beyond 5 years.
- No correlation was found between specific IAA anatomic types and survival rates.
- Adolescent survival rates were 7% for IAA with VSD and 14% for isolated IAA, with no statistical difference in age at death between these groups.
Implications:
- Survival into adulthood for interruption of the aortic arch is rare but possible.
- The presence of a ventricular septal defect significantly impacts long-term survival in IAA patients.
- Further research is needed to understand IAA survival and potential interventions.