Loeys-Dietz syndrome
Congenital Malformations, Deformations and Chromosomal Abnormalities·Other specified congenital malformation syndromes affecting multiple systems·subcategory·FY 2027
Birth Defects: A birth defect is a problem that happens while a baby is developing in the mother's body. Most birth defects happen during the first 3 months of pregnancy. One out of every 33 babies in the United States is born with a birth defect.
Read more about birth defects on MedlinePlus · Source: MedlinePlus, National Library of Medicine
Also indexed as: Syndrome, Loeys-Dietz
Instructional notes
Excludes1: not coded here, use a different code
- arterial tortuosity syndrome (Q87.82)
- Ehlers-Danlos syndrome (Q79.6)
- Marfan syndrome (Q87.40-Q87.43)
- other systemic involvement of connective tissue (M35)
Also applies, from Q87: Other specified congenital malformation syndromes affecting multiple systems
These notes sit on Q87 and apply to every code under it, including Q87.A.
Use additional code: add a second code for this, listed after this one
- code(s) to identify all associated manifestations
Where Q87.A sits
MS-DRGs
As the principal diagnosis, this code groups to these MS-DRGs (v44.0). Which one applies depends on procedures performed, secondary diagnoses (CC/MCC), and the patient's age and discharge status.
Code history
- FY 2026: replaced Q87.89 (Other specified congenital malformation syndromes, not elsewhere classified)