2024 ICD-10-CM Diagnosis Code Q87.4

by

Marfan syndrome

  • Q87.4 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • ICD-10-CM Q87.4 is a revised 2024 ICD-10-CM code that became effective on October 1, 2023.
  • This is the American ICD-10-CM version of Q87.4 – other international versions of ICD-10 Q87.4 may differ.

The following code(s) above Q87.4 contain annotation back-references

 that may be applicable to Q87.4:

  • Q00-Q99  Congenital malformations, deformations and chromosomal abnormalities
  • Q87  Other specified congenital malformation syndromes affecting multiple systems

Clinical Information

  • A genetic syndrome inherited as an autosomal dominant trait. It is caused by mutations in the fbn1 gene. It is characterized by tall stature, elongated extremities, mitral valve prolapse, aortic dilatation, aortic dissection, and sublaxation of the lens.
  • A hereditary disorder of connective tissue characterized by tall stature, elongated extremities, dilatation of the ascending aorta, and pigeon breast.
  • An autosomal dominant disorder of connective tissue with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include mitral valve prolapse, dilation of the aorta, and aortic dissection. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged dura mater (dural ectasia). Marfan syndrome is associated with mutations in the gene encoding fibrillin, a major element of extracellular microfibrils of connective tissue.
  • Disorder of connective tissue characterized by tall stature, elongated extremities, subluxation of the lens, dilatation of the ascending aorta, and “pigeon breast”; inherited as an autosomal dominant trait.
  • Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels and other organs. One of these proteins is fibrillin. A problem with the fibrillin gene causes marfan syndrome. Marfan syndrome can be mild to severe, and the symptoms can vary. People with marfan syndrome are often very tall, thin and loose jointed. Most people with marfan syndrome have heart and blood vessel problems, such as a weakness in the aorta or heart valves that leak. They may also have problems with their bones, eyes, skin, nervous system and lungs. nih: national institute of arthritis and musculoskeletal and skin diseases

Code History

  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
  • 2024 (effective 10/1/2023): Revised code
    • New description: Marfan syndrome
    • 2023 description: Marfan’s syndrome

Code annotations containing back-references to Q87.4: