ICD-10-CM Code Q87.40

Marfan's syndrome, unspecified

Version 2020 Billable Code POA Exempt

Valid for Submission

Q87.40 is a billable code used to specify a medical diagnosis of marfan's syndrome, unspecified. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code Q87.40 might also be used to specify conditions or terms like blindness, scoliosis, arachnodactyly syndrome, congenital anomaly of subcutaneous tissue, congenital nephritis, genetic lipodystrophy, marfanoid facies, marfanoid habitus with autosomal recessive intellectual disability syndrome, etc The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

ICD-10:Q87.40
Short Description:Marfan's syndrome, unspecified
Long Description:Marfan's syndrome, unspecified

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code Q87.40 are found in the index:


Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Blindness, scoliosis, arachnodactyly syndrome
  • Congenital anomaly of subcutaneous tissue
  • Congenital nephritis
  • Genetic lipodystrophy
  • Marfanoid facies
  • Marfanoid habitus with autosomal recessive intellectual disability syndrome
  • Marfanoid physique
  • Marfanoid physique
  • Marfanoid physique
  • Marfan's syndrome
  • Marfan's syndrome
  • Marfan's syndrome affecting skin
  • Microcephalus, glomerulonephritis, marfanoid habitus syndrome
  • Neonatal Marfan syndrome
  • Painful orbital and systemic neurofibroma, marfanoid habitus syndrome
  • Plexiform neurofibroma
  • Progeroid and marfanoid aspect, lipodystrophy syndrome
  • X-linked intellectual disability with marfanoid habitus

Clinical Information

  • MARFAN SYNDROME-. 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.

Diagnostic Related Groups

The ICD-10 code Q87.40 is grouped in the following groups for version MS-DRG V37.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC).
applicable from 10/01/2019 through 09/30/2020.

  • 306 - CARDIAC CONGENITAL AND VALVULAR DISORDERS WITH MCC
  • 307 - CARDIAC CONGENITAL AND VALVULAR DISORDERS WITHOUT MCC

Present on Admission (POA)

Q87.40 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here .

CMS POA Indicator Options and Definitions
POA Indicator CodePOA Reason for CodeCMS will pay the CC/MCC DRG?
YDiagnosis was present at time of inpatient admission.YES
NDiagnosis was not present at time of inpatient admission.NO
UDocumentation insufficient to determine if the condition was present at the time of inpatient admission.NO
WClinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.YES
1Unreported/Not used - Exempt from POA reporting. NO

Convert Q87.40 to ICD-9

  • 759.82 - Marfan syndrome (Approximate Flag)

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • Other congenital malformations (Q80-Q89)
      • Oth congenital malform syndromes affecting multiple systems (Q87)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients


Marfan Syndrome

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.

There is no single test to diagnose Marfan syndrome. Your doctor may use your medical history, family history, and a physical exam to diagnose it. Marfan syndrome has no cure, but treatments can help delay or prevent complications. Treatments include medicines, surgery, and other therapies.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases


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Marfan syndrome Marfan syndrome is a disorder that affects the connective tissue in many parts of the body. Connective tissue provides strength and flexibility to structures such as bones, ligaments, muscles, blood vessels, and heart valves. The signs and symptoms of Marfan syndrome vary widely in severity, timing of onset, and rate of progression.Because connective tissue is found throughout the body, Marfan syndrome can affect many systems, often causing abnormalities in the heart, blood vessels, eyes, bones, and joints. The two primary features of Marfan syndrome are vision problems caused by a dislocated lens (ectopia lentis) in one or both eyes and defects in the large blood vessel that distributes blood from the heart to the rest of the body (the aorta). The aorta can weaken and stretch, which may lead to a bulge in the blood vessel wall (an aneurysm). Stretching of the aorta may cause the aortic valve to leak, which can lead to a sudden tearing of the layers in the aorta wall (aortic dissection). Aortic aneurysm and dissection can be life threatening.Many people with Marfan syndrome have additional heart problems including a leak in the valve that connects two of the four chambers of the heart (mitral valve prolapse) or the valve that regulates blood flow from the heart into the aorta (aortic valve regurgitation). Leaks in these valves can cause shortness of breath, fatigue, and an irregular heartbeat felt as skipped or extra beats (palpitations).Individuals with Marfan syndrome are usually tall and slender, have elongated fingers and toes (arachnodactyly), loose joints, and have an arm span that exceeds their body height. Other common features include a long and narrow face, crowded teeth, an abnormal curvature of the spine (scoliosis or kyphosis), stretch marks (striae) not related to weight gain or loss, and either a sunken chest (pectus excavatum) or a protruding chest (pectus carinatum). Some individuals develop an abnormal accumulation of air in the chest cavity that can result in the collapse of a lung (spontaneous pneumothorax). A membrane called the dura, which surrounds the brain and spinal cord, can be abnormally enlarged (dural ectasia) in people with Marfan syndrome. Dural ectasia can cause pain in the back, abdomen, legs, or head. Most individuals with Marfan syndrome have some degree of nearsightedness (myopia). Clouding of the lens (cataract) may occur in mid-adulthood, and increased pressure within the eye (glaucoma) occurs more frequently in people with Marfan syndrome than in those without the condition.The features of Marfan syndrome can become apparent anytime between infancy and adulthood. Depending on the onset and severity of signs and symptoms, Marfan syndrome can be fatal early in life; however, with proper treatment, many affected individuals have normal lifespans.
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