Hereditary Hemorrhagic Telangiectasia (HHT)

We are looking for some insight into the manifestations of this. We have a case of one admitted with HHT with epistaxis and AVM's, who underwent APC of 15 colon AVM's. Discussion between the coder and CDI involved whether the AVM's would just get coded as HHT, or do we code both the HHT and the AVM. If we code both the HHT and the AVM's, are the AVM's acquired or congenital? We asked the opinion of our Physician Advisor, who responded with: INteresting point. I would say acquired because the disease is congenital (meaning had at birth) but the AVMs were not present at birth. Just like if you had hypothyroidism. If you have it AT birth its congenital but if develops later its acquired.Or if you have a congenital heart defect at birth, the "heart failure" from the congenital heart disease may not develop until after birth which then means its acquired.

Acquired vs Congenital AVM's alters the DRG impact and we are looking for guidance on how to properly capture this. In addition, hereditary epistaxis codes to HHT...so, would that mean the AVM's would be congential too??

Thank you!

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Comments

  • HHT (Osler-Weber-Rendu syndrome) is characterized by bleeding telangiectasias and AVM throughout the body (CNS, lungs, GI tract, etc).
    The GI AVM's are a manifestation of this hereditary condition and are not 'acquired' even if they did not manifest at birth. They are 'congenital' AVM's that result from the abnormal blood vessel development these patients exhibit. 
    An example of acquired AVM occurs in patient with advanced CKD and ESRD and are the second most common cause of GI bleed in these patients, just behind ulcers/erosion. 
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