Coding Tips: New Codes for Carcinoid Heart Syndrome/Hedinger Syndrome
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New Codes for Carcinoid Heart Syndrome

(Valid for October 1, 2024 discharges)

Code E34.0 was expanded to create new codes to identify Carcinoid syndrome:

•  E34.0 Carcinoid syndrome
•  E34.00 Unspecified
•  E34.01 Carcinoid heart syndrome
•  E34.09 Other Carcinoid syndrome

What is Carcinoid heart syndrome?  Carcinoid heart disease (CaHD), also known as Hedinger syndrome, is a rare cardiac condition that typically occurs in patients with primary neuroendocrine tumors (PNETs) with metastatic disease to the liver. CaHD can be confirmed by undergoing a transthoracic echocardiogram (TTE). Results include, but are not limited to, thickened, retracted, and fixed valve leaflets, valvular regurgitation, and dilated right chambers of the heart. However, as the disease progresses, all four valves of the heart can be affected and increase the chance of heart failure.

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