2027 ICD-10-CM Diagnosis Code E34.01Carcinoid heart syndrome

ICD-10-CM Codes›E00–E89›E20-E35›E34

ICD-10-CM E34.01
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

E34.01 is a billable ICD-10-CM diagnosis code for carcinoid heart syndrome. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 643 through 645. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 11 closely related codes. Coders also document this condition as carcinoid heart disease. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified endocrine disorders.

For Medicare Advantage risk adjustment, E34.01 maps to CMS-HCC Category 17 (Cancer Metastatic to Lung, Liver, Brain, and Other Organs; Acute Myeloid Leukemia Except Promyelocytic) under the V28 model, adding a risk factor of about 4.209 for a community, non-dual, aged beneficiary in payment year 2027.

Code Identity

ICD-10-CM Code
E34.01
Billable Status
Yes — Valid for Submission
Code Describes
Carcinoid heart syndrome
Short Description
Carcinoid heart syndrome
Same as the full description in the CMS dataset.
Parent Code
Carcinoid syndrome

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE20-E35Disorders of other endocrine glands
CategoryE34Other endocrine disorders
This CodeE34.01Carcinoid heart syndrome

Medicare Risk Adjustment (HCC)Billing

E34.01 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.

CMS-HCC V28 Category (Payment Model)
HCC 17— Cancer Metastatic to Lung, Liver, Brain, and Other Organs; Acute Myeloid Leukemia Except Promyelocytic
Payment HCC · PY 2027 one of 78 ICD-10-CM codes in this category
Risk Adjustment Factor (RAF) Weight
+4.209
community, non-dual, aged · ranges 1.952–4.235 across segments
Hierarchy
Supersedes HCC 18, HCC 19, HCC 20, HCC 21, HCC 22, and HCC 23
less severe related categories are not paid alongside HCC 17
Prior Model (CMS-HCC V24)
HCC 12
V24 retired V28 pays 100% of MA risk scores since PY 2026
Other CMS Models
PACE (CMS-HCC V22): HCC 12 · ESRD (V21): HCC 12 · ESRD (V24): HCC 12
ESRD V21 weights: 0.046 dialysis, 0.156–0.194 functioning graft · ESRD V24 weights: 0.045 dialysis, 0.162–0.219 functioning graft
Part D (RxHCC)
RxHCC 22 — Prostate, Breast, Bladder, and Other Cancers and Tumors
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Carcinoid heart disease
  • Serotonin level above reference range

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to E34.01: its own notes plus those printed at E34.0, E34, block E20-E35, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

  • Carcinoid heart disease
  • Hedinger syndrome

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From E34 Other endocrine disorders applies to 18 codes
  • pseudohypoparathyroidism (E20.1)
From Block E20-E35 Disorders of other endocrine glands applies to 99 codes
From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Code Also

A second code may be needed to fully describe the condition; the note gives no sequencing direction.

From E34.0 Carcinoid syndrome applies to 3 codes
  • the underlying disorder, such as:
  • primary neuroendocrine tumors (C7A.-)
  • secondary neuroendocrine tumors (C7B.-)

Notes

General instructions on how these codes are used.

From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name E34.01, its category, or a range that includes it.

Code First 1

These codes say to code first a condition named in the note, and E34.01 is one of the conditions named.

  • G63 Polyneuropathy in diseases classified elsewhere
    endocrine disease, except diabetes (E00-E07, E15-E16, E20-E34) names E20-E34, which includes this code

Notes 1

These notes mention E34.01.

  • Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases
    All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere. names E34.-, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END015
Other specified and unspecified endocrine disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Carcinoid Syndrome

    a syndrome characterized by signs and symptoms caused by advanced carcinoid tumors. they include skin flushing, diarrhea, wheezing, and tachycardia.

Patient EducationClinical

Carcinoid Tumors

Carcinoid tumors are rare, slow-growing cancers. They usually start in the lining of the digestive tract or in the lungs. They grow slowly and don't produce symptoms in the early stages. As a result, the average age of people diagnosed with digestive or lung carcinoids is about 60.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Code History & ChangesHistory

Replacement E34.01 replaces the following previously assigned code(s):

  • E34.0 - Carcinoid syndrome
FY 2025AddedAdded to the ICD-10-CM code setEffective October 1, 2024.
FY 2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About E34.01Overview

What is the ICD-10 code for carcinoid heart syndrome?

The ICD-10-CM code for carcinoid heart syndrome is E34.01 (sometimes written as E3401). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is E34.01 (Carcinoid syndrome) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report carcinoid heart syndrome on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does E34.01 group to?

When carcinoid heart syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 644, 645, 643, with relative weights from 0.7461 to 1.6138 depending on complications. Higher weights mean higher Medicare reimbursement.

Is E34.01 a CC or MCC?

CMS lists E34.01 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 11 closely related codes in its exclusion list.

Can E34.01 be reported with E20.1?

Not as a rule. The Excludes1 note at E34, which applies to E34.01, lists "pseudohypoparathyroidism (E20.1)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

What HCC is E34.01?

E34.01 (carcinoid heart syndrome) maps to CMS-HCC Category 17 (Cancer Metastatic to Lung, Liver, Brain, and Other Organs; Acute Myeloid Leukemia Except Promyelocytic), commonly written as HCC 17, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 12 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 22.

Does E34.01 risk-adjust for Medicare Advantage payment?

Yes. When documented and reported on a Medicare Advantage encounter, E34.01 adds a risk adjustment factor of about 4.209 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 1.952 to 4.235 depending on the payment segment). HCC 17 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 17 category page.