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International Classification of Disease (ICD)

The US Department of Health and Human Services (HHS) has mandated the replacement of the ICD-9-CM code sets used by medical coders and billers to report health care diagnoses and procedures with ICD-10 codes, as of October 1, 2015 E78.71 became a billable code for Barth syndrome as a PRIMARY DIAGNOSIS. Supplemental coding information is provided below. Please note it is very important for Barth syndrome code E78.71 to be entered as the primary diagnosis (PDX).

How To Find (Barth syndrome) ICD-10 Diagnostic Code
Endocrine, nutritional and metabolic diseases (E00 – E89)
Metabolic disorders (E70 – E88)
Disorders of lipoprotein metabolism and other lipidemias (E78)
Disorders of bile acid and cholesterol metabolism (E78.7)
Barth syndrome = E78.71 (with dot)
Code Type: Diagnosis
Description: Barth syndrome

ICD-10 E7871/E78.71 (Barth Syndrome Diagnosis) to ICD-9 General Equivalence Mapping (GEM)

Source ICD-10

 

Target ICD-9

E7871 (Diagnosis) Barth syndrome

 

75989 (Diagnosis) – Specified congenital anomaly NEC (Other specified congenital anomalies)

E78.71 (Diagnosis) Barth syndrome

 

759.89 (Diagnosis) – Specified congenital anomaly NEC (Other specified congenital anomalies)

 

U.S. Department of Health and Human Services Health Centers for Medicare and Medicaid Services (CMS) Insurance Prospective Payment System (HIPPS) Codeset

Medicare Diagnostic Category  (MDC)

  • Musculoskeletal System and Connective Tissue = 08

Diagnostic Related Group (DRG)

  • Other Musculoskeletal Systems and Connective Tissue Diagnosis with MCC = 564

Medical Severity-DRG Code and Title (MS-DRG)

  • Primary Diagnosis (PDX) Barth syndrome = E7871 (without dot)

  • Primary Diagnosis (PDX) Collection = 1089:19

  • Barth syndrome = E7871 (without dot)

Orphanet Classification of Rare Heart Disease

  • Barth Syndrome: Orpha111

Acronyms:
ADX: Admitting diagnosis
CC: Complications and Comorbidities
CMS: Centers for Medicare and Medicaid Services
DRGs or DRG: Diagnosis Related Groups
DX: Diagnosis
HCPCS: Healthcare Common Procedure Coding System
ICD-10: International Classification of Diseases 10th Revision
MCC: Major Complications and Comorbidities
MDC: Medicare Diagnostic Category
MS: Medical Severity
PCS: Procedure Coding System
PDX: Principal diagnosis
PPX: Principal Procedure
SDX: Secondary Diagnosis

ICD-9 to ICD-10 Mapping

The following codes have previously been used as a PDX; however, now that Barth syndrome has a distinct diagnostic code, it is important for these codes to be entered as secondary diagnosis code.

 Cardiology Codes

ICD-9 Description

ICD-9 Code

 

ICD-10 Description

ICD-10 Code

Cardiomegaly

429.3/4293

 

Cardiomegaly

I51.7/I1517

Endocardial Fibroelastosis

425.3/4253

 

Endocardial Fibroelastosis
NOTE: Predominantly associated with Restrictive cardiomyopathy

I42.4/I424

Other Primary Cardiomyopathies

425.4/4254

 

Dilated cardiomyopathy

I42.0/I420

Hypertrophic obstructive cardiomyopathy

425.11/42511

 

Obstructive hypertrophic cardiomyopathy

I42.1/I421

Other hypertrophic cardiomyopathy

425.18/42518

 

Other hypertrophic cardiomyopathy

I42.2/I422

 Hematology Codes

Congenital Neutropenia

288.01/28801

 

Congenital Neutropenia

D70.0/D700

Cyclic Neutropenia      

288.02/28802

 

Cyclic Neutropenia

D70.4/D704

Neutropenia, unspecified

288.00/28800

 

Neutropenia, unspecified
Appropriate for Chronic Neutropenia

D70.9/D709

 Metabolism Codes

Specified congenital anomaly NEC
(Other specified congenital anomalies)

759.89/75989

 

BARTH SYNDROME   

E78.71/E7871

Disturbances of branched-chain amino acid metabolism

270.3/2703

 

3-methylglutaconic aciduria

E71.111

 

Helpful Links
HIPAASpace:  http://www.hipaaspace.com
ICD10Data.com:  www.icd10data.com
ICD-10 Code Search:  www.icd10codesearch.com
World Health Organization International Classification of Diseases (ICD):  http://www.who.int/classifications/icd/
Orphanet Disease Search:  http://www.orpha.net/consor/cgi-bin/Disease.php?lng=EN

Last Updated: 2016-10-24

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