Summary & Overview
CPT 0614U: Blue Native PAGE for Mitochondrial Enzyme Function
CPT code 0614U designates a Proprietary Laboratory Analyses (PLA) test — Blue Native Polyacrylamide Gel Electrophoresis (PAGE) — performed exclusively by the Children's Hospital Colorado Laboratory to assess mitochondrial enzyme function in frozen tissue for suspected primary mitochondrial disease. As a PLA code, 0614U is linked to a single manufacturer/lab assay and signals a specialized diagnostic service used in complex metabolic and neuromuscular evaluations. Nationally, PLA codes like 0614U matter because they identify proprietary tests with unique clinical utility, distinct billing considerations, and concentrated provider availability.
Key payers discussed include Aetna, Blue Cross Blue Shield, Cigna Health, UnitedHealthcare, and Medicare. Readers will find a concise overview of the clinical purpose and service setting for 0614U, plus discussion of payer coverage patterns and benchmarking context where available. The publication summarizes billing and coding considerations tied to proprietary laboratory tests, clarifies the test’s clinical role in evaluating suspected primary mitochondrial disease, and outlines what to expect in payer engagement for specialized reference-lab assays. Data not provided in the input — such as common modifiers, associated taxonomies, specific ICD-10 codes, related codes, and detailed service-line classification — are noted as not available.
Billing Code Overview
CPT code 0614U is a Proprietary Laboratory Analyses (PLA) code that applies only to a single, specific laboratory test. Report 0614U only for Blue Native Polyacrylamide Gel Electrophoresis (PAGE) performed by the Children's Hospital Colorado Laboratory. The test evaluates mitochondrial enzyme function for suspected primary mitochondrial disease by analyzing four enzyme complexes in frozen tissue samples using a specialized electrophoresis method and reporting a qualitative diagnostic result.
Service type: Specialized laboratory diagnostic testing for mitochondrial enzyme function
Typical site of service: Reference laboratory / hospital laboratory (Children's Hospital Colorado Laboratory)
Clinical & Coding Specifications
Clinical Context
A pediatric or adult patient with suspected primary mitochondrial disease (PMD) presents with multisystem findings such as unexplained exercise intolerance, progressive muscle weakness, neurodevelopmental delay, lactic acidosis, or multisystem neurologic and cardiac involvement. Neurology, genetics, or metabolic medicine obtains informed consent and arranges a diagnostic muscle or other frozen tissue biopsy. The biopsy specimen is preserved and shipped to Children's Hospital Colorado Laboratory under frozen conditions per lab instructions. Laboratory personnel perform Blue Native Polyacrylamide Gel Electrophoresis (PAGE) to evaluate mitochondrial enzyme complexes I–IV (and potentially associated supercomplexes) and interpret results qualitatively (e.g., normal, decreased, absent, or abnormal banding patterns). Results are reported to the ordering clinician to support diagnosis, guide further genetic testing, and inform clinical management decisions such as additional metabolic evaluation or referral to specialty services.
Coding Specifications
| Modifier | Description | When to Use |
|---|---|---|
26 | Professional component | Use when billing only the professional interpretation component separate from the technical component, if applicable and allowed by payer. |
TC | Technical component | Use when billing only the technical component (laboratory processing and testing) and the professional interpretation is billed separately. |
59 | Distinct procedural service | Use when another separate, unrelated procedure or service was performed on the same day and must be reported as distinct. |
90 | Reference (outside) laboratory | Use when the specimen is sent to an outside, independent laboratory for testing and billing is done by that outside lab. |
91 | Repeat clinical diagnostic laboratory test | Use when the same lab test is repeated on the same day for validation or confirmation per payer policy. |
52 | Reduced services | Use when the service performed is partially reduced or not completed as described by the full code. |
73 | Discontinued outpatient procedure prior to anesthesia | Not commonly used for lab tests but applicable if a specimen collection or procedure was initiated and then discontinued prior to completion in outpatient setting. |
74 | Discontinued outpatient procedure after anesthesia/analgesia | Rare for lab tests; use only if applicable when a procedural collection requiring anesthesia was discontinued. |
24 | Unrelated E/M service by same physician during postoperative period | Use when an unrelated evaluation and management service is provided during a global period while the lab testing is unrelated to the original procedure. |
22 | Increased procedural services | Use when the laboratory service required substantially greater resources or time than typical and payer allows modifier 22 documentation and approval. |
| Taxonomy Code | Specialty | Notes |
|---|---|---|
207RN0000X | Neurology | Neurologists commonly order mitochondrial diagnostic testing for neuromuscular and neurodegenerative presentations. |
2080P0207X | Pediatrics | Pediatricians and pediatric subspecialists (e.g., pediatric neurology, metabolic medicine) frequently manage suspected PMD and arrange tissue biopsy. |
2084P0800X | Medical Genetics | Clinical geneticists and genetic counselors coordinate diagnostic testing and interpret results in the context of genetic evaluation. |
207K00000X | Pathology | Pathologists and laboratory directors oversee tissue processing and correlate electrophoretic findings with histopathology. |
208000000X | Internal Medicine | Adult medicine physicians (including neurologists and metabolic specialists) may order mitochondrial diagnostic testing for adult-onset presentations. |
Related Diagnoses
| ICD-10 Code | Description | Clinical Relevance |
|---|---|---|
E88.40 | Mitochondrial myopathy, unspecified | Directly related to suspected primary mitochondrial disease evaluated by mitochondrial enzyme testing. |
G71.3 | Mitochondrial myopathy, not elsewhere classified | Reflects myopathic presentations prompting muscle biopsy and biochemical analysis. |
R94.31 | Abnormal results of oxidative phosphorylation studies | Captures abnormal biochemical testing results such as those from Blue Native PAGE. |
R56.9 | Unspecified convulsions | Seizures are a common neurologic manifestation of mitochondrial disease and may prompt mitochondrial testing. |
E78.5 | Hyperlipidemia, unspecified | Metabolic derangements can be seen in mitochondrial disorders; included when metabolic evaluation is performed. |
G31.84 | Mild cognitive impairment, so stated | Neurodevelopmental and cognitive symptoms may be part of multisystem mitochondrial presentations that trigger diagnostic workup. |
Related CPT Codes
| CPT Code | Description | Relationship to This Procedure |
|---|---|---|
0614U | Blue Native PAGE for mitochondrial enzyme complex analysis (Children's Hospital Colorado Laboratory) | This proprietary PLA code specifically reports the Blue Native PAGE assay performed at Children's Hospital Colorado for qualitative evaluation of mitochondrial enzyme complexes. |
88305 | Level IV surgical pathology, gross and microscopic examination | Used for initial histopathologic examination of muscle biopsy tissue prior to or in conjunction with specialized biochemical testing. |
83013 | Creatine kinase (CK) assay | Common laboratory test ordered in evaluation of myopathic processes and muscle injury accompanying workup for mitochondrial myopathy. |
82370 | Lactic acid (serum) | Serum lactate is commonly ordered in patients suspected of mitochondrial disorders to assess metabolic dysfunction. |
81406 | Targeted genomic sequence analysis for mitochondrial genes (example panel) | Molecular genetic testing of mitochondrial and nuclear genes encoding mitochondrial proteins is commonly performed following abnormal biochemical testing to identify causative variants. |
88346 | Immunohistochemistry, per specimen; different antibodies (one or more) | May be used to evaluate specific proteins or mitochondrial markers on muscle biopsy in parallel with biochemical electrophoresis. |