Helix Insight

Documentation / Screening / Tier System

Tier System

After scoring, every variant is assigned to one of four priority tiers. Tier assignment considers both the total weighted score and individual component peaks -- a single exceptional signal can elevate a variant to Tier 1. Clinical boosts from patient context can further promote variants to higher tiers.

Tier Definitions

Tier 1High Priority

Immediate clinical review required. These variants have the strongest evidence for clinical relevance to this patient. Tier 1 is capped at 20 variants by default to keep the review set manageable.

Tier 2Moderate Priority

Should be reviewed if time permits. These variants have moderate evidence for clinical relevance and may become Tier 1 with additional clinical information.

Tier 3Low Priority

Future consideration. These variants have weak evidence for immediate clinical relevance but may be worth monitoring for reclassification or new evidence.

Tier 4Very Low Priority

Likely not clinically relevant. Excluded from results by default. Can be included via settings for comprehensive review.

Base Tier Assignment

The base tier is determined from the weighted total score and individual component peaks. Peak-based promotion ensures that a single exceptional signal (for example, a highly constrained gene with pLI = 0.99) elevates a variant to Tier 1 even if other components are moderate.

TierCondition
Tier 1Total score >= 0.80, OR any of constraint / dosage / deleteriousness / age_relevance >= 0.9
Tier 2Total score >= 0.50
Tier 3Total score >= 0.20
Tier 4Total score < 0.20

Clinical Boosts

After base scoring, patient-specific clinical context adds priority boosts. Boosts are additive to the base score and capped at a total of 1.0. These boosts can promote variants to higher tiers than their base score alone would justify.

BoostConditionAmount
ACMG ClassPathogenic classification+0.30
ACMG ClassLikely Pathogenic classification+0.20
Phenotype TierTier 1 phenotype match+0.25
Phenotype TierTier 2 phenotype match+0.15
Family HistoryCancer gene + family history+0.25
Family HistoryCardiac gene + family history+0.20
Sex-LinkedX-linked gene in male patient+0.30
Sex-LinkedX-linked gene in female patient+0.10
ConsanguinityHomozygous variant + consanguinity+0.25
EthnicityAshkenazi Jewish founder gene+0.15 to +0.20
De NovoTrio sample in highly constrained gene+0.20
PregnancyPrenatal actionable gene + pregnant+0.20

Final Tier Promotion

After all boosts are applied, the final tier is recalculated. Certain conditions guarantee Tier 1 placement regardless of the base score:

Final TierCondition
Tier 1Any Pathogenic or Likely Pathogenic variant (ACMG boost > 0)
Tier 1Tier 1 phenotype match (phenotype boost >= 0.25)
Tier 1Boosted score >= 0.70
Tier 2Tier 2 phenotype match OR boosted score >= 0.50
Tier 3Boosted score >= 0.20
Tier 4Boosted score < 0.20

Clinical Actionability Labels

Each variant also receives a clinical actionability label indicating the recommended clinical response:

LabelCriteria
ImmediatePathogenic or Likely Pathogenic in an ACMG Secondary Findings gene (81 genes including BRCA1/2, MYBPC3, KCNH2, LDLR)
MonitoringPathogenic or Likely Pathogenic in other gene, or total score > 0.7
FutureTotal score > 0.4
ResearchEverything else