Q22.6 Hypoplastic right heart syndrome

Billable ICD-10-CM code, FY2026. Acts as a Major Complication or Comorbidity (MCC) as a secondary diagnosis. Exempt from present-on-admission (POA) reporting.

MS-DRG index

Defines the logic of DRG(s) 306-307 in MDC 05.

Approximate ICD-9-CM (GEMs)

• 746.1 Tricuspid atresia and stenosis, congenital (approximate)

Code annotations containing back-references to Q22.6

Back-references to Q22

• Type 2 Excludes: I08 Multiple valve diseases ("multiple valve disease specified as nonrheumatic (I34.-, I35.-, I36.-, I37.-, I38.-, Q22.-, Q23.-, Q24.8-)")
• Code Also: I27.21 Secondary pulmonary arterial hypertension ("congenital heart disease (Q20-Q28)")
• Type 2 Excludes: P29 Cardiovascular disorders originating in the perinatal period ("congenital malformations of the circulatory system (Q20-Q28)")
• Code Also: Z71.87 Encounter for pediatric-to-adult transition counseling ("congenital malformations of the circulatory system (Q20-Q28)")

Diagnosis Index entries for Q22.6

• Anomaly, anomalous (congenital) (unspecified type) › heart › obstructive NEC
• Hypoplasia, hypoplastic › right heart syndrome
• Syndrome › right › heart, hypoplastic

Nearby codes

• Q22.1 Congenital pulmonary valve stenosis
• Q22.2 Congenital pulmonary valve insufficiency
• Q22.3 Other congenital malformations of pulmonary valve
• Q22.4 Congenital tricuspid stenosis
• Q22.5 Ebstein's anomaly
• Q22.6 Hypoplastic right heart syndrome (this code)
• Q22.8 Other congenital malformations of tricuspid valve
• Q22.9 Congenital malformation of tricuspid valve, unspecified