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August 01, 2022

Congenital Heart Disease code updates

New 2022 codes for Exchange plans and commercial plans

Updated Sept. 2022: This article has been updated with new codes

Beginning Jan. 1, 2022, there are new Current Procedural Terminology (CPT®) codes in place for congenital heart disease:

  • Endovascular Repair of Congenital Heart and Vascular Defects (33894, 33895, and 33897)
  • Cardiac Catheterization for Congenital Defects (93593, 93594, 93595, 93596, 93597) – replacing codes 93530-93533, which are no longer valid.

Affected States
These CPT code changes affect all commercial plans and UnitedHealthcare Individual Exchange plans, also referred to as Individual and Family, plans in the following states:

  • Alabama
  • Arizona
  • Florida
  • Georgia
  • Illinois
  • Louisiana
  • Maryland
  • Massachusetts
  • Michigan
  • Nevada
  • New York
  • North Carolina
  • Oklahoma
  • Tennessee
  • Texas
  • Virginia
  • Washington

The new codes and their descriptions are listed below.

Cardiac catheterization for congenital defects CPT codes

CPT code Code description
93593 Right heart catheterization for congenital heart defect(s) including imaging guidance by the proceduralist to advance the catheter to the target zone; normal native connections
93594 Right heart catheterization for congenital heart defect(s) including imaging guidance by the proceduralist to advance the catheter to the target zone; abnormal native connections
93595 Left heart catheterization for congenital heart defect(s) including imaging guidance by the proceduralist to advance the catheter to the target zone, normal or abnormal native connections
93596 Right and left heart catheterization for congenital heart defect(s) including imaging guidance by the proceduralist to advance the catheter to the target zone(s); abnormal native connections
93597 Right and left heart catheterization for congenital heart defect(s) including imaging guidance by the proceduralist to advance the catheter to the target zone(s); abnormal native connections
93598 Cardiac output measurement(s), thermodilution or other indicator dilution method, performed during cardiac catheterization for the evaluation of congenital heart defects (List separately in addition to code for primary procedure)

Endovascular repair of congenital heart and vascular defects CPT codes

CPT code Code description

33894

Endovascular stent repair of coarctation of the ascending, transverse, or descending thoracic or abdominal aorta, involving stent placement; across major side branches
33895 Endovascular stent repair of coarctation of the ascending, transverse, or descending thoracic or abdominal aorta, involving stent placement; not crossing major side branches
33897 Percutaneous transluminal angioplasty of native or recurrent coarctation of the aorta

CPT codes to be added to UnitedHealthcare commercial plans

CPT code Code description
93582 Percutaneous transcatheter closure of patent ductus arteriosus
33250 Operative ablation of supraventricular arrhythmogenic focus or pathway (eg, Wolff-Parkinson-White, atrioventricular node re-entry), tract(s) and/or focus (foci); without cardiopulmonary bypass
33253 Operative incisions and reconstruction of atria for treatment of atrial fibrillation or atrial flutter (eg, maze procedure)
33468 Tricuspid valve repositioning and plication for Ebstein anomaly
33390 Valvuloplasty, aortic valve; open, with cardiopulmonary bypass
33391 Valvuloplasty, aortic valve; open, with cardiopulmonary bypass
93583 Percutaneous transcatheter septal reduction therapy (eg, alcohol septal ablation) including temporary pacemaker insertion when performed
33620 Application of right and left pulmonary artery bands (eg, hybrid approach stage 1)
33622 Reconstruction of complex cardiac anomaly (eg, single ventricle or hypoplastic left heart) with palliation of single ventricle with aortic outflow obstruction and aortic arch hypoplasia, creation of cavopulmonary anastomosis, and removal of right and left pulmonary bands (eg, hybrid approach stage 2, Norwood, bidirectional Glenn, pulmonary artery debanding)
33782 Aortic root translocation with ventricular septal defect and pulmonary stenosis repair (ie, Nikaidoh procedure); without coronary ostium reimplantation
33783 Aortic root translocation with ventricular septal defect and pulmonary stenosis repair (ie, Nikaidoh procedure); with reimplantation of 1 or both coronary ostia
33925 Repair of pulmonary artery arborization anomalies by unifocalization; without cardiopulmonary bypass
33926 Repair of pulmonary artery arborization anomalies by unifocalization; with cardiopulmonary bypass
33465 Replacement, tricuspid valve, with cardiopulmonary bypass
33472 Valvotomy, pulmonary valve, open heart; with inflow occlusion
33722 Closure of aortico-left ventricular tunnel
33813 Obliteration of aortopulmonary septal defect; without cardiopulmonary bypass
33814 Obliteration of aortopulmonary septal defect; with cardiopulmonary bypass
33824 Repair of patent ductus arteriosus; by division, 18 years and older
33741 Transcatheter atrial septostomy (TAS) for congenital cardiac anomalies to create effective atrial flow, including all imaging guidance by the proceduralist, when performed, any method (eg, Rashkind, Sang-Park, balloon, cutting balloon, blade)
33745 Transcatheter intracardiac shunt (TIS) creation by stent placement for congenital cardiac anomalies to establish effective intracardiac flow, including all imaging guidance by the proceduralist, when performed, left and right heart diagnostic cardiac catherization for congenital cardiac anomalies, and target zone angioplasty, when performed (eg, atrial septum, Fontan fenestration, right ventricular outflow tract, Mustard/Senning/Warden baffles); initial intracardiac shunt
33746 Transcatheter intracardiac shunt (TIS) creation by stent placement for congenital cardiac anomalies to establish effective intracardiac flow, including all imaging guidance by the proceduralist, when performed, left and right heart diagnostic cardiac catherization for congenital cardiac anomalies, and target zone angioplasty, when performed (eg, atrial septum, Fontan fenestration, right ventricular outflow tract, Mustard/Senning/Warden baffles); each additional intracardiac shunt location (List separately in addition to code for primary procedure)

ICD 10 diagnosis codes to be added to UnitedHealthcare commercial plans

ICD 10 CM Diagnosis code Code description
Q25.21 Interruption of aortic arch
Q25.29  Other atresia of aorta
Q25.41 Absence and aplasia of aorta
Q25.42 Hypoplasia of aorta
Q25.43 Congenital aneurysm of aorta
Q25.44 Congenital dilation of aorta
Q25.45 Double aortic arch 
Q25.46 Tortuous aortic arch
Q25.47 Right aortic arch
Q25.48 Anomalous origin of subclavian artery
Q25.49 Other congenital malformations of aorta
I27.83 Eisenmenger's syndrome

CPT codes to be added to Individual Exchange plans

CPT code Description
93582 Percutaneous transcatheter closure of patent ductus arteriosus
33202 Lead and Dual chamber pacemaker implantation
33213 Lead and Dual chamber pacemaker implantation
33253 Operative incisions and reconstruction of atria for treatment of atrial fibrillation or atrial flutter (eg, maze procedure)
33468 Tricuspid valve repositioning and plication for Ebstein anomaly
33390 Valvuloplasty, aortic valve; open, with cardiopulmonary bypass
33391 Valvuloplasty, aortic valve; open, with cardiopulmonary bypass
93580 Percutaneous transcatheter closure of congenital interatrial communication (ie, Fontan fenestration, atrial septal defect) with implant
93583 Percutaneous transcatheter septal reduction therapy (eg, alcohol septal ablation) including temporary pacemaker insertion when performed
33620 Application of right and left pulmonary artery bands (eg, hybrid approach stage 1)
33622 Reconstruction of complex cardiac anomaly (eg, single ventricle or hypoplastic left heart) with palliation of single ventricle with aortic outflow obstruction and aortic arch hypoplasia, creation of cavopulmonary anastomosis, and removal of right and left pulmonary bands (eg, hybrid approach stage 2, Norwood, bidirectional Glenn, pulmonary artery debanding)
33925 Repair of pulmonary artery arborization anomalies by unifocalization; without cardiopulmonary bypass
33926 Repair of pulmonary artery arborization anomalies by unifocalization; with cardiopulmonary bypass
33465 Replacement, tricuspid valve, with cardiopulmonary bypass
33472 Valvotomy, pulmonary valve, open heart; with inflow occlusion
33813 Obliteration of aortopulmonary septal defect; without cardiopulmonary bypass
33814 Obliteration of aortopulmonary septal defect; with cardiopulmonary bypass
33824 Repair of patent ductus arteriosus; by division, 18 years and older

ICD 10 diagnosis codes to be added to Individual Exchange plans

ICD 10 CM Diagnosis code Description
Q20.0 Common arterial trunk
Q20.1 Double outlet right ventricle
Q20.2 Double outlet left ventricle
Q20.3 Discordant ventriculoarterial connection
Q20.4 Double inlet ventricle
Q20.5 Discordant atrioventricular connection
Q20.6 Isomerism of atrial appendages
Q20.8 Other congenital malformations of cardiac chambers and connections
Q20.9 Congenital malformation of cardiac chambers and connections, unspecified
Q21.0 Ventricular septal defect
Q21.1 Atrial septal defect
Q21.2 Atrioventricular septal defect
Q21.3 Tetralogy of Fallot
Q21.4 Aortopulmonary septal defect
Q21.8 Other congenital malformations of cardiac septa
Q21.9 Congenital malformation of cardiac septum, unspecified
Q22.0 Pulmonary valve atresia
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
Q22.8 Other congenital malformations of tricuspid valve
Q22.9 Congenital malformation of tricuspid valve, unspecified
Q23.0 Congenital stenosis of aortic valve
Q23.1 Congenital insufficiency of aortic valve
Q23.2 Congenital mitral stenosis
Q23.3 Congenital mitral insufficiency
Q23.4 Hypoplastic left heart syndrome
Q23.8 Other congenital malformations of aortic and mitral valves
Q23.9 Congenital malformation of aortic and mitral valves, unspecifie
Q24.0 Dextrocardia
Q24.1 Levocardia
Q24.2 Cor triatriatum
Q24.3 Pulmonary infundibular stenosis
Q24.4 Congenital subaortic stenosis
Q24.5 Malformation of coronary vessels
Q24.6 Congenital heart block
Q24.8 Other specified congenital malformations of heart
Q24.9 Congenital malformation of heart, unspecified
Q25.0 Patent ductus arteriosus
Q25.1 Coarctation of aorta
Q25.2  
Q25.21 Interruption of aortic arch
Q25.29  Other atresia of aorta
Q25.3 Supravalvular aortic stenosis
Q25.4  
Q25.41 Absence and aplasia of aorta
Q25.42 Hypoplasia of aorta
Q25.43 Congenital aneurysm of aorta
Q25.44 Congenital dilation of aorta
Q25.45 Double aortic arch 
Q25.46 Tortuous aortic arch
Q25.47 Right aortic arch
Q25.48 Anomalous origin of subclavian artery
Q25.49 Other congenital malformations of aorta
Q25.5 Atresia of pulmonary artery
Q25.6 Stenosis of pulmonary artery
Q25.71 Coarctation of pulmonary artery
Q25.72 Congenital pulmonary arteriovenous malformation
Q25.79 Other congenital malformations of pulmonary artery
Q25.8 Other congenital malformations of other great arteries
Q25.9 Congenital malformation of great arteries, unspecified
Q26.0 Congenital stenosis of vena cava
Q26.1 Persistent left superior vena cava
Q26.2 Total anomalous pulmonary venous connection
Q26.3 Partial anomalous pulmonary venous connection
Q26.4 Anomalous pulmonary venous connection, unspecified
Q26.5 Anomalous portal venous connection
Q26.6 Portal vein-hepatic artery fistula
Q26.8 Other congenital malformations of great veins
Q26.9 Congenital malformation of great vein, unspecified
Q27.0 Congenital absence and hypoplasia of umbilical artery
Q27.1 Congenital renal artery stenosis
Q27.2 Other congenital malformations of renal artery
Q27.31 Arteriovenous malformation of vessel of upper limb
Q27.32 Arteriovenous malformation of vessel of lower limb
Q27.33 Arteriovenous malformation of digestive system vessel
Q27.34 Arteriovenous malformation of renal vessel
Q27.39 Arteriovenous malformation, other site
Q27.8 Other specified congenital malformations of peripheral vascular system
Q27.9 Congenital malformation of peripheral vascular system, unspecified
Q28.2 Arteriovenous malformation of cerebral vessels
Q28.3 Other malformations of cerebral vessels
I27.83 Eisenmenger's syndrome

Questions?
For more information, review the American College of Cardiology’s article announcing the new codes. 

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