Slide1:
Congenital Heart Anomaly Procedures  
Proposals for ICD-10-PCS Changes
ICD Coordination and Maintenance Committee Meeting
March 18, 2015

Slide 2:
Issue: Difficulty Coding Procedures
Difficulty coding aspects of complex heart anomaly correction procedures identified by multiple Children’s Hospitals during their ICD10 preparations
ICD-9-CM: usually one code to describe all parts of the procedure
ICD-10-PCS: requires multiple codes to describe all parts of the procedure
Children’s Hospitals would like to code as specifically as possible
– not use generic “Repair” root operation or “Heart” body part value

Slide 3:
CHA’s Involvement
Children’s Hospital Association became involved due to close ties with our member hospitals
– Regular Coding Roundtable discussions
No proposals presented to ICD C&M Committee to correct or question coding issues
Created special Coding Workgroup to determine gaps and create proposals
– Representatives from 8 large children’s hospitals from across the nation
– Comprised of coding managers, lead coders, and ICD10 project managers
– Most participants are AHIMA approved ICD-10-CM and ICD-10-PCS trainers


Slide 4:
Congenital Heart Anomaly Procedures
All procedures presented are current standard of care for treatment of a variety of congenital heart defects:
– Modified Blalock-Taussig Shunt
– Arterial Switch
– Rastelli Procedure
– Repair of Common Atrioventricular Canal Defect (Endocardial Cushion Defect)
– Repair of Truncus Arteriosus
– Balloon Atrial Septostomy

Slide5:
Modified Blalock-Taussig Shunt
Patient with cyanotic heart defects – Tetralogy of Fallot, pulmonary stenosis or atresia, tricuspid atresia
Palliative procedure – create a systemic to pulmonary shunt to increase pulmonary blood flow until corrective procedure can be performed
Procedure Description: 
– Graft (usually Gore-tex) placed between either the innominate, subclavian or carotid arteries to a pulmonary artery to re-route blood flow
Patient may or may not need cardiopulmonary bypass 

Slide6: 
Arterial Switch w/Repositioning of Coronary Arteries
Patient with Transposition of the Great Vessels – Aorta and Pulmonary Trunk 
Corrective procedure performed in first 2 weeks of life
Patient may have other heart anomalies – ASD, VSD, PDA repaired at same episode
Gap found when coding procedures in ICD-10-PCS for excision of the coronary “buttons” from the anatomic aorta and reattaching them into the “neo-aorta” created when repositioning the aorta and pulmonary trunk
Reposition – desired root operation 

Slide 7:
Arterial Switch with Repositioning of Coronary Arteries – Procedure Description
• Aorta and Pulmonary Trunk transected
• Left and right ostia coronary ostia excised from aorta with a “button” of aortic wall
• Lecompte maneuver performed bringing pulmonary artery in front of aorta
• Reconstruction of aorta with former pulmonary trunk artery
• Coronary “Buttons” reattached to new “neo-aorta” 
• Reconstruction of pulmonary artery trunk
• Cardiopulmonary bypass & TEE utilized

Slide 8:
Rastelli Procedure & Accompanying Procedures
Surgical treatment for patients with numerous congenital conditions
– Transposition of great vessels and pulmonary stenosis
– Pulmonary atresia with VSD 
– Double outlet right ventricle with pulmonary stenosis or atresia
– Some patients have had a previously placed Blalock-Taussig shunt
Gaps found when coding procedures in ICD-10-PCS for accompanying procedures
– Ligation & division of BT shunt
– Oversewing pulmonary valve

Slide 9:
Rastelli Procedure – Procedure Description 
Right ventriculotomy performed
Ventricular Septal Defect closed with graft material
Pulmonary artery transected with oversewing of the pulmonary valve
Valved homograft conduit anastomosed from pulmonary bifurcation/trunk to the right ventricle outflow tract
Other procedures: ligation and division of BT shunt  and closure atrial septal defect
Cardiopulmonary bypass & TEE utilized

Slide 10:
Repair Complete Common Atrioventricular Canal Defect
Common AV Valve Defect is a combination of congenital heart defects caused by failure of normal development of the endocardial cushion
– Atrial Septal Defect
– Ventricular Septal Defect
– Common atrioventricular valve fails to separate into the Mitral and Tricuspid Valves
Often associated with Down’s syndrome patients
Surgical intervention undertaken in early infancy
Patients may need further interventions to correct/repair AV valve problems throughout lifetime
Coding gaps found when attempting to code procedures to repair the AV canal and valves

Slide 11:
Repair Complete Common Atrioventricular Canal Defect – Procedure Description 
Right atriotomy performed
Ventricular Septal Defect closed with patch material (Dacron or pericardial) incorporating AV Valve tissue to divide the valve into left and right sides
Cleft in left AV valve leaflets closed with sutures
Atrial Septal Defect closed with patch material (Dacron or pericardial) with suture of valve to ASD patch
Valves tested for leakage 
Cardiopulmonary bypass & TEE utilized

Slide 12:
Persistent Truncus Arteriosus Repair
Occurs when truncal artery fails to separate into the ascending aorta and pulmonary trunk/arteries during gestation
Patient is born with a single great vessel straddling a large outlet ventricular septal defect
Spectrum of severity based on the origin of pulmonary arteries from the truncal artery:
– Type I: main pulmonary arises from the truncal artery
– Type II: branch pulmonary arteries arise separately but in close proximity to truncal artery
– Type III: branch pulmonary arteries may arise widely separate from the lateral aspects of truncus artery
Truncal valve abnormalities are common and valve function must be assessed preoperatively and may require intervention at time of surgery
Coding gaps found when attempting to code procedures to the truncal valve and artery

Slide 13:
Persistent Truncus Arteriosus Repair – Procedure Description
Pulmonary artery separated from truncal root
Truncal vessel transected if necessary to repair truncal valve
Repairs to truncal valve, if necessary – i.e. replacement, repair, valvotomy, commissuroplasty, excision, etc.
Truncal vessel repair – i.e. direct closure or with patch (neo-aorta)
Right ventriculotomy
Closure of ventricular septal defect with patch
Valved conduit created between right ventricle to pulmonary trunk/bifurcation
Cardiopulmonary bypass and TEE utilized	

Slide 14:
Balloon Atrial Septostomy
• Performed on patients born with dextro-transposition of the great arteries or another cyanotic heart defect with life-threatening low oxygen levels (hypoxemia)
• Performed via endovascular approach
• Used primarily for newborns under 6 weeks of age 
• Allows for more oxygen-rich blood to circulate in the body
• Palliative, temporary procedural measure until further corrective surgery can be performed – can also be performed in utero
• Atrial Septal Defect: may be present in patients
• Small/ restrictive ASD/Patent Foramen Ovale: enlarged during procedure
• No ASD/PFO: opening is created during procedure	
• Coding gap found when attempting to code this procedure

Slide 15:
Balloon Atrial Septostomy – Procedure Description
Catheterization performed; usually through umbilical vein
Guidewire and sheath advanced into Right atrium
Guidewire advanced across restrictive atrial septum or small ASD
Balloon catheter advanced across atrial septum, inflated and forcefully pulled back through the atrial septum 
Procedure may be repeated until the septal defect is adequate in size or additional septal defects may be created in multiple areas of the atrial septum
Performed under fluoroscopic and echocardiographic imaging

Slide 16:
References
• Pediatric Heart Surgery – A Ready Reference for Professionals – 5th Edition, Maxishare – Children’s Hospital of Wisconsin; L. Eliot May, PA-C
• Case Study Operative Reports from:
• Children’s Hospital of Philadelphia
• Children’s Hospital of Wisconsin
• Children’s Hospitals and Clinics of Minnesota

Slide 17:
Thank you for your consideration!
Presenter: 
Amber Davidson, RHIT, CCS, CCS-P
Health Information Specialist
Amber.Davidson@childrenshospitals.org
913-981-4140

