Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Complex Congenital Heart Disease. 
Treatment:  Transcatheter Pulmonic Valve-in-Valve Replacement. 

The insurer denied coverage for: Transcatheter Pulmonic Valve Replacement, CPT code(s): 93451; 93566; 93568; 33477; 93593; 93594; 93462; 93596; and 93597. 

The denial is: overturned. 

Asked to review the case of a patient with complex congenital heart disease. The patient was born with Tetralogy of Fallot (TOF) and underwent complete repair with transannular patch at 8 months of age. The patient required open surgical pulmonary valve replacement 12 years ago as a result of severe pulmonary valve regurgitation and severe right ventricular dilatation. In recent echocardiogram demonstrated moderate bioprosthetic pulmonary valve regurgitation and moderate valve stenosis with a transvalvular gradient of 35 millimeter of mercury (mmHg).  Dilatation of the right ventricle was also noted.  Cardiac magnetic resonance imaging (MRI),  demonstrated a dilated right ventricle with an right ventricle/left ventricle (RV/LV) ratio of 2:1 and an RVEDVi (right ventricular end diastolic volume index) of 178 milliliter (ml)/meter squared (m2). Moderate pulmonary regurgitation with a regurgitant fraction of 35% and a dilated tricuspid valve with moderate tricuspid regurgitation (TR) was also noted.  Computed tomography (CT) scan demonstrated a severely dilated right ventricle with an RVEDVi of 199 ml/m2. The patient reported increasing fatigue and lower extremity swelling over the past several months. Examination demonstrated a 2/6 systolic ejection murmur over the left sternal border. 12 lead electrocardiogram (EKG) demonstrated normal sinus rhythm and right bundle branch block (RBBB) with a QRS duration of 164 milliseconds (msec). The patient was referred for transcatheter pulmonary valve-in-valve replacement. 

This patient has developed symptoms of right sided heart failure and multiple imaging studies reveal a failing bioprosthetic pulmonic valve with moderate pulmonic stenosis and regurgitation, severe right ventricular dilatation, tricuspid annular dilatation and concomitant tricuspid valve regurgitation. Current American Hospital Association (AHA)/ACC (American College of Cardiology) guidelines recommend replacement of failing bioprosthetic heart valves and specifically mention valve-in-valve prosthesis for failing pulmonic valves. (Reference 1-2). Reports from a recent multicenter prospective single arm study enrolled 58 patients with congenital heart disease and previous sternotomy with indications for transcatheter pulmonic valve replacement. They found that transcatheter pulmonic valve replacement was safe and effective in patients with previously implanted valves in the pulmonic position. (Reference 3) AHA guidelines focused on valve replacement in young patients with congenital heart disease support transcatheter pulmonic valve replacement in Tetralogy of Fallot (TOF) repair patients with moderate pulmonary regurgitation with a level IIa indication. (Reference 4). 

Current procedure terminology (CPT) code(s) 93451; 93566; 93568; 33477; 93593; 93594; 93462; 93596; and 93597 are medically necessary for this patient. The health plans guidelines and American Hospital Association (AHA)/ACC (American College of Cardiology) support the use of cardiac catheterization with transcatheter pulmonic valve replacement. (Reference 4-5)  The United guidelines themselves specify that patients with moderate pulmonary valve stenosis with a gradient greater than or equal to 35 millimeter of mercury (mmHg) are candidates covered for transcatheter pulmonary valve repair. (Reference 5)  

Based on the above, the insurer's denial must be overturned. The health care plan did not act reasonably and with sound medical judgment and in the best interest of the patient. The medical necessity for procedure to replace pulmonic valve, current procedure terminology (CPT) code(s) 93451; 93566; 93568; 33477; 93593; 93594; 93462; 93596; and 93597 is substantiated.