There are four (4)
primary drug categories addressed in this policy that are approved to treat
pulmonary arterial hypertension (PAH). Coverage criteria for each of the drugs
within each category are as below and prescribed by a pulmonologist or
cardiologist.
1)
Phosphodiesterase Type 5 (PDE-5) inhibitors
a.
Revatio
(Sildenafil)
i.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class
II-III symptoms AND
ii.
Confirmed
by right heart catheterization AND
iii.
No
concurrent nitrate therapy AND
iv.
Patient 18
years of age or older AND
v.
If
prescribing oral tablets, only generic is covered AND
vi.
If
prescribing oral suspension, must document why generic oral tablet cannot be
used AND
vii.
If
prescribing IV dosage form, must document why oral dosage form cannot be used.
b.
Adcirca
(Tadalafil)
i.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2), WHO Group 1, NYHA Class
II-III symptoms AND
ii.
Confirmed
by right heart catheterization AND
iii.
No
concurrent nitrate therapy AND
iv.
Patient is
18 years of age or older
2)
Endothelin
Receptor Antagonists
a.
Tracleer
(Bosentan)
i.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2) , WHO Group I, NYHA Class
II-IV symptoms AND
ii.
Confirmed
by right heart catheterization AND
iii.
Patient is
not pregnant AND
iv.
Patient not
receiving concomitant cyclosporine or glyburide therapy AND
v.
Provider
and patient enrolled in TAP Restricted Distribution Program
b.
Letairis
(Ambrisentan)
i.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class
II-III symptoms AND
ii.
Confirmed
by right heart catheterization AND
iii.
Patient is
not pregnant AND
iv.
Patient is
not diagnosed with idiopathic pulmonary fibrosis AND
v.
Provider
and female patients are enrolled in Letairis REMS program
c.
Opsumit (Macitentan)
i.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class
II-III symptoms AND
ii.
Confirmed
by right heart catheterization AND
iii.
Patient is
not pregnant AND
iv.
Provider
and female patients are enrolled in Opsumit REMS program
3)
Prostacyclin Analogues/Prostacyclin Agonists
a.
Epoprostenol (Flolan, Veletri)
i.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class
III-IV symptoms AND
ii.
Confirmed
by right heart catheterization
b.
Ventavis
(Iloprost)
i.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class III
symptoms AND
ii.
Confirmed
by right heart catheterization
c.
Treprostinil (Remodulin, Tyvaso, Orenitram)
i.
Remodulin
and Tyvaso are covered if meeting the criteria below; Orenitram is not covered.
ii.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class
II-III symptoms AND
iii.
Confirmed
by right heart catheterization
d.
Selexipag
(Uptravi)
i.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class
II-III symptoms AND
ii.
Confirmed
by right heart catheterization
4)
Guanylate
Cyclase Stimulators
a.
Adempas
(Riociguat)
i.
Diagnosis
of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class
II-III symptoms AND
ii.
Confirmed
by right heart catheterization AND
iii.
Patient is
not pregnant OR
iv.
Diagnosis
of persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH)
WHO Group 4 following surgery or when surgery is not an option AND
v.
Patient is
not pregnant
Initial
authorization shall be for six (6) months. Reauthorization for continued use
shall be reviewed at least every 12 months to confirm that the patient continues
to meet coverage criteria and has achieved an objective response to therapy
demonstrated by one of the following:
1)
Improvement
in exercise capacity from baseline (e.g. improved 6MW, O2 saturation, etc.) OR
2)
Improvement
in the signs/symptoms of PAH or lack of disease deterioration