Diagnosis: Pulmonary Arterial Hypertension.
Treatment: Sildenafil.

The insurer denied coverage for Sildenafil. The denial is overturned.


The patient is male with a history of Tetralogy Fallot repair as a child and recent pulmonic and tricuspid valve surgery. This patient suffers from pulmonary arterial hypertension (PAH)r that is present following corrective surgery for congenital heart disease (Tetralogy of Fallot). As per Simonneau et al. (2009), the patient's condition is classified as World Health Organization (WHO) Group 1, type D- pulmonary arterial hypertension (PAH) involving congenital systemic to pulmonary shunts. Following this patient's corrective surgery for pulmonic and tricuspid valve regurgitation, the patient developed shortness of breath and lower extremity edema. The patient's echocardiogram revealed severe right ventricular dilatation with severe tricuspid regurgitation (TR) and paravalvular pulmonic valve regurgitation. The patient's estimated pulmonary artery pressure was 60mmHg (millimeter of mercury) plus right atrial pressure on echocardiogram. On physical exam, the patient demonstrated findings consistent with severe right-sided volume overload. The patient was prescribed with diuretics and sildenafil. The patient's prior right heart catheterization revealed severe pulmonary artery hypertension with mean pulmonary artery pressures of 30mmHg. These clinical findings support a diagnosis of WHO Group I and WHO functional class III PAH.

As per Galie et al. (2016), current guidelines support a class I indication for treatment of such patients with phosphodiesterase type 5 inhibitors such as sildenafil. Multiple randomized controlled trials have demonstrated that these agents are beneficial. (3-5)

Based on the review of the medical record and literature, sildenafil is considered medically necessary for this patient.

The health plan did not act reasonably with sound medical judgment, and in the best interest of the patient.

The insurer's denial of coverage for sildenafil to treat PAH should be reversed. The medical necessity is substantiated.