Management of infants with mutations in ENPP1 is symptomatic (standard treatment of cardiac failure).
Management of vascular calcifications, in particular, involves treatment with bisphosphonates. In an analysis of 43 patients who survived their first day of life, mortality was 35% in those treated with bisphosphonates, and 69% for those not treated with bisphosphonates.
Management for hypertension is standard.
For management of rickets supplementation with phosphorus and calcitriol is recommended. Of note, worsening of vascular calcification has not been seen with judicious treatment.
Surveillance includes at least yearly audiology examination, and yearly retinal examination. Lateral cervical spine X-rays should be obtained prior to intubation if patients need surgery, given the potential (though rare) complication of spinal fusion.