Pulmonary hypertension in a 12mo.Submitted by John T. Eanes II
Denial Type: Denial of a Pharmaceutical Hospital Course:This is a 12 month old former 25 week premature female with severe bronchopulmonary dysplasia and technology dependence. The cardiologist noted, “Her degree of PH [Pulmonary Hypertension] has been stable for a few months now, in the mild to moderate range. We have not seen much improvement in her echo over the last 2-3 months, and she is still on very high respiratory support, and seem to have very little reserve (documented that she desaturates very quickly when NC comes off). For this reason, we think she may benefit from starting dual therapy for management of her PH.” At this time she was started on bosentan. The prescription of bosentan was denied during a forensic review as an experimental therapy. The denials team provided the following information: Bosentan is an endothelin receptor antagonist used for pulmonary artery hypertension associated with congenital heart disease. Per “A systemic review and meta-analysis – The efficiency of endothelin receptor antagonist bosentan for pulmonary artery hypertension associated with congenital heart disease. Kuang et al., Medicine (2018) 97:10(e0075)” Bosentan in [Pulmonary Artery Hypertension – Congestive Heart Disease] is well established [although] still requires clinical trials for an identification of its efficiency on [Congestive Heart Disease] patients for an optimized period lessening a serious complication and the common [Adverse Events]. In addition per the AHA/ATS Guideline, Pediatric Pulmonary Hypertension, Guidelines from the American Heart Association and American Thoracic Society, Circulation,2015; 132: 2037-2099. DOI 10.116/CIR.0000000000000329. “ Bosentan, a dual ERA, lowers PAP and PVR and improves exercise capacity in adults with PAH,286 and similar results have been reported in children.315,340–346 Bosentan lowers PAP and PVR and is well tolerated in children with IPAH or PAH associated with CHD.343,344,347 Elevated hepatic aminotransferase levels occur in ≈11% of adults and 3% of children treated with bosentan. In a 12-week study, bosentan was well tolerated and lowered the PAP and PVR children with IPAH or PAH related to CHD.343 A retrospective study of 86 children on bosentan for a median exposure of 14 months, with and without concomitant therapy, reported that bosentan caused sustained clinical and hemodynamic improvement and was well tolerated, with 2-year survival estimates of 91%.342 Follow-up of these patients at 4 years revealed that the Kaplan- Meier estimate of disease progression in patients on bosentan was high (54%), with a survival estimate of 82%.348 Bosentan therapy provided short-term improvement in WHO functional class and 6MWD test in children and adults with PAH and systemic-to-pulmonary shunt.349 This beneficial response progressively declined after 1 year, with a more rapid decline observed in children, who tended to have more severe disease at baseline than adults.349” pp.2064-2065 Appeal Strategy:The team concentrated on medical documentation and references, and eventually won on contractual obligations and allowances from Medicaid. This is an example where quoting a definitive medical guideline supported the position of the care team and hospital. |