Background: Patients with coarctation of the aorta have a high prevalence of intracranial aneurysms (IA) and suffer subarachnoid hemorrhage (SAH) at younger ages than the general population. American Heart Association/American College of Cardiology guidelines recommend IA screening, but appropriate age and interval of screening and its effectiveness remain a critical knowledge gap.
Methods and results: To evaluate the benefits and cost-effectiveness of magnetic resonance angiography screening for IA in patients with coarctation of the aorta, we developed and calibrated a Markov model to match published IA prevalence estimates. The primary outcome was the incremental cost-effectiveness ratio. Secondary outcomes included lifetime cumulative incidence of prophylactic IA treatment and mortality and SAH deaths prevented. Using a payer perspective, a lifetime horizon, and a willingness-to-pay of $150 000 per quality-adjusted life-year gained, we applied a 3% annual discounting rate to costs and effects and performed 1-way, 2-way, and probabilistic sensitivity analyses. In a simulated cohort of 10 000 patients, no screening resulted in a 10.1% lifetime incidence of SAH and 183 SAH-related deaths. Screening at ages 10, 20, and 30 years led to 978 prophylactic treatments for unruptured aneurysms, 19 procedure-related deaths, and 65 SAH-related deaths. Screening at ages 10, 20, and 30 years was cost-effective compared with screening at ages 10 and 20 years (incremental cost-effectiveness ratio $106 841/quality-adjusted life-year). Uncertainty in the outcome after aneurysm treatment and quality of life after SAH influenced the preferred screening strategy. In probabilistic sensitivity analysis, screening at ages 10, 20, and 30 years was cost-effective in 41% of simulations and at ages 10 and 20 in 59% of simulations.
Conclusions: Our model supports the American Heart Association/American College of Cardiology recommendation to screen patients with coarctation of the aorta for IA and suggests screening at ages 10 and 20 or at 10, 20, and 30 years would extend life and be cost-effective.
Keywords: aorta; intracranial aneurysm; magnetic resonance angiography; quality of life; subarachnoid hemorrhage.