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Introduction:Sustained fetal supraventricular tachycardia (SVT) is associated with significant morbidity and mortality if untreated, yet the optimal management strategy remains unclear.Methods:Retrospective cohort study including all fetuses diagnosed with sustained SVT (&gt50% of the diagnostic echocardiogram) between 1985-2018. Fetuses with congenital heart disease were excluded.Results:Sustained SVT was diagnosed in 65 fetuses at a median gestational age of 30 weeks (range 14-37). Atrioventricular reentrant tachycardia (AVRT) and atrial flutter (AFL) were the most common diagnoses, seen in 41 and 16 cases, respectively. Moderate/severe ventricular dysfunction was present in 20 fetuses, and hydrops fetalis was present in 13. Of the 57 fetuses initiated on transplacental drug therapy, 47 received digoxin first-line yet 39/57 (68%) required advanced therapy with sotalol, flecainide, and/or amiodarone (10 primary, 29 secondary). Treatment success was achieved in 47/57 treated fetuses. Hydrops (OR 4.44, 95% CI 1.2-17.0, p=0.03) was associated with preterm delivery but not treatment failure. There were no cases of fetal demise. Later gestational age at fetal diagnosis (OR 1.1, 95% CI 1.01-1.2, p=0.046) and moderate/severe fetal ventricular dysfunction (OR 5.0, 95% CI 1.5-16.7, p=0.008) were associated with postnatal SVT requiring discharge on anti-arrhythmic medication. A single postnatal death occurred in an infant with fetal AVRT born at 30 weeks and 1.2 kg with severe hydrops and ventricular dysfunction.Conclusions:Fetuses with structurally normal hearts and sustained SVT can be effectively managed with transplacental drug therapy with minimal risk of fetal demise. Treatment often requires multiple anti-arrhythmic agents. Hydrops predicts preterm delivery but not treatment failure. Later gestational age at fetal diagnosis and the presence of depressed fetal ventricular function predict postnatal arrhythmia burden.

Introduction:Patients with coarctation of the aorta (CoA) have a high prevalence of intracranial aneurysms (IA) and suffer subarachnoid hemorrhage (SAH) at significantly younger ages than the general population. The 2018 AHA/ACC Guideline for Adults with Congenital Heart Disease recommends IA screening, but the timing (start and stop ages), frequency of IA screening and its effectiveness remain a critical knowledge gap.Methods:To evaluate the benefits and cost-effectiveness of magnetic resonance angiography screening strategies for IA in patients with CoA, we developed a Markov model and calibrated the model to match published IA prevalence estimates at ages 29, 33, and 41 years. The primary outcome was the incremental cost-effectiveness ratio (ICER) and secondary outcomes included lifetime cumulative incidence of prophylactic IA treatment and resulting mortality, SAH deaths prevented, and life-years saved. Using a payer perspective, a lifetime horizon, and a willingness to pay of $150,000 per quality-adjusted life year (QALY) gained, we applied a 3% annual discounting rate to costs and effects and performed 1-way, 2-way, and probabilistic sensitivity analyses.Results:In a simulated cohort of 10,000 CoA 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 980 prophylactic treatments for unruptured aneurysms and 18 procedure-related deaths but prevented 118 deaths from SAH and resulted in a net gain of 2,300 life-years. Screening at ages 10, 20 and 30 years was cost-effective vs. no screening (ICER $58,000/QALY) and vs. screening at ages 10 and 20 (ICER $108,000/QALY). Other strategies were inferior. Screening beyond age 40 did not increase QALYs. In sensitivity analysis, uncertainty in the outcome after aneurysm treatment and rate of aneurysm growth 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 ACC/AHA recommendation to screen patients with CoA for IA and suggests screening at ages 10 and 20 or at 10, 20 and 30 years would extend life and be cost-effective.