Background: To support decision-making in aortic valve replacement in children and adults, we provide a comprehensive overview of outcome after the Ross procedure. Methods and Results: A systematic search was conducted for publications reporting clinical outcome after the Ross procedure, published between January 1, 2000, and November 22, 2017. Reported event rates and time-to-event data were pooled and entered into a microsimulation model to calculate life expectancy and lifetime event risk. Ninety-nine publications were included (13 129 patients; total follow-up: 93 408 patient-years, pooled mean follow-up: 7.9±5.3 years). Pooled mean age at surgery was 9.4±5.5 years for children and 41.9±11.4 for adults. For children and adults, respectively, pooled early mortality risk was 4.19% (95% CI, 3.21–5.46) and 2.01% (95% CI, 1.44–2.82), late mortality rate was 0.54%/y (95% CI, 0.42–0.70) and 0.59%/y (95% CI, 0.46–0.76), autograft reintervention 1.28%/y (95% CI, 0.99–1.66) and 0.83%/y (95% CI, 0.68–1.01), and right ventricular outflow tract reintervention 1.97%/y (95% CI, 1.64–2.36) and 0.47%/y (95% CI, 0.37–0.59). Pooled thromboembolism and bleeding rates were low and comparable to the general population. Lifetime risks of autograft and right ventricular outflow tract reintervention were, respectively, 94% and 100% for children and 49% and 19% for a 45-year-old. Estimated life expectancy after surgery was 59 years for children (general population: 64 years) and 30 years for a 45 years old (general population: 31 years). Conclusions: Through excellent survival and avoidance of the burden of anticoagulation, the Ross procedure provides a unique opportunity for patients whose preferences do not align with the outcome provided by mechanical valve replacement and for growing children who also benefit from autograft diameter increase along with somatic growth. On the downside, almost all pediatric and many adult Ross patients will require a reintervention in their lifetime.

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This study demonstrates that outcome after mechanical AVR in non-elderly adults is characterized by suboptimal survival and considerable lifetime risk of anticoagulation-related complications, but also reoperation. Non-elderly adult patients who are facing prosthetic valve selection are entitled to conveyance of evidence-based estimates of the risks and benefits of both mechanical and biological valve options in a shared decision-making process.

This systematic review illustrates that all currently available aortic valve substitutes are associated with suboptimal results in children, reflecting the urgent need for reliable and durable repair techniques and innovative replacement solutions for this challenging group of patients.