Congenital Anomalies of the Aortic Root

Neil Venardos, MD, Harma K. Turbendian, MD

Key Points

  • Congenital anomalies of the aortic root include aortic root aneurysms and dissections (often associated with connective tissue disorders such as Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome), sinus of Valsalva aneurysms, and aorto-left ventricular tunnels. Each has distinct pathophysiology and clinical implications.
  • Many aortic root anomalies are linked to genetic syndromes impacting connective tissue integrity, leading to progressive dilation, aneurysm formation, and increased risk for dissection or rupture - all of which contribute to determination of timing for surgical intervention.
  • Medical therapy (e.g., beta blockers, ACE inhibitors) may slow progression of aortic root aneurysms in select cases, but surgical intervention is the definitive treatment when size or growth thresholds are met, or complications arise. Valve-sparing Root Replacement or the Florida Sleeve Operation are preferred when feasible; outcomes are generally excellent with appropriate patient selection.
  • Personalized external aortic root support (PEARS) is currently being used as a pre-emptive intervention for aortic root dilation in the setting of connective tissue disorders. Precise indications are still being defined, and the device has not yet received FDA approval for use in the United States of America.
  • Sinus of Valsalva aneurysms and aorto-left ventricular tunnels are rare lesions which require surgical intervention.

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