Surgical Repair of Regurgitant Quadricuspid Aortic Valve

Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect that was first classified by Hurwitz and Roberts following autopsy studies in 1973 (1). Because of its rarity and characteristics, the natural history and long-term outcomes are still poorly defined. Recently, Tsang et al. demonstrated a quadricuspid aortic valve diagnostic frequency of 0.006% (2). In their population, aortic dilatation was present in 29% of the patients, and 26% of those patients had moderate or severe aortic valve regurgitation, which was the most frequent reason for surgery. The general principles of aortic valve repair can also be applied to quadricuspid aortic valves. However, surgical records demonstrate that repair of quadricuspid valves is attempted in only 23% of patients (3). The high variability of the anatomy and the lack of standardized surgical techniques could explain this low percentage of valve repair.

The authors present the case of a 32-year-old man with a quadricuspid aortic valve. The standardization principles of conservative aortic valve surgery by Lansac et al. were applied, addressing the three elements that support aortic valve physiology: cusps, root diameters (aortic annular base and sinotubular junction), and root dynamics. The annulus dilation was corrected by applying an expansible aortic ring in a subvalvular position. The authors remodeled the aortic root, reducing the sinotubular junction diameter and respecting the root dynamics with the Yacoub technique, and successfully performed the bicuspidization and re-suspension of the leaflets.

The authors believe that this technique could be a safe, easy, and standardized method to correct two of the lesions that cause aortic regurgitation in patients with quadricuspid aortic valve and aortic aneurysm, limiting the variability of the repair to the quality and morphology of the four cusps.

References:

1. Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol. 1973;31:623-6.
2. Tsang MY, Abudiab MM, Ammash NM, Naqvi TZ, Edwards WD, Nkomo VT, et al. Quadricuspid Aortic Valve: Characteristics, Associated Structural Cardiovascular Abnormalities, and Clinical Outcomes. Circulation 2015; first published on December 3 2015 as doi:10.1161/CIRCULATIONAHA.115.017743.
3. Idrees JJ, Roselli EE, Arafat A, Johnston DR, Svensson LG, Sabik JF 3rd, et al. Outcomes after repair or replacement of dysfunctional quadricuspid aortic valve. J Thorac Cardiovasc Surg. 2015;150:79-82.
4. Lansac E, Di Centa I, Raoux F, Raffoul R, Al Attar N, Rama A, et al. Aortic annuloplasty: towards a standardized approach of conservative aortic valve surgery. Multimed Man Cardiothorac Surg. 2007;329:mmcts.2006.001958.