Quadricuspid aortic valve: a rare cause of severe aortic regurgitation

Excerpt

Quadricuspid aortic valve (QAV) is a rare congenital heart disease with an incidence of approximately 0.006% in patients undergoing transthoracic echocardiography [1]. It is typically associated with isolated aortic regurgitation (AR) which may leads to left ventricular dilatation and dysfunction, commonly occurring after the age of 40, as seen in this 58-year-old male patient with advanced stage chronic obstructive pulmonary disease (COPD) [2]. He was referred for cardiological assessment due to worsening dyspnea despite receiving optimal medical COPD treatment. Echocardiographic assessment revealed a QAV type A (Hurwitz and Roberts classification) [3] with a central coaptation deficit measuring 0.5 cm² (Fig. 1A) and a 3D vena contracta area of 0.4 cm², causing severe regurgitation and subsequent severe left ventricular dilatation. The coaptation deficit is evident in the 3D-echocardiographic reconstruction (Fig. 1D) as in the 3D reconstruction from the pre-operative computerized tomography which was performed for anatomical and coronary artery assessment (Fig. 1C). Additionally, the aortic regurgitation jet (Fig. 1B) was partially impacting the anterior mitral valve leaflet (AMVL) causing premature middiastolic rebound into the left ventricular inflow tract (Fig. 1E and F).

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