Clinical characteristics and surgical outcomes of dysfunctional quadricuspid aortic valve

Quadricuspid aortic valve (QAV) is a rare and poorly understood congenital cardiac abnormality. This study aims to evaluate the clinical features and surgical outcomes of dysfunctional QAV.

From January 2011 to May 2017, 36 (mean age 50.7 ± 11.9 y, 19 males) of a total of 3855 patients who underwent aortic valve surgery were identified as having dysfunctional QAV (frequency 0.9%). All patients presented moderate or severe aortic regurgitation, and nine patients (25.0%) had concomitant aortic stenosis. The ascending aortic diameter was over 40 mm in seven patients (19.4%) and over 45 mm in two patients (5.6%). The most common QAV morphology was type B (n = 12, 33.3%) according to the Hurwitz-Roberts classification.

All patients underwent aortic valve replacement and two required concomitant ascending aortic replacement. The mean follow-up time was 20.6 ± 14.2 mo. There was no early or late postoperative mortality or major complications. Pathological analysis of dilated ascending aorta demonstrated a relatively normal appearance. The ascending aorta did not grow after surgery (37.3 ± 4.1 mm versus 36.1 ± 2.5 mm, P = 0.084). Both the end-diastolic (58.1 ± 7.0 mm versus 50.0 ± 6.3 mm, P < 0.001) and end-systolic (37.7 ± 6.7 mm versus 32.8 ± 6.0 mm, P < 0.001) left ventricular dimensions were significantly decreased.

Aortic insufficiency is the predominant pathology in dysfunctional QAV patients. The incidence and extent of aortic dilation is not significant in QAV and not associated with aortic valve phenotypes. Short- and mid-term surgical outcomes were found to be satisfactory in this study.