Aggravating factors associated with the severity of aortic regurgitation and clinical characteristics of patients with quadricuspid aortic valve.

BACKGROUND AND AIM OF THE STUDY: Quadricuspid aortic valve (QAV) is a rare congenital anomaly. Aortic regurgitation (AR) has been suggested to be the major functional abnormality of QAV. The study aim was to define the clinical characteristics of patients with QAV through echocardiography, thereby identifying factors related to the severity of AR. METHODS: Patients diagnosed with QAV by echocardiography were enrolled from electronic medical records obtained between November 1994 and May 2012. RESULTS: Twenty-six patients diagnosed with QAV were identified. AR was present in 20 of these patients (77%), among which 12 (46%) exhibited significant AR. Aortic stenosis was rare (n = 1; 4%). Patient age and severity of AR were weakly correlated. The most frequent valve morphology type was three equally sized cusps and one smaller cusp (n = 12; 46%), followed by two larger, equally sized cusps and two smaller, equally sized cusps (n = 6; 23%). The latter morphology type was accompanied by a high prevalence of significant AR (5/6; 83%). The initial clinical symptoms of the patients included dyspnea and chest pain (n = 14; 54%), other congenital abnormalities (n = 4; 15%), and arrhythmias (n = 4; 15%). Among the patients with significant AR, 10 were symptomatic. CONCLUSION: Approximately half of the patients with QAV had significant AR at the time of initial diagnosis by echocardiography. The severity of AR appeared to be correlated with patient age and valve morphology. These factors should be considered during echocardiographic assessment and follow up treatment for QAV.