If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
A 35-year-old woman presented with a history of gradually worsening dyspnea on exertion. Clinical examination revealed a diastolic murmur heard loudest along the upper left sternal border, which did not radiate to the carotids or the precordium. Transthoracic echocardiography demonstrated a quadricuspid aortic valve (type A, Hurwitz and Robert's classification) with an aorta-to-right atrium fistula (sinus 2) through a ruptured right-anterior sinus of Valsalva (Figure 1, A). In addition, the coronary orifices originated from sinus 1 (right coronary artery) and sinus 4 (left main coronary artery), and the aortic regurgitation was moderate (Figure 1, A and B). A diagnosis of a congenital quadricuspid aortic valve associated with ruptured sinus of Valsalva aneurysm was made, and the patient was referred for surgical correction. During the procedure, the aortic valve was confirmed to have 4 leaflets with associated sinuses, and a ruptured right-anterior sinus of Valsalva without coronary orifices was identified (Figure 2, A). The proximal windsock orifice was below the right-anterior sinus of Valsalva, and a right angle forceps could easily be passed through the aorta-to-right atrium fistula into the right atrium (Figure 2, B). The ruptured sinus of Valsalva aneurysm with an associated leaflet was resected. A 4-0 polypropylene suture was used to reconstruct the aortic root with 3 leaflets and associated sinuses. Postoperative transesophageal echocardiography showed normal aortic valvular function with no aortic insufficiency or residual shunt (Figure 3, A and B). The patient recovered well and was discharged a few days later, with no further complications.
Published online: July 18, 2019
Received in revised form:
This work was supported by the 2017 Wuhan Young and Middle-Aged Medical Backbone Training Program; the Health and Family Planning Commission of Wuhan municipality scientific research project (WX17Q35); and the Wuhan Science and Technology Bureau (2016060101010036).
Drs Song, Xu, and Xiao contributed equally to the article.
Disclosures: Authors have nothing to disclose with regard to commercial support.
Quadricuspid aortic valves (QAVs) are very rare but have been well described since the early 1970s.1 Even less recognized but documented is the association of QAVs with a potential congenital structural aortopathy. In the largest series of QAVs to date, 42% of patients had ascending aortic diameters greater than or equal to 4 cm.2 Most patients with QAVs present with aortic insufficiency, aortic stenosis, or a combination of these. In this issue of the Journal, Xu and associates3 report on a 35-year-old woman who presented with a QAV associated with a ruptured sinus of Valsalva aneurysm.