2024/12/16 by Jie Wang, Ping Hu, Bin Bai +1 · 1 voice
Medicine · #Infective Endocarditis Diagnosis and Management #Coronary Artery Anomalies #Cardiac Valve Diseases and Treatments
paper · doi:10.1093/ehjci/jeae323
openalex publication_date 2024/12/16 · openalex created_date 2025/10/10 · openalex updated_date 2026/07/22
A 2-year-old preschool child was admitted to the hospital due to a heart murmur detected during a school entry physical examination. Clinical examination revealed a 3/6 diastolic murmur at two to four intercostal spaces along the left sternal border. Transthoracic echocardiography demonstrated a quadruple aortic valve (QAV, Type B). Color Doppler imaging showed continuous shunting from the accessory sinus (Panels A and B, Sinus 4, arrow; Supplementary data online, Videos S1–S3) to the membranous ventricular septal aneurysm (asterisk), predominantly during diastole, with a shunt velocity of 5 m/s. Severe regurgitation was noted from the accessory cusp. Computed tomography angiography revealed a QAV with three equally sized sinuses and a smaller accessory sinus (Panels C and D, Sinus 4) communicating with the membranous ventricular septal aneurysm (Panels E and F, asterisk). The patient underwent surgical correction, and intraoperatively, a mid-cusp tissue defect of the accessory sinus was identified, leading to severe aortic valve regurgitation (Panel G, arrow; Supplementary data online, Video S4). Postoperatively, the patient recovered well and was discharged a few days later without complications. QAV is a rare congenital heart defect, and its association with aortic sinus aneurysm rupture is even rarer. In our case, the partial absence of the accessory cusp and rupture into the membranous aneurysm posed significant diagnostic challenges due to similarities with aorto-left ventricular tunnel (ALVT). Multimodal imaging was crucial for differentiation, accurately displaying the ‘X’ configuration of the QAV and the bidirectional blood flow signals of ALVT. This case underscores the importance of multimodal imaging in diagnosing and managing rare and complex congenital heart defects such as QAV with aortic sinus aneurysm rupture. Supplementary data are available at European Heart Journal - Cardiovascular Imaging online. Funding: This work was funded by the Wuhan Clinical Medical Research Center for Cardiovascular Imaging and the Open Fund of Imaging Center (Project No. CMRC202302). Ethical approval: Hospital exempted from ethics review in view of retrospective cases. Consent: This report has obtained the patient's informed consent for the publication of their anonymized clinical data. Data availability: The data that support the findings of this study are available from the corresponding author.