Visualization of perimembranous ventricular septal defect with ruptured sinus of Valsalva aneurysm by three-dimensional transesophageal echocardiography and multidetector three-dimensional computed tomography

Excerpt

A 31-year-old man was referred to our institution for the evaluation of cardiac murmur without symptoms. Auscultation revealed a loud continuous systolo-diastolic murmur over the left sternal border and a thrill through the precordium. His blood pressure was 124/60, electrocardiogram (ECG) was normal, and chest X-ray demonstrated mild cardiomegaly and slightly increased pulmonary vascular markings. Transthoracic echocardiography showed a sinus of Valsalva aneurysm at the right coronary cusp (RCC), left to right shunt, and mild biventricular enlargement. Multidetector three-dimensional computed tomography (MD3DCT) revealed aorta to right ventricle (RV) shunt through the ruptured sinus of Valsalva aneurysm at the RCC in diastolic phase, and left ventricular outflow tract to RV shunt through a perimembranous ventricular septal defect (PVSD) in systolic phase (Fig. 1a, b, reconstructed from the ECG-gated four-dimensional data). These findings were also confirmed by three-dimensional transesophageal echocardiography (3DTEE) (Fig. 1c–h: c–f were reconstructed from three-dimensional image data; Supplemental Videos S1–S4). The sinus of Valsalva wall protruded through the PVSD into the RV, forming an aneurysm, and finally ruptured. Aortic valve leaflet covered the aortic side entry of the aneurysm and a small subaortic PVSD arose just under the cusp in systolic phase. The location of the ruptured aneurysm was classified as type II of the Sakakibara and Konno classification [1], which originates in the mid portion of the RCC, away from the pulmonary valve. The PVSD measured 6 × 7 mm on MD3DCT and 5 × 8 mm on 3DTEE in diastolic phase. The ratio of pulmonary to aortic flow was 1.7 at cardiac catheterization by oximetry.

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