Multilobulated Sinus of Valsalva Aneurysm Dissecting into the Interventricular Septum (DAIS) and Rupturing into Left Ventricle: A Case Report

Abstract

Background: Dissecting aneurysm of sinus of Valsalva (SOV) into the interventricular septum is a rare entity. Multilobulated form of dissection rupturing into the left ventricle (LV) has never been reported in the literature.

Case summary: A 52-year-old male presented with dyspnoea and palpitation with wide pulse pressure and peripheral signs of distal run-off and a continuous murmur along the left parasternal area. Echocardiography revealed dilated right coronary cusp (RCC), which burrowed into the interventricular septum (IVS), forming multi-loculated cystic lesion which ruptured into LV with associated restrictive ventricular septal defect (VSD) and severe aortic regurgitation. Computed tomography (CT) angiography confirmed a 4.8 cm × 5.3 cm × 5.4 cm multiseptated aneurysm. The surgery involved excision of the aortic valve (AV) with the sinus, ligation of its penetrating portion at the crest of IVS, closure of VSD, and AV replacement. Postoperative echocardiography showed the complete collapse of the IVS component of the SOV aneurysm and the normally functioning mechanical AV.

Discussion: Dissecting aneurysm into the IVS is a rare variant of SOV aneurysm, usually arising from RCC. It is mostly congenital in origin and has wide variety of presentations like congestive heart failure, palpitations, recurrent syncope, chest pain, sudden cardiac arrest, infective endocarditis, cerebral infarction, or asymptomatic. Aortic regurgitation is present in 30-50% of cases. Conduction disturbances from first-degree block to complete heart block are common. Echocardiogram, CT angio, and magnetic resonance imaging are useful for diagnosis. Surgical repair is the only option for treatment.

Keywords: Computed tomography (CT) angiography; Dissecting aneurysm of the interventricular septum (DAIS); Echocardiography; Rupture of the sinus of Valsalva; Sinus of Valsalva aneurysm.

Figure 1

Electrocardiogram showing normal sinus rhythm with bifascicular block.

Figure 2

Two-dimensional echocardiography and colour Doppler images. (A) Dilated and loculated right coronary sinus and left coronary cusp in parasternal short-axis (PSAX) view. (B) Lobulated aneurysmal sinus of Valsalva burrowing into interventricular septum in apical four-chamber view. (C) Colour Doppler showing aortic regurgitation into left ventricle due to fenestration/rupture into left ventricle in apical 4-chamber (A4C) view.

Figure 3

Computed tomography angiography images (A–C) showing large multiseptated aneurysm (An) arising (dashed arrow depicting neck of the aneurysm) from the right sinus of Valsalva (#) and is seen burrowing into the basal and mid-cavity interventricular septum (black arrows) with mass effect on left ventricular outflow (*). An, aneurysm; Ao, ascending aorta; LA, left atrium; LCA, left coronary artery; LV, left ventricle; RCA, right coronary artery.

Figure 4

Postoperative images after excision of sinus of Valsalva aneurysm and replacement with a 23mm SJM mechanical valve in A5C view (A) and parasternal long-axis (PLAX) view (B).