Mitral Regurgitation Caused by Concomitant Pseudoaneurysms of the Sinus of Valsalva and Ascending Aorta

Introduction

Sinus of Valsalva (SOV) pseudoaneurysms are rare vascular anomalies that can arise from congenital defects, infection, trauma, or iatrogenic causes [1,2]. Their clinical presentation ranges from incidental detection to life-threatening complications such as rupture, fistula formation, or severe valvular dysfunction [2,3]. While isolated pseudoaneurysms involving a single sinus are already uncommon, reported in less than 0.1% of the general population and approximately 0.14% of surgical cases [4], the occurrence of multiple SOV pseudoaneurysms is even rarer. Simultaneous involvement of multiple SOV pseudoaneurysms with an ascending aortic pseudoaneurysm is exceedingly rare, and to our knowledge no case series have quantified its incidence; only isolated case reports exist [5,6].

Case Report

A 35-year-old man was referred to our institution with progressive shortness of breath over the past 3 months. Before his presentation, he had been treated for a febrile illness at a rural healthcare center in his home region of Africa, shortly before traveling for Hajj pilgrimage. The fever resolved with supportive treatment, and there was no documented bacteremia at that time. Upon admission to our center, the infectious disease workup was repeated, including multiple sets of blood cultures, all of which were negative. Routine laboratory tests revealed no leukocytosis, elevated inflammatory markers, or other abnormalities. However, given the patient’s history of prior antibiotic treatment, these negative findings were interpreted with caution; they ruled out active uncontrolled bacteremia but did not definitively exclude a diagnosis of healed or culture-negative infective endocarditis.

Upon admission, the patient was hemodynamically stable and was managed with standard heart failure optimization protocols, including diuretics and afterload reduction. Echocardiography revealed severe mitral valve regurgitation, with a pulsatile sac measuring 3.9×2.5 cm abutting the mitral valve annulus (Figure 1A). Moreover, the aortic root contained another sac that narrowed the left ventricular outflow tract and invaded the interventricular septum without compromising aortic valve competence (Figure 1B). A computed tomography (CT) scan confirmed the presence of a pseudoaneurysm of the left (6.3×7.0 cm) and right (3.9×2.4 cm) sinuses of Valsalva, as well as an additional pseudoaneurysm of the ascending aorta (2.5 cm diameter) that mimicked an aortic dissection flap (Figure 2A, 2B).

The patient was promptly operated on with cardiopulmonary bypass and moderate hypothermia. Both antegrade and retrograde cold-blood cardioplegia were used to arrest the heart. The mitral valve was approached via a left atriotomy. Mitral valve analysis revealed no obvious structural lesions. The leaflets and subvalvular apparatus were grossly normal. The mitral valve regurgitation was attributed to dynamic annular distortion created by the left SOV pseudoaneurysm. A 30-mm Carpentier-Edwards Physio II ring (Edwards Lifesciences, Irvine, USA) was placed. The valve geometry was restored, and valve competence was achieved, as confirmed by the saline test and postoperative transesophageal echocardiography.

The openings of the right and left SOV pseudoaneurysms were immediately beneath the corresponding coronary ostiums. The right SOV pseudoaneurysm was located within the interventricular septum, and an intramural thrombus was removed. The left SOV pseudoaneurysm was very large and invaded the basal to mid-segment of the inferolateral left ventricular wall, with a membrane-like component projecting into the left ventricular cavity and an exterior portion harboring the circumflex coronary artery (Figure 2C).

The ostia of both the right and left sinuses were closed using bovine pericardial patches with a running 5-0 Prolene suture. Furthermore, the body of the large left SOV pseudoaneurysm was externally plicated along the left ventricular lateral wall. This maneuver was performed under meticulous direct vision to obliterate the cavity and relieve the mass effect. Crucially, the suture line was placed strictly along the aneurysm wall, keeping the course of the circumflex coronary artery external to the repair to ensure its patency and prevent distortion.

The aortic valve leaflets were appropriately coapted. The ascending aortic pseudoaneurysm was excised. Intraoperative assessment of the aortic wall at the resection margins demonstrated pliable, robust tissue lacking the fragility associated with active aortitis. This tissue quality permitted a tension-free, direct end-to-end reconstruction without the need for a prosthetic graft. Pathological specimens from the ascending aorta showed nonspecific inflammatory changes. Histopathological and biochemical testing were not remarkable for syphilis or any other specific infectious etiology. The patient had a good recovery after surgery. Predischarge echocardiography confirmed the integrity of the aortic root and SOV repairs, showing a well-seated mitral annuloplasty ring with only trace residual mitral regurgitation. He was discharged on postoperative day 10 on a regimen of anti-failure and antiplatelet therapy. The operating surgeon remains in direct contact with the patient, who is currently in very good clinical condition several years after surgery.

Discussion

Sinus of Valsalva (SOV) pseudoaneurysms are extremely rare and can result from either a congenital or acquired etiology. Endocarditis, syphilis, arteriosclerosis, connective tissue diseases, and trauma have all been associated with SOV pseudoaneurysms [1,2]. In our patient, while the definitive etiology remains unproven due to a negative microbiological workup, the clinical picture characterized by a prior febrile illness followed by the development of multiple pseudoaneurysms raises the strong suspicion of healed or culture-negative infective endocarditis [2]. While systemic conditions such as vasculitis (eg, Behçet’s disease) and syphilis were considered, the absence of specific clinical stigmata and negative serological workup made these unlikely [5]. However, alternative mechanisms such as unrecognized congenital weakness or sub-clinical inflammatory aortitis cannot be definitively excluded. The “nonspecific inflammatory changes” observed on pathology further support a resolved infectious or inflammatory process.

We believe that the mitral valve regurgitation in our patient resulted from the mass effect caused by the left SOV pseudoaneurysm. Depending on the location and size of SOV pseudoaneurysms, various clinical scenarios can arise [5]. Previous case reports have documented pseudoaneurysms rupturing into neighboring cardiac chambers, coronary arteries, and even the inferior vena cava [3,4]. In our case, the right SOV pseudoaneurysm invaded the interventricular septum. The large left SOV pseudoaneurysm likely interfered with mitral valve competency due to its mass effect on the mitral valve annulus. Additionally, the circumflex coronary artery was displaced off the left ventricular wall into the roof of the left SOV pseudoaneurysm (Figure 2C).

In contemporary practice, multimodality imaging (TTE/TEE, CT angiography, and, when available, CMR with 3-D reconstructions) is central to therapy selection in SOV pseudoaneurysms, defining the neck/orifice, proximity to coronary ostia and aortic cusps, thrombus burden, and interaction with the LVOT or mitral apparatus [1,3,5–8]. In our case, transthoracic echocardiography clarified the mechanism of severe mitral regurgitation (annular distortion without intrinsic leaflet disease), while CT delineated multi-sinus and ascending aortic involvement and the circumflex artery’s relationship to the left SOV sac, collectively guiding a surgical rather than endovascular approach.

Management strategies depend heavily on the anatomical complexity and clinical presentation (Figure 3). Surgical intervention is the definitive treatment for complex cases like this one, where conservative management is not feasible due to symptoms, mass effect, or multi-component involvement [3,5,8]. While endovascular closure is feasible in selected anatomies – classically, when a discrete neck is remote from the coronary ostia and tissue quality permits device stability [6,8] – it was not suitable for this patient. The involvement of multiple sinuses, the proximity of the orifices to the coronary ostia, and the need to surgically relieve the mass effect on the mitral annulus mandated an open approach. Although uncommon, conservative management with medical optimization and structured imaging surveillance may be appropriate in carefully selected patients (eg, small, stable, or minimally symptomatic lesions or prohibitive surgical risk); in a contemporary cohort, nonsurgical patients had no late complications attributable to the SVA during follow-up [7]. Finally, it is important to emphasize that the mechanism of mitral regurgitation described here is highly specific to this unique anatomical configuration and should not be generalized to all patients with aortic root pathology.

Conclusions

This case highlights an exceptionally rare constellation of multiple sinus of Valsalva pseudoaneurysms occurring simultaneously with an ascending aortic pseudoaneurysm. The unique anatomical relationship of the left sinus pseudoaneurysm with the mitral annulus produced severe regurgitation through annular distortion rather than intrinsic leaflet disease. This underscores the importance of diagnostic vigilance; careful preoperative imaging review is essential to identify such secondary mechanisms, as they fundamentally alter the surgical strategy. Prompt surgical repair, including patch closure of the sinus orifices, excision of the ascending aortic pseudoaneurysm, and mitral annuloplasty, successfully corrected the anatomical defects and restored hemodynamic function. Recognition of such unusual mass effects is critical, as timely intervention is required to prevent irreversible heart failure.