Unruptured Sinus of Valsalva Aneurysms and Severe Aortic Valve Regurgitation Combined with Multiple Intracranial and Iliac Artery Aneurysms

Abbreviations

AVR, Aortic valve regurgitation

CTA, Computed tomography angiography

LVEF, Left ventricular ejection fraction

SVAs, Sinus of Valsalva aneurysms

INTRODUCTION

Sinus of Valsalva aneurysms (SVAs) combined with multiple aneurysms in other locations are extremely rare. Concomitant severe aortic valve regurgitation and unruptured SVAs needs surgical management. This case report summarizes successful surgical treatment with a modified Bentall procedure in a 69-year-old female patient who had unruptured left and right SVAs with severe aortic valve regurgitation (AVR). The left main coronary artery was widened and computed tomography angiography (CTA) demonstrated multiple intracranial and iliac artery aneurysms which need close observation and follow-up.

CASE

A 69-year-old female was admitted to the hospital due to intermittent chest tightness and dyspnea for over 4 years, with aggravation for more than 20 days, without chest pain or radiating pain. The patient’s medical history included hypertension, with the highest blood pressure of 180/90 mmHg, type 2 diabetes, old myocardial infarction, old cerebral infarction and subclinical hypothyroidism. No family history was reported. No relatives manifested similar vascular lesions. Physical examination revealed a heart rate of 74 beats per minute, regular rhythm, and a diastolic murmur at the right second intercostal space.

After admission, echocardiography showed huge left and right SVAs, a severe aortic regurgitation with a regurgitant jet width of 0.74 cm, PGmax: 17 mmHg, left ventricular ejection fraction (LVEF): 50%, and estimated pulmonary artery systolic pressure about 22 mmHg (Figure 1A).

Figure 1.

Preoperative echocardiography, coronary CTA, intracranial and neck CTA, and total aortic CTA. (A) Echocardiography showed left and right SVAs and a severe aortic regurgitation with a measured regurgitant jet width of 0.74 cm. (B) Coronary CTA demonstrated the left and right SVAs (white arrows) and widened LMCA (yellow arrow). (C) Preoperative intracranial and neck CTA demonstrated multiple aneurysms in the internal carotid artery, middle cerebral artery and vertebrobasilar artery (yellow arrows). (D) Preoperative total aortic CTA (MIP reconstruction) indicated multiple aneurysms in aneurysms in left and right internal iliac artery (yellow arrows). CTA, computed tomography angiography; LMCA, left main coronary artery; MIP, maximum intensity projection; SVAs, sinus of Valsalva aneurysms.

A total aortic and coronary CTA was performed. The lumen of left main coronary artery was widened, with the widest diameter measured 1.3 × 1.2 cm. The cross-section of the dilated sinus of Valsalva was approximately 5.7 cm (right to non coronary sinus top) × 4.6 cm (left to non coronary sinus top) × 6.7 cm (right to left sinus top) (Figure 1B). CTA of the intracranial and neck arteries indicated multiple dissecting aneurysms in the right internal carotid artery, middle cerebral artery and vertebrobasilar artery (Figure 1C). Besides, arterial aneurysms were observed in both internal iliac arteries (Figure 1D). CTA also showed that atherosclerotic plaque formation was observed in the abdominal aorta, bilateral iliac and cervical arteries.

According to management of unruptured intracranial aneurysms and abdominal aorto-iliac artery aneurysms, the multiple intracranial aneurysms carry a significant risk of rupture and need digital subtraction angiography or further interventional operation. The iliac artery aneurysms require follow-up observation.^1,2 In this case, we have taken the patient’s individual circumstances into full consideration. Firstly, it is difficult to determine the duration and recent progression of the multiple peripheral artery aneurysms. While the patient had no neurological or abdominal symptoms, the AVR and SVAs — which are responsible for the recent worsening of dyspnea — require prioritized treatment. Secondly, peripheral arterial surgery demands adequate cardiac function support, and it would be dangerous to perform such surgery before correcting the AVR. We also considered a combined single-stage procedure which was rejected because the Bentall procedure itself is lengthy, and combining it with an interventional surgery would further prolong operative time and increase surgical risk. After multidisciplinary discussion involving Neurology, Vascular Surgery, and Anesthesiology departments, a consensus was reached to prioritize treatment of the AVR and SVAs.

The patient underwent Bentall procedure and left atrial appendage closure under general anesthesia. During the operation, the diseased calcification valve leaflets were removed, and 17 double-headed needles with spacers were sutured along the aortic valve annulus and attached to a 23 mm Magna Ease Carpentier-Edwards pericardial aortic valve and a 28 mm woven double velour vascular graft (maquet). Continuous suturing was performed with 5-0 prolene to anastomose the openings of the left and right coronary arteries to the side of the artificial blood vessel. The left atrial appendage was ligated with double 10# sutures. The operation was successful. Postoperative treatment included diuresis, anticoagulation and infection prevention. At the 10th day after operation, echocardiology showed that the patient’s recovery was smooth, with an LVEF of 45%, unobstructed blood flow in the artificial blood vessel, and no significant regurgitation signal in the aortic valve (Figure 2A, 2B). Pathology of the aortic valve showed mucoid degeneration of valve tissue (Figure 2C). Two months after the operation, echocardiology showed a stable artificial aortic valve without regurgitation, intact vascular graft without collapse and LVEF: 52%. The patient declared no headache, no dizzy or other neurological related symptoms (Figure 2D).

Figure 2.

Postoperative examinations and the diagnostic flowchart. Postoperative echocardiology 10 days after surgery (A) and chest radiograph 1 month after surgery (B). Pathology of the aortic valve showed mucoid degeneration of valve tissue (C). Echocardiology 2 months after the operation (D). The process of dignosis in this case (E). COL3A1, collagen type III alpha 1 chain; CRP, C reactive protein; CTA, computed tomography angiography; FBN-1, fibrillin-1; NF1, neurofibromatosis type 1; TGFBR, transforming growth factor beta receptor.

DISCUSSION

SVAs is a rare cardiac condition which exact prevalence is difficult to determine. However, autopsy studies indicate that SVA occurs in less than 0.1% in general population. The co-occurrence of multiple aneurysms at other large vessels with SVA is even rarer. Acquired SVAs often involve the aortic valve disorder or aortic root lesion. Small and localized aneurysms can be surgically repaired with Dacron patches. If multiple coronary sinuses or the ascending aorta are involved, Bentall procedrue or valve-sparing aortic root replacement may be required. Postoperative complications may include residual shunt, new-onset or aggravated aortic regurgitation, arrhythmias, infective endocarditis, among others. According to statistics, the overall operative mortality for surgical repair is as high as 3.6%, while the 15-year survival rate is close to 90%.³

Sinus of Valsalva or ascending aortic aneurysms combined with multiple aneurysms at other large vessels is extremely rare. Such cases should be differentiated from several other diseases. Hereditary connective tissue disease, including Marfan syndrome, Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, and neurofibromatosis type 1, among others could present as multiple aneurysms. Genetic testing can provide evidence for hereditary connective tissue diseases.^4,5 However, genetic testing was not performed for the patient in this case due to practical limitations. Nevertheless, the patient exhibited normal physical development, absence of characteristic facial features, and no skin manifestations such as café-au-lait spots or neurofibromas. Her clinical presentation does not meet the diagnostic criteria for the aforementioned hereditary connective tissue disorders. Furthermore, differentiation from systemic vasculitis such as Behçet’s disease is also necessary. This patient did not have typical symptoms such as recurrent oral ulcers, genital ulcers, uveitis, and characteristic skin lesions. Considering the 4-year history of intermittent chest tightness and shortness of breath and history of hypertension and diabetes mellitus which were risk factors for atherosclerosis, severe atherosclerosis may be the cause of the disease in this case (Figure 2E).

The long-term management of multiple aneurysms should include the following components: firstly, a multidisciplinary team should lead the development of a personalized treatment plan with lifelong follow-up. Follow-up intervals should be determined based on risk stratification, with imaging examination performed at least every 6-12 months. Key warning signs for aneurysms include rapid growth, morphological changes and new symptoms. Daily risk factor control and pharmacological management include blood pressure management and oral medication selection such as beta-blockers or angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers, which reduce hemodynamic stress on the vessel wall. Besides, absolute smoking cessation, weight control and lipid management are also critical. Psychological support should be provided to help patients alleviating unnecessary anxiety.

In summary, this is an extremely rare case of severe aortic regurgitation and giant SVAs, combined with multiple intracranial and iliac artery aneurysms. The patient underwent bentall procedure and left atrial appendage closure. Pre-operative symptoms, including intermittent chest tightness and shortness of breath, disappeared after the operation.

LEARNING POINTS

• Early diagnosis and intervention: Surgical intervention should be prioritized once a SVA is diagnosed, particularly when accompanied by aortic regurgitation or rupture risk.

• Systemic evaluation: Cardiovascular pathologies with multisystem aneurysms necessitate comprehensive imaging examinations and exclusion of genetic disorders (e.g., Marfan syndrome).

• Individualized treatment: Surgical approaches (e.g., Bentall procedure, valve-sparing surgery) should be tailored to anatomical features.

DECLARATION OF CONFLICT OF INTEREST

All authors declare no conflict of interests.

ETHICS

This case report has been approved by Ethics Committee of Shandong Provincial Hospital affiliated to Shandong First Medical University. We have obtained written informed consent from patient, patient’s brother and her husband (all participants).

DATA AVAILABILITY

The data underlying this article are available in the article.

FUNDING

This work was supported by the grants from the Nature Science Foundation of Shandong Province (ZR2023 QH254; ZR2024QH317).