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REVIEW ARTICLE
Year : 2023 | Volume
: 24
| Issue : 3 | Page : 148-152
Surgical or percutaneous repair of sinus of valsalva rupture: Case series and literature review
Mohammad Paymard1, Mark Daniel Higgins2, Ajay Sinhal3, Muntaser D Musameh2
1 Department of Cardiology, The Canberra Hospital, Canberra, Australian Capital Territory, Mackay, Queensland, Australia
2 Depatment of Cardiology, Mackay Base Hospital, Mackay, Queensland, Australia
3 Department of Cardiology, Flinders Medical Centre, Adelaide, South Australia, Australia
Correspondence Address:
Dr. Mohammad Paymard
Department of Cardiology, Canberra Hospital, Yamba Drive, Garran, Canberra, ACT 2605
Australia
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/heartviews.heartviews_96_22
Abstract
The rupture of the sinus of the Valsalva aneurysm is a rare but very serious condition. Rapid and accurate diagnosis and prompt treatment are critical for these cases. We present two cases of sinus of Valsalva ruptures. One case was managed with open surgical repair and the second case was treated percutaneously. We have discussed these two therapeutic approaches available to treat sinus of Valsalva rupture.
Keywords: Rupture of sinus of Valsalva, sinus of Valsalva aneurysm, percutaneous repair
Introduction 
Rupture of the sinus of the Valsalva aneurysm (ROSOVA) is a rare condition that could be fatal if the diagnosis is delayed. The rupture usually occurs in the context of aneurysmal dilatation of the sinuses that has either congenital or acquired etiologies. Congenitally, structural deficiency of the aortic media leads to the lack of continuity between the aorta and the aortic annulus which in turn causes saccular outpouching formation.[1]
Connective tissue disorders such as Marfan's syndrome, Ehlers–Danlos syndrome, and Loeys–Dietz syndrome are also associated with sinus of Valsalva aneurysm (SOVA).[2] The most common acquired forms of SOVA include bacterial endocarditis, chest trauma, syphilis, and vasculitis.[3]
Patients may present with symptoms of acute heart failure, acute coronary syndrome, hemodynamic compromise, or even cardiac arrest.[4] Due to the rarity of the condition and nonspecific presenting symptoms, a fast and accurate clinical diagnosis could be challenging. A cardiac imaging modality is usually required to confirm the diagnosis. Conventionally, the management has been the surgical repair of the defect. However, percutaneous closure has also recently become available as an alternative therapeutic option. The latter option could be also suitable for patients presenting with the chronic form of ROSOV.
In this article, we present two cases of ROSOV. The first case presented with acute ROSOV and was managed by urgent surgical repair. The second case presented with chronic ROSOV and was managed by a percutaneous closure device (Amplatzer, Duct Occluder 1; St Jude Medical, Saint Paul, MN, USA)). We then discuss the clinical presentations, diagnosis, and management of ROSVO focusing on the safety and efficacy of percutaneous closure therapeutic option.
Case Reports Case 1
A previously well, 54-year-old obese man presented with sudden-onset dyspnea, central chest tightness, diaphoresis, and palpitation. His past medical history included hypertension. On examination in the emergency department, he appeared diaphoretic and in respiratory distress. The blood pressure was 139/67 mmHg, and the pulse rate was 106 bpm. The oxygen saturation was 90% on room air and the respiratory rate was 22/min. The Jugular venous pressure was elevated to the angle of the jaw and there were bibasal inspiratory crackles on auscultation. The cardiac auscultation revealed a Grade 4/6 continuous murmur over the left sternal border. The electrocardiogram demonstrated sinus tachycardia with 1 mm of ST-segment elevation in lead augmented vector right with widespread ST depression in precordial and limb leads. Serum troponin was elevated at 112 ng/L (normal <20 ng/L).
The patient was in acute pulmonary edema for which he received standard treatment. He then underwent an urgent coronary angiography which showed normal coronaries.
Subsequent urgent transthoracic echocardiography (TTE) followed by transesophageal echocardiography (TEE) demonstrated a ruptured sinus of Valsalva at the location of the right coronary cusp (RCC) that was associated with a shunt from the RCC into the right atrium and the right ventricle [Figure 1]a and [Figure 1]b. There was no evidence of valvular vegetation. The left ventricular systolic function was preserved. The computed tomography (CT) aortogram demonstrated normal size aortic root and there was no evidence of aortic dissection.
Figure 1: (a) The short axis view of the aortic valve of TEE. The right coronary cusp is perforated and is associated with a shunt from the aortic root into the right atrium and right ventricle. (b) The 2D image of aortic valve in short axis view demonstrating the defect in the right coronary cusp. RCC: Right coronary cusp, NCC: Noncoronary cusp, LCC: Left coronary cusp, RA: Right atrium, RV: Right ventricle, 2D: Two-dimensionalThere were no clinical features of connective tissue diseases. There was no history of chest trauma and the syphilis-screening test was negative.
The patient then underwent an urgent surgical repair. Intraoperative examination revealed a defect of 8 mm width in the RCC. The cusp appeared to be thin, but the aortic root looked normal in size and diameter. The postoperative course was uneventful, and the patient recovered very well.
Case 2
A 31-year-old man with known partial deletion of chromosome 12 was under routine follow-up for mild-to-moderate aortic regurgitation when he was found to have a continuous machinery murmur. Of note the patient was asymptomatic. Further investigation with a TTE revealed an aneurysmal RCC of the sinus of Valsalva with communication and shunting between the sinus and the right ventricular outflow tract (RVOT). The TEE and CT aortogram confirmed the diagnosis of ROSOV with the left to right flow into the RVOT adjacent to the pulmonary valve. There was evidence of mild aortic regurgitation. The left ventricle was mildly dilated associated with mildly reduced systolic function.
The case was reviewed at the heart team meeting of our Tertiary Centre. Considering that the patient was asymptomatic and hemodynamically stable, the heart team recommended an attempt to close the defect percutaneously and if failed to consider him for open surgical repair.
The procedure was performed under general anesthesia and guided by fluoroscopy and TEE. The right femoral artery and right femoral vein accesses were used. The defect was successfully closed with a 10 × 8 Amplatzer Duct Occluder 1 [Figure 2] and [Figure 3]. The intraoperative TEE and fluoroscopy showed a remarkable reduction in the left-to-right shunting flow. The patient recovered well and was discharged home 2 days later. The patient reported feeling more energetic and active on follow-up 6 months following after the procedure.
Figure 2: (a) The short axis view of the aortic valve demonstrating that the Amplatzer device is deployed in the RCC. (b) The Amplatzer device is seen in the RCC from the long-axis aortic root long intraoperatively. RCC: Right coronary cusp
Figure 3: This right anterior oblique view of the heart demonstrates that the Amplatzer occluder device is deployed in the right coronary cusp of sinus of Valsalva Discussion SOVA is a rare cardiac accounting for 0.1%–3.5% of all congenital heart defects and 0.14% of all cardiac operations.[5] The exact prevalence is unclear but based on an autopsy study, the prevalence has been estimated to be 9/10,000 in the general population.[6]
This abnormality results from the discontinuity between the aortic media and the aortic annulus, facilitating the formation of a fibrous, aneurysmal sac.[1] They could be congenital or acquired in etiology.
Moustafa et al. reported that patients with bicuspid aortic valves are more likely to develop SOVA.[7] They also reported the association between ventricular septal defect (31%) and aortic regurgitation in patients with SOVA.[7]
In the general population, the condition predominantly affects the male gender (4:1) and also has been reported to have a higher incidence among the Asian population.[8]
Most commonly, ROSOVA originates from the RCC (65%–85%) followed by noncoronary cusp (10%–30%) and left coronary cusp (<5%).[5],[9],[10] Due to the proximity of the RCC and noncoronary cusp, the right ventricle is the receiver chamber following the rupture in most cases.[5]
According to the literature, the majority of patients who suffered ROSOVA are aged between 20 and 40 years.[5],[10]
Patients with unruptured SOVA are usually asymptomatic and they are detected incidentally.[4] Presenting symptoms of ROSOVA vary and are determined by the rapidity of the rupture, the size of the orifice created by the rupture, and the chamber into which the shunt is directed.
Commonly, symptoms follow physical stress. The most common presenting is acute onset dyspnea followed by fatigue, chest pain, palpitation, and sudden cardiac death.[4],[7],[11] It sometimes could be challenging to promptly diagnose the condition owing to diverse presenting symptoms. Clinically following ROSVOA, in the majority of cases there is a loud continuous murmur that is best heard along the left sternal border.[9],[12] The electrocardiogram may show sinus tachycardia, voltage criteria for left ventricular hypertrophy, and ST-T segment deviations.[13]
Historically, cardiac catheterization used to be the diagnostic tool of ROVOSA-via visualizing the shut during aortography-before advancement in noninvasive cardiac imaging.[14]
In modern medicine, however, noninvasive imaging modalities play a pivotal role in the diagnosis of ROVOSA.
Once the clinical suspicion is high, an urgent TTE is warranted. In most cases, a TEE is also required to confirm the diagnosis. TTE is a highly useful imaging modality that examines the sinuses of Valsalva and also could exclude other associated or precipitated cardiac anomalies.[15] In addition, TEE provides the anatomy preoperatively which would be indispensable, particularly in assessing cases with distorted anatomy due to underlying pathology.
Cardiac CT and cardiac magnetic resonance (CMR) are two excellent diagnostic methods that could be used to confirm the condition when TTE and TEE findings are inconclusive. Cardiac CT and CMR are also very useful for surgical planning by providing high-resolution 3-dimensional cardiac images.[4]
Urgent surgical repair is the standard approach when a case of ROSOVA is encountered. In 1957, the first successful surgical repair of ROSOVA was performed.[16] Since then, different surgical approaches have been developed. In general, surgical correction of ROSOVA is a highly successful procedure with a low mortality rate of 1.9%–3.6%.[3],[4] Patients who undergo successful surgical repair could have a life expectancy near the aged/sex match their healthy counterparts in the general population with a 10-year survival rate of 90%–95%.[10],[17]
An attractive alternative to the open surgical intervention is transcatheter closure (TCC) which was first successfully performed in 1994.[18] Recent studies have demonstrated the mid-term and long-term safety and efficacy of TCC.[19],[20],[21]
TCC is considered to be a highly successful procedure when performed by experienced operators and patients are well selected. This has been demonstrated by studies published by Zhao et al. and Kerkar et al. where the success rates were 100% (10 out of 10 cases) and 91% (20 out of 22 cases), respectively.[19],[22] In terms of complications of TCC, in the cohort reported by Zhong et al., 2 cases out of 22 required emergent surgical aortic valve replacement due to TCC-related severe aortic regurgitation. On both occasions, the aortic opening size was equal to or >9 mm. The same investigators reported only a small residual shunt in two out of twenty cases of successfully performed TCC in 6 months of follow-up.[21]
The defect can be occluded either anterogradely or retrogradely.[19],[23] Amplatz Duct Occluder (ADO; St Jude Medical, Saint Paul, MN, USA) has been predominantly used to close the defect percutaneously. In case 2, we successfully deployed 10 × 8 Amplatzer Duct Occluder 1 with a very good result.
The appropriate patient selection is critical before contemplating the TCC of ROSOV. Zhong et al. reported that in their experience, using the TCC technique defects with >9 mm diameters could be repaired with a very high success rate. In their cohort, 19/22 patients with a defect diameter of 9 mm were successfully treated with the TCC technique. The failure rate increases when the diameter is >9 mm.[21]
The distance between the rupture site and from coronary artery ostium is also another factor that can affect the patient selection for TCC. The ADO that is selected to occlude the defect is 2–5 mm in diameter. Therefore, to avoid the coronary ostium being covered by ADO when deployed, the distance of the coronary ostium from the defect should be more than 5 mm.[21] Currently, there are no guidelines to recommend how to select the size and type of the occluding device.
Conclusion ROSOV is a rare but potentially treatable condition. The presenting symptoms for acute ruptures are usually similar to those of acute coronary syndrome or acute pulmonary edema. Once it is diagnosed, the standard treatment is open surgical repair. However, TCC is another effective and safe therapeutic option in properly selected patients particularly with chronic forms of ROSOV.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References 
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