This systematic review was conducted according to the latest Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines (10). On July 12, 2022, we performed a broad systematic search in the Scopus, PubMed, and Embase electronic databases. The main search terms were “Sinus of Valsalva”, “Rupture/Aneurysm”, and “Transcatheter/Catheter/Device”. The search syntax of each database is reported in Supplementary Table S1. Our search was limited to articles in the English language. All clinical studies, composed of original articles, case series, case reports, and letters to the editor, were retrieved. Additionally, references were searched for further related studies.

The investigations selected were clinical studies on pediatric or adult patients with RSOV or SOVA and case reports or case series with TCC as the treatment strategy.

The investigations excluded were animal studies, review articles, commentaries, editorials, and conference articles. Also excluded were studies with overlapping patients. (Newer investigations with more patients were included in this case, and previous studies were excluded.) Additionally, studies where the intervention attempt was unsuccessful in the cath lab resulting in referral for surgery and investigations where RSOV was caused by previous interventions or endocarditis were excluded.

The search results were screened based on the relevance of their abstracts and titles. The full text of the selected studies was obtained. Two independent authors (AA and NT) further assessed the included studies based on the inclusion criteria. The reviewers consulted a senior author (AH) to achieve a consensus in the event of differing opinions. The studies that seemed to report overlapping cases were excluded. The selected articles are sorted in Table 1 based on study type and year. The authors identified 15 main variables from the final articles: the name of the author, the year of publication, the type of study, the location of the study, the number of studied patients, the study population's mean age, the studied patients' gender, defect sizes and defect sites, occluder device types and sizes, follow-up durations, complications, accompanying lesions, and success rates.

A report of the search results based on the PRISMA guideline is presented in Figure 1. Our search in the 3 databases yielded 437, 273, and 307 articles from PubMed, Scopus, and Embase, respectively. The removal of duplicates left 668 articles. Another 530 studies were excluded based on our title and abstract relevance criterion. Afterward, the full texts of the remaining 138 articles were obtained, and their reference lists were searched for further related studies, leading to the addition of 2 new articles. Despite multiple attempts to contact both the author and the journal, obtaining the full text of 1 article proved impossible. Then, full-text reviews of the selected articles excluded another 46 investigations based on our inclusion criteria (Supplementary Table S2).

Ultimately, the current review was conducted on 94 articles: 23 case series and 71 case reports. The included articles are summarized in Table 1, with the case report combined in the last row. A complete list of the included case reports is available in (Supplementary Table S3).

Patients with unruptured SOVA are often symptom-free, whereas a rupture into a heart chamber can significantly change hemodynamics and create severe symptoms (15). Dyspnea, palpitations, chest pain, fatigue, and peripheral edema are the most frequently reported symptoms after SOVA rupture. The onset of these symptoms can be acute or gradual (3, 101).

The pressure difference between the aorta and the low-pressure heart chamber causes continuous machinery murmurs, frequently heard in patients with RSOV. The murmurs become more intense when a fistula grows more prominent (102).

Since the first reported case of the TCC of RSOV in 1994, mounting evidence (mostly case reports and a small number of case series) has indicated the effectiveness of TCC as a potential alternative to surgical intervention. TEE proves its effectiveness during the intervention by providing live visualization of cardiac structures, especially the aortic valve. Multiple imaging modalities have made percutaneous approaches a more feasible treatment option than open-heart surgery. Still, surgical intervention is unavoidable in RSOV cases with accompanying heart defects, infections, arrhythmias, or outflow tract obstruction. Moreover, prompt surgery might become necessary when TCC results in major complications, such as significant residual shunts, aortic insufficiency, and device embolization.

Precise patient selection criteria are critical before TCC. Xiao et al. (15) considered patients to have TCC if they have a body weight exceeding 10 kg, if the non-coronary sinus or the right coronary sinus is the origin of the defect rupturing into the right ventricle or the right atrium, if the defect size is less than 10 mm, if RSOV does not affect the aortic valve and has more than a 7 mm distance from the annulus of the aortic valve, if a gap of more than 5 mm exists between the ostium of the right coronary sinus and the ruptured site, and if surgery is needed in the absence of other cardiac anomalies.

Lui et al. (22) also suggested that RSOV patients with a European System for Cardiac Operative Risk Evaluation II (EuroSCORE II) score exceeding 20% would benefit from TCC. Nevertheless, controversy remains over the indications for TCC in patients with RSOV since this method is new and the prevalence of RSOV is low. Further studies are, therefore, required to validate the indications or contraindications of TCC in patients with RSOV (11).

Although there are no stringent guidelines on RSOV imaging modalities, recent studies have proposed a multimodality imaging approach to RSOV (103, 104).

Transthoracic echocardiography (TTE) is the first-line modality for diagnosing RSOV (104). A primary TTE examination can detect aortic root dilation at the level of the Valsalva sinus (7) and assess the RSOV dimension.

Color Doppler echocardiography can visualize the blood flow through the RSOV fistula from the aorta into a heart chamber (105).

TEE provides higher-resolution images due to its proximity to the heart and the thoracic aorta. During corrective interventions, TEE can visualize RSOV (106). Further, the relative accuracy of TEE in defect sizing has reduced the need for balloon occlusive diameter methods during TCC (107).

Multidetector computed tomography and cardiac magnetic resonance imaging (CMR) can assess the aorta fully by forming a 3D reconstruction. Multidetector computed tomography or CMR are also capable of determining the dimensions, morphology, and complications of RSOV.

Electrocardiogram-gated CT or CMR can augment imaging accuracy by controlling motion artifacts (108, 109).

Invasive transcatheter angiography is occasionally necessary to differentiate RSOV from other coronary disorders (7).

The first case of the TCC of RSOV was performed utilizing a Rashkind Umbrella via an arterial approach (110). However, the transvenous approach has been performed more frequently because of its higher maneuverability and easier access to the Valsalva sinus. Recent decades have seen the use of other devices, including Amplatzer ductal occluders, PDA occluders, muscular ventricular septal defect occluders, septal occluders, and coils, depending on the anatomy of the defect.

Amplatzer-type ductal occluder devices have been in frequent use due to the conformity of their shape with RSOV morphology (20). Some studies have reported a preference for PDA occluder use. Other than the characteristics of the defect, the interventionist's familiarity with the device, the availability of the device, and financial concerns are taken into consideration in device selection (111).

TCC involves creating an arteriovenous loop by passing a Judkins right coronary catheter from the ascending aorta through the defect into the right atrium or the right ventricle with the aid of an angled-tip guidewire (25, 110). The guidewire is, then, exchanged for a long guidewire, and a gooseneck snare is introduced through venous access to snare the long guidewire in the right atrium or the pulmonary artery with a view to avoiding entrapment and damage to the chordae tendineae of the tricuspid valve, which could result in postprocedural tricuspid regurgitation. Subsequently, a long wire is exteriorized from venous access to form a stable arteriovenous loop crossing the RSOV. Thereafter, a delivery sheath is introduced through the femoral vein and is negotiated from the RSOV to the ascending aorta while precaution is taken to avoid damage to the surrounding tissues, causing heart blocks or defect dilation (111). In the next step, an appropriate-sized device is selected and connected to a delivery cable. The device is inserted through this sheath, and the aortic disk is opened in the aorta. Next, the delivery sheath and the delivery cable are pulled back so that the disk closes the aortic side of the RSOV. After the confirmation of the appropriate placement, the other disk is deployed in the associated right chamber (110, 112).

It is essential to assess all the steps of the procedure with intraprocedural TEE for the confirmation of the complete blockade of the shunt. Possible residual shunting, aortic regurgitation, tricuspid regurgitation, and other possible procedural complications should also be assessed following complete device emplacement.

Although shown to be effective, the TCC of RSOV can be associated with severe complications on rare occasions. Possible complications can be prevented or readily addressed through efficient measures. A summary of reported complications is presented in Table 4.

The new onset or progression of an existing aortic regurgitation is the most critical complication in the TCC of RSOV. Aortic insufficiency can be due to traction on the aortic valve annulus or an aortic valve injury during the procedure. In cases of severe aortic insufficiency, surgical intervention is unavoidable.

Compression of the coronary arteries after device insertion can also be a life-threatening complication, which can be avoided by visualizing coronary artery flow during the intervention via angiography. A new-onset ST depression after device implantation can also be due to coronary artery compression. If left undetected, the condition could cause myocardial infarction (109). Embolization of the occluder device can also be a possible complication. Glaceza et al. (109) reported the embolization of undersized devices in 3 cases; they were, however, able to retrieve the migrated devices percutaneously.

Hemolysis is also a rare complication of TCC caused by the inserted device (46).

In rare situations, disturbances in the conduction pathways might result in arrhythmias, including atrioventricular or bundle branch blocks. These blocks are caused by possible traction or compressions on conductive pathways. Later inflammations could also be a cause of these arrhythmias (112).

As with any percutaneous intervention, complications associated with catheterization sites, including atrioventricular fistulae, hematoma, and bleeding events, are also possible.

The lack of large-scale studies and clinical trials was the principal limitation of the present study.