Introduction Sinus of Valsalva aneurysms SVA is a rare cardiovascular malformation that was first described in detail by Antonio Maria Valsalva, a leading Italian anatomist and pathologist. The dilatation of the aortic sinus at the junction of the aortic valve and sinus duct was defined as an aortic sinus aneurysm, and a case of ruptured aortic sinus aneurysm was first reported by Hope in 1839. A year later, Thurnam described multiple cases of unruptured aortic sinus aneurysms. Aortic sinus aneurysms can be congenital or acquired; congenital aortic sinus aneurysms are more common and often result from weak development of the middle layer of the aortic wall at the fibrous tissue junction with the annulus; degenerative changes in the elastic fibers of the aortic wall secondary to atherosclerosis or infection are the main cause of acquired aortic sinus aneurysms. Sinus aneurysms most often originate from the right coronary sinus of the aorta or from the noncoronary sinus, and less frequently involve the left coronary sinus. The anatomic location of the sinus aneurysm determines the chambers into which it may break, and the literature has reported that sinus aneurysms can break into any cardiac chamber, including extracardiac structures. Embryology: Edwards and Burchell describe the structural defect of SVA as a continuous absence of the middle layer of the aortic wall and the aortic valve annulus, followed by progressive development of intimal avulsion and sinus aneurysm formation. Congenital SVA often occurs locally in a sinus of the aorta, which is weakly developed and subjected to high pressure from the aortic root, first forming a small asymptomatic sac. Other congenital malformations associated with aortic sinus aneurysms include ventricular septal defect (30%-60%), aortic diastasis (10%), aortic regurgitation, pulmonary stenosis, aortic constriction, atrial septal defect, and subaortic aneurysmal dilatation; it has been proposed that the association of anaplastic and right coronary sinus aneurysms with ventricular septal defect stems from incomplete fusion of the right and left distal bulbous septum during fetal life. Epidemiology: SVA is clinically rare, accounting for 0.1% to 3.5% of all congenital heart disease and 0.14% of all open-heart procedures; patients with SVA may have no clinical symptoms, so the exact incidence is not known, but an autopsy study of 8,138 cases suggested a prevalence of 0.09%; patients with SVA usually have no clear family history, but Johnson also reported that both brothers had The incidence of SVA is significantly higher in males than females (4:1), with a higher incidence in Asian populations, and the incidence of SVA with septal defects is higher in Eastern populations and is often combined with intracrural septal defects, whereas in Western populations it is more often combined with perimembranous septal defects. Acquired SVAs are less common than congenital SVAs and occur secondary to injury to the aortic wall, including infection (syphilis, bacterial or fungal endocarditis, or tuberculosis); degenerative disease (atherosclerosis, connective tissue disorders, and capsular endocardial apoptosis); or chest trauma. Acquired SVA often involves multiple coronary sinuses. However, some congenital factors including inherited connective tissue anomalies such as Ehlers-Danlos syndrome or Marfan syndrome can also lead to widespread diffuse dilatation of the aortic sinuses rather than limited aneurysmal protrusion. Sinus aneurysm formation due to aortitis and leukoaraiosis has also been described in the literature. Much rarer is the medical origin of SVA, including after aortic valve replacement and after resection of extensive calcification of the aortic valve. Unlike congenital SVAs, acquired SVAs often have a number of features: the aneurysm is often extracardiac and can involve any aortic sinus and continue upward; they are often associated with acquired cardiac infections and rarely with congenital malformations of the heart; left coronary sinus aneurysms are the least common but are often acquired, especially when the left coronary sinus breaks into the left ventricle. One explanation for the rare involvement of the left coronary sinus in congenital SVAs is that the left coronary artery is supported by the left coronary sinus wall, which travels within the myocardial septum soon after emanation. Unruptured SVA Unruptured SVAs are often asymptomatic and not easily detected. However, with the development of echocardiography and other noninvasive imaging techniques, more unruptured SVAs are being detected. Rarely, a continuous murmur may be heard in the chest prior to SVA rupture, which is caused by turbulent flow in and out of the SVA pocket. Patients with unruptured SVAs may also have exertional dyspnea, palpitations, and angina-like chest pain, although this may not be related to the sinus tumor. Several cases have reported significant anatomic and physiologic disturbances due to unruptured SVA; severe arrhythmias including ventricular tachycardia and atrial fibrillation, as well as complete AV block due to compression of the AV node or conduction bundle by a sinus tumor projecting into the ventricular septum. Myocardial ischemia due to obstruction of coronary flow by SVA is rare, and Chipps first reported a case of a 35-year-old man with syphilitic isolated aortic left coronary sinus aneurysm compressing the left main trunk causing infarction. A large sinus tumor is a good site for thrombosis and thrombus dislodgement leads to ectopic embolism. Sinus aneurysm rupture Most sinus aneurysm ruptures occur after puberty between the ages of 20 and 40 years, with the average time to undergo surgery being 35 to 39 years. Very few cases are in infants, children and elderly patients. The physiologic consequences of rupture depend on the speed at which it occurs, the size of the rupture, and the heart cavity into which it ruptures. The opposite clinical presentation often leads us to a dilemma in the diagnosis of sinus tumor rupture. Two modalities of SVA rupture lead to two clinical patterns: acute rupture of a giant sinus tumor and slow penetration of a smaller sinus tumor. Acute, massive ruptures result in severe clinical syndromes: severe subxiphoid pain, epigastric pain, and severe dyspnea. Blunt chest injuries and cardiac catheterization have also been reported in the literature to be predisposing factors for sinus tumor rupture. Progressively increasing dyspnea, fatigue, terminal breathing, and nocturnal paroxysmal dyspnea are also common, as the heart is unable to adapt to the sudden increase in volume load in a very short period of time. Regardless of the location of the sinus tumor rupture, resulting in a left-to-right or left-to-left shunt, overload of the left heart inevitably occurs. Acute symptoms of heart failure may last for hours or days and then improve or worsen. Smaller penetrations of sinus tumors may be more insidious. Patients may remain asymptomatic for months or years until the onset of congestive heart failure. The causes of sudden death due to ruptured sinus tumors are pericardial compression, myocardial ischemia, conduction block, or malignant arrhythmias. Rupture of a sinus tumor into the pericardial cavity (very rare, 2%, without coronary sinus rupture) almost inevitably leads to fatal pericardial compression. Rupture of the sinus tumor leading to compression of the left main trunk opening causing myocardial ischemia or malignant arrhythmias can also lead to sudden death. In a review of 26 patients with ruptured sinus tumors penetrating the base of the septum, more than 50% developed congestive heart failure and complete AV block. Hope described the murmur as “a very low, superficial, saw-like continuous murmur overlying the first and second heart sounds”. The typical murmur seen in 45% to 95% of patients is a low, machine-like continuous murmur that differs in systolic and diastolic intensity and is most likely to be heard at the base of the heart. Unlike the murmur in patent ductus arteriosus, which peaks at the second heart sound, the murmur may diminish in intensity at the second heart sound and increase again in diastole, producing a reciprocal rhythm. The electrocardiogram suggests leftward deviation of the electrical axis and abnormalities of the ST segment and T wave. Chest radiographs may reveal increased pulmonary blood or pulmonary stasis and enlargement of the atria of the heart. In the early years, aortic sinus aneurysm rupture was diagnosed by cardiac catheterization and imaging. 35 cases of aortic sinus aneurysm rupture diagnosed by imaging were reported by De Bakey et al. in 1967. On imaging, the ruptured aortic sinus aneurysm protruded into the draining heart cavity like a finger or windbag, and the draining heart cavity was visualized with contrast. Aortic angiography may also show aortic regurgitation. Right heart catheterization can indicate pulmonary artery pressure and resistance and provide quantitative analysis of left-to-right shunt flow. Rothbaum et al. first reported the echocardiographic manifestations of SVA rupture in 1974, and since then two-dimensional and Doppler echocardiography have been widely used in the diagnosis and evaluation of SVA. Two-dimensional echocardiography combined with color Doppler and spectral techniques can accurately evaluate ruptured aortic sinus aneurysms and other coexisting malformations. Multi-sectional imaging can evaluate the location and size of sinus aneurysm pockets. Parasternal long-axis and short-axis views (at the level of the aortic root) are considered to be the best locations to show the aortic sinus. In a ruptured SVA, color flow imaging combined with pulsed and continuous Doppler techniques can show systolic-diastolic continuous turbulent flow through the rupture into the draining heart chambers. Careful visualization of the left ventricular outflow tract and aortic root in short-axis views can help to differentiate septal defects combined with membranous aneurysms from SVAs. Transesophageal echocardiography (TEE) is highly sensitive in diagnosing and localizing SVA ruptures. TEE can provide further information in differentiating SVA from ventricular septal defects combined with membranous aneurysms. However, it is not superior to transthoracic echocardiography and aortic root angiography in evaluating the extent of aortic regurgitation and left-to-right shunts. Enhanced CT scan of the aorta is not widely used in the diagnosis of aortic sinus aneurysms. However, MRI is useful in some difficult cases. MRI, including MRI movies, provides a more accurate three-dimensional anatomy of the SVA and can show variations in the direction of flow during the cardiac cycle. In contrast to aortic root angiography, MRI can show the thickness of the aortic wall, detect the presence of thrombus, and is more sensitive to smaller penetrations. Transthoracic Doppler echocardiography should be the first-line noninvasive method for the diagnosis of ruptured or unruptured SVA. We recommend TEE as a second step complementary method when the diagnosis is in doubt, especially if the tumor capsule is small or combined with other congenital malformations. Cardiac catheterization and angiography can confirm the diagnosis of an SVA while evaluating the hemodynamic changes and combined malformations after rupture and, more importantly, can simultaneously show the coronary artery anatomy. If multiple congenital malformations are combined, or if treatment requires more detailed anatomical details, we recommend MRA or CTA as a useful addition. Treatment Surgery Untreated ruptured aortic sinus aneurysms heal poorly, and traditional surgical repair is the primary treatment. The number of procedures, including unruptured SVA repair, has increased in recent years as the diagnosis has improved. morrow and Bigelow and Barnes independently reported the first aortic sinus aneurysm repair under hypothermic perfusion in the mid-1950s. lillehei et al. performed the first aortic sinus aneurysm repair under conventional extracorporeal circulation, which has since been widely used. Depending on the location and size of the aneurysm, the location of the rupture, the cavity into which the aneurysm has broken, and the combined malformation, direct suturing, patching, and aortic root replacement may be used. In the early years, the repair route was through the sinus aneurysm, whereas in the modern surgical era, the majority of cardiac centers expose both chambers, and both the aortic root and the involved chambers are incised, facilitating finer suturing of the breach, better visualization of the combined malformations, and avoidance of aortic valve coiling and late aortic valve insufficiency. Several studies have suggested that direct suturing of the breach may have a higher chance of sinus aneurysm recurrence. Regardless of the size of the aneurysm or breach, many cardiac centers use patches to repair sinus aneurysms to avoid excessive tension on the aortic annulus. Surgical treatment has a high rate of surgical success and low surgical risk. The expected survival rate of patients undergoing surgical treatment for ruptured aortic sinus aneurysms is close to that of the normal population (10-year survival rate 90 to 95 percent). In the largest series involving 129 patients, the perioperative mortality rate was 3.9%, and most of the deaths were associated with preoperative sepsis or infective endocarditis. In complex cases, aortic root replacement or ROSS surgery may be required. Late complications include prosthetic valve dysfunction, infective endocarditis of the prosthetic valve, sinus aneurysm recurrence, and bleeding due to anticoagulation. Aortic regurgitation usually complicates the pathophysiology and clinical presentation of patients with SVA. the incidence of aortic regurgitation in patients with SVA is approximately 30%, whereas the chance of valve replacement is around 50%, with moderate-to-large regurgitation, aortic valve contracture and thickening, and bileaflet aortic valve, then aortic valve replacement may be necessary. Aortic valve insufficiency alone heals well, but aortic valve insufficiency combined with ventricular septal defect and SVA heals poorly, and early aortic valve insufficiency at discharge is a predictor of long-term aortic regurgitation. Transcatheter aortic sinus aneurysm occlusion With the rapid development of cardiovascular imaging techniques and occlusion devices, transcatheter intracardiac defect occlusion has become an alternative to surgical treatment. 1994 saw the first report by Cullen on the use of the Rashkind atrial septal defect occluder for the interventional treatment of congenital aortic sinus aneurysm rupture. Eight cases were reported by Ramesh A in 2004, and 10 cases were reported by Prof. Zhao Shihua of Fu Wai Hospital in Beijing in 2008. In the early years, the occlusion devices used included Rashkind atrial septal defect occluder, Gianturco spring plug, Gianturco Grifka vascular occluder, and more recently, AGA arterial catheterization occluder, and in China, domestic arterial catheterization and ventricular septal defect occluders are more commonly used. Since most of the cases are right aortic sinus or uncrowned sinus breaking into the right atrium or right ventricle, the operation process is similar to septal defect occlusion, generally need to establish a femoral artery, an aortic sinus aneurysm, a break into the heart cavity, a vein track, and a vein from the venous side to place the occluder. Ideal indications for transcatheter occlusion include the presence of aortic sinus aneurysm (to the right heart) confirmed by TTE or TEE; the aneurysm does not involve the aortic valve or annulus and does not obstruct the right ventricular outflow tract; the edge of the sinus aneurysm to the aortic annulus and coronary opening is ≥5 mm; cardiac function is resistant to intervention; and other serious cardiac anomalies are excluded. Relative indications include: combination of other congenital malformations that can be treated with simultaneous intervention; acute rupture with poor cardiac function that cannot tolerate surgery; infertile women who may require valve replacement later. Contraindications include: severe pulmonary hypertension leading to right-to-left shunt; combination of other lesions requiring surgery; thrombus at the placement of the blocker, thrombosis at the catheter insertion route; endocarditis and bleeding disorders, sepsis, and severe infection within 1 month prior to blocking. A total of 21 patients with ruptured aortic sinus aneurysms were treated with interventions from April 2005 to December 2012 at Beijing Fu Wai Hospital, the largest group of cases retrievable to date. Among them, 14 were male and 7 were female; age ranged from 6 to 71 years (36.8 years ± 17.44). 16 cases were right coronary sinus aneurysms, with 5 cases ruptured into the right ventricle and 11 cases into the right atrium; 5 cases were non-coronary sinus aneurysms, with 1 case ruptured into the right ventricle and 4 cases into the right atrium. The combined malformations included: aortic diastasis in 3 cases, ventricular septal defect in 2 cases, and moderate aortic regurgitation (2 cases). The diameter of the sinus aneurysm rupture: 3 mm-4 mm (5.5 mm ± 2.50); mean pulmonary artery pressure: 17 mmHg-38 mmHg, including 3 cases of moderate pulmonary hypertension and 3 cases of mild pulmonary hypertension. 20 cases had a right femoral artery-aortic sinus aneurysm-right heart-right femoral vein track established, and 1 case had a right radial artery-aortic sinus aneurysm-right heart-femoral vein track established, and all were implanted by the femoral vein route. The occluders were implanted by the femoral vein route. A total of 21 occluded arterial catheters and one symmetrical ventricular septal defect occluder were used, and in one case, two occluders were inserted due to multiple breaches. Two cases had immediate postoperative residual shunts, and the rest were completely occluded, with one case having a slight increase in aortic regurgitation compared with the preoperative period. At follow-up, there was no recurrence of sinus aneurysm rupture and no significant increase in aortic regurgitation requiring aortic valve replacement surgery. It can be affirmed that transcatheter occlusion of strictly selected cases of ruptured aortic sinus aneurysms is less invasive, with positive near-term efficacy, shorter treatment time, and less patient pain, and is worthy of clinical application. Large-scale, multicenter studies and long-term follow-up are needed to further validate the clinical value of interventional procedures in aortic sinus aneurysm rupture and to assess the cost-effectiveness ratio.