Ruptured aneurysm of the right sinus of valsalva associated with a ventricular septal defect and an anomalous coronary artery

Aneurysms of the sinus of Valsalva are extremely rare. Ruptured aneurysms of the sinus of Valsalva are frequently associated with other congenital defects, particularly with ventricular septal defect, aortic valve regurgitation, and bicuspid aortic valve. We describe the case of a 26-year-old man who had a ruptured aneurysm of the right coronary sinus, a ventricular septal defect, and an anomalous origin of the right coronary artery. Successful surgical correction of the aneurysm and ventricular septal defect was performed with patch repair and aortic valve replacement. A review of the English-language medical literature revealed only 1 other case of a sinus of Valsalva aneurysm associated with a ventricular septal defect and an anomalous coronary artery. Previously published reports of the coexistence of a single coronary artery with a sinus of Valsalva aneurysm or with a ventricular septal defect, and their management, are discussed herein.     

主动脉窦瘤破裂的外科治疗

目的:总结1981年1月～2005年6月156例主动脉窦瘤破裂(RSVA)的外科治疗经验。方法:156例RSVA患者,男102例,女54例,平均年龄27(8～59)岁。其中并发室间隔缺损91例,主动脉瓣关闭不全(AI)86例,感染性心内膜炎8例,右室流出道狭窄或右室双腔心16例,瘤破裂直接结扎11例,直接缝合43例,补片修补102例。室间隔缺损均采用补片修补,主动脉瓣整形15例,主动脉瓣置换28例。余并发症均同期予以处理。结果:围术期死亡1例,远期死亡4例。再次手术5例,其中急诊2次手术1例。RSVA修补术后残余分流3例,室间隔缺损修补术后残余分流5例。其余患者心功能明显改善,AI减轻,临床效果良好。结论:RSVA是少见的心脏疾病,手术是惟一有效的治疗方法。采用主动脉及心腔双切口利于心肌保护和确切修补主动脉窦瘤,纠正并发畸形;并发AI时应注意探查,大多数重度AI患者最终需行主动脉瓣置换术。     

Aortic sinus of Valsalva aneurysms simulating primary right-sided valvular heart disease.

While acute regurgitation is the most common valvular lesion associated with aortic sinus of Valsalva aneurysms, this report describes the pathological findings in five patients in whom primary right-sided valve dysfunction was simulated by sinus of Valsalva aneurysms. In two patients aneurysms of the noncoronary sinus projected into the right atrium at the level of the tricuspid valve and caused tricuspid incomptence. In three patients aneurysms of the right sinus of Valsalva projected into the right ventricle immediately below the pulmonic valve and caused pulmonary outflow tract obstruction. In only one of these patients was the aneurysm perforated at necropsy. Although valvular dysfunction was evident clinically, in none was there a clinical suspicion of a sinus of Valsalva aneurysm. In addition to valvular dysfunction, two patients had conduction disturbances and two, right coronary arterial occlusions found to be the result of aneurysms. The risks of valve dysfunction, arrythmias, aneurysm rupture, and sudden death and the ability to correct this lesion surgically make it especially important to consider aortic sinus of Valsalva aneurysm as a cause of obscure right-sided valvular disease.     

Aortic root replacement in Behçet disease

The patient presented with a history of recurrent aphthous stomatitis, genital ulceration, and a family history of positive for collagen disease. Echocardiography and retrograde aortography revealed aneurysm formation of the sinus of Valsalva, and dilatation of the aortic valve annulus with severe aortic regurgitation. On diagnosis of an aneurysm of the sinus of Valsalva and aortic regurgitation associated with Beh?et's disease, aortic root replacement with the modified Bentall technique was successfully performed.     

Unruptured right coronary sinus to left ventricle aneurysm diagnosed by cross sectional echocardiography.

A 37 year old Chinese man presenting with features of chest infection was diagnosed as having severe aortic regurgitation. Cross sectional echocardiography showed an intact right coronary sinus of Valsalva aneurysm, which protruded into the left ventricle. The changes in appearance of the aneurysm during the cardiac cycle were recorded by cross sectional echocardiography. The opening of the aneurysm was successfully closed and the aortic valve replaced with a mechanical prosthesis.     

Unruptured right coronary sinus to left ventricle aneurysm diagnosed by cross sectional echocardiography

A 37 year old Chinese man presenting with features of chest infection was diagnosed as having severe aortic regurgitation. Cross sectional echocardiography showed an intact right coronary sinus of Valsalva aneurysm, which protruded into the left ventricle. The changes in appearance of the aneurysm during the cardiac cycle were recorded by cross sectional echocardiography. The opening of the aneurysm was successfully closed and the aortic valve replaced with a mechanical prosthesis.     

Unruptured aneurysm of the sinus of valsalva with Beh?et's disease.

A 52-year-old Japanese man who had suffered from Beh?et's disease since the age of 45 years was admitted to hospital for evaluation of syncope and heart murmur. Echocardiography and aortography revealed severe aortic regurgitation and cystic masses under the right coronary cusp and the left ventricular outflow tract, but no shunt jet. He was diagnosed with unruptured aneurysm of the sinus of Valsalva, and surgical closure of the orifice of the aneurysm was performed. The diameter of the orifice was 11 mm and the aneurysm was 15 mm in depth, and consisted of 2 chambers. Because the aortic regurgitation was reduced after patch closure of the orifice, aortic valve replacement was not performed. Unruptured aneurysm of the sinus of Valsalva is a rare clinical lesion, but patients with active inflammatory disease of the aorta, such as in Beh?et's disease, should have periodic echocardiography for early detection of an aneurysm or valvular involvement, even if there are not any symptoms.     

Non-coronary sinus of Valsalva aneurysm diagnosed after a road traffic accident

A 38 year old man with a huge unruptured sinus of Valsalva aneurysm, complicated with severe valvar aortic regurgitation, is described. The aneurysm was detected by echocardiography in the asymptomatic patient who presented with an intense precordial diastolic rumble after a road traffic accident. The patient had successful surgery for the aneurysm and aortic valve replacement. Possible aetiologies for the aneurysm and a brief revision of clinical aspects and treatment are discussed.


Keywords: sinus of Valsalva aneurysm; valvar disorders; trauma     

Transesophageal Echocardiography Findings of an Infected Quadricuspid Aortic Valve with an Anomalous Coronary Artery

We describe a patient with severe aortic regurgitation in whom transesophageal echocardiography coupled with cardiac catheterization and pathological examination confirmed the diagnosis of a quadricuspid aortic valve, anomalous coronary artery, and aneurysm of the noncoronary sinus of Valsalva.     

Aortic Valve Regurgitation that Resolved after a Ruptured Coronary Sinus Aneurysm Was Patched

Sinus of Valsalva aneurysms appear to be rare. They occur most frequently in the right sinus of Valsalva (52%) and the noncoronary sinus (33%). More of these aneurysms originate from the right coronary cusp than from the noncoronary cusp. Surgical intervention is usually recommended when symptoms become evident.We report the case of a 34-year-old woman who presented with a congenital, ruptured sinus of Valsalva aneurysm that originated from the noncoronary cusp. Moderate aortic regurgitation was associated with this lesion. Simple, direct patch closure of the ruptured aneurysm resolved the patient''s left-to-right shunt and was associated with decreased aortic regurgitation to a degree that valve replacement was not necessary. Only trace residual aortic regurgitation was evident after 3 months, and the patient remained free of symptoms after 6 months.Our observations support the idea that substantial runoff blood flow in the immediate supra-annular region can be responsible for aortic regurgitation in the absence of a notable structural defect in the aortic valve, and that restoring physiologic flow in this region and equalizing aortic-cusp closure pressure can largely or completely resolve aortic insufficiency. Accordingly, valve replacement may not be necessary in all cases of ruptured sinus of Valsalva aneurysms with associated aortic valve regurgitation.Key words: Aortic aneurysm/congenital/etiology/radiography/surgery, aortic valve insufficiency, cardiac surgical procedures/methods, differential diagnosis, sinus of Valsalva/pathology/radiography/surgery, treatment outcomeSinus of Valsalva aneurysm (SVA) was first described in 1839.¹ This lesion is encountered during 0.14% to 0.96% of all open-heart procedures and in 0.09% of postmortem studies.² Right coronary cusp aneurysms are the most frequent form of SVA (52% of all SVA cases), and noncoronary cusp aneurysms are diagnosed in 33% of SVA cases.³ We present the case of a woman who had a congenital, ruptured SVA of the noncoronary cusp and moderate aortic regurgitation (AR). We discuss our simple, direct patch closure of the aneurysm, and we present our reasoning as to how this treatment eliminated the patient''s AR without the need to replace the aortic valve.     

Alternative Surgical Approach to Repairing a Giant Sinus of Valsalva Aneurysm

We report our method of surgically repairing an unruptured giant aneurysm of the right coronary sinus of Valsalva, a lesion that caused moderate aortic regurgitation but no symptoms in a 61-year-old woman. We excised the aneurysm, reconstructed the right sinus of Valsalva with use of a patch, performed mechanical aortic valve replacement directly through the excised aneurysm''s cavity, and constructed a single bypass graft to the right coronary artery. The patient was discharged from the hospital after 5 days. Twelve months postoperatively, her clinical and echocardiographic results were normal, and she was doing well. To our knowledge, our surgical approach to this repair has not been described previously.     

Double orifice mitral valve in an asymptomatic adult with an unusual combination of congenital malformations: a case report.

We report a case of an asymptomatic adult patient, with several congenital malformations including an infrequent variant of double orifice mitral valve, postductal aortic coarctation, bicuspid aortic valve and an aneurysm of the right Valsalva sinus. The loss of support of the right coronary cusp of the aortic valve caused major aortic regurgitation. With the exception of the mitral valve, which was left untouched because it was neither stenotic nor regurgitant, all the other abnormalities were successfully corrected, in a two-step surgical approach.     

Rupture of an aneurysm of the aortic sinus of Valsalva into the right ventricle. A case presenting an unusual hemodynamic manifestation

Rupture of an aneurysm of the aortic sinus of Valsalva into the right ventricle is described. Surgery revealed a small aneurysm ruptured into the right ventricle near the tricuspid valve without aneurysmal involvement of the tricuspid valve. The cineangiogram revealed a shunt from the aortic sinus into the right atrium not during systole but during diastole, and increased oxygen saturation was found in the right atrium. The unusual hemodynamics in this case was most probably due to the closure of the small aneurysmal opening due to myocardial contraction during systole, so that the shunt flow through the aneurysmal tract occurred only during diastole and was directed toward the right atrium due to the location of the aneurysm. Thus, even without aneurysmal involvement of the tricuspid valve or tricuspid regurgitation, the oxygen saturation increased in the right atrium.     

Diagnostic paradox: Ruptured aneurysm of sinus of Valsalva simulating tricuspid valve endocarditis

Rupture of aneurysm of sinus of Valsalva into the right atrium mimicking tricuspid valve endocarditis is a rare presentation. We review a case of spontaneous rupture of aneurysm of sinus of Valsalva into the right atrium presenting as a murmur. Transthoracic echocardiogram showed a mobile mass that appeared to be attached to the tricuspid valve leaflet with moderate tricuspid regurgitation suggestive of tricuspid valve endocarditis. The diagnosis was confirmed as spontaneous rupture of noncoronary sinus in to the right atrium by transesophageal echocardiogram. Patient recovered completely after surgical repair.     

38例主动脉窦瘤破裂的外科治疗

目的 :通过研究 38例主动脉窦瘤破裂治疗经过 ,总结主动脉窦瘤破裂外科治疗经验。方法 :1987年 1月至 2 0 0 1年 9月 ,对 38例主动脉窦瘤破裂病人行手术治疗 ,及时准确闭合主动脉窦瘤及矫正合并的心脏畸形。对于合并主动脉瓣关闭不全病例 ,轻度不予处理 ,中度行成形术 ,重度行主动脉瓣置换术。结果 :早期死亡 1例 ,死亡率为 2 6 %。术后随访 2个月～ 14年 ,随访者心脏功能恢复到Ⅰ～Ⅱ级 (NYHA)。结论 :主动脉窦瘤破裂一经诊断 ,应及早手术 ,同时矫正合并的心脏畸形 ,可获得满意的近期和远期效果。     

Ruptured sinus of Valsalva aneurysm associated with noncompaction of the ventricular myocardium.

Rupture of a sinus of Valsalva aneurysm is a rare, but life-threatening cardiac abnormality that requires surgical correction when diagnosed, and is frequently associated with other congenital defects, particularly with ventricular septal defect, aortic valve regurgitation, and bicuspid aortic valve. We present the case of a 21-year-old man who had a ruptured aneurysm of the noncoronary sinus into the right atrium, a ventricular septal defect, a persistent left superior vena cava and a noncompaction of the ventricular myocardium diagnosed by two-dimensional echocardiography. Surgical repair was carried out and the patient made an uneventful recovery.     

彩色多普勒超声心动图对主动脉窦瘤的诊断价值

目的 :旨在探讨彩色多普勒超声心动图 (CDE)诊断主动脉窦瘤的临床价值。方法 :观察分析 115例主动脉窦瘤CDE图像特征及规律性并与手术对照。结果 :95例源于右冠状动脉窦 ,19例源于无冠状动脉窦 ,1例源于左冠状动脉窦 ;窦瘤破入右心室 95例 ,破入右心房 18例 ,破入左心室 1例 ,破入室间隔 1例。合并室间隔缺损 79例 ,主动脉瓣脱垂及反流 5 5例 ,心内膜炎 2 9例 ,其它房间隔缺损、动脉导管未闭分别为 4、9例。术前CDE诊断结论与手术符合率达 99% ,合并室间隔缺损的检出率为 91%。本组合并室间隔缺损占整个病例的 68% ,占窦瘤破入右心室的 84%之多。合并主动脉瓣脱垂或反流本组检出率高于手术所见 (62 /5 5 )。结论 :CDE可以判明主动脉窦瘤的精确的解剖 ,包括窦瘤的来源部位、形态、大小、血流动力学影响 ,是否合并其它心脏畸形等。患者经CDE检查后不必作其他辅助性诊断检查就进行手术是安全、准确、可靠的。     

Unruptured aneurysm of the right sinus of Valsalva: a case report

A previously healthy and asymptomatic 45-year-old woman was referred for evaluation of a heart murmur and an unusual dilatation in the aortic root. Transthoracic and transesophageal echocardiography, computed tomography, magnetic resonance imaging, and cardiac catheterization revealed an aneurysm with a maximum diameter of 6.0 cm in the right sinus of Valsalva. Aortic regurgitation and stenosis at the right ventricle outflow tract were associated with the aneurysm. Surgery and histological study demonstrated that the sinus of Valsalva aneurysm was enormously dilated with idiopathic degenerative change in the aortic media. The aneurysm was tremendously large compared to any previously reported. Decrease in pressure load during diastole caused by aortic regurgitation probably resulted in the growth of this huge aneurysm without rupture.     

Ruptured sinus of Valsalva aneurysm: 10-year Indian surgical experience

Sinus of Valsalva aneurysm is a rare anomaly with a higher incidence in Eastern than Western populations. Recent improvements in diagnostic techniques have resulted in more patients undergoing surgical repair. Uncorrected, the intracardiac shunts and frequently associated cardiac lesions cause a preventable deterioration in heart function. We retrospectively analyzed the data of 33 patients who underwent repair of ruptured sinus of Valsalva aneurysm from May 2000 to January 2010. The aneurysms originated from the right coronary sinus in 24 patients and from the noncoronary sinus in 9, and ruptured into the right ventricle in 21, right atrium in 10, and left ventricle in 2. Operative procedures included simple plication (1), patch repair (32), and aortic valve replacement (10). There were 3 early deaths. The 30 survivors were followed up for 5.4 ± 2.6 years; all had an improvement in functional class, with superior results in those with no aortic regurgitation. Surgical treatment of ruptured sinus of Valsalva aneurysm has an acceptably low operative risk and good long-term symptom-freedom survival. An early aggressive approach is recommended to prevent worsening symptoms and more extensive disease.     

小儿双动脉瓣下室间隔缺损46例临床─X线分析

经手术证实的双动脉瓣下室间隔缺损４６例。结合临床、Ｘ线胸片共分为３组：①无并发症的单纯缺损组２８例；②合并肺动脉高压组１２例（其中１例伴动脉导管未闭及主动脉右瓦氏窦瘤）；③合并主动脉右瓣脱垂５例（其中伴主动脉瓣关闭不全４例）及主动脉右瓦氏窦瘤１例。与其它类型的室间隔缺损相比较，讨论了胸部平片诊断的价值、并发症的成因及心血管造影的表现，并强调主动脉根部造影的重要性。