Unruptured Sinus of Valsalva Aneurysm with Right Ventricular Outflow Tract Obstruction and Supracristal Ventricular Septal Defect: A Rare Case

Unruptured right sinus of Valsalva aneurysm that causes severe obstruction of the right ventricular outflow tract is extremely rare. We describe the case of a 47-year-old woman who presented with exertional dyspnea. Upon investigation, we discovered an unruptured right sinus of Valsalva aneurysm with associated right ventricular outflow tract obstruction and a supracristal ventricular septal defect. To our knowledge, only 2 such cases have previously been reported in the medical literature.Although treatment of unruptured sinus of Valsalva aneurysm remains debatable, surgery should be considered for extremely large aneurysms or for progressive enlargement of the aneurysm on serial evaluation. Surgery was undertaken in our patient because there was clear evidence of right ventricular outflow tract obstruction, right-sided heart dilation, and associated exertional dyspnea.     

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.     

Huge calcified aneurysm of the sinus of Valsalva

Aneurysms of the sinus of Valsalva often remain undiagnosed until they rupture. A 61-year-old man had a huge, heavily calcified unruptured aneurysm, originating from the right sinus of Valsalva, detected incidentally on a chest radiograph taken for the diagnosis of cardiomegaly. Two-dimensional echocardiography revealed pericardial effusion with a huge calcified mass compressing the right ventricular outflow tract. The Doppler color-flow echocardiogram showed blood flow from the aortic root into the aneurysm. A chest computed tomographic scan revealed a large thrombosed aneurysm originating from the aortic root and measuring 10x10 cm. After pericardiocentesis, cardiac catheterization was performed, which showed that the right ventricular systolic pressure had elevated to 80 mmHg. Aortic root aortography demonstrated a huge unruptured calcified aneurysm in the sinus of Valsalva arising from the right coronary sinus. The patient underwent surgical correction to prevent aneurysmal rupture and to relieve the right ventricular outflow obstruction.     

Unruptured Sinus of Valsalva Aneurysm with Right Ventricular Outflow Tract Obstruction and Ventricular Septal Defect—A Rare Combination

Sinus of Valsalva aneurysms are rare cardiac anomalies that may be acquired or congenital. We describe the case of a patient who had an unruptured sinus of Valsalva aneurysm causing right ventricular outflow obstruction, with an associated subaortic ventricular septal defect. Sinus of Valsalva aneurysms rarely present until rupture occurs. However, the unruptured aneurysm of the right sinus of Valsalva caused significant right ventricular outflow tract obstruction, resulting in exertional dyspnea. Intraoperative multiplane transesophageal echocardiography and color Doppler helped in precise identification of structural anomalies, shunt location, and definitive surgical repair.     

Unruptured aneurysm of the sinus of Valsalva presenting with right ventricular outflow obstruction

Sinus of Valsalva aneurysms rarely present until rupture occurs. We describe the case of a patient who presented with acute right heart failure and upon investigation was found to have an unruptured sinus of Valsalva aneurysm causing right ventricular outflow obstruction; there was an associated subaortic ventricular septal defect. To the best of our knowledge, only 1 other case with these features has been reported in the medical literature. The diagnosis was made by transthoracic echocardiography and cardiac catheterization. Through an aortic and right ventricular approach, we successfully excised the aneurysmal right coronary sinus, closed the ventricular septal defect, and replaced the aortic valve.     

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。     

Echocardiographic diagnosis of a ruptured aneurysm of the sinus of Valsalva: operation without catheterisation in seven patients

A ruptured aneurysm of the sinus of Valsalva was diagnosed by Doppler, colour, and cross sectional echocardiography in a consecutive series of seven patients. The diagnoses were confirmed at operation without cardiac catheterisation. Examination by pulsed and continuous Doppler echocardiography showed continuous turbulence in six patients with aneurysms rupturing into the right ventricular outflow tract and in the patient with rupture of an aneurysm of the non-coronary sinus into the right atrium. Colour Doppler echocardiography showed turbulent flow across the defects in all seven patients. A ventricular septal defect with aortic regurgitation was detected in one patient and an associated ventricular septal defect in another. Doppler, colour, and cross sectional echocardiography were useful non-invasive techniques for diagnosing a ruptured aneurysm of the sinus of Valsalva without the need for cardiac catheterisation.     

Echocardiographic diagnosis of a ruptured aneurysm of the sinus of Valsalva: operation without catheterisation in seven patients.

A ruptured aneurysm of the sinus of Valsalva was diagnosed by Doppler, colour, and cross sectional echocardiography in a consecutive series of seven patients. The diagnoses were confirmed at operation without cardiac catheterisation. Examination by pulsed and continuous Doppler echocardiography showed continuous turbulence in six patients with aneurysms rupturing into the right ventricular outflow tract and in the patient with rupture of an aneurysm of the non-coronary sinus into the right atrium. Colour Doppler echocardiography showed turbulent flow across the defects in all seven patients. A ventricular septal defect with aortic regurgitation was detected in one patient and an associated ventricular septal defect in another. Doppler, colour, and cross sectional echocardiography were useful non-invasive techniques for diagnosing a ruptured aneurysm of the sinus of Valsalva without the need for cardiac catheterisation.     

Unruptured congenital aneurysm of the sinus of valsalva presenting with pulmonary stenosis

Congenital aneurysm of the sinus of Valsalva is a rare cardiovascular anomaly. It is usually silent until rupture occurs. The natural history of unruptured aneurysm of the sinus of Valsalva is still not clear, and the therapeutic strategy is uncertain. Here we reported a case of unruptured aneurysm of the sinus of Valsalva which was correctly diagnosed before invasive diagnostic procedures. A 30-year-old female noted mild palpitation and dyspnea for 1 month. Physically, a grade 3/6 systolic ejection murmur at upper left sternal border was detected. Echocardiography revealed dilatation and irregular protrusion of the right sinus of Valsalva encroaching on right ventricular outflow tract to cause obstruction. With these findings, unruptured aneurysm of the sinus of Valsalva with pulmonary stenosis was diagnosed. Cardiac catheterization and angiography confirmed the diagnosis. The aneurysm was repaired with a Dacron patch with good results. It is concluded that sinus of Valsalva aneurysm can be diagnosed by echocardiography before its rupture so as to render a proper management for this potentially life-threatening anomaly. Cathet. Cardiovasc. Intervent. 46:210–213, 1999. © 1999 Wiley-Liss, Inc.     

Normal Pregnancy and Birth Despite Ruptured Sinus of Valsalva Aneurysm: Another Case for the Record

Sinus of Valsalva aneurysm is a rare cardiac abnormality. Ruptured sinus of Valsalva aneurysms in pregnancy are of course rarer still. We present a case in which an aneurysm ruptured into the right ventricular outflow tract during pregnancy.In 2012, a 26-year-old Chinese woman, in the 18th week of pregnancy and with no apparent evidence of cardiac problems, was diagnosed with atrioventricular septal defects and a sinus of Valsalva aneurysm that had ruptured into her right ventricular outflow tract. After an uncomplicated full-term pregnancy, she gave birth to a healthy baby boy by cesarean section. Fifty days postpartum, the patient underwent surgical repair of the ruptured aneurysm and other cardiac defects. Her surgical outcome was good. As of May 2013, the patient and her baby were healthy.Ruptured sinus of Valsalva aneurysm in pregnancy can be asymptomatic, and women with such a rupture can have a normal full-term pregnancy and give birth to healthy babies. Cesarean section is preferable for pregnant women with ruptured sinus of Valsalva aneurysm because the hemodynamic changes associated with labor can aggravate the aneurysm. Surgical repair should be performed as soon as the patient''s condition allows.     

Ruptured aneurysm of the sinus of valsalva coexisting with a ventricular septal defect and single coronary artery.

A 26-year-old man had been diagnosed with a cardiac murmur from birth. In 1998, he was admitted to hospital because of slight fatigue. A grade 5/6 continuous murmur was audible near the right sternal border at the second intercostal space. Doppler echocardiography detected an abnormal flow that suggested that an aneurysm of the right coronary sinus of Valsalva had ruptured into the right ventricular inflow tract. Blood tests showed a 19% step-up in oxygen saturation value between the right atrium and right ventricle, indicating a ventricular septal defect with left to right shunt. Coronary angiography revealed a single coronary artery. Surgical repair was carried out and the patient made an uneventful recovery. This rare combination of a ruptured aneurysm of the sinus of Valsalva coexisting with a ventricular septal defect and a single coronary artery has not been reported previously.     

Familial unruptured sinus of Valsalva aneurysm obstructing the right ventricular outflow tract

A case demonstrating compression of the right ventricular outflow tract by an unruptured coronary sinus of Valsalva aneurysm in which repair resulted in symptomatic improvement is presented. The pathology report revealed that the patient’s younger brother had died from a ruptured aneurysm of the coronary sinus of Valsalva. The present report is the first to describe a familial unruptured coronary sinus of Valsalva aneurysm raising questions regarding the screening of relatives of patients with sinus of Valsalva aneurysms of unknown etiology.     

The chance finding of an aneurysm of the right sinus of Valsalva in an 11-year-old child with a ventricular septal defect and a pericardial effusion

Ventricular septal defects can occur as part of other congenital cardiac malformations or as an isolated finding. Aneurysms of the sinus of Valsalva are rare, most commonly involving the right or noncoronary sinuses. They can be congenital or acquired through infection, trauma, or degenerative diseases. They frequently co-exist with ventricular septal defects, aortic valve dysfunction, or other cardiac abnormalities. More commonly, sinus of Valsalva aneurysms are diagnosed after the clinical sequelae of rupture. Several etiologic factors may lead to the development of pathologic pericardial effusion and the detection of pericardial effusion was one of the first applications of echocardiography to gain widespread acceptance. We present a case of a chance finding of an aneurysm of the right sinus of Valsalva in an 11-year-old Italian child with a ventricular septal defect and a pericardial effusion.     

Echocardiography diagnosis of ruptured aneurysm of sinus of Valsalva. Report of two cases.

Echocardiographic features of two cases of ruptured congenital aneurysm of Valsalva sinus with (case1) and without (case 2) a supracristal ventricular defect were studied before and after surgery by standard echocardiography. M-mode scan and two-dimensional echocardiography. Discontinuity was observed in the echo from the aneurysm wall of the Valsalva sinus in case 1 with ruptured aneurysm, but was not observed in a similar case of unruptured aneurysm. Herniation of the right coronary cusp of the aortic valve into the right ventricular outflow tract was also observed in case 1. After surgical correction the echo from the aneurysm wall and from the herniated right coronary cusp of the aortic valve disappeared. Discontinuity in the echo from the anterior aortic wall and the interventricular septum also disappeared. In case 2, discontinuity in the echo from the anterior aortic wall, and tricuspid flutter with an abnormally low early diastolic peak were observed. These abnormalities disappeared after surgery. The ability of echocardiography to detect ruptured Valsalva aneurysm is discussed.     

Ventricular tachycardias arising from the aortic sinus of valsalva: an under-recognized variant of left outflow tract ventricular tachycardia

OBJECTIVES: To describe a normal heart left bundle branch block, inferior axis ventricular tachycardia (VT), that could not be ablated from the right or left ventricular outflow tracts. BACKGROUND: Whether these VTs are epicardial and can be identified by a specific electrocardiographic pattern is unclear. METHODS: Twelve patients with normal heart left bundle branch block, inferior axis VT and previously failed ablation were included in this study. Together with mapping in the right and left ventricular outflow tracts, we obtained percutaneous epicardial mapping in the first five patients and performed aortic sinus of Valsalva mapping in all patients. RESULTS: No adequate pace mapping was observed in the right and left ventricular outflow tracts. Earliest ventricular activation was noted in the epicardium and the aortic cusps. All patients were successfully ablated from the aortic sinuses of Valsalva (95% CI 0% to 18%). The electrocardiographic pattern associated with this VT was left bundle branch block, inferior axis and early precordial transition with Rs or R in V2 or V3. Ventricular tachycardia from the left sinus had rS pattern in lead I, and VT from the noncoronary sinus had a notched R wave in lead I. None of the patients had complications and all remained arrhythmia-free at a mean follow-up of 8 +/- 2.6 months. CONCLUSIONS: Normal heart VT with left bundle branch block, inferior axis and early precordial transition can be ablated in the majority of patients from either the left or the noncoronary aortic sinus of Valsalva.     

Unruptured sinus of valsalva aneurysm presenting as acute coronary syndrome

An unusual presentation of sinus of Valsalva aneurysm causing right ventricular outflow tract obstruction and presenting as acute coronary syndrome is reported. A 38-year-old lady presented with ischemic chest pain, probably due to embolization from an unruptured sinus of Valsalva aneurysm.     

Multiplane transesophageal echocardiography in the preoperative evaluation of the sinus of Valsalva fistula to right chambers]

INTRODUCTION AND OBJECTIVES: Ruptured sinus of Valsalva aneurysm to right cardiac chambers is an uncommon lesion in Western countries. The prognosis is usually serious unless the condition is promptly treated surgically. For this reason an accurate anatomical and functional evaluation is necessary. The main purpose of this report is to compare the usefulness of multiplane transesophageal echocardiography with transthoracic echocardiography and angiocardiography in the preoperative evaluation of ruptured sinus of Valsalva aneurysm to right chambers. PATIENTS AND METHOD: Since January 1990, 9 patients (mean age 36,3 18 yr, 6 males) with ruptured sinus of Valsalva aneurysm to right chambers were studied. The pathogenesis was congenital aneurysm in 6 patients, aortic prosthesis endocarditis in one and two cases of iatrogenia: during a percutaneous mitral valvuloplasty and after cardiac surgery. Transthoracic echocardiography was performed in all cases, transesophageal echocardiography in 7 and angiocardiography in 8. Two patients died before surgery, and 7 were successfully operated on. RESULTS: Transesophageal echocardiography was more useful when compared to transthoracic echocardiography and angiocardiography in detecting: a) the fistula; b) the sinus involved; c) the right chamber affected; d) congenital aneurysms morphology and size; e) aneurysm prolapse through a ventricular septal defect, y f) the identification of other cardiac congenital or acquired anomalies. CONCLUSIONS: Multiplane TEE is the most accurate tool in the preoperative evaluation of ruptured sinus of Valsalva aneurysm to right chambers.     

Fixed subaortic stenosis with congenital aneurysm of sinus of Valsalva--cross-sectional and Doppler echocardiographic diagnosis of a rare association

A 13-year-old girl was diagnosed by cross-sectional and Doppler echocardiography to have the rare combination of congenital aneurysm of the right sinus of Valsalva rupturing into the right ventricular outflow tract and a discrete subaortic fibrous shelf with severe subvalvar stenosis, moderate aortic regurgitation and left ventricular dysfunction. The findings were confirmed at cardiac catheterisation and surgery.     

Cross-sectional echocardiographic diagnosis of discrete subaortic stenosis, ruptured congenital aneurysm of the right sinus of Valsalva, unruptured aneurysm of non-coronary sinus and a rudimentary left aortic leaflet

A 7-year-old asymptomatic male child was found to have a shelf-like subaortic stenosis, congenital aneurysm of the right sinus of Valsalva communicating with the right ventricular outflow tract, an unruptured aneurysm of the non-coronary sinus and a small left aortic sinus with a rudimentary valvar leaflet causing moderate aortic regurgitation. The diagnosis was made by cross-sectional and Doppler echocardiography and confirmed by cineangiocardiography.     

Unruptured sinus of Valsalva aneurysm with right ventricular outflow tract obstruction associated with ventricular septal defect

We describe an aneurysm originating from the right sinus of Valsalva, with right ventricular outflow tract obstruction associated with ventricular septal defect, in a 9-year-old boy. Diagnosis was made by echocardiography and cardiac catheterization. Successful surgical repair of ventricular septal defect and aortic valvuloplasty resulted in symptomatic improvement. © 1996 Wiley-Liss, Inc.