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.     

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.     

Severe Right Ventricular Outflow Obstruction by Right Sinus of Valsalva Aneurysm

Aneurysms of the sinus of Valsalva are rarely diagnosed cardiac anomalies, occurring in 0.14%–0.96% of patients who have undergone open heart surgical procedures. The most common congenital anomalies accompanying sinus of Valsalva aneurysm (SVA) are ventricular septal defect, bicuspid aortic valve, atrial septal defect, and coarctation of aorta. We report a patient with an unruptured right SVA presenting with severe right ventricular outflow tract (RVOT) obstruction, and coexisting patent foramen ovale (PFO) with a right to left shunt. It could be assumed that the increase in right atrial pressure due to RVOT obstruction had led to a right to left shunt across the patent foramen ovale. (Echocardiography 2010;27:341‐343)     

A sinus of valsalva aneurysm: more complicated than imagined

Sinus of Valsalva aneurysms mostly remain silent. Here, we report a case with sinus of Valsalva aneurysm which caused right ventricular outflow obstruction and ruptured into the main pulmonary artery in the setting of DeBakey type I aortic dissection.     

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.     

Unruptured congenital aneurysm of the left sinus of Valsalva presenting as acute right ventricular failure.

A patient with unruptured congenital aneurysm of the left coronary sinus of Valsalva presented with acute right-sided heart failure due to right ventricular outflow tract obstruction. The mechanism for such an acute presentation may have been a sudden increase in the size of the aneurysm. The surgical importance of this lesion is the combined aortocameral approach which is seldom required for correction of such aneurysms.     

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.     

Rare cause of right heart failure: contained rupture of a sinus of Valsalva aneurysm associated intraventricular septal aneurysm.

Sinus of Valsalva aneurysm (SVA) is a rare congenital lesion described first in 1840 by John Thurnam (Cited by Boutefou JM, Moret PR, Hahn C, Hanf E. Aneurysms of the sinus of Valsalva: report of seven cases and review of the literature. Am J Med 1978;65:18-24). In most cases unruptured Sinus of Valsalva aneurysm (SVA) is clinically silent; however, if it progressively enlarges it may cause coronary artery compression, complete heart block, or right ventricular outflow tract obstruction (Meier JH, Seward JD, Miller FA, Oh J, Sarano ME. Aneurysms in the left ventricular outflow tract: clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr 1998;11:729-45; D'Silva SA, Dalve BV, Lokhandwala YY, Kale PA, Tendolkar AG. Unruptured congenital aneurysm of the left sinus of Valsalva presenting as acute right heart failure. Chest 1992;101:578-79) or is a potential source of cerebrovascular emboli. (Shahrabani RM, Jairaj PS. Unruptured aneurysm of the sinus of Valsalva: a potential source of cerebrovascular embolism. Br Heart J 1993;69:266-67). In this report, we describe a case of right coronary sinus of Valsalva aneurysm with a contained rupture. The containing rupture is in intraventricular septal aneurysm; the patient presents with right-sided heart failure.     

Unruptured sinus of Valsalva aneurysm with right ventricular outflow obstruction

The recommended operative management of unruptured sinus of Valsalva aneurysm consists of closure of the mouth of the aneurysm with or without aortic valve surgery. We report a case of unruptured aneurysm producing right ventricular outflow tract obstruction. Closure of the mouth of the aneurysm failed to relieve the obstruction, which was subsequently achieved by excising the aneurysmal wall overlying the outflow tract.     

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.     

Isolated unruptured sinus of Valsalva aneurysm producing right ventricular outflow obstruction

An unusual case of right ventricular outflow obstruction and right heart failure due to an isolated unruptured congenital sinus of Valsalva aneurysm originating from the right coronary sinus in a 75-year-old-man is described. The diagnosis was made by two-dimensional echocardiography and cardiac catheterization. Successful surgical resection of the aneurysm resulted in dramatic symptomatic improvement.     

Unruptured sinus of Valsalva aneurysm with right ventricular outflow obstruction diagnosed by two-dimensional and Doppler echocardiography

This report presents a case of an unusually large unruptured sinus of Valsalva aneurysm complicated by right ventricular outflow tract obstruction, right coronary artery occlusion and incomplete right bundle branch block. Two-dimensional and Doppler echocardiography were instrumental in preoperative diagnosis and postoperative follow-up.     

Unruptured sinus of Valsalva aneurysm presenting as acute coronary syndrome

Sinus of Valsalva aneurysm is an infrequent cardiac anomaly. In the United States, it accounts for only 0.15 to 1.5% of all cardiopulmonary bypass procedures. Sinus of Valsalva aneurysms can be acquired, secondary to infectious, degenerative or traumatic processes. This paper describes the case report of an 81-year-old woman, with an unruptured aneurysm of the right Valsalva sinus, who was admitted to the emergency department with a history of chest pain. After surgical repair of the sinus ofValsalva aneurysm, the prognosis is usually good, and the risk of recurrence is rare.     

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.     

Sinus of Valsalva Aneurysm—An Unusual Cause of Syncope

Sinus of Valsalva aneurysms (SOVA) are rare anomalies which often only become apparent at the time of rupture. Syncope associated with unruptured aneurysms is a recognized symptom but in most previously reported cases the mechanism has been either a brady‐ or tachyarrhythmia. We report a case of a large SOVA affecting the right coronary cusp which presented with syncope secondary to hypotension thought to be resulting from reduced left atrial filling as a consequence of transient right ventricular outflow tract (RVOT) obstruction. The case demonstrates how echocardiographic and angiographic images correlate and also an unusual mechanism of syncope. SOVA are rare anomalies that often only become apparent at the time of rupture. Syncope associated with unruptured aneurysms is a recognized symptom but in most previously reported cases the mechanism has been either a brady‐ or tachyarrhythmia. We report a case of a large SOVA affecting the right coronary cusp, which presented with syncope secondary to hypotension thought to be resulting from reduced left atrial filling as a consequence of transient RVOT obstruction. The case demonstrates how echocardiographic and angiographic images correlate and also an unusual mechanism of syncope. (Echocardiography 2010;27:E60‐E61)     

Aneurysms of the sinuses of Valsalva

Sinus of Valsalva aneurysms are rare cardiac anomalies which may be acquired or congenital, most commonly involving the right or noncoronary sinuses. The congenital aneurysms are more common and often caused by weakness at the junction of the aortic media and the annulus fibrosus. Acquired aneurysms are caused by conditions affecting the aortic wall, such as infections (syphilis, bacterial endocarditis, or tuberculosis), trauma, or connective tissue disorders. Unruptured aneurysms are usually found incidentally during diagnostic studies. More commonly, sinus of Valsalvaaneurysms are diagnosed after clinical sequelae of rupture. Diagnosis of sinus of Valsalva aneurysm is facilitated by echocardiography, contrast aortography, and more recently, magnetic resonance imaging. Repair is generally required for ruptured aneurysms; unruptured aneurysms encroaching on nearby structures, causing myocardial ischemia, or having the potential to rupture warrant repair. A review of the literature is presented focusing on anatomy, clinical presentation of ruptured and unruptured aneurysms, noninvasive diagnostic modalities, and techniques for repair of this anomaly.     

Unruptured aneurysms of the sinus of Valsalva disclosed by syncopal disorders of cardiac excitability. Apropos of 2 cases

Aneurysms of the sinus of Valsalva are rarely diagnosed before rupture into the cardiac cavities which usually leads to the appearance of a continuous murmur and cardiac failure. In the two cases described, the presenting symptom of the aneurysm was syncope due to cardiac hyperexcitability: ventricular tachycardia in the first and paroxysmal tachyarrhythmia in the second case. The presenting symptoms of unruptured aneurysms of the sinus of Valsalva were analysed. In general, they are: uncontinuous cardiac murmurs: either diastolic murmurs of aortic regurgitation, systolic murmurs of mitral or tricuspid regurgitation, or, as in our first case, of obstruction to right ventricular ejection; arrhythmias: the commonest are conduction defects, which can be syncopal; hyperexcitability (especially ventricular) seems to be very care. Echocardiography is a valuable tool for the diagnosis of sinus of Valsalva aneurysms. The appearances of unruptured aneurysms in our two patients are described. The presence of syncopal cardiac hyperexcitability, possibly associated with one of the preceding auscultatory abnormalities is an indication for echocardiography which may lead to the diagnosis of this condition.     

Aneurysms of sinus of Valsalva: two-dimensional echocardiographic diagnosis and recognition of rupture into the right heart cavities

The aim of this study was to evaluate the role of echocardiography in the diagnosis of sinus of Valsalva aneurysms projecting toward the right heart cavities. Three patients who had a ruptured aneurysm of a sinus of Valsalva diagnosed by echocardiography and confirmed by catheterization underwent cardiac surgery. In two patients, the aneurysm originated from the right coronary sinus and had perforated into either the inflow or outflow tract of the right ventricle. In the third patient, the aneurysm, which originated from the noncoronary sinus, ruptured into the atrium. A fourth patient was also investigated and had an unruptured aneurysm of the right coronary sinus projecting into the right ventricular outflow tract. M-mode, two-dimensional and contrast echocardiographic studies were performed before cardiac catheterization in all patients and after surgery in three patients. M-mode echocardiography was useful only when the aneurysm had an anterior projection, whether or not the aneurysm was ruptured. Conversely, two-dimensional echocardiography was always able to identify the aneurysmal sac which appeared as an abnormal circular thin-walled structure protruding into the right heart cavities. By using multiple views, it was possible to investigate the whole abnormal structure and locate the sinus from which the aneurysm originated. The use of the echo contrast technique allowed more precise definition of the aneurysmal sac and diagnosis of a left to right shunt by demonstrating a negative contrast image in the right cavities. On the other hand, no negative contrast image was recorded in the patient with an unruptured aneurysm or in the two instances of a successful surgically reconstructed aorta.     

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.