Congenital submitral aneurysm and sinus of valsalva aneurysm

Submitral aneurysm (SMA) is congenital outpouching of the left ventricular (LV) wall invariably occurring adjacent to the posterior leaflet of mitral valve. SMA is typically diagnosed in young adults who present with severe mitral regurgitation, heart failure, systemic embolism, and sudden cardiac death. African blacks account for the majority of the reported SMA cases. Our report of the very rare combination of SMA with aortic sinus aneurysm lends support to the congenital origin of this pathology occurring due to developmental deficiency in the fusion of myocardium and cardiac fibro skeleton. Complete diagnosis was made by transthoracic echocardiography.     

Rupture of sinus of valsalva aneurysm. Differentiation from cardiac contusion (author's transl)

The diagnostic aspects of rupture sinus of Valsalva aneurysm are discussed. The indication for corrective surgery can only be derived by angiocardiography. Important is the demonstration of pathophysiological hemodynamics, due to the cardiovascular anomaly. The importance of echocardiographic findings in an early phase of the clinical examination is stressed.     

Ruptured sinus of valsalva aneurysm Clinical features

From our experience with two patients with ruptured sinus of Valsalva aneurysms, we believe that there are so many unusual features in this form of cardiac disease that the diagnosis can be suspected not only before rupture of the aneurysm but also the march of events after rupture will indicate the site of rupture. Our first patient was young and she compensated for the sudden alteration in hemodynamics long enough to allow for both elaborate studies and elective surgery which was successful. Our second patient was older and she deteriorated rapidly. Surgery was planned but could not be instituted until too late and there was no opportunity for other than simple investigative procedures. It seems to us that when presented with this latter situation, immediate operation is required and the site of rupture can be predicted with some certainty from clinical and electrocardiographic findings.     

Treatment and outcome of sinus of valsalva aneurysm

BACKGROUND: In order to understand the long-term outcome after surgical repair of sinus of Valsalva aneurysms or fistulas (SVAF), the operative results for patients treated at Shanghai Chest Hospital were retrospectively assessed. METHODS: Between 1960 and 1999, 216 patients were operated on for SVAF. Of these 143 (66.2%) had a ventricular septal defect (VSD), 60 (28.0%) had aortic regurgitation and of these 12 underwent aortic valve replacement (AVR). RESULTS: There were eight in-hospital deaths (3.7%). Another patient had aortic regurgitation postoperatively requiring AVR. CONCLUSION: Resection and repair of SVAF entails an acceptably low operative risk and yields long-term freedom from symptoms. Early, aggressive operation is recommended to prevent development of complications.     

Ruptured aneurysm of the noncoronary sinus of valsalva

Sinus of Valsalva aneurysm is a rare disorder. It is usually congenital, but other origins have been described. It may be asymptomatic, or it may present as angina or with symptoms of valvular insufficiency or outflow obstruction. Once ruptured, it often produces hemodynamic instability. Diagnosis should be pursued with transesophageal echocardiography or catheterization. A 50-year-old man was admitted with symptoms of congestive heart failure. His functional capacity had declined over 3 years, worsening over the last 3 weeks. His medical history was significant only for gunshot to the right hemithorax, at age 25. He was found to be in distress, with signs of heart failure, S3 and S4 gallop, and systolic and diastolic murmur at the right sternal border. Two-dimensional echocardiography revealed an ejection fraction of 0.25, dilated left and right atria, and a sinus of Valsalva aneurysm with an aortic-right atrial shunt. Left and right heart catheterization revealed metallic fragments in the anterior chest wall, normal coronary arteries, and a step-up in the oxygen saturation from the inferior vena cava to the pulmonary artery. Aortography revealed that the aneurysm had ruptured into the right atrium. Surgical repair was performed. Ruptured sinus of Valsalva aneurysm demands prompt diagnosis and treatment. Patients are often men, in the 3rd or 4th decade of life when rupture occurs. In our patient, the position of the bullet and its fragments, leaflet perforation, and lack of a predisposing infection suggest a causal association between the gunshot wound and the aneurysm.     

Transcatheter closure of ruptured sinus of valsalva aneurysm

Congenital sinus of Valsalva aneurysm is a rare cardiovascular anomaly. Rupture of sinus of Valsalva (RSOV) is a rare occurrence, occurring in 10–20% of cases. Rupture into right atrium is further a rare occurrence. The rupture is usually a catastrophic event presenting as acute onset congestive cardiac failure in an apparently healthy individual in the 2^nd or 3^rd decade of life. Surgery used to be the mainstay of treatment of RSOV aneurysm, however, in past few years several reports of transcatheter closure of RSOV have come into light. We report a patient, who underwent device closure with a Duct Occluder device. Device was manipulated and deployed with an angle so as to prevent aortic valve dysfunction. © 2010 Wiley‐Liss, Inc.     

Transcatheter closure of ruptured sinus of valsalva aneurysm

Percutaneous transcatheter closure of ruptured sinus of valsalva aneurysm was attempted in eight patients between January 1995 and March 2003 as an alternative strategy to surgery as this technique at present is an accepted therapeutic modality for various intracardiac defects. The age range was 14-35 years, all were male, seven in symptomatic class III and one in class IV on medical treatment. Two-dimensional and color Doppler echocardiography revealed rupture of an aneurysm of right coronary sinus into right ventricle in five and noncoronary sinus into right atrium in three and none had associated ventricular septal defect. The echo estimated size of the defect was 7-12 mm. On cardiac catheterization left ventricular end-diastolic pressure ranged from 20 to 40 mmHg and the calculated Qp/Qs ratio was 2-3.5. In all patients the defect was crossed retrogradely from the aortic side and over an arterio-venous wire loop after balloon sizing, devices were successfully deployed by antegrade venous approach (Rashkind umbrella device in two and Amplatzer occluders in six, which included Amplatzer duct occluder in five and Amplatzer septal occluder in one). One patient who had residual shunt developed hemolysis on the next day and was taken up for reintervention. That patient continued to have intermittent hemolysis and was sent for surgical repair. On follow-up (2-96 months), there was no device embolization, infective endocarditis, and aortic regurgitation. One patient died of progressive congestive heart failure while other six are asymptomatic. These data highlight that transcatheter closure is feasible and effective, especially safe with the available Amplatzer devices. Definitely, it has the advantage of obviating open heart surgery but complete occlusion is mandatory to prevent hemolysis and infective endocarditis.     

Rupture of the sinus of valsalva into the pulmonary artery

Congenital aneurysms of the sinus of Valsalva are rare lesions that can rupture into any cardiac chamber, due to the central position of the aortic root. Rupture into the pulmonary artery, however, is very rare. We encountered an 18-year-old girl with rupture of the right coronary sinus into the pulmonary artery. During surgical correction, the girl was also found to have a small outlet ventricular septal defect, which was obscured by multiple hypertrophied septal trabeculae in the right ventricular outflow tract.     

Ruptured left sinus of valsalva aneurysm to right atrium

A 6-year-old child presented with left sinus of Valsalva aneurysm opening in right atrium. Origin of sinus of Valsalva from left aortic sinus and its opening into right atrium is extremely rare. The anomaly was corrected surgically by patch closure at the aortic end. Follow-up echocardiography did not reveal any residual shunt in right atrium.     

An unusual cause for unruptured sinus of valsalva aneurysm

Sinus of valsalva aneurysm is considered to be one of the rarest complications of inflammatory aortitis. Herewith, we are reporting a young male patient who presented to us with severe aortic regurgitation. On evaluation, he was found to have unruptured sinus of valsalva aneurysm. CT angiography and magnetic resonance imaging have shown value in the diagnosis of sinus of valsalva aneurysm.     

Ruptured sinus of valsalva aneurysm in a pregnant woman

A ruptured sinus of Valsalva aneurysm is a rare cardiac anomaly, usually of a congenital nature. There are few documented cases of this condition during pregnancy, which renders unclear the therapeutic options. We report the case of a 35-year-old pregnant woman who presented at the hospital for cardiac evaluation due to the presence of a heart murmur. The patient was asymptomatic and in her 4th month of pregnancy. Transthoracic echocardiography revealed a ruptured sinus of Valsalva aneurysm and a fistula to the right ventricle with evidence of a continuous left-to-right shunt.     

Rupture of a right sinus of valsalva aneurysm into the right ventricle during vaginal delivery: a case report

A case is reported of a right sinus of Valsalva aneurysm rupture into the right ventricle during vaginal delivery in a 34-year-old healthy woman in her third pregnancy. Pregnancy was carried to term and a healthy baby was delivered vaginally. On day 7 following vaginal delivery she was admitted to hospital for dyspnea and cough, with clinical signs of severe heart failure. The diagnosis of the right sinus of Valsalva aneurysm rupture into the right ventricle was established by transthoracic and transesophageal echocardiography. Clinical recognition and early echocardiographic diagnosis followed by immediate surgical repair proved lifesaving in our patient.     

Transcatheter closure of a ruptured sinus of valsalva aneurysm.

BACKGROUND: Ruptured sinus of Valsalva aneurysms (RSVA) can be associated with ventricular septal defects or isolated lesions. Surgical repair has been the traditional treatment of for the RSVA. The results of transcatheter closure of the RSVA in 4 patients are reported. METHODS AND RESULTS: From 2003 to 2004, 4 patients (2 males and 2 females) aged from 18 years to 47 years with RSVA were identified. The diagnosis of RSVA was made based on a combination of several imaging modalities. The drainage site of the RSVA was right ventricle in 2, and right atrium in remaining 2. All patients underwent general anesthesia and transesophageal echocardiographic (TEE) monitoring during the procedure. Transcatheter closure with an Amplatzer duct occluder was attempted in all 4 patients. The size of the Amplatzer duct occluder selected was up to 2 mm larger than the maximal diameter of the aortic opening site of the RSVA as measured on TEE images. The attempt to deploy an Amplatzer duct occluder was successful in 3 and a Gianturco coil was deployed in 1. Follow-up (3-18 months) echocardiography showed neither residual shunt nor aortic regurgitation in any of the patients. CONCLUSION: The transcatheter technique is a safe alternative in the treatment of RSVA; however, a longer follow-up is mandatory.