Sinus of Valsalva aneurysm
Sinus of Valsalva aneurysm
Description, Causes and Risk Factors:
A congenital thin-walled out pouching with an entirely intracardiac course, usually in the right or non-coronary sinus, that may rupture into the right, or rarely, the left heart chambers to form an aortocardiac fistula.
Sinus of Valsalva aneurysms are rare cardiac anomalies which may be acquired or congenital, most commonly involving the right or non-coronary sinuses. The congenital aneurysms are more common and often caused by weakness at the junction of the aortic media and the annulus ?brosus. Acquired aneurysms are caused by conditions affecting the aortic wall, such as infections (syphilis, bacterial endocarditis, or tuberculosis), trauma, or connective tissue disorders (CTD). Unruptured aneurysms are usually found incidentally during diagnostic studies.
In most cases, the aneurysm arises from the right coronary cusp, which is normally adjacent to the right ventricular outflow tract. These aneurysms may be congenital, due to a deficiency in the muscular and elastic tissues at the base of the aorta, or acquired after infective endocarditis, atherosclerosis, or aortic dissection. Unruptured aneurysms of the right coronary cusp have been found to cause pulmonary insufficiency (by distorting the pulmonary valve leaflets), myocardial ischemia (by obstructing the right coronary ostia), conduction disturbances, and tricuspid incompetence. A coexisting ventricular septal defect has also been reported. In such an event, the ventricular septal defect is situated immediately below the aneurysm. This association has been explained by the structural abnormality in the distal bulbar septum.
Aneurysms of the sinus of Valsalva are very uncommon, with an incidence ranging from 0.1 to 3.5% of all congenital heart defects. Such aneurysms account for only 0.14% of all open heart surgical procedures. So far only 2 cases have been reported in the literature that presented with involvement of ?2 sinuses.
A good prognosis depends on early diagnosis and prompt surgical intervention. A bicameral operative approach is probably the best option in such patients.
If unruptured, this type aneurysm may be asymptomatic and therefore go undetected until symptoms appear or medical imaging is performed for other reasons. A ruptured aneurysm typically leads to an aortocardiac shunt and progressively worsening heart failure.
An aneurysm of the aortic sinus may rupture due to infective endocarditis involving the aortic wall and tertiary-stage syphilis.The manifestations appear depending on the site where the sinus has ruptured. For example, if the sinus ruptures in a low pressure area like the right atrium or right ventricle then a continuous type of murmur is heard. The murmur is located in the left parasternal region mainly confined to the lower sternum. It is also accompanied by a superficial thrill. A ruptured Sinus of Valsalva abscess represents a surgical emergency.
More commonly, sinus of Valsalva aneurysms 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.
Previously healthy individuals with small perforationsof SVA may present with an asymptomatic continuousmurmur, a hallmark of a ruptured aneurysm into the rightside of the heart. Hope described `a very loud, super?cial sawing murmur prolonged continuously over the?rst and second heart sounds. The classic murmur isdescribed in 40-95% patients presenting with an aneurysm, as a loud, machinery-like, continuous murmur,varying in intensity with systole and diastole, best heardat the base of the heart. The murmur does notpeak around the second heart sound, as does the murmurof patent ductus arteriosus. The intensity of the murmurmay be diminished around the second heart sound, onlyto increase again in diastole, creating a `to-and-fro' cadence.
The electrocardiogram usually shows voltage criteriafor left ventricular hypertrophy and ST-T wave abnormalities. Chest X-ray may reveal a bulge to the rightof the caval shadow in the postero-anterior view (PA view) and appear as an excessive anterior-superior bulge in the leftanterior oblique view.
The mainstay of treatment for ruptured SVA is surgical intervention, since prognosis of patients without urgent surgical repair is very poor. Early aggressive treatment is recommended for ruptured SVA in order to prevent endocarditis or enlargement of the lesion, which might necessitate more extensive repair. Because of recent advances in diagnostic technique, the number of patients undergoing surgery has been increasing, including patients with unruptured aneurysms.
In the current surgical era, dual chamber exposure has been favored by most surgical centers. A combined approach, in which both the involved chamber and aortic root are opened, allows meticulous closure of the rupture, better evaluation of coexistent anomalies, avoidance of distortion of the aortic valve cusps, and reduction in the incidence of late aortic insuf?ciency. In addition, aortotomy allows selective perfusion of the coronary ostea in larger patients. Several investigators have suggested that direct closure with sutures may be associated with a higher incidence of aneurysm recurrence. Many centers have been using patch closure for all SVA repairs, regardless of size, to avoid tension on the annulus, especially in complex cases.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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