Discussion
Sinus of Valsalva aneurysm is a dilation of the aortic wall between the aortic valve and the sinotubular junction that is caused by the lack of continuity between the middle layer of the aortic wall and the aortic valve.1 Cases of SVA are rare cardiac anomalies with prevalence of 1% in patients undergoing open-heart surgery.2 Between 65% and 85% of SVA cases originate from the right coronary sinus, 10% to 20% from the noncoronary sinus, and < 5% from the left coronary sinus.3
Sinus of Valsalva aneurysm is usually congenital, although cases associated with syphilis, bacterial endocarditis, trauma, Behçet disease, and aortic dissection have been reported. Structural defects associated with congenital SVAs include ventricular septal defect, bicuspid aortic valve, and aortic regurgitation. It is less commonly associated with pulmonary stenosis, coarctation of the aorta, patent ductus arteriosus, tricuspid regurgitation, and atrial septal defects.
The most common complication of the SVA is rupture into another cardiac chamber, frequently the right ventricle (60%) or RA (29%) and less frequently into left atrium (6%), left ventricle (4%), or pericardium (1%).1 Patients with ruptured SVA mainly develop dyspnea and chest pain, but cough, fatigue, peripheral edema, and continuous murmur have been reported.1
Atrial septal aneurysm is an uncommon finding in adults, with an incidence of 2.2 % in the general population, and it is often associated with atrial septal defect and PFO.1,4 Although ASA formation can be secondary to interatrial differences in pressures, it can be a primary malformation involving the region of the fossa ovalis or the entire atrial septum.4 Atrial septal aneurysm may be an isolated anomaly, but often is found in association with other structural cardiac anomalies, including SVA and PFO.4,5
Conclusion
Although coexistence of SVA and ASA has been reported previously, the case reported here, a ruptured noncoronary SVA that was associated with a large ASA and a PFO, has not been previously documented in the English literature. This patient’s anomalies are most likely congenital in origin. Progressive dyspnea and chest pain in the presence of a continuous loud murmur should raise the suspicion of ruptured sinus of Valsalva. Although no significant aortic regurgitation was noted on echocardiography, the pistol shot sound heard over the femoral artery was believed to be due to the rapid diastolic runoff into the RA through the ruptured SVA.
The significant increase in the RA pressure made the ASA and PFO more prominent. A TEE, left and right heart catheterizations with shunt study are vital for the diagnosis of SVA. If left untreated, SVA has an ominous prognosis. Surgical repair of ruptured SVA has an accepted risk and good prognosis with 10-year survival rate of 90%, whereas the mean survival of untreated ruptured SVA is about 4 years.6,7 Hence, the patient in this study was referred to a tertiary care center for surgical intervention.