Case Presentation

A 67-year-old male was asymptomatic until 3 years prior to this evaluation when he began experiencing precordial pain provoked by exercise and presyncopal episodes, which became more frequent until virtually any physical activity became impossible to carry out. In addition, the patient presented with symptoms of right-sided congestive heart failure (jugular venous distention, hepatomegaly and ascites). Physical examination disclosed a grade IV/VI systolic murmur in the 2^nd left intercostal space and a holosystolic III/VI murmur in the 5^th left intercostals space that increased with inspiration. The ECG demonstrated atrial fibrillation and complete right bundle branch block with signs of ischemia in the right precordial leads. The chest x-ray was normal. Transthoracic echocardiographic images showed aneurysmal dilatation of the aortic right sinus of Valsalva (parasternal long axis view) that measured 78 x 80 mm with spontaneous echo-contrast consistent with stagnant flow within the aneurysm (Figure 1).

Figure 1

Figure 1: Parasternal long axis view showing a gigantic dilatation of the right sinus of Valsalva with thin walls and spontaneous echo contrast within the 78 x 78 mm aortic aneurysm.

The short axis views showed that the dilatation of the sinus of Valsalva was associated with right atrial and right ventricular enlargement and right ventricular outflow tract obstruction with a peak instantaneous pressure gradient of 84 mm Hg by Doppler techniques (Figure 2).

Figure 2

Figure 2: Short axis view showing the right ventricular outflow tract obstruction provoked by the sinus of Valsalva aneurysm, with a peak instanteous pressure gradient of 84 mm Hg.

The diagnosis of sinus of Valsalva aneurysm without rupture was confirmed by cardiac catheterization and aortography (patent right coronary ostium ;Figure 3).

Figure 3

Figure 3: This is a 60 left anterior oblique view of the aortography showing the opacification of the sinus of Valsalva aneurysm by contrast

The aneurysm was surgically repaired (aorto-cameral access) with Dacron patch closing of the aneurysm’s neck and plication of the aneurismal sac. It was not necessary to replace the aortic valve. The postoperative follow up was uneventful and subsequent echocardiographic images showed a satisfactory closure of the neck of the aneurysm and no residual obstruction in the right ventricular outflow tract (Figure 4).

Figure 4

Figure 4: Echocardiographic image showing the Dacron patch (arrow) following closure of the neck of the aneurysm as part of the aorto-cameral surgical correction approach.

Discussion

Congenital sinus of Valsalva aneurysms seem to result from the lack of continuity of the aortic media at the ring of the aortic valve.¹ These aneurysms involve the right aortic sinus 69% of the time, the non-coronary aortic sinus in 26% of patients and the left aortic sinus in 5% of cases. Rarely, more than one sinus is involved in the same patient.² These aneurysms can be associated with other anomalies, such as ventricular septal defects, aortic regurgitation, patent foremen, ovale and coronary artery malformations. Because of their intracardiac locations, these aneurysms exhibit expansion and rupture involving sites in relation to the adjacent structures.⁴ In the case of right aortic sinus, the aneurysms project toward the right ventricle and right atrium, whereas those involving the non-coronary sinus abut the left atrium. Rarely, the aneurysm can protrude into the right ventricular outflow tract causing obstruction.⁵ The most frequent complication of sinus of Valsalva aneurysms is rupture into the cardiac chambers (usually right-sided chambers), which commonly occur in men of ages 30 to 40 in whom congestive heart failure (acute on gradual) ensues. Development of fistulae is rarely associated with symptoms. Other complications include infection, right ventricular outflow tract obstruction like in our patient, extension of the aneurysm into the ventricular septum leading to complex dysrrythmias, conduction abnormalities and thrombosis in situ that can lead to coronary ostia obstruction.⁶

Echocardiography provides a useful diagnostic approach for detection of sinus of Valsalva aortic aneurysms without rupture in order to define possible complications, prognosis, and is also helpful for clinical follow-up.^7,8,9