A 41 year old man was admitted with an eight day history of exertional dyspnoea and peripheral oedema. Clinical findings were consistent with right ventricular failure. Echocardiogram showed a ruptured aneurysm of the right sinus of Valsalva protruding into the right ventricular outflow tract (fig 1A). The aneurysm induced right ventricular to pulmonary trunk obstruction with a peak Doppler velocity of 3.4 m/s. The aortic valve was quadricuspid, with mild to moderate aortic regurgitation (fig 1B). A moderate sized subarterial ventricular septal defect was seen, with left to right shunt. Both ventricles were enlarged. The findings were confirmed during surgery. Parasternal short axis echocardiogram showing quadricuspid aortic valve.

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Figure 1

(A)Parasternal short axis view echocardiogram at the level of pulmonary artery (PA) showing the location of the aneurysmal sac (An) obstructing the right ventricular outflow tract (RVOT) immediately below the pulmonic valve (arrow). (B) Parasternal short axis view showing quadricuspid aortic valve.

Congenital sinus of Valsalva aneurysms have been reported in association with other conotruncal malformations including bicuspid aortic valve and bulbar interventricular septal defects. To our knowledge, no case of sinus of Valsalva aneurysm with right ventricular outflow tract obstruction associated with quadricuspid aortic valve has ever been reported.