A Rare Case - Sinus of Valsalva Aneurysm in Tetralogy of Fallot from West
African Patient
The overall incidence of the Sinus of Valsalva aneurysm (SOVA) is 0.09% in the general population and comprise 0.1% to 3.5% of all congenital cardiac defects. It is associated with only 2% of Tetralogy of Fallot (TOF) patients, thus making this combination of unruptured SOVA with TOF unique. We present the case of a 4½- year-old West African boy who developed exertional dyspnea and frequent squatting episodes since the age of ten months. He was not having any other major medical/ genetic history or phenotypically syndromic features. Preoperative diagnosis was confirmed with Trans Esophageal Echocardiography (TEE) features of TOF with SOVA arising from right coronary cusp (RCC) bulging but not distorting Right Ventricular Outflow Tract (RVOT). The narrowest portion of the aneurysm being 0.65 cm. Postoperative Adequacy of TOF repair was demonstrated by ruling out residual Ventricular Septal Defect (VSD), morphological distortion as stenosis/ regurgitation across both Left Ventricular Outflow Tract (LVOT) and RVOT. The measurements of RVOT and LVOT at different levels were compared with the Z- scores provided by the Detroit data. TEE was useful not only in pointing out the adequacy of repair but also predicted not requiring additional intervention especially to aortic valve in the near future.