Ruptured Sinus of Valsalva Found Incidentally in a Patient with Tetralogy of Fallot

Abstract

Ruptured sinus Valsalva aneurysm (SVA) in a patient with tetralogy of Fallot is a very rare cardiac complication. This report describes the case of a 10-year-old Hispanic girl who presented to her cardiologist for a routine transthoracic echocardiography (TTE), which showed a ruptured sinus of Valsalva, with left-to-right shunting noted from the right cusp to the right ventricular outflow tract and pulmonic insufficiency. The patient underwent right and left heart catheterization with transesophageal echocardiography guidance and closure of the ruptured sinus of Valsalva with an Amplatzer muscular ventricular septal defect (VSD) device. Later that day, after the procedure, it was noted that the patient had a recurrence of continuous murmur. The TTE did not visualize the Amplatzer VSD device, and chest X-ray showed that the device had embolized to the right pulmonary artery. The device was successfully retrieved percutaneously. The patient returned 2 weeks later for surgical repair of a ruptured SVA and pulmonary valve replacement, which went well. This case report is the first to describe congenital rupture of a Valsalva aneurysm in a patient with tetralogy of Fallot. The conventional treatment for a ruptured sinus of Valsalva is surgical, although transcatheter approaches have been used increasingly in recent years.

Ruptured sinus of Valsalva aneurysm with tetralogy of Fallot in an adult

“…Although RSOV existed in our patient, he was relatively asymptomatic because of the associated TOF which limited the increased runoff into the pulmonary circuit. Only four similar case reports exist in the literature …”

“…A ruptured sinus of Valsalva aneurysm (RSOV) is frequently associated with other congenital cardiac defects, particularly ventricular septal defects (VSD) . Its association with tetralogy of Fallot (TOF) is rare . We report a 32‐year patient with RSOV and TOF.…”

Ruptured aneurysm of the right coronary sinus of Valsalva in a child with Down syndrome

“…6 Rupture of an aneurysm of sinus of Valsalva has been observed in Noonan syndrome, 22q11 microdeletion syndrome, and in tetralogy of Fallot. 7,8 The occurrence of a ruptured aortic sinus aneurysm in tetralogy of Fallot, 22q11 microdeletion syndrome, and Down syndrome may be a reflection of a joint underlying connective tissue abnormality. Progressive aortic root and ascending aortic dilatation is typical for syndromes such as Marfan, Loeys-Dietz, Ehlers-Danlos, and Turner syndrome, 9 but may be found also in Noonan syndrome or chromosome 22q11 deletion syndrome.…”

Assessment of the structure and function of the aorta by echocardiography

“…Over half occur in association with ventricular septal defects, but they may present as isolated lesions; 14 association with other CHD has also been described. [15][16][17] Echocardiography allows the diagnosis of these lesions with a high degree of sensitivity and specificity. 14 A sinus of Valsalva aneurysm is typically seen as a thin saccular structure arising from the aortic sinus just superior to the aortic valve annulus.…”

“…– 13 They may be congenital or occur as a complication of diseases such as Marfan syndrome or endocarditis. Over half occur in association with ventricular septal defects, but they may present as isolated lesions; 14 association with other CHD has also been described 15 17 Echocardiography allows the diagnosis of these lesions with a high degree of sensitivity and specificity 14 .…”