Aorta-right atrial tunnel is a rare entity characterized by a iatrogenic vascular fistula, which connects aortic root and right atrium. It is frequently associated with congestive heart failure (HF).
A 54 y female with an history of atrial septal defect (ASD) type ostium secundum closure and a recent mitral and tricuspid annuloplasty surgery, presents to the hospital with significant decrease in exercise tolerance and progressive shortness of breath.
At the presentation, physical examination was significant for tachycardia, continuous murmur over precordium and lower extremity edema. ECG showed right ventricular pressure overload with repolarization abnormality.
TTE showed moderate systolic flattening of the septum and dilated right sections. Non coronary cusp of aortic valve demonstrated a perforation extending to the right atrium (RA).
ETE confirmed the connection between non-coronary aortic cusp and RA, furthermore revealed unkown sinus venosus type ASD. TC angiography showed a marked dilation of the pulmonary artery trunk (egg and banana sign) and a connection between aorta and RA.
She was treated with surgical closure of aorta- right atrial tunnel and ASD through detached and pledget stitch closure. Follow-up reveals significant functional recovery of shortness of breath and exercise tolerance.
Aorta– right atrial tunnel is a rare entity characterized by a vascular fistula, which connects one of the sinuses of Valsalva and the RA. It is a rare complication of tricuspid plastic surgery. It is classified according to its relation to the aorta to anterior or posterior.
The clinical presentation ranges from asymptomatic continuous murmur to the symptoms of right sided HF. In our case, the presence of ASD accelereted and worsened the manifestations of right HF. It is usually diagnosed by TTE or ETE, TC angiography and aortography.
The treatment of this condition is important even in asymptomatic cases due to the risk of aortic valve dysfunction, likely regurgitation, congestive right sided HF followed by left sided HF, calcification, infective endocarditis, aneurysmal dilatation and pulmonary hypertension.
The possibility of complications of aorta-right atrium fistula necessitates early therapy, even in asymptomatic patients or those with a hemodynamically insignificant shunt. Available treatment is surgical closure. It has a high mortality rate if it is not diagnosed adequately and surgically treated without delay.