Baylor University Medical Center Proceedings July 2017 - 301

Aorto-right atrial fistula after Bentall repair
Charles E. Howard, MD, Carlos E. Velasco, MD, Christina P. Roullard, OMS, and Aldo Rafael, MD

We describe a man with the Marfan syndrome and a prior ascending
aortic aneurysm resection who presented with knee pain and concern of
endocarditis. Transesophageal echocardiogram showed no vegetations,
and computed tomography angiogram of the heart showed a possible
pseudoaneurysm. Cardiac catheterization and aortogram revealed the
diagnosis of an aorto-right atrial fistula, which was then operatively repaired. This case highlights the role that cardiac catheterization with
aortogram can play in the detection of aorto-atrial fistula.

CASE REPORT
A 49-year-old morbidly obese (body mass index 41 kg/m2)
white man with the Marfan syndrome who previously had an
ascending aneurysm repair with Bentall procedure at age 38 and
aortic valve replacement (St. Jude Medical mechanical prosthesis),
gastric bypass, and bilateral knee replacements presented with
left knee pain. Three weeks prior to admission, he developed
worsening left knee pain associated with subjective fever. The left
knee was painful with motion, swollen, and erythematous. Due
to concern of endocarditis, a transthoracic echocardiogram was
obtained, which showed no evidence of vegetation. A transesophageal echocardiogram was then obtained, which showed a possible
ascending aortic pseudoaneurysm. Computed tomography of the
heart revealed a thin linear density within the lumen of the left
and right sides of the ascending aorta along the course of the
graft, concerning for possible pseudoaneurysm, and also depicted
possible dilation of the left main coronary artery. A cardiology
consult was requested, and physical examination revealed loud
systolic and diastolic murmurs. The patient underwent right and
left heart catheterization, which showed a mean pulmonary capillary wedge pressure of 22, pulmonary artery pressure of 55/22/32,
right ventricular pressure of 55/10, and mean right atrial pressure
of 10 mm Hg. Two separate oxygen saturation runs were unable
to show any clear evidence of an intracardiac shunt; however, an
ascending aortogram (left anterior oblique with mild cranial angulation projection) discovered the presence of a ﬁstula between the
ascending aorta and right atrium (Figure 1). The patient's coronary
arteries were normal; no left main abnormalities were identiﬁed.
The patient was taken for repeat repair of the ascending aorta
and closure of the aorta-right atrial ﬁstula. Intraoperative transesophageal echocardiogram showed the ﬁstula with the anterior
Proc (Bayl Univ Med Cent) 2017;30(3):301-302

Figure 1. Ascending aorta angiogram showing a shunt from the aorta to the
right atrium (arrow).

surface of the ascending aortic graft communicating to the right
atrium (Figure 2). There was a pseudoaneurysm of the aortic root
with evidence of old thrombus adhered to the aortic wall. A previously implanted composite graft was explanted, and the aortic
root was reconstructed with an aortic homograft. The right and
left coronary buttons were reimplanted and a distal anastomosis
was created between the homograft and the ascending aorta. The
aorto-right atrial ﬁstula was identiﬁed and successfully closed with
a bovine pericardium Peri-Guard patch. The patient was weaned
oﬀ cardiopulmonary bypass and eventually discharged home.
Although initially concerned about endocarditis, the patient remained without a recorded fever or leukocytosis, and all cultures
returned negative. The patient was doing well as of last follow-up.
DISCUSSION
Since ﬁrst described in 1924, aorto-atrial ﬁstulas have been reported in association with acute aortic dissection, pseudoaneurysm,
From Baylor University Medical Center at Dallas, Baylor Heart and Vascular
Hospital, and Texas A&M College of Medicine, Dallas, Texas.
Corresponding author: Carlos E. Velasco, MD, 3600 Gaston Avenue, Wadley
Suite 851, Dallas, TX 75246 (e-mail: Carlos.Velasco@BSWHealth.org).
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