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Aorto-Atrial Fistulas
A Contemporary Review
Elizabeth A. Fierro, OMS-II,* Rutuja R. Sikachi, MBBS, DNB,† Abhinav Agrawal, MD,‡ Isha Verma, MD,§
Marcin Ojrzanowski, MD,¶ and Sonu Sahni, MD*‡
FIGURE 1. Potential causes of aorto-atrial fistulas (AAF). AVR indicates aortic valve replacement; MVR, mitral valve replacement;
TIPS, transjugular intrahepatic portosystemic shunts.
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FIGURE 2. Transesophageal echocardiogram in short axis (A) and long axis (B) views, showing the proximity of the left ven-
tricular outflow tract, aortic valve, and ascending aorta to the right and left atrium. C, Computed tomography of the chest with
contrast showing the proximity of the left ventricular outflow tract and ascending aorta to the right and left atrium. Ao indicates
aorta; AV, aortic valve; LA, left atrium; LV, left ventricle; LVOT, left ventricular outflow tract; RA, right atrium; RV, right ventricle;
RVOT, right ventricular outflow tract.
right atrium. We have observed there to be a propensity for ADs to requiring an expeditious recognition and aggressive management.
fistulize into the right atrium over the left. Out of 17 cases of AAF Risk factors for IE include rheumatic heart disease, congenital heart
related to AD, 13 (76.5%) affected the right atrium. Our observa- disease, intravenous drug use, sclerotic valve disease, prosthetic dis-
tion is similar to that of Lindsay,22 in which the right atrium was ease, and nosocomial infection.39 When the infection spreads beyond
the receiving chamber in 75% of cases. This is thought to be due to vascular structures, it can lead to periannular complications, such as
the anatomic relationship between the ascending aorta and the right AAFs. It has been reported that the occurrence of AAF in the setting
atrium. The right atrium abuts the right lateral and posterior aspect of of IE is 1–2%.40
several centimeters of the ascending aorta beginning at the sinus of In our search of the literature, we identified 29 cases of AAF
Valsalva.22 In addition, AD most frequently begins on the right lateral caused by IE, including 18 males and 11 females, although there is
aspect of the aortic wall, with the initial injury to the intima occur- no perceived gender bias in IE. We found the average age of AAF
ring on the right side, making the right atrium most vulnerable.38 due to IE to be 43.6 ± 3.1 years, with older patients having a history
Management of AAF in the setting of AD is often surgical. of prior cardiac events or surgeries and younger patients more com-
Simultaneous AD repair is performed along with closure of the fis- monly having infectious causes or a history of intravenous drug use.
tula. The technique of AD repair and closure is determined on a case- The cases of AAF associated with IE are outlined in Table 4.2–4,41–66
by-case basis. The most common bacteria associated with AAF in the setting of
IE is the Staphlococcus species, which has been reported in up to 58% of
Infective Endocarditis cases,67 followed by Streptococcus species in 28%, Enterococcus species
IE is a spectrum of diseases that results in infection of internal in 7%, and 7% of cases being polymicrobial.41 In our review of cases,
structures of the heart. IE may be caused by a variety of organisms, we found a similar distribution of causative organisms, which have been
TABLE 2. Cases of Aorto-Atrial Fistulas in the Setting of Valve Replacement Surgeries
Author Age (Sex) Type of Valve Replacement Atrium (L/R) Intervention Outcome
Aoyagi et al16 57 M AVR R Surgical repair Successful
Tayama et al17 58 F AVR and dissection R Surgical repair Successful
Badak et al15 49 F AVR R Surgical repair (Goretex patch and atrial sutures) Successful
Ananthasubramaniam et al18 66 M AVR L Surgical repair Successful
Menon et al14 73 F Rigid tricuspid annuloplasty. R Surgical repair Successful
Odaro et al19 79 M AVR R No intervention Patient died
Ahmad et al9 71 M AVR L Surgical repair Successful
Yesin et al13 41 F MVR L Surgical repair with sutures and AVR Successful
Raut et al20 48 M MVR L Surgical repair with sutures and AVR Successful
Luc et al12 70 F AVR Surgical repair Successful
Alkhouli et al10 84 M AVR R Transcatheter closure with ADO-III occluder Successful
AVR indicates aortic valve replacement; MVR, mitral valve replacement.
shown in Table 5. Despite the heterogeneity of the causative organisms, the spread of abscesses and fistula formation, making fistulas in this
the nature of the pathogen has not been shown to affect prognosis.68 area common when IE is already present.43 Previous literature states
Often, IE originates in the valvular structures of the heart. that periannular abscesses have been noted in up to 80% in patients
Spread from the affected valve to local tissue is often the initial step in with aortic valve endocarditis,70 whereas intracardiac fistula forma-
the pathologic cascade leading to AAF formation. Spread to the local tion is seen in 14% of patients.69 However, in our search, we found
tissue may result in abscess formation, leading to local inflammation that 19 (65.5%) cases of AAF cases had associated abscess formation.
and degradation of tissue. When the abscess ruptures, it erodes the The treatment for AAF caused by IE must be aggressive and
valve and leads to a fistula into the nearby cardiac chamber, such as timely to avoid its lethal consequences. Treatment includes broad-
the atrium.42 Due to the anatomical considerations, fistula formation spectrum intravenous antibiotics and surgical correction of the fistu-
most commonly occurs between the aorta and the right atrium.5 Our lous connection. Fistulas lead to a very high rate of complications,
literature review revealed that in AAF caused by IE, there were 15 with more than 60% of patients developing significant heart failure
(51.7%) cases with fistula formation to the right atrium. and more than 40% ending in death.52 Surgical mortality is very high,
Paravalvular abscess formation is seen more commonly in approximately 40%.5 In our search, 8 (27.5%) cases ended in patient
prosthetic valve endocarditis than in native valve endocarditis.3 In mortality, most of which were due to advanced disease at presenta-
prosthetic valves, the bacteria first begin at the prosthetic cuff and tion. Factors associated with adverse outcomes include septic shock,
then invade the outside apparatus, resulting in valvular dehiscence paravalvular leakage, hemodynamic instability, and congestive heart
and abscess formation. Endocarditis is common after prosthetic failure. The Amplatzer plug technique allows for percutaneous clo-
valve implantations, occurring in 2–4% of patients.69 In our search, sure of fistulous connections, as previously mentioned.10,55
we found 9 (31.3%) of AAF cases in the setting of IE to be pros-
thetic valves. An example has been shown in Figure 3A and B.
Infection is more capable of spreading when there is a lack of PRIMARY CAUSES
vascularization, leading to a paucity of immunological mediators to
mount an immune response. The “mitral-aortic intervalvular fibrosa” Genetic and Connective Tissue Disorders
is the junctional zone between the mitral and aortic valve annulus. Due Although uncommon, some genetic causes and connective
to the avascular nature of this area, it provides very little resistance to tissue disorders related to AAF have been documented. Congenital
140 | www.cardiologyinreview.com © 2017 Wolters Kluwer Health, Inc. All rights reserved.
FIGURE 3. Transesophageal echocardiogram in short axis (A) and long axis (B) views, showing an extensive aortic root abscess
around a bioprosthetic aortic valve, forming a fistulous communication to left atrium. ABSC indicates abscess; AML, anterior
mitral leaflet; BPAV, bioprosthetic aortic valve; LA, left atrium; MASS, mass in LA; LV, left ventricle; RA, right atrium.
transcatheter closure of atrial septal defects,78–80 and other diagnostic such as catheter ablation should raise concern for an AEF. These
arterial and venous catheterizations.81,82 patients should have a prompt diagnostic study followed by imme-
In the case of AAF caused by cardiac stent implantation, stent diate surgical intervention.
struts are believed to cause friction against the atrial septum, lead-
ing to fistula formation.83 It is also believed that the fistula forma-
tion may occur during insertion of endoprosthesis.81 These fistulas CONCLUSIONS
have been documented in the setting of self-expanding prostheses AAFs represent a potentially life-threatening complication,
implanted to treat stenosis in the vascular anastomosis between the which, if not expeditiously treated, could lead to volume overload
suprahepatic veins and the inferior vena cava83 and in the creation of the heart and eventually death. Its diagnosis is often delayed due
of a transjugular intrahepatic portosystemic shunt.84 In these cases, to the nonspecific nature of presenting symptoms and only 50%
after the initial procedure, the patient usually presents with a new sensitivity for diagnosis on TTE. Diagnosis requires both radiologi-
continuous murmur and worsening congestive heart failure. Immedi- cal (TTE and imaging) and more invasive techniques such as TEE
ate surgical consultation is required to close the fistula. Management and cardiac catheterizations. Upon diagnosis, expeditious manage-
in iatrogenic causes of AAF requires removal of the offending proce- ment is necessary to reduce mortality. The management option often
dure or prosthesis and surgical intervention. With early detection and involves surgical intervention, especially if percutaneous options are
management, the prognosis is relatively good. not viable. Success is often achieved with the closure of AAFs, albeit
with the potential for postsurgical complications. It is important for
Other Considerations physicians to be aware of this possible pathologic entity, as the diag-
Iatrogenic causes of AAF often are a result of intravascu- nosis requires a high degree of suspicion.
lar cardiac procedures such as catheter ablation.76,77 In addition
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