Review Article
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*‡
Abstract: Aorto-atrial fistulas (AAFs) are a relatively rare, but potentially
life-threatening condition, where an anomalous connection forms between
the aortic structures and the cardiac atria. AAFs are most often the result of
an underlying condition concerning the cardiac structures. It may be congeni-
tal, secondary to conditions such as aortic dissection, infective endocarditis,
or valve replacement, or iatrogenic in nature. Secondary causes incite local
deterioration of cardiac wall integrity leading to formation of fistulous con-
nections, whereas iatrogenic causes are more traumatic in nature. Signs and
symptoms include those of volume overload and heart failure, with patients
often presenting with fever, regurgitative murmurs, cardiac chamber dilation,
and pedal edema. The diagnosis of AAFs requires a high degree of clinical
suspicion necessitating imaging techniques such as echocardiography, com-
puter tomography, and more invasive procedures. Management is dependent
on underlying conditions and include the use of antibiotics, percutaneous clo-
sure, and in many cases, open heart surgery. It is important for physicians
to be aware of this pathological condition to aid in timely management and
favorable outcomes. This review attempts to summarize the various causes
and clinical presentations of AAFs over the past decades.
Key Words: aorto-atrial fistula, aorta-cameral fistula, infective endocarditis,
aortic dissection, valvular disease, aortoesophageal fistula
(Cardiology in Review 2018;26: 137–144)
A orto-atrial fistulas (AAF) are a relatively rare but very seri-
ous condition, where an anomalous connection forms between
the aortic structures and the cardiac atria. Abnormal connections
between the aorta and the cardiac chambers were first reported in
1924 by Boyd as an incidental finding on autopsy.1 AAFs are most
often the result of an underlying condition concerning the cardiac
structures. It may be congenital, secondary to conditions such as aor-
tic dissection (AD), infective endocarditis (IE), or valve replacement,
or iatrogenic in nature. The conditions that may be associated with
fistula formation have been outlined in Figure 1. The exact incidence
and prevalence are unknown as diagnosis is often made postmortem.
Patients who develop AAF present with signs and symptoms per-
taining to the underlying cause, the most common being dyspnea, fever,
weakness, and chest pain. If not detected, AAF may lead to cardiac over-
load and eventual death. Diagnosis relies heavily on clinical suspicion
From the *Touro College of Osteopathic Medicine, New York, NY; †Department
of Anaesthesia, Deenanath Mangeshkar Hospital & Research Center, Pune,
Maharashtra, India; ‡Department of Pulmonary, Critical Care and Sleep Medi-
cine, Hofstra Northwell School of Medicine—Northwell Health System, New
Hyde Park, NY; §Division of Cardiology, Hartford Hospital, Hartford, CT; and
¶Department of Cardiology, Bieganski Hospital, Medical University of Lodz,
Łódź, Poland.
Disclosure: The authors declare no conflict of interest.
Correspondence: Sonu Sahni, MD, Department of Primary Care, Touro College
of Osteopathic Medicine, 230 W 125th Street. New York, NY 10027. E-mail:
sahni.sonu@gmail.com.
Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
ISSN: 1061-5377/18/2603-0137
DOI: 10.1097/CRD.0000000000000182
Cardiology in Review  •  Volume 26, Number 3, May/June 2018 www.cardiologyinreview.com  |  137
Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
and imaging modalities. AAFs are best visualized with echocardiog-
raphy (transthoracic [TTE] or transesophageal [TEE]). Ultimately,
a timely diagnosis and expeditious treatment are imperative to avoid
fatality.2 Management is dependent on the underlying cardiac condition
and suspected cause of fistula formation. Options may include percuta-
neous closure, minimally invasive surgery, or thoracotomy. Herein, this
review article attempts to create a consensus on disease characteristics,
causes, and various management strategies.
METHODS
A search was conducted of the National Library of Medi-
cine’s Medline/PubMed with the objective of identifying all articles
published in English language between January 1980 and July 2017
with “aorto-atrial fistula” or “aortoatrial fistula” in the title. Pseud-
onyms such as “aortocameral fistula” and “aortocavitary fistula”
were also included in our search. Combinations of medical subject
heading terms associated with AAF were also searched, including
“infective endocarditis,” “aortic dissection,” and “connective tissue
disease”. We mainly selected recent publications but did not exclude
any older works that were widely referenced. We also searched
the reference lists of all articles identified by this search strategy
and selected those we judged to be relevant. All pertinent reports
were retrieved and the relative reference lists were systematically
searched to identify any potential additional studies that could be
included. All data were accessed between January and June 2017.
Our comprehensive PubMed/Medline search revealed a total of
188 manuscripts, of which 104 were duplicates, not of the English
language, or not related to our focus and were excluded from our
review. This yielded a total of 84 manuscripts on AAFs that were
completely assessed and incorporated into this review.
SIGNS AND SYMPTOMS OF AAF
AAF causes shunting of blood from the aorta to either the
right or left atria due to a high pressure gradient. This leads to volume
overload, with subsequent failure of the right or the left ventricle.
The patients, thus, present usually with signs and symptoms of heart
failure. AAF can also commonly lead to a continuous murmur due
to the presence of a gradient between the aorta and the low pressure
atria in both systole and diastole.3 Patients with associated aortic val-
vular dysfunction may have a diastolic murmur due to aortic regur-
gitation. Patients with biventricular failure also demonstrate pedal
edema, anasarca, and congestive hepatopathy.4 The signs and symp-
toms most commonly associated with AAF are presented in Table 1.
DIAGNOSIS OF AAF
Diagnosing patients with AAFs can be challenging depending
on the size of the shunt. It requires a high degree of clinical suspi-
cion in patients with sudden onset of congestive heart failure symp-
toms who have a recent history of an invasive or surgical cardiac
procedure. The median duration of symptoms to echocardiographic
detection of fistulas is about 25 days.5 After clinical suspicion, a TTE
Fierro et al Cardiology in Review  •  Volume 26, Number 3, May/June 2018
FIGURE 1.  Potential causes of aorto-atrial fistulas (AAF). AVR indicates aortic valve replacement; MVR, mitral valve replacement;
TIPS, transjugular intrahepatic portosystemic shunts.
TABLE 1.  Signs and Symptoms Associated With Aorto-Atrial
Fistulas
Signs Symptoms
Murmurs: systolic, diastolic Shortness of breath
Fever Lethargy
Congestive heart failure Fever
Tachycardia Chills
Aortic regurgitation Pedal edema
Dilated left ventricle
Pulmonary edema
Congestive hepatopathy
is the initial choice for investigation.6 The limitation of TTE is its
inability to characterize early abscesses when their densities are simi-
lar to those of surrounding tissues. With a low sensitivity of detec-
tion, TTE is able to detect fistulous tracts in 50% of cases. However,
when combined with TEE, detection rates increase to 97% due to the
high pressure differences across the aorta and atria and the anatomi-
cal positions of the atria and esophagus, leading to easily detectable
fistula flow by use of continuous Doppler monitoring.7 Thus, TEE
provides a higher sensitivity and specificity and is the gold standard
for diagnosis of AAF. TEE also allows for optimal characterization
of the fistula tract, thus, providing precise anatomic information that
is extremely important for surgical planning. An example of TEE
displaying the proximity of the left ventricular outflow tract, aortic
valve, and ascending aorta to the right and left atrium are shown in
Figure 2A and B. After the diagnosis of the AAF, disease-specific
diagnostic measures can be taken to assess the underlying cause. For
example, in the setting of AD, computer tomography of the chest
with contrast, or rarely an aortography, may be performed.8 An exam-
ple of computer tomography of the chest with contrast displaying the
proximity of the left ventricular outflow tract and ascending aorta to
the right and left atrium is shown in Figure 2C. Cardiac catheteriza-
tion may also be utilized to determine atrial pressures and saturations
in a certain subset of these patients. These patients should also obtain
an electrocardiogram to look for underlying conduction defects.
SECONDARY CAUSES OF AAF
Valve Replacements and Surgeries
AAFs are rare but serious complications of aortic, mitral, or
tricuspid valve replacement and surgeries. The risk factors of AAF
138  |  www.cardiologyinreview.com © 2017 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
after valve replacement surgery include the presence of infected tis-
sue before the surgery, connective tissue abnormality, and oversized
prosthetics.9,10 During aortic valve surgery, several mechanisms of
injury have been proposed to help explain what leads to predispo-
sition to AAFs, including inadvertent injury to the membranous
septum during dissection below the noncoronary cusp, perivalvular
damage due to improper retraction, and excessive debridement of
calcium.11 Injury to the atrial wall during aortomy closure can also
lead to AAF.10 On the other hand, if a sutureless valve is implanted,
its self-expanding nature, combined with a predisposing weakness
of vasculature, such as a bicuspid aortic valve, can lead to an AAF
due to distortion of the aortic root.12 Similarly, in mitral and tricus-
pid valve surgeries, deep sutures or suture tracts can lead to a fistula
between aorta and the left atrium.13,14 Cases of AAFs caused by the
abovementioned surgeries are outlined in Table 2.9,10,12–20
Surgery is the standard of treatment for these kinds of AAF.
Surgical intervention consists of repairing the affected aortic seg-
ment, replacing prosthesis if the valve is destroyed, annular debride-
ment in the setting of abscess, and suture of the fistula.15 Alkhouli
et al10 successfully deployed an Amplatz Ductal Occluder-II device
for the closure of the AAF. Although a review of literature revealed
a number of transcatheter closures of AAF with excellent short-term
outcomes, no long-term data are available regarding the use of these
devices. A thorough assessment of the valve leaflet is essential before
the device is released. Other possible complications of a transcatheter
closure include device embolization, leading to stroke or coronary
artery obstruction.
Aortic Dissection
Dissection of the aorta is a potentially life-threatening condi-
tion that occurs due to a tear in the intimal layer of the aorta, causing
blood to flow into the media of the aorta, forcing the layers apart. The
vast majority of ADs originate in either the ascending aorta (65%),
the aortic arch (10%), or just distal to the ligamentum arteriosum in
the descending thoracic aorta (20%).21 Acute AD may lead to serious
complications, including rupture to neighboring structures, aortic
insufficiency, hemothorax, hemopericardium, and occlusion of the
major vessels originating from the aorta. Free rupture of AD into the
pleural and pericardial spaces often results in sudden cardiovascular
collapse and death. Another rare complication is fistulization to the
cardiac atria (right atrium, left atrium), which is a rare and often fatal
occurrence.22,23 Cases of AAF in the setting of AD have been outlined
in Table 3.8,17,24–37
Our review had revealed a total of 17 cases since 1980 that
describe fistula formation between the aorta and either the left or
Cardiology in Review  •  Volume 26, Number 3, May/June 2018 Aorto-Atrial Fistulas

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
fistulize into the right atrium over the left. Out of 17 cases of AAF
related to AD, 13 (76.5%) affected the right atrium. Our observa-
tion is similar to that of Lindsay,22 in which the right atrium was
the receiving chamber in 75% of cases. This is thought to be due to
the anatomic relationship between the ascending aorta and the right
atrium. The right atrium abuts the right lateral and posterior aspect of
several centimeters of the ascending aorta beginning at the sinus of
Valsalva.22 In addition, AD most frequently begins on the right lateral
aspect of the aortic wall, with the initial injury to the intima occur-
ring on the right side, making the right atrium most vulnerable.38
Management of AAF in the setting of AD is often surgical.
Simultaneous AD repair is performed along with closure of the fis-
tula. The technique of AD repair and closure is determined on a case-
by-case basis.
Infective Endocarditis
IE is a spectrum of diseases that results in infection of internal
structures of the heart. IE may be caused by a variety of organisms,
requiring an expeditious recognition and aggressive management.
Risk factors for IE include rheumatic heart disease, congenital heart
disease, intravenous drug use, sclerotic valve disease, prosthetic dis-
ease, and nosocomial infection.39 When the infection spreads beyond
vascular structures, it can lead to periannular complications, such as
AAFs. It has been reported that the occurrence of AAF in the setting
of IE is 1–2%.40
In our search of the literature, we identified 29 cases of AAF
caused by IE, including 18 males and 11 females, although there is
no perceived gender bias in IE. We found the average age of AAF
due to IE to be 43.6 ± 3.1 years, with older patients having a history
of prior cardiac events or surgeries and younger patients more com-
monly having infectious causes or a history of intravenous drug use.
The cases of AAF associated with IE are outlined in Table 4.2–4,41–66
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
cases,67 followed by Streptococcus species in 28%, Enterococcus species
in 7%, and 7% of cases being polymicrobial.41 In our review of cases,
we found a similar distribution of causative organisms, which have been

© 2017 Wolters Kluwer Health, Inc. All rights reserved. www.cardiologyinreview.com | 139

Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

Fierro et al Cardiology in Review  •  Volume 26, Number 3, May/June 2018

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.

TABLE 3.  Cases of Aorto-Atrial Fistulas in the Setting of Aortic Dissection

Author Age (Sex) Prior Cardiac History Atrium (L/R) AAF Outcome
Hurley et al
24
75 M CABG × 3 R Surgery; survived
Henze et al25 45 M AVR R Surgery; survived
Tayama et al17 58 F AVR R Surgery; survived
Scalia et al26 61 F MVR R Surgery; survived
76 M CABG × 3 R Surgery; survived
Fujii et al27 70 F Ascending aorta replacement R Surgery; survived
Chung et al8 52 M AVR; aortic root replacement R Surgery; survived
Nakano et al28 65 M AVR R Surgery; survived
Caruso et al29 41 M None L Surgery; survived
Caruso et al30 59 F Heart transplant R Surgery; patient died
Russo et al31 70 F Aortic replacement R Surgery; survived
Patsouras et al32 70 M AVR L No intervention; patient died
Matsumoto et al33 71 F MVR L Surgery; survived
Haddad et al34 66 M Giant cell arteritis R Surgery; survived
Shah et al35 54 F AVR; aortic dissection repair L Surgery; survived
Pagni et al36 69 F MI; CABG R Surgery; survived
Sytnik et al37 63 M Ascending aorta replacement R Surgery; survived
AAF indicates aorto-atrial fistulas; AVR, aortic valve replacement; CABG, coronary artery bypass grafting; MI, myocardial infarction; MVR, mitral valve replacement.

shown in Table 5. Despite the heterogeneity of the causative organisms,
the nature of the pathogen has not been shown to affect prognosis.68
Often, IE originates in the valvular structures of the heart.
Spread from the affected valve to local tissue is often the initial step in
the pathologic cascade leading to AAF formation. Spread to the local
tissue may result in abscess formation, leading to local inflammation
and degradation of tissue. When the abscess ruptures, it erodes the
valve and leads to a fistula into the nearby cardiac chamber, such as
the atrium.42 Due to the anatomical considerations, fistula formation
most commonly occurs between the aorta and the right atrium.5 Our
literature review revealed that in AAF caused by IE, there were 15
(51.7%) cases with fistula formation to the right atrium.
Paravalvular abscess formation is seen more commonly in
prosthetic valve endocarditis than in native valve endocarditis.3 In
prosthetic valves, the bacteria first begin at the prosthetic cuff and
then invade the outside apparatus, resulting in valvular dehiscence
and abscess formation. Endocarditis is common after prosthetic
valve implantations, occurring in 2–4% of patients.69 In our search,
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
vascularization, leading to a paucity of immunological mediators to
mount an immune response. The “mitral-aortic intervalvular fibrosa”
is the junctional zone between the mitral and aortic valve annulus. Due
to the avascular nature of this area, it provides very little resistance to
the spread of abscesses and fistula formation, making fistulas in this
area common when IE is already present.43 Previous literature states
that periannular abscesses have been noted in up to 80% in patients
with aortic valve endocarditis,70 whereas intracardiac fistula forma-
tion is seen in 14% of patients.69 However, in our search, we found
that 19 (65.5%) cases of AAF cases had associated abscess formation.
The treatment for AAF caused by IE must be aggressive and
timely to avoid its lethal consequences. Treatment includes broad-
spectrum intravenous antibiotics and surgical correction of the fistu-
lous connection. Fistulas lead to a very high rate of complications,
with more than 60% of patients developing significant heart failure
and more than 40% ending in death.52 Surgical mortality is very high,
approximately 40%.5 In our search, 8 (27.5%) cases ended in patient
mortality, most of which were due to advanced disease at presenta-
tion. Factors associated with adverse outcomes include septic shock,
paravalvular leakage, hemodynamic instability, and congestive heart
failure. The Amplatzer plug technique allows for percutaneous clo-
sure of fistulous connections, as previously mentioned.10,55
PRIMARY CAUSES
Genetic and Connective Tissue Disorders
Although uncommon, some genetic causes and connective
tissue disorders related to AAF have been documented. Congenital

140  |  www.cardiologyinreview.com © 2017 Wolters Kluwer Health, Inc. All rights reserved.

Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

Cardiology in Review  •  Volume 26, Number 3, May/June 2018 Aorto-Atrial Fistulas

TABLE 4.  Cases of Infective Endocarditis Associated Aorto-Atrial Fistulas

LA/ Coronary
Author Age (Sex) Prior Cardiac History RA Abscess Murmur Sinus Management; Outcome
Schwartz et al 44
18 (M) None LA Yes 1/6 Diastolic Aortic root Surgery; successful
Karalis et al43 28 (M) AVR LA Yes None Aortic root Surgery; patient died
Benham45 21 (M) None LA Yes Continuous LCS Surgery; successful
Thomas et al2 53 (M) Tricuspid aortic valve RA Yes Continuous RCS Surgery; successful
Kelion et al46 39 (F) AVR LA Yes 3/6 Continuous NCS/LCS Surgery; successful
Archer et al47 61 (M) None LA Yes 2/6 Systolic NCS None; patient died
Gharagozloo et al48 76 (M) AVR LA Yes 3/6 Systolic NCS/RCS Surgery; successful
Ananthasubramaniam and 50 (F) AVR, CABG RA Yes 2/6 ejection systolic, Aortic root Surgery; patient died
Karthikeyan3 2/6 early diastolic
Farouque et al49 39 (F) None RA No 4/6 Continuous RCS Surgery; successful
Darwazah et al50 23 (M) AVR RA No None NCS/LCS Surgery; successful
Stechert and Kellermeier51 65 (M) AVR LA Yes None NCS Surgery; successful
Ozer et al52 41 (F) AVR RA Yes 3/6 Continuous N/A None; patient died
Chen et al53 50 (M) Tricuspid aortic valve RA No 3/6 Continuous RCS/LCS Antibiotics only; patient died
Kawahira et al54 67 (M) Incomplete AVB Mobitz type II RA Yes 3/6 Systolic NCS Surgery; successful
Swampillai et al4 16 (F) None RA Yes Diastolic NCS/LCS Surgery; successful
Estevez-Loureiro et al55 44 (M) AVR, MVR LA No None NCS Surgery (percutaneous);
successful
Candan et al56 55 (F) 3 mitral valve operations, 1 MVR RA No 2–3/6 Holosystolic NCS Surgery; patient died
Dias et al57 54 (M) Bicuspid aortic valve RA Yes 3/6 Systolic N/A Surgery; successful
Chandra et al58 20 (M) None LA No Early diastolic NCS/LCS Surgery; successful
Gunaranthe et al59 28 (M) Bicuspid Aortic Valve, sepsis RA Yes Diastolic NCS Surgery; successful
John et al60 21 (F) IV drug use RA Yes None NCS Surgery; successful
Villablanca et al42 51 (F) Tricuspid valve IE RA No 4/6 Pansystolic NCS Antibiotics alone; successful
Agrawal et al41 68 (F) AVR LA Yes 3/6 Systolic, 2/6 Aortic Root Surgery; patient died
Diastolic
Sabzi et al61 37 (F) None LA Yes None LCS Surgery; successful
Noyes et al62 35 (M) IV drug use, bicuspid aortic valve LA Yes 4/6 Systolic LCS Surgery; successful
Valero et al63 60 (F) Type A AD, Ascending aorta LA No 4/6 Diastolic Aortic Root Surgery; successful
reconstruction
Agrawal et al64 57 (M) None LA Yes 3/6 Systolic LCS None; patient died
Ikeda et al65 45 (M) Asymptomatic rupture of Sinus of RA No Continuous RCS Surgery; successful
Valsava aneurysm
Kim et al66 42 (M) None RA No 3/6 Continuous LCS Surgery; successful
AD indicates aortic dissection; AVB, atrioventricular block; AVR, aortic valve replacement; CABG, coronary artery bypass graft; IE, infective endocardiditis; IV, intravenous; LA,
left atrium; LCS, left coronary sinus; MVR, mitral valve replacement; NCS, noncoronary sinus; RA, right atrium; RCS, right coronary sinus.

arthritis, and Ehlers–Danlos syndrome type IV. Behcet’s disease is a

TABLE 5.  Infectious Agents Causing Infective Endocarditis
Infectious Agent
Stapholococcus species
Streptococcus species
Enterococcus species
Other species (Brucella, Candida, Proteus, N/A)
fistulas have been documented in cases with anomalous branches of
the aorta communicating directly with one of the atria. These patients
present at a younger age with smoothly communicating vessels, with
the presence of a second similar but much smaller vessel and the lack
of fibrous tissue or adhesions.71 In a reported case of a 4-year-old male
admitted for investigation of a cardiac murmur, a congenital fistula
between the descending thoracic aorta and left atrium was discovered.71
A second case includes a 5-month-old male presenting with a history of
difficulty in breastfeeding, failure to thrive, and distressed breathing. In
this patient, a fistula between the descending aorta and left atrium was
reported in association with the aortopulmonary window and secundum
atrial septal defect.72 In congenital cases, early corrective surgery is per-
formed to ligate the aberrant vessels and abolish the fistula.
Patients with inherited connective tissue disorders are predis-
posed to vascular and digestive ruptures, most typically arterial rup-
tures.73 AAF has been documented in Behcet’s disease, rheumatoid
chronic, multisystemic inflammatory disorder characterized by wide-
N (%) spread vasculitis of arterial and venous circulation. It is believed that
infection could act as a trigger to cardiac events, such as fistula forma-
10 (34.4)
tion, in individuals with Behcet’s disease.74 Reported AAF formation
9 (31.0)
2 (6.9)
in the setting of rheumatoid arthritis is believed to occur through the
8 (27.6 regression of a granuloma in the aortic wall or spontaneous aortic
rupture and erosion into the right atrium.75 Ehlers–Danlos syndrome
type IV is caused by abnormal synthesis of collagen type III, which
predisposes individuals to vascular and digestive tract ruptures. In the
reported case of AAF in the setting of Ehlers–Danlos syndrome, the
arterial rupture led to the intramural wall of the right atrium, gradually
producing the AAF.73 Treatment of AAF in the setting of connective
tissue disorders includes surgical closure of the fistula through intro-
duction of sutures along with a patch in the atrial wall.
Iatrogenic
Iatrogenic causes of AAF are often limited to intravascular
procedures involving or in proximity to the cardiac structures. AAF
has been documented as a complication of cardiac catheterization
and percutaneous transluminal angioplasty. A potentially lethal com-
plication of cardiac catheterization is perforation of the heart or great
vessels, leading to fistula formation. AAF caused by cardiac cath-
eterization has been reported in a variety of settings such as cath-
eterization ablation for history of abnormal cardiac conduction,76,77

© 2017 Wolters Kluwer Health, Inc. All rights reserved. www.cardiologyinreview.com | 141

Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

Fierro et al Cardiology in Review  •  Volume 26, Number 3, May/June 2018

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
arterial and venous catheterizations.81,82
In the case of AAF caused by cardiac stent implantation, stent
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
have been documented in the setting of self-expanding prostheses
implanted to treat stenosis in the vascular anastomosis between the
suprahepatic veins and the inferior vena cava83 and in the creation
of a transjugular intrahepatic portosystemic shunt.84 In these cases,
after the initial procedure, the patient usually presents with a new
continuous murmur and worsening congestive heart failure. Immedi-
ate surgical consultation is required to close the fistula. Management
in iatrogenic causes of AAF requires removal of the offending proce-
dure or prosthesis and surgical intervention. With early detection and
management, the prognosis is relatively good.
Other Considerations
Iatrogenic causes of AAF often are a result of intravascu-
lar cardiac procedures such as catheter ablation.76,77 In addition
to AAFs, other communicating lesions originating from the aorta
may form as a complication of these procedures. Left atrial cath-
eter ablation has been linked to aortoesophageal fistulas (AEF).85
Due to its anatomic location relative to the posterior left atrium,
the anterior esophagus is particularly prone to surgical injury.86
Patients with AEF often present with signs and symptoms of IE
or cerebral air emboli due to communication between the esopha-
gus and the left atrium. In addition, massive hematemesis may be
seen in these patients due to flow of blood from the left atrium
to the esophagus. These fistulous tracts are difficult to diagnose,
especially in patients with active bleeding. Diagnostic studies
may often show gaseous bubbles in the left atrium.87 In a study
by Doll et al,88 it was noted that up to 1% of patients had esopha-
geal perforation through the aortic wall after intraoperative radio-
frequency ablation; 75% of these patients had an unremarkable
postoperative course, followed by sudden onset of neurologic
symptoms from esophagoatrial air embolization. In patients with
AEF with severe complications, the mortality rate can be as high
as 93%.87 Thus, clinical signs and symptoms of stroke, hemateme-
sis, and signs of endocarditis or sudden onset of cardiovascular
decompensation in patients who underwent a vascular procedure
such as catheter ablation should raise concern for an AEF. These
patients should have a prompt diagnostic study followed by imme-
diate surgical intervention.
CONCLUSIONS
AAFs represent a potentially life-threatening complication,
which, if not expeditiously treated, could lead to volume overload
of the heart and eventually death. Its diagnosis is often delayed due
to the nonspecific nature of presenting symptoms and only 50%
sensitivity for diagnosis on TTE. Diagnosis requires both radiologi-
cal (TTE and imaging) and more invasive techniques such as TEE
and cardiac catheterizations. Upon diagnosis, expeditious manage-
ment is necessary to reduce mortality. The management option often
involves surgical intervention, especially if percutaneous options are
not viable. Success is often achieved with the closure of AAFs, albeit
with the potential for postsurgical complications. It is important for
physicians to be aware of this possible pathologic entity, as the diag-
nosis requires a high degree of suspicion.
REFERENCES
1. Boyd L. A study of four thousand cases of aneurysm of the thoracic aorta. Am
J Med Sci 1924;168:654–658.
2. Thomas MR, Monaghan MJ, Michalis LK, et al. Aortoatrial fistulae diagnosed
by transthoracic and transesophageal echocardiography: advantages of the
transesophageal approach. J Am Soc Echocardiogr. 1993;6:21–29.
3. Ananthasubramaniam K, Karthikeyan V. Aortic ring abscess and aortoatrial
fistula complicating fulminant prosthetic valve endocarditis due to Proteus
mirabilis. J Ultrasound Med. 2000;19:63–66.
4. Swampillai J, Liang M, Fisher R, et al. Enterococcus gallinarum causing
native valve endocarditis and aorto-atrial fistula: a case report and literature
review. Echocardiography. 2012;29:873–875.
5. Anguera I, Miro JM, Vilacosta I, et al.; Aorto-cavitary Fistula in Endocarditis
Working Group. Aorto-cavitary fistulous tract formation in infective endocar-
ditis: clinical and echocardiographic features of 76 cases and risk factors for
mortality. Eur Heart J. 2005;26:288–297.
6. Thuny F, Grisoli D, Collart F, et al. Management of infective endocarditis:
challenges and perspectives. Lancet. 2012;379:965–975.
7. Hill EE, Herijgers P, Claus P, et al. Abscess in infective endocarditis: the value
of transesophageal echocardiography and outcome: a 5-year study. Am Heart
J. 2007;154:923–928.
8. Chung DA, Page AJ, Coulden RA, et al. Aorto-atrial fistula after operated type
A dissection. Eur J Cardiothorac Surg. 2000;17:617–619.

142  |  www.cardiologyinreview.com © 2017 Wolters Kluwer Health, Inc. All rights reserved.

Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

Cardiology in Review  •  Volume 26, Number 3, May/June 2018 Aorto-Atrial Fistulas
9. Ahmad T, Chithiraichelvan S, Patil TA, et al. Aortic root to left-atrial fistula
after aortic valve replacement: a rare complication and its intraoperative man-
agement. Ann Card Anaesth. 2014;17:155–156.
10. Alkhouli M, Almustafa A, Kawsara A, et al. Transcatheter closure of an aor-
toatrial fistula following a surgical aortic valve replacement. J Card Surg.
2017;32:186–189.
11. Eng MH, Garcia JA, Hansgen A, et al. Percutaneous closure of a para-pros-
thetic aorto-right ventricular fistula. Int J Cardiol. 2007;118:e31–e34.
12. Luc JGY, Maruyama M, Sidhu S, et al. Aorto-right atrial fistula after suture-
less valve implantation. Can J Cardiol. 2017;33:688.e9–688.e11.
13. Yesin M, Kalçık M, Gürsoy MO, et al. Aorto-left atrial fistula after mitral valve
surgery. Echocardiography. 2015;32:1446–1448.
14. Menon PR, Doenst T, Ender J, et al. Surgical treatment of an aorto-right atrial
fistula as a complication of tricuspid valve repair. Thorac Cardiovasc Surg.
2011;59:250–252.
15. Badak O, Akdeniz B, Aslan O, et al. Rare complication of aortic valve replace-
ment: aorto-right atrial fistula. Anadolu Kardiyol Derg. 2003;3:373.
16. Aoyagi S, Akashi H, Kawara T, et al. False aneurysm of the ascending aorta
with fistula to the right atrium. Noninvasive diagnosis by computed tomo-
graphic scan and two-dimensional echocardiography with successful repair.
Thorac Cardiovasc Surg. 1994;42:58–60.
17. Tayama K, Aoyagi S, Akashi H, et al. Aortic dissection after aortic valve
replacement. Report of a case with an aortocameral fistula. Thorac Cardiovasc
Surg. 1995;43:299–301.
18. Ananthasubramaniam K. Clinical and echocardiographic features of aorto-
atrial fistulas. Cardiovasc Ultrasound. 2005;3:1.
19. Odaro E, Jain M, Horne M, et al. Aortoatrial fistula. Am J Respir Crit Care
Med. 2013;187:e9.
20. Raut N, Potdar A, Sharma S. Aorto - Left atrial fistula after mitral valve
replacement. Indian Heart J. 2016;68(suppl 2):S138–S139.
21. Criado FJ. Aortic dissection: a 250-year perspective. Tex Heart Inst J.
2011;38:694–700.
22. Lindsay J Jr. Aortocameral fistula: a rare complication of aortic dissection. Am
Heart J. 1993;126:441–443.
23. Matsuhisa H, Obo H, Nakagiri K, et al. Aorto-right atrial fistula caused by type
A aortic dissection. Ann Thorac Surg. 2004;78:2173–2175.
24. Hurley DV, Nishimura RA, Schaff HV, et al. Aortic dissection with fistula to
right atrium. Noninvasive diagnosis by two-dimensional and Doppler echo-
cardiography with successful repair. Case report and review of the literature.
J Thorac Cardiovasc Surg. 1986;92:953–957.
25. Henze AC, Thorelius JB, Borowiec JW, et al. Ascites after rupture of
dissecting aortic aneurysm into the right atrium. Ann Thorac Surg.
1991;51:125–127.
26. Scalia D, Rizzoli G, Scomparin MA, et al. Aorto-right atrial fistula: a rare
complication of aortic dissection type A. A report of two cases. J Cardiovasc
Surg (Torino). 1997;38:619–622.
27. Fujii H, Oka T, Kawaguchi H, et al. Aorto-atrial fistula associated with recur-
rent aortic dissection after ascending aorta replacement. J Cardiovasc Surg
(Torino). 1998;39:817–819.
28. Nakano H, Takahara Y, Sudou Y, et al. Aortic dissection compli-
cated with aorto-right atrium fistula. Jpn J Thorac Cardiovasc Surg.
2000;48:531–533.
29. Caruso A, Iarussi D, Materazzi C, et al. Aortic dissection with fistula to left
atrium: diagnosis by transesophageal echocardiography with successful repair.
J Am Soc Echocardiogr. 2000;13:69–72.
30. Caruso A, Iarussi D, Materazzi C, et al. Aortic dissection with fistula to right
atrium after heart transplantation: diagnosis by transthoracic and transesopha-
geal echocardiography. Echocardiography. 2000;17:337–340.
31. Russo C, De Chiara F, Bruschi G, et al. Aorto-atrial fistula through the septum
in recurrent aortic dissection. Ann Thorac Surg. 2001;72:921–922.
32. Patsouras D, Argyri O, Siminilakis S, et al. Aortic dissection with aorto-left
atrial fistula formation soon after aortic valve replacement: a lethal complica-
tion diagnosed by transthoracic and transesophageal echocardiography. J Am
Soc Echocardiogr. 2002;15:1409–1411.
33. Matsumoto K, Yamashita M, Toyohira H, et al. Stanford type A aortic dis-
section which ruptured into the left atrium: report of a case. Surg Today.
2003;33:114–116.
34. Haddad F, El-Rassi I, Haddad FG, et al. Aorto-atrial fistula 10 days after dis-
section repair in giant cell arteritis. Ann Thorac Surg. 2008;86:1672–1674.
35. Shah BN, Livesey SA, Rakhit DJ. Aorto-atrial fistula in the absence of infec-
tive endocarditis: diagnosis by 2- and 3-dimensional transesophageal echocar-
diography. Tex Heart Inst J. 2012;39:146–147.
© 2017 Wolters Kluwer Health, Inc. All rights reserved. www.cardiologyinreview.com | 143
Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
36. Pagni S, Mascio C, Trivedi J, et al. Type A aortic dissection complicated with
fistulization into the right atrium and right-to-left shunt. Interact Cardiovasc
Thorac Surg. 2013;16:909–911.
37. Sytnik P, White CW, Nates W, et al. Type A aortic dissection complicated by an
aorto-right atrial fistula. J Card Surg. 2015;30:173–178.
38. Crawford ES. The diagnosis and management of aortic dissection. JAMA.
1990;264:2537–2541.
39. Moreillon P, Que YA. Infective endocarditis. Lancet. 2004;363:139–149.
40. Anguera I, Miro JM, San Roman JA, et al.; Aorto-Cavitary Fistula in
Endocarditis Working Group. Periannular complications in infective endocar-
ditis involving prosthetic aortic valves. Am J Cardiol. 2006;98:1261–1268.
41. Agrawal A, Amor MM, Iyer D, et al. Aortico-left atrial fistula: a rare compli-
cation of bioprosthetic aortic valve endocarditis secondary to Enterococcus
faecalis. Case Rep Cardiol. 2015;2015:473246.
42. Villablanca PA, Sukhal S, Maitas O, et al. Aorto-right atrial fistula: Late
complication of tricuspid valve infective endocarditis. World J Cardiol.
2014;6:1122–1126.
43. Karalis DG, Chandrasekaran K, Wahl JM, et al. Transesophageal echocardio-
graphic recognition of mitral valve abnormalities associated with aortic valve
endocarditis. Am Heart J. 1990;119:1209–1211.
44. Schwartz DR, Belkin RN, Pucillo AL, et al. Aneurysm of the mitral-aor-
tic intervalvular fibrosa complicating infective endocarditis: preoperative
characterization by two-dimensional and color flow Doppler echocardiog-
raphy, magnetic resonance imaging, and cineangiography. Am Heart J.
1990;119:196–199.
45. Behnam R. Aortico-left atrial fistula in aortic valve endocarditis. Chest.
1992;102:1271–1273.
46. Kelion AD, Chambers JB, Deverall PB. Aorto-left atrial fistula in prosthetic
aortic endocarditis. J Heart Valve Dis. 1993;2:481–484.
47. Archer TP, Mabee SW, Baker PB, et al. Aorto-left atrial fistula. A reversible
cause of acute refractory heart failure. Chest. 1997;111:828–831.
48. Gharagozloo F, McReynolds SR, Dausmann MJ. Surgical management of
endocarditis-induced aorto-atrial and aorto-ventricular fistula: a new tech-
nique. J Cardiovasc Surg (Torino). 1997;38:131–135.
49. Farouque HM, Worthley SG, Yeend RA. Aortico-atrial fistula secondary to
bacterial endocarditis. Heart. 2001;86:498.
50. Darwazah A, Kiswani M, Ismail H, et al. Aorto-right atrial fistula: a complica-
tion of prosthetic aortic valve endocarditis. A case report. J Heart Valve Dis.
2006;15:142–145.
51. Stechert MM, Kellermeier JP. Aorto-atrial fistula: an important complication
of aortic prosthetic valve endocarditis. Anesth Analg. 2007;105:332–333.
52. Ozer N, Deniz A, Atalar E, et al. Aorta-right atrial fistula: a rare complica-
tion of prosthetic aortic valvular endocarditis. J Am Soc Echocardiogr
2007;20:538.e5–538.e6.
53. Chen MY, Zhong DD, Ying ZQ. Aorta-to-right atrium fistula, an unusual com-
plication of endocarditis. J Zhejiang Univ Sci B. 2009;10:230–232.
54. Kawahira T, Iwahashi K, Okada M. Aortocavitary fistula without aneurysm
and transient incomplete atrioventricular block due to infective endocarditis.
Gen Thorac Cardiovasc Surg. 2010;58:45–48.
55. Estévez-Loureiro R, Salgado Fernández J, Vázquez-González N, et al.
Percutaneous closure of an aorto-atrial fistula after surgery for infective endo-
carditis. JACC Cardiovasc Interv. 2012;5:e15–e17.
56. Candan O, Gecmen C, Guler A, et al. Aorto-right atrial fistula secondary to
infective endocarditis presenting with cardiogenic shock. J Cardiovasc Med
(Hagerstown). 2012;13:65–67.
57. Dias A, Mankodi D, Smith S. Aorta to right atrial fistula presenting as a rare
complication of native bicuspid aortic valve endocarditis. J Invasive Cardiol.
2013;25:61.
58. Chandra S, Ameta D, Kharwar RB, et al. Three-dimensional echocardiographic
delineation of an acquired aorto-left atrial fistula complicating native aortic
valve endocarditis - “advantage of three dimensions”. Echocardiography.
2013;30:E326–E330.
59. Gunarathne A, Hunt J, Gershlick A. Aorto-right atrial and right ventricular
fistulae: a very rare complication of native bicuspid aortic valve endocarditis.
Heart. 2013;99:1708.
60. John ES, Boyer J, Ledzian B, et al. A rare case of sinus of valsalva-right atrial
fistula secondary to an abscess perforation from underlying aortic valve endo-
carditis. J Cardiothorac Surg. 2014;9:124.
61. Sabzi F, Heidari A, Faraji R. A rare case of aortic sinuses of valsalva fistula
to multiple cardiac chambers secondary to periannular aortic abscess for-
mation from underlying Brucella endocarditis. GMS Hyg Infect Control.
2015;10:Doc14.
Fierro et al Cardiology in Review  •  Volume 26, Number 3, May/June 2018
62. Noyes AM, Ramu B, Parker MW, et al. Extracorporeal membrane oxy-
genation as a bridge to surgery for infective endocarditis complicated
by aorto-atrial fistula and cardiopulmonary collapse. Tex Heart Inst J.
2015;42:471–473.
63. Valero E, Santas E, Machado F, et al. Pseudoaneurysm with fistulization to the
left atrium: A very rare late complication of previous aortic root surgery. Int J
Cardiol. 2016;212:145–147.
64. Agrawal Y, Konda M, Kalavakunta JK. Aorto-left atrial fistula: Rare cause of
acute cardiac failure in a previously healthy individual. J Saudi Heart Assoc.
2016;28:270–273.
65. Ikeda A, Nakajima T, Konishi T, et al. Infective endocarditis of an aorto-right
atrial fistula caused by asymptomatic rupture of a sinus of Valsalva aneurysm:
a case report. Surg Case Rep. 2016;2:43.
66. Kim GS, Kim DW, Jeong IS, et al. Aorta-right atrial tunnel in a patient with
multivalvular endocarditis. J Card Surg. 2016;31:738–739.
67. Kang N, Wan S, Ng CS, et al. Periannular extension of infective endocarditis.
Ann Thorac Cardiovasc Surg. 2009;15:74–81.
68. Anguera I, Miro JM, Cabell CH, et al.; ICE-MD investigators. Clinical char-
acteristics and outcome of aortic endocarditis with periannular abscess in the
International Collaboration on Endocarditis Merged Database. Am J Cardiol.
2005;96:976–981.
69. Sabik JF, Lytle BW, Blackstone EH, et al. Aortic root replacement with
cryopreserved allograft for prosthetic valve endocarditis. Ann Thorac Surg.
2002;74:650–659; discussion 659.
70. Bashore TM, Cabell C, Fowler V Jr. Update on infective endocarditis. Curr
Probl Cardiol. 2006;31:274–352.
71. Nihoyannopoulos P, Sapsford R, Oakley CM. Congenital fistula between the
aorta and left atrium. Br Heart J. 1987;57:387–390.
72. Sarkar AK, Sanjeeva NG, Waghmare NS. Association of congenital descend-
ing aorto-left atrial fistula with the aortopulmonary window and atrial septal
defect. Cardiol Young. 2014;24:143–144.
73. Jiang S, Gao C, Ren C, et al. Successful surgical treatment of intramural aor-
toatrial fistula, severe aortic regurgitation, mitral prolapse, and tricuspid insuf-
ficiency in a patient with Ehlers-Danlos syndrome type IV. Heart Surg Forum.
2012;15:E156–E157.
74. Melua A, Campbell N, McCluskey D, et al. Aorto-atrial fistula without aneu-
rysm formation in Behçet’s disease. Heart. 1998;80:200–201.
144  |  www.cardiologyinreview.com © 2017 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
75. Crean PA, Reid DS, Buchanan J. Aorto-atrial fistula in rheumatoid arthritis.
Ann Rheum Dis. 1983;42:203–205.
76. Aligeti VR, South HL, Hirsh JB, et al. Aorto-right atrial fistula following trans-
septal catheterization and catheter ablation for atrial fibrillation. J Cardiovasc
Electrophysiol. 2012;23:659–661.
77. Tsang MY, Hagler DJ, Dearani JA, et al. Aorto-right atrial fistula: a rare com-
plication of trans-septal puncture and catheter ablation for atrial fibrillation.
Eur Heart J Cardiovasc Imaging. 2014;15:115.
78. Bartus S, Sorysz D, Siudak Z, et al. Spontaneous closure of aorta-to-
right atrium fistula after septal occluder implantation. J Cardiovasc Med
(Hagerstown). 2008;9:744–746.
79. Knirsch W, Dodge-Khatami A, Balmer C, et al. Aortic sinus-left atrial fistula
after interventional closure of atrial septal defect. Catheter Cardiovasc Interv.
2005;66:10–13.
80. Sey MS, Manlucu J, Myers KA. Intravascular hemolysis secondary to aorto-
atrial fistula presenting as red urine. J Gen Intern Med. 2010;25:1370–1374.
81. Danenberg HD, Hasin Y, Milgalter E, et al. Aorto-atrial fistula following inter-
nal jugular vein catheterization. Eur Heart J. 1995;16:279–281.
82. Ladowski JS, Hardesty RL. Repair of an iatrogenic aortoatrial fistula. Cathet
Cardiovasc Diagn. 1984;10:43–46.
83. Barrio-López MT, Martín-Trenor A, Mastrobuoni S, et al. Iatrogenic atrial
septal defect and aortoatrial fistula in a patient with endovascular prosthesis in
the inferior vena cava. Ann Thorac Surg. 2012;93:e23–e25.
84. Sehgal M, Brown DB, Picus D. Aortoatrial fistula complicating transjugular
intrahepatic portosystemic shunt by protrusion of a stent into the right atrium:
radiologic/pathologic correlation. J Vasc Interv Radiol. 2002;13:409–412.
85. Yousuf T, Keshmiri H, Bulwa Z, et al. Management of Atrio-Esophageal
Fistula Following Left Atrial Ablation. Cardiol Res. 2016;7:36–45.
86. Eitel C, Rolf S, Zachäus M, et al. Successful nonsurgical treatment of esoph-
agopericardial fistulas after atrial fibrillation catheter ablation: a case series.
Circ Arrhythm Electrophysiol. 2013;6:675–681.
87. Pappone C, Oral H, Santinelli V, et al. Atrio-esophageal fistula as a complica-
tion of percutaneous transcatheter ablation of atrial fibrillation. Circulation.
2004;109:2724–2726.
88. Doll N, Borger MA, Fabricius A, et al. Esophageal perforation during left
atrial radiofrequency ablation: Is the risk too high? J Thorac Cardiovasc Surg.
2003;125:836–842.