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\begin{document}

\title{Congenital aorto-right ventricular fistula in a 31-year-old female}



\author[1]{Arnaldo Okino}%
\author[2]{Renan Petinelli}%
\author[3]{Marcos Pires}%
\author[4]{João Fonseca}%
\author[3]{Kalil Khalil}%
\author[3]{Jaqueline Luvizotto}%
\affil[1]{Affiliation not available}%
\affil[2]{Irmandade Santa Casa de Londrina}%
\affil[3]{Hospital Norte Paranaense}%
\affil[4]{Caixa de Assistência ao Servidor do Estado do Mato Grosso do Sul}%


\vspace{-1em}



  
  \date{June 13, 2024}


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\selectlanguage{english}
\begin{abstract}
Aorto-right ventricular fistula is a rare condition that communicates
the ascending aorta to the right ventricle. This case describes a
31-year-old female attended at the outpatient clinic with a complain of
dyspnea and edema in the lower limbs lasting about 2 months. Physical
evaluation showed a holosystolic heart murmur on cardiac auscultation.
Echocardiography and cardiac catheterization revealed a communication
between the right coronary sinus to the right ventricular outflow tract.
The patient underwent cardiac surgical treatment to correct the defect,
in which fistula tissue was resected and the orifice was closed with
bovine pericardium. The patient was discharged at the fifht day after
procedure, and a postsurgical echocardiography did not show
communication between the right sinus of valsalva to the right ventricle
anymore.%
\end{abstract}%



\sloppy


\textbf{INTRODUCTION}

Aorto-Right Ventricular Fistula (ARVF) is a very rare condition which
communicates the ascending aorta to the cavity of the right
ventricle\textsuperscript{{[}1--4{]}}. The Sinus of Valsalva fistula was
first described in 1839 and has a general incidence about 0.14\% to
0.96\% of all cardiac operations\textsuperscript{{[}3{]}}. The structure
most frequently involved is the right coronary sinus, accounting for
64\%, followed by the noncoronary sinus\textsuperscript{{[}1{]}}. It's
known that the perforation often communicates the aorta to the right
ventricle, but it can also do, rarely, to the right atrium, left
chambers or even to pericardium\textsuperscript{{[}1,5{]}}.

This condition is mostly like associated to ruptured aneurysm of Sinus
of Valsalva, complication of endocarditis, chest trauma, aorta
dissection or complication of another cardiac procedures as valve
replacement, Ventricular Septal Defect (VSD) correction and
Transcatheter Aortic Valve Replacement (TARV), among
others\textsuperscript{{[}2,6,7{]}}. This case describes an adult with
ARVF without any of these causes above and with no other abnormalities
on the aortic valve and root, ascending aorta or ventricular septum.

\textbf{CASE REPORT}

A 31-year-old women with unremarkable medical history was consulted at
the ambulatory complaining of dyspnea and edema of the lower limbs
lasting about 2 months. The patient had no history of endocarditis,
trauma, previous surgery or other heart disease diagnosed so far. On
physical evaluation, cardiac auscultation showed a rude, high-frequency
holosystolic heart murmur, pancardiac, 4+/6+ according to Levine's
scale, and bilateral lower limbs edema. Transthoracic echocardiography
was performed, which showed (I) moderate atrial dilatation, (II)
secondary pulmonary hyperflow and (III) high-pressure blood flow
directed from the right Valsalva sinus to the right ventricle, with
intense retrograde flow over pulmonary valve, resulting in its
functional insufficiency.

Decided for her admission to clinical compensation and diagnostic
complementation. After clinical improvement, cardiac catheterization of
the left and right chambers was performed, which revealed (I) coronary
arteries free of atherosclerotic disease, (II) hyperflow from the aorta
to right ventricular cavity, (III) right ventricular diastolic overload,
(IV) moderate functional tricuspid valve incompetence and (IV)
appearance of pulmonary hyperflow. Hence, it was decided for surgical
approach to correct the defect.

Surgical access was performed by median longitudinal sternotomy,
installation of extracorporeal circulation circuit with aortic cannula
positioned in ascending aorta and bicaval venous drainage. Hypotermia up
to 32\selectlanguage{ngerman}ºC, transverse aortotomy, identification of the orifice in the
right Valsalva sinus of the right coronary leaflet, with communication
to the right ventricle outflow tract.

The fistula tissue was resected and the orifice was closed with bovine
pericardium and prolene 5.0 thread.

The surgery had no complications during operation, and the patient was
discharged at the fifth day after the procedure. In the postsurgical
outpatient evaluation, the patient was asymptomatic, and the
echocardiography did not show communication between the right sinus of
valvalsa to the right ventricle anymore.

\textbf{DISCUSSION}

The Congenital Aorto-Right Ventricle Fistula is a defect on the wall of
the aorta and, although there is no definitive etiology for this
anomaly, it is probably caused by its weakness throughout the
development. The abnormalities of the Sinus of Valsalva are
characterized into three different groups: (1) unruptured aneurysm of
the Sinus of Valsalva, (2) aneurysm of the Sinus of Valsalva with
intracardiac or extracardiac rupture, and (3) intracardiac or
extracardiac fistula of the Sinus of
Valsalva\textsuperscript{{[}1,5{]}}.

Meyer and colleagues\textsuperscript{{[}1{]}} made a survey about the
procedures realized at the Texas Heart institute from April, 1956, to
September, 1973. They found 45 patients with aneurysm and/or fistula of
the Sinus of Valsalva, representing 0.43\% of all procedures which were
required cardiopulmonary bypass. Of the 45 patients in the group, 22 had
an unruptured aneurysm, whereas 10 and 13 had a ruptured aneurysm and a
simple fistulous communication, respectively. On the other hand,
according to Walpot and contributors\textsuperscript{{[}6{]}} and Nishi
et al.\textsuperscript{{[}5{]}}, the major cause of an aorto-cardiac
fistula is a rupture of an aortic sinus aneurysm, accounting for 76\% of
the cases.

Many patients can be asymptomatic, but the typical symptoms are like
Congestive Heart Failure (CHF). A pain in the chest and a sudden onset
of the symptoms point to a ruptured aneurysm. It is possible to hear a
``to-and-fro'' murmur on cardiac auscultation with a systolic and
diastolic thrill. The diagnosis is usually taken by transthoracic or
transesophageal echocardiography, cardiac catheterization with
aortography, computed tomography or magnetic resonance imaging (MRI),
revealing a left-to-right shunt.

It is important to differentiate the ARVF from the Aorto-Right
Ventricular Tunnel (ARVT). This condition, as the first, communicates
the aorta to the right ventricle, however the second one's perforation
arises above the sinutubular junction instead. In the ARVF, the
communication is localized below the sinutubular
structure.\textsuperscript{{[}8{]}} Most part of the aorto-ventricle
tunnel communicates the aorta to the left ventricle (90\%), and just a
small part of the cases do to the right
ventricle\textsuperscript{{[}9{]}}.

The surgical correction is the treatment of choice. If promptly
diagnosed and treated, it is possible to avoid major morbidity resulting
from CHF caused by left-to-right shunt\textsuperscript{{[}2{]}}. Even in
asymptomatic patients, closure of the fistula is recommended in low
surgical risk person due to the low rate of procedure complications and
the risk of heart failure, bacterial endocarditis, pulmonary vascular
disease, aneurysm formation and spontaneous
rupture\textsuperscript{{[}3{]}}. Currently, an alternative of treatment
is transcatheter closure, but it is required more studies to analyze the
optimal management\textsuperscript{{[}2,5{]}}.

\textbf{FINANCIAL SUPPORT}

No financial support.

\textbf{CONFLICT OF INTERESTS}

No conflict of interest.

\textbf{CONSENT STATEMENT}

The patient gave her consent for clinical information relating to this
case to be reported in a medical publication.

\textbf{REFERENCES}

{[}1{]} Meyer J, Wukasch DC, Hallman GL, Cooley DA. Aneurysm and fistula
of the sinus of valsalva. Ann Thorac Surg. 1975 Feb;19(2):170--9. doi:
10.1016/s0003-4975(10)63999-9.

{[}2{]} Tiwari KK, Salvati AC, di Summa M, Barbieri A, Portella G,
Strada G. Aorta-to-right ventricular outflow tract fistula with coronary
cusp prolapse. Asian Cardiovasc Thorac Ann. 2013 Apr;21(2):193--5.
doi:10.1177/0218492312449011

{[}3{]} Desai S, Flores E, Loli A, Maki P. Sinus of valsalva fistula to
the right ventricle along with coronary artery fistula to the pulmonary
artery in a young native american female. Case Reports in Cardiology.
2013 Nov;2013(3)1--3. doi:10.1155/2013/674608.

{[}4{]} De Viti D, Santoro F, Raimondo P, Brunetti ND, Memmola C.
Congenital aorto-right ventricular fistula associated with pulmonary
hypertension in an old female patient. J Cardiovasc Echogr. 2018
Apr-Jun;28(2):141--2. doi: 10.4103/jcecho.jcecho\_58\_17.

{[}5{]} Nishi I, Ishizu T, Sakamoto K, Eda K, Ishimitsu T, Watanabe S,
Yamaguchi I. A case of sinus of Valsalva-right ventricle fistula without
a typical aneurysm, and a single origin of the coronary arteries. Int
Heart J. 2005 May;46(3):531-6. doi: 10.1536/ihj.46.531.

{[}6{]} Walpot J, Klazen C, Hokken R, Sorgedrager J, Hoevenaar M, den
Braber J. Aorto-right ventricular fistula as an occasional finding. Eur
J Echocardiogr. 2005 Jan;6(1):65-6. doi: 10.1016/j.euje.2004.08.009.

{[}7{]} Pinaud F, Pezard P, Merheb M, Sibileau E, Baufreton C.
Congenital aorto-right ventricular fistula in an adult. Eur Heart J.
2009 Sep;30(17):2116. doi: 10.1093/eurheartj/ehp206.

{[}8{]} Mckay R, Anderson RH, Cook AC. The aorto-ventricular tunnels.
Cardiol Young. 2002 Dec;12(6):563--80. doi:10.1017/S1047951102001038

{[}9{]} McKay R. Aorto-ventricular tunnel. Orphanet J Rare Dis. 2007 Oct
8;2:41. doi: 10.1186/1750-1172-2-41.

\textbf{Hosted file}

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