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Case report
Congenital aorto-right ventricular fistula in a 31 year old female
Fístula aortoventricular derecha en una mujer de 31 años
Arnaldo Akio Okinoa,b,
Corresponding author
arnaldo.okino@gmail.com

Corresponding author.
, Marcos Alfredo de Andrade Piresb, Jaqueline Maria de Oliveira Lima Luvizottob, Renan Pontes Petinellia, João Luiz Itagiba Fonsecac, Kalil Hussein Khalilb
a Department of Cardiovascular Surgery, Irmandade Santa Casa de Londrina, Londrina, Paraná, Brazil
b Department of Cardiovascular Surgery, Hospital Norte do Paraná, Arapongas, Paraná, Brazil
c Department of Cardiovascular Surgery, Hospital da Caixa de Assistência dos Servidores do Mato Grosso do Sul, Nova Andradina, Mato Grosso do Sul, Brazil
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Aorto-right ventricular fistula &#40;ARVF&#41; is a very rare condition which communicates the ascending aorta to the cavity of the right ventricle&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#8211;4</span></a> The Sinus of Valsalva fistula was first described in 1839 and has a general incidence about 0&#46;14&#8211;0&#46;96&#37; of all cardiac operations&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> The structure most frequently involved is the right coronary sinus&#44; accounting for 64&#37;&#44; followed by the noncoronary sinus&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> It&#39;s known that the perforation often communicates the aorta to the right ventricle&#44; but it can also do&#44; rarely&#44; to the right atrium&#44; left chambers or even to pericardium&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;5</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">This condition is mostly like associated to ruptured aneurysm of Sinus of Valsalva&#44; complication of endocarditis&#44; chest trauma&#44; aorta dissection or complication of another cardiac procedures as valve replacement&#44; Ventricular Septal Defect &#40;VSD&#41; correction and Transcatheter Aortic Valve Replacement &#40;TARV&#41;&#44; among others&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">2&#44;6&#44;7</span></a> This case describes an adult with ARVF without any of these causes above and with no other abnormalities on the aortic valve and root&#44; ascending aorta or ventricular septum&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0015" class="elsevierStylePara elsevierViewall">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&#46; The patient had no history of endocarditis&#44; trauma&#44; previous surgery or other heart disease diagnosed so far&#46; On physical evaluation&#44; cardiac auscultation showed a rude&#44; high-frequency holosystolic heart murmur&#44; pancardiac&#44; 4&#43;&#47;6&#43; according to Levine&#39;s scale&#44; and bilateral lower limbs edema&#46; Transthoracic echocardiography was performed&#44; which showed &#40;I&#41; moderate atrial dilatation&#44; &#40;II&#41; secondary increased pulmonary blood flow and &#40;III&#41; high-pressure blood flow directed from the right Valsalva sinus to the right ventricle&#44; with intense retrograde flow over pulmonary valve&#44; resulting in its functional insufficiency&#44; see <a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A and B&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Decided for her admission to clinical compensation and diagnostic complementation&#46; After clinical improvement&#44; cardiac catheterization of the left and right chambers was performed&#44; which revealed &#40;I&#41; coronary arteries free of atherosclerotic disease&#44; &#40;II&#41; increased blood flow from the aorta to right ventricular cavity&#44; &#40;III&#41; right ventricular diastolic overload&#44; &#40;IV&#41; moderate functional tricuspid valve incompetence and &#40;V&#41; appearance of increased pulmonary blood flow&#46; Hence&#44; it was decided for surgical approach to correct the defect&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Surgical access was performed by median longitudinal sternotomy&#44; installation of extracorporeal circulation circuit with aortic cannula positioned in ascending aorta and bicaval venous drainage&#46; Hypotermia up to 32<span class="elsevierStyleHsp" style=""></span>&#176;C&#44; transverse aortotomy&#44; identification of the orifice in the right Valsalva sinus of the right coronary leaflet&#44; with communication to the right ventricle outflow tract&#46; The fistula tissue was resected and the orifice was closed with bovine pericardium and prolene 5&#47;0 suture&#44; see <a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A and B&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">The surgery had no complications during operation&#44; and the patient was discharged at the fifth day after the procedure&#46; In the postsurgical outpatient evaluation&#44; the patient was asymptomatic&#44; and the echocardiography three months later showed&#58; &#40;I&#41; left atrium&#58; 36<span class="elsevierStyleHsp" style=""></span>mm&#59; &#40;II&#41; diameter of right ventricle&#58; 18<span class="elsevierStyleHsp" style=""></span>mm&#59; &#40;III&#41; final diastolic diameter of left ventricle&#58; 42<span class="elsevierStyleHsp" style=""></span>mm&#59; &#40;IV&#41; final systolic diameter of left ventricle&#58; 28<span class="elsevierStyleHsp" style=""></span>mm&#59; &#40;V&#41; ejection fraction of left ventricle&#58; 62&#37;&#59; &#40;VI&#41; soft aortic valve regurgitation&#59; &#40;VII&#41; absence of flow between the sinus of valsalva to the right ventricle&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">The Congenital Aorto-Right Ventricle Fistula is a defect on the wall of the aorta and&#44; although there is no definitive etiology for this anomaly&#44; it is probably caused by its weakness throughout the development and is present at birth but may manifest clinically at any age&#46; The abnormalities of the Sinus of Valsalva are characterized into three different groups&#58; &#40;1&#41; unruptured aneurysm of the Sinus of Valsalva&#44; &#40;2&#41; aneurysm of the Sinus of Valsalva with intracardiac or extracardiac rupture&#44; and &#40;3&#41; intracardiac or extracardiac fistula of the Sinus of Valsalva&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Meyer and colleagues<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> made a survey about the procedures realized at the Texas Heart institute from April 1956&#44; to September 1973&#46; They found 45 patients with aneurysm and&#47;or fistula of the Sinus of Valsalva&#44; representing 0&#46;43&#37; of all procedures which were required cardiopulmonary bypass&#46; Of the 45 patients in the group&#44; 22 had an unruptured aneurysm&#44; whereas 10 and 13 had a ruptured aneurysm and a simple fistulous communication&#44; respectively&#46; On the other hand&#44; according to Walpot and contributors<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">6</span></a> and Nishi et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a> the major cause of an aorto-cardiac fistula is a rupture of an aortic sinus aneurysm&#44; accounting for 76&#37; of the cases&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Many patients can be asymptomatic&#44; but the typical symptoms are like Congestive Heart Failure &#40;CHF&#41;&#46; A pain in the chest and a sudden onset of the symptoms point to a ruptured aneurysm&#46; It is possible to hear a &#8220;to-and-fro&#8221; murmur on cardiac auscultation with a systolic and diastolic thrill&#46; The diagnosis is usually taken by transthoracic or transesophageal echocardiography&#44; cardiac catheterization with aortography&#44; computed tomography or magnetic resonance imaging &#40;MRI&#41;&#44; revealing a left-to-right shunt&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">It is important to differentiate the ARVF from the Aorto-Right Ventricular Tunnel &#40;ARVT&#41;&#46; This condition&#44; as the first&#44; communicates the aorta to the right ventricle&#44; however the second one&#39;s perforation arises above the sinutubular junction instead and runs into extra-cardiac tissues addressed to ventricular chambers&#44; forming a conduit that by-passes the sinutubular junction&#46; According to McKay et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> the origin of the tunnel from the ascending aorta serves to differentiate it from rupture of an aneurysmal Sinus of Valsalva&#46; Most part of the aorto-ventricle tunnel communicates the aorta to the left ventricle &#40;90&#37;&#41;&#44; and just a small part of the cases do to the right ventricle&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a> In the other hand&#44; some surgeons consider that apart from fistulae secondary to infection&#44; trauma or post surgical&#44; most cases are a manifestation of a ruptured Sinus of Valsalva&#46; While some authors use the term aorto-ventricular tunnel&#44; others say that these terms are sinonimous and do not correspond to a different pathology&#46; In their opinion the right term is ruptured Sinus of Valsalva&#46; In the ARVF&#44; the communication is localized below the sinutubular structure&#44; where the abnormal pathway remains completely within the heart&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The surgical correction is the treatment of choice&#46; If promptly diagnosed and treated&#44; it is possible to avoid major morbidity resulting from CHF caused by left-to-right shunt&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a> Even in asymptomatic patients&#44; 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&#44; bacterial endocarditis&#44; pulmonary vascular disease&#44; aneurysm formation and spontaneous rupture&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> Currently&#44; an alternative of treatment is transcatheter closure&#44; but it is required more studies to analyze the optimal management&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">2&#44;5</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Consent statement</span><p id="par0060" class="elsevierStylePara elsevierViewall">The patient gave her consent for clinical information relating to this case to be reported in a medical publication&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Financial support</span><p id="par0065" class="elsevierStylePara elsevierViewall">No financial support&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conflict of interests</span><p id="par0070" class="elsevierStylePara elsevierViewall">No conflict of interest&#46;</p></span></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La f&#237;stula aortoventricular derecha es una entidad poco com&#250;n que comunica la aorta ascendente con el ventr&#237;culo derecho&#46; Se describe el caso de una mujer de 31 a&#241;os que acudi&#243; a consulta externa con un cuadro de disnea y edema en miembros inferiores de aproximadamente 2 meses de duraci&#243;n&#46; La evaluaci&#243;n f&#237;sica mostr&#243; un soplo card&#237;aco holosist&#243;lico en la auscultaci&#243;n card&#237;aca&#46; La ecocardiograf&#237;a y el cateterismo card&#237;aco revelaron una comunicaci&#243;n entre el seno coronario derecho y el tracto de salida del ventr&#237;culo derecho&#46; La paciente fue intervenida para corregir el defecto&#59; en la operaci&#243;n se resec&#243; tejido de la f&#237;stula y se cerr&#243; el orificio con pericardio bovino&#46;</p></span>"
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos