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      Aorta-right Atrial Tunnel Presenting with Heart Failure in an Adult

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          Abstract

          Aorta-right atrial tunnel (ARAT) is a rare congenital anomaly characterized by extracardiac tunnel-like vascular connection between aorta and right atrium. Patients are usually asymptomatic, but patient may present with palpitation, dyspnea, and fatigue on exertion. Diagnosis can be made by transthoracic echocardiography during investigations for continuous murmur. Diagnosis can be confirmed by computed tomography, aortography, and coronary angiography. We report a case of a 26-year-old man diagnosed with ARAT arising from right sinotubular junction extending anteriorly and opening into right atrium near the opening of superior vena cava complicated with cardiomegaly and heart failure.

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          Most cited references13

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          Congenital malformations of the coronary arteries: the Texas Heart Institute experience.

          Coronary artery anomalies, some of which are considered clinically insignificant, can be associated with other congenital heart defects, myocardial ischemia, and reduced life expectancy. We conducted a retrospective study to determine the efficacy of surgical treatment in 191 patients who had a total of 202 coronary artery anomalies, which were classified as anomalies of origin (88 patients), termination (93), or distribution (10). Of the 88 patients with anomalies of origin, 60 had a coronary artery arising from the pulmonary artery, 18 had a right coronary artery arising from the left anterior descending artery, and 10 had a coronary artery arising from the contralateral sinus of Valsalva. All patients with an anomaly of termination had a coronary arteriovenous fistula, and all patients with an anomaly of origin had a single coronary artery. The diagnostic and operative techniques for each of the defects are evaluated. Based on our experience, early diagnosis and surgical intervention can yield satisfactory results in patients with coronary artery anomalies, with most experiencing relief of symptoms. The operative procedures were associated with a low early and late mortality; in addition, few patients experienced complications.
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            Aorta-right atrial tunnel: clinical presentation, diagnostic criteria, and surgical options.

            Aorta-right atrial tunnel is a rare and distinct congenital anomaly. To the spectrum of aorta-right atrial communications, we want to add this separate entity of aorta-right atrial tunnel by presenting the clinical features, morphologic aspects, diagnostic criteria, surgical techniques, and outcome in various clinical settings for better understanding of this developmentally intriguing, clinically complex, and therapeutically challenging disorder. From June 1994 through October 2004, 9 patients were treated for aorta-right atrial tunnel at our institution. Ages ranged from 9 to 45 years. There were 5 male subjects and 4 female subjects. Morphologically, 2 types of tunnels were identified in relationship to the ascending aorta: the anterior type in 3 patients and the posterior type in 6 patients. In all patients the diagnosis was established by means of 2-dimensional echocardiography and transesophageal echocardiography and confirmed by means of angiography. Eight patients were treated surgically, and 1 patient was treated with coil embolization. Postoperative echocardiograms obtained for all patients before discharge confirmed complete obliteration of the tunnel. One patient died perioperatively, and the other 8 patients were discharged in stable condition. During follow-up at 3 months, 6 months, and 1 year, all patients were in New York Heart Association class I, and echocardiography showed no residual shunts. In patients with aorta-right atrial tunnel, 2-dimensional echocardiography and transesophageal echocardiography are enough to establish clinical diagnosis, but ascending aortography is necessary to differentiate from more common clinical conditions, like ruptured sinus of Valsalva aneurysm and coronary cameral fistula. The rarity of this condition is established by the fact that during the same period of time, we have treated in our institution 66 cases of ruptured sinus of Valsalva aneurysm, which is the most common aorta-right atrial communication. Treatment options are simple ligation or ligation with implantation of coronary ostium or coil embolization. The location of the coronary ostium dictates technical details. Follow-up reveals excellent functional recovery.
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              Aorta--right atrial communication. Report of an unusual case.

              An unusual aneurysmal aorta--right atrial communication was found in a 25-year-old man with a rounded mass in the right cardiophrenic angle. Differential diagnoses included ruptured aneurysm of the sinus of Valsalva, coronary arteriovenous fistula, rupture of a dissecting aneurysm of the ascending aorta into the right atrium, and pseudoaneurysm of the right coronary artery followed by formation of a fistula between the aneurysm and the right atrium. Closure of the communication was performed under cardiopulmonary bypass. Complete absence of the right superior vena cava with a large persistent left superior vena cava draining to the coronary sinus was an unexpected finding at operation. The origin of the communication is not known; possible anomalous coronary and noncoronary origins are considered.
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                Author and article information

                Journal
                Heart Views
                Heart Views
                HV
                Heart Views : The Official Journal of the Gulf Heart Association
                Wolters Kluwer - Medknow (India )
                1995-705X
                0976-5123
                Oct-Dec 2018
                : 19
                : 4
                : 152-155
                Affiliations
                [1]Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra, India
                [1 ]Department of Medicine, Government Medical College, Nagpur, Maharashtra, India
                Author notes
                Address for correspondence: Dr. Jyoti Jain, Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha - 442 102, Maharashtra, India. E-mail: jyotijain@ 123456mgims.ac.in
                Article
                HV-19-152
                10.4103/HEARTVIEWS.HEARTVIEWS_74_18
                6487293
                63584297-8277-4876-9013-40a3109534d8
                Copyright: © 2019 Heart Views

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                Categories
                Case Report

                Cardiovascular Medicine
                aorta-right atrial tunnel,congenital anomaly,computed tomography angiography,echocardiography,right atrium

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