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      Diagnosis and management of aorto-left ventricular tunnel

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          Abstract

          Background:

          Aorto-left ventricular tunnel (ALVT) is a rare congenital extracardiac channel with progressive left ventricular dilatation needs early correction.

          Materials and Methods:

          This is a report of diagnosis and management of aorto-left ventricular tunnel (ALVT) over a period of 11 years from a single institution. Seven patients (age range: 7 days-45 years) presented with heart failure. The diagnosis of ALVT was made by transthoracic echocardiogram in all cases.

          Results:

          Treatment was refused by two patients who died during follow-up. Surgical closure of the tunnel was done in four cases, of which one needed Bentall procedure. Two patients had residual leak after the surgery. Transcatheter closure using Amplatzer muscular device was performed in two cases (for postoperative residual leak in one and primary procedure in the other). Significant hemolysis developed in one of them, necessitating the removal of the device and closed surgically. This child underwent aortic valve replacement two years later. All the remaining patients were doing well during the median follow-up of 30 months (range: 1.5-9 years).

          Conclusion:

          ALVT is a rare and potentially fatal anomaly that is ideally managed surgically. Catheter closure has a limited role.

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          Most cited references24

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          Aortico-left ventricular tunnel: 35-year experience.

          The purpose of this study was to review our 35 years of experience with aortico-left ventricular tunnel (ALVT), with emphasis on diagnosis, surgical details, and follow-up. Aortico-left ventricular tunnel is a rare congenital anomaly. Neonatal surgery has been advocated in all due to long-term concern of valvar aortic regurgitation (AR). We identified 11 patients from 1963 to August 2002. Clinical, echocardiographic, catheterization, and surgical details were reviewed. Eight of 11 patients presented at less than six months old (six with congestive heart failure) and three later with a murmur, all with clinical evidence of AR. Associated lesions, most commonly aortic valve and coronary artery anomalies, were present in 45%. Catheter occlusion was considered but not performed in five. Spontaneous occlusion was documented in one. Ten had surgery (nine in our institution), seven with direct suture and two by patch closure of the aortic end of the AVLT. At follow-up (median, 5 years; 1 month to 35 years), all were asymptomatic; three had residual ALVT (one moderate, two small/trivial), with at most mild AR. Aortico-left ventricular tunnel is a rare cardiac malformation with a good post-operative long-term outcome. Associated lesions occurred in 45%. Catheterization should be reserved for patients with unclear non-invasive findings or transcatheter closure. We recommend surgery for most patients. We report spontaneous closure in one patient, prompting consideration of conservative follow-up in rare small, asymptomatic AVLT.
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            Aorto-ventricular tunnel

            Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinutubular junction to the cavity of the left, or (less commonly) right ventricle. The exact incidence is unknown, estimates ranging from 0.5% of fetal cardiac malformations to less than 0.1% of congenitally malformed hearts in clinico-pathological series. Approximately 130 cases have been reported in the literature, about twice as many cases in males as in females. Associated defects, usually involving the proximal coronary arteries, or the aortic or pulmonary valves, are present in nearly half the cases. Occasional patients present with an asymptomatic heart murmur and cardiac enlargement, but most suffer heart failure in the first year of life. The etiology of aorto-ventricular tunnel is uncertain. It appears to result from a combination of maldevelopment of the cushions which give rise to the pulmonary and aortic roots, and abnormal separation of these structures. Echocardiography is the diagnostic investigation of choice. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks gestation. Aorto-ventricular tunnel must be distinguished from other lesions which cause rapid run-off of blood from the aorta and produce cardiac failure. Optimal management of symptomatic aorto-ventricular tunnel consists of diagnosis by echocardiography, complimented with cardiac catheterization as needed to elucidate coronary arterial origins or associated defects, and prompt surgical repair. Observation of the exceedingly rare, asymptomatic patient with a small tunnel may be justified by occasional spontaneous closure. All patients require life-long follow-up for recurrence of the tunnel, aortic valve incompetence, left ventricular function, and aneurysmal enlargement of the ascending aorta.
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              Aortico-left ventricular tunnel: a clinical review and new surgical classification.

              This is a collective review of aortico-left ventricular tunnel (ALVT) in the English-language literature. We include the long-term follow-up of a previously reported patient, and a report on 2 new patients. To date, 37 cases of ALVT have been reported. Controversies regarding the definition, etiology, local anatomy, and treatment are discussed. The ages of the patients ranged from 1 day to 25 years old, and the male to female ratio was 2:1. There were associated anomalies in 27% of the patients, and moderate to severe heart failure in 59% of them. Mortality was 100% in the medically managed group; the surgical mortality was 16%. Previous surgical techniques utilized were simple closure, patch closure of the aortic end, and obliteration of the tunnel on both ends. Progressive aortic incompetence seems to be a common, but not well-documented problem on long-term follow-up. We have classified the lesion into four types (I, II, III, and IV) that have a bearing on the appropriate surgical techniques of repair, and describe a new technique for the repair of type III ALVT in which septal aneurysm is present.
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                Author and article information

                Journal
                Ann Pediatr Cardiol
                Ann Pediatr Cardiol
                APC
                Annals of Pediatric Cardiology
                Medknow Publications & Media Pvt Ltd (India )
                0974-2069
                0974-5149
                May-Aug 2015
                : 8
                : 2
                : 103-107
                Affiliations
                [1]Department of Cardiac Surgery, Gutti, Consultant Pediatric Cardiologist, Care Hospital, Hyderabad, Telangana, India
                [1 ]Chief Cardiac Surgeon, Care Hospital, Hyderabad, Telangana, India
                [2 ]Consultant Pediatric Cardiac Surgery, Care Hospital, Hyderabad, Telangana, India
                [3 ]Consultant Pediatric Cardiologist, G. Kuppuswamy Naidu Memorial Hospital, Coimbatore, Tamil Nadu, India
                [4 ]Consultant Cardiologist, Care Hospital, Hyderabad, Telangana, India
                [5 ]Chief Pediatric Cardiologist, Care Hospital, Hyderabad, Telangana, India
                Author notes
                Address for correspondence: Dr. Nageswara Rao Koneti, Chief Pediatric Cardiologist, Road No: 1, Banjara Hills, Hyderabad - 500 031, Telangana, India. E-mail: drkoneti@ 123456yahoo.com
                Article
                APC-8-103
                10.4103/0974-2069.157021
                4453176
                26085759
                f9b026c0-5412-46be-8b22-f73f8719f31e
                Copyright: © Annals of Pediatric Cardiology

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Original Article

                Cardiovascular Medicine
                aortic disease,aortico- left ventricular tunnel,congenital heart defect,non valvular aortic regurgitation

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