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      Correction of Double-Chambered Right Ventricle 40 Years after Pulmonary Valvuloplasty

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          Abstract

          Background Double-chambered right ventricle is a partial separation of the right ventricle by pathological tissue. This anomaly is described in infants and children but can also be diagnosed in young adults.

          Case Description A 47-year-old patient presented with chest pain and exercise intolerance. Further evaluation revealed a double-chambered right ventricle. During the surgical procedure, fibrotic tissue was transected, and a pulmonary valve replacement including enlargement of the main pulmonary artery was performed.

          Conclusion Subpulmonary obstruction of the right ventricle can be related to double-chambered right ventricle even in adulthood. Treatment options include surgical resection with pulmonary valve replacement.

          Most cited references6

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          Double-chambered right ventricle: a review.

          A double-chambered right ventricle is a rare heart defect in which the right ventricle is separated into a high-pressure proximal and low-pressure distal chamber. This defect is considered to be congenital and typically presents in infancy or childhood but has been reported to present rarely in adults. It can be caused by the presence of anomalous muscle tissue, hypertrophy of the endogenous trabecular bands, or an aberrant moderator band; all of which will typically result in progressive obstruction of the outflow tract. In this paper, we will discuss the general anatomy of the right ventricle, the relevant embryology of the heart, and the presentation, diagnosis, and treatment of a double-chambered right ventricle.
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            Rapid progression of midventricular obstruction in adults with double-chambered right ventricle.

            The purpose of this study was to determine the rate of progression of midventricular obstruction in adolescents and adults with double-chambered right ventricle. Clinical and echocardiographic findings in 45 patients (mean age 26 +/- 6 years, range 15-44) diagnosed with double-chambered right ventricle were retrospectively analyzed. Twenty patients underwent surgical repair before the age of 15 years. The relationship between Doppler midventricular pressure gradient and patient age was analyzed in 25 patients without previous repair. Sequential change in midventricular obstruction was determined for patients with 2 or more Doppler echocardiographic examinations performed within at least a 2-year interval. Right midventricular pressure gradient in nonrepaired patients was 70 +/- 38 mm Hg (range 25-150). A significant relationship between midventricular obstruction and patient age (r = 0.64, P /= 25 years (P <.03). After the initial study, 5 patients underwent surgical repair and 13 patients without repair were followed up for a period of 6.1 +/- 2.7 years (range 2-9), in which midventricular pressure gradient increased from 32 +/- 26 mm Hg to 67 +/- 35 mm Hg (P <.001). The slope of the change in midventricular pressure gradient was 6.2 +/- 3 mm Hg per year of follow-up. Seven more patients underwent surgical repair during follow-up due to progression of the obstruction. There was no mortality nor residual midventricular obstruction in surgically repaired patients. Mild right midventricular obstruction shows a fast rate of progression in adolescents and young adults. Thus, close clinical and echocardiographic follow-up is advised, and surgical repair should be considered if significant progression of obstruction is detected.
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              Outcomes of surgical repair of double-chambered right ventricle.

              We reviewed our experience with surgical repair of double-chambered right ventricle and long-term outcome.
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                Author and article information

                Journal
                Thorac Cardiovasc Surg Rep
                Thorac Cardiovasc Surg Rep
                10.1055/s-00024355
                The Thoracic and Cardiovascular Surgeon Reports
                Georg Thieme Verlag KG (Stuttgart · New York )
                2194-7635
                2194-7643
                15 July 2015
                December 2015
                : 4
                : 1
                : 31-33
                Affiliations
                [1 ]Department of Cardiothoracic Surgery, Heart Center Cologne, Cologne, Germany
                Author notes
                Address for correspondence Elmar W. Kuhn, MD Department of Cardiothoracic Surgery Heart Center Cologne, Kerpener Str. 62 50937Cologne, Cologne 50937Germany elmar.kuhn@ 123456uk-koeln.de
                Article
                150157crc
                10.1055/s-0035-1556814
                4670311
                aa7e8af4-507e-47eb-986a-9d0f7d717adf
                © Thieme Medical Publishers
                History
                : 13 February 2015
                : 21 April 2015
                Categories
                Article

                congenital heart disease,heart valve disease,pulmonary valve

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