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      Characteristics of Double-Chambered Right Ventricle in Adult Patients

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          Abstract

          Background/Aims

          This study evaluated the clinical features of double-chambered right ventricle (DCRV) in adults. Most cases of DCRV are diagnosed and treated during childhood. Consequently, very few reports include cases in which its clinical characteristics are evident in adults.

          Methods

          We reviewed the clinical data for 10 adult patients (age ≥ 18 years) with DCRV.

          Results

          Electrocardiogram showed right ventricular hypertrophy in 3 DCRV patients. All cases were associated with ventricular septal defect (VSD; 7 for perimembranous, 2 for muscular outlet, and 1 for the subarterial type). Surgical correction was done for 7 DCRV patients all of whom survived operations. Their follow-up echocardiogram showed the pressure gradient in their right ventricle was significantly decreased from 69.4 ± 17.2 mmHg preoperatively to 10.2 ± 5.0 mmHg postoperatively ( p < 0.05). In the short-term follow-up, there was no significant increase in the pressure gradient in the right ventricle.

          Conclusions

          There are lots of cases of DCRV that are not diagnosed accurately in adults. In our experience, all DCRV cases had VSD and surgical correction of these cases showed excellent results. Therefore, accurate diagnosis of DCRV is necessary so that DCRV is not overlooked and operations are enabled within an appropriate time.

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          Double-chambered right ventricle presenting in adulthood.

          Doff B McElhinney, Kanu Chatterjee, V.Mohan Reddy (2000)
          Double-chambered right ventricle is a form of right ventricular outflow tract obstruction that develops over time, often in patients with an abnormally short distance between the moderator band and pulmonary valve. This lesion typically presents in childhood or adolescence and is often accompanied by a ventricular septal defect. Only a handful of previous cases have been described in which double-chambered right ventricle occurred in adulthood. Since 1992, three patients more than 30 years old (38, 43, and 66 years of age) have presented at our institution with unusual symptoms or a previous incorrect diagnosis. We reviewed the clinical data in these patients. Presenting symptoms included syncope, angina, and severe dyspnea resembling pulmonary hypertension. In 1 patient, disease was categorized as New York Heart Association class IV, and in the other 2 as class III. Coexisting anomalies included a patent foramen ovale or secundum atrial septal defect in 2 patients, a small ventricular septal defect in 1 (with a probable history of ventricular septal defect in another), and mild aortic regurgitation in 1. All patients required urgent or emergent operations, with peak pressures in the proximal right ventricular chamber of 135 to 180 mm Hg and severely depressed left ventricular function in 1 patient. Resection of the anomalous right ventricular muscle bundles was achieved through a right atrial approach in all patients. All patients were alive with improved functional status at follow-up, which was between 15 and 40 months. Right ventricular outflow tract obstruction resulting from a double-chambered right ventricle is rare in adults, but when it does occur it can present with unusual symptoms. When evaluating the patient with signs or symptoms of primary right heart failure, cardiologists should make an effort to image the entire right heart complex. Subcostal echocardiography can facilitate adequate visualization of the right ventricle when it is difficult to distinguish the subpulmonary outflow tract from the parasternal and apical windows.
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            The role of echocardiography in diagnosing double chambered right ventricle in adults.

            W. Wojcik, Lynn J Rozanski, Eric Hoffman … (2004)
            To evaluate the utility of echocardiography in diagnosing double chambered right ventricle (DCRV) in adults. A retrospective study. Department of Congenital Heart Diseases and Department of Cardiac Surgery, Institute of Cardiology, Warsaw, Poland. 32 patients, mean age 32.5 years, 21 female, and 11 male. Transthoracic (TTE) and transoesophageal (TOE) echocardiographic examination in patients with suspected DCRV. Direct inspection during surgical treatment of 28 patients diagnosed as having DCRV as an isolated lesion or associated with other pathologies. Echocardiography allowed the final diagnosis of DCRV in 26 patients (81%) out of 32 studied. TTE was diagnostic in 5 (15.6%) whereas TOE was diagnostic in 21 of 21 studied by this technique. Of 6 patients with negative TTE, DCRV was identified by cardiac catheterisation in 3 and directly during surgery in the remaining 3. Of 26 patients diagnosed by echocardiography, the anomalous muscle bundle was discrete in 20 (77%) and diffuse in 6 (23%). In 23 patients (88%) right ventricular outflow obstruction was localised low in the right ventricle, and in the remaining 3 (11.5%), the obstruction was localised high in the right ventricle. Abnormal bundles localised high were discrete, bundles localised in the lower part of right ventricle were discrete in 17 (74%) and diffuse in the remaining 6 (26%). DCRV was an isolated lesion in only 2 patients (6.2%). In all subjects surgical inspection confirmed echocardiographic data. Echocardiography was very useful to diagnose DCRV in adults as well as to identify its anatomic type. Comparing two different approaches, TOE better defines the entire scope of pathology, including estimation of the resultant systolic pressure gradient within the right ventricular cavity.
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              Rapid progression of midventricular obstruction in adults with double-chambered right ventricle.

              Emily J Oliver, Marta Mateos Mazón, F. CARCELLER BENITO … (2003)
              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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                Author and article information

                Journal
                Journal ID (nlm-ta): Korean J Intern Med
                Journal ID (publisher-id): KJIM
                Title: The Korean Journal of Internal Medicine
                Publisher: The Korean Association of Internal Medicine
                ISSN (Print): 1226-3303
                ISSN (Electronic): 2005-6648
                Publication date (Print): June 2010
                Publication date (Electronic): 01 June 2010
                Volume: 25
                Issue: 2
                Pages: 147-153
                Affiliations
                [1 ]Division of Cardiology, Department of Internal Medicine, Eulji University School of Medicine, Daejeon, Korea.
                [2 ]Division of Cardiology, Department of Internal Medicine, Cardiovascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
                Author notes
                Correspondence to Seung Woo Park, M.D. Department of Internal Medicine, Samsung Medical Center, 50 Irwon-dong, Gangnam-gu, Seoul 135-710, Korea. Tel: 82-2-3410-3849, Fax: 82-2-3410-3419, swpark@ 123456smc.samsung.co.kr
                Article
                DOI: 10.3904/kjim.2010.25.2.147
                PMC ID: 2880687
                PubMed ID: 20526387
                SO-VID: b188d2b5-0302-4c27-9321-e659447cff4a
                Copyright © Copyright © 2010 The Korean Association of Internal Medicine
                History
                Date received : 20 November 2008
                Date accepted : 21 July 2009
                Categories
                Subject: Original Article

                ScienceOpen disciplines: Internal medicine
                Keywords: heart ventricles,heart defects, congenital,ventricular outflow obstruction,double outlet right ventricle
                Data availability:
                ScienceOpen disciplines: Internal medicine
                Keywords: heart ventricles, heart defects, congenital, ventricular outflow obstruction, double outlet right ventricle

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