Long-term natural history and postoperative outcome of double-chambered right ventricle—Experience from two tertiary adult congenital heart centres and review of the literature Peter C. Kahr a, ⁎, Rafael Alonso-Gonzalez b , Aleksander Kempny b,c , Stefan Orwat a , Anselm Uebing b , Konstantinos Dimopoulos b,c , Lorna Swan b , Helmut Baumgartner a , Michael A. Gatzoulis b,c , Gerhard-Paul Diller a,b,c a Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany b Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Foundation Trust, London, UK c National Heart and Lung Institute, Imperial College School of Medicine, London, UK abstract article info Article history: Received 19 February 2014 Received in revised form 14 April 2014 Accepted 17 April 2014 Available online xxxx Keywords: Adult congenital heart disease Double-chambered right ventricle Outcome Background: Double-chambered right ventricle (DCRV) is a rare form of congenital heart disease. Little is known about the outcome during adult life. Here we report the combined experience of two tertiary Adult Congenital Heart Disease Centres and systematically review the published literature. Results: 50 patients (32 female, median age 39 years [IQR: 27;53]) with DCRV under follow-up could be identi- fied. A retrospective review of medical records was performed. Almost all patients (96%) had ventricular septal defects as underlying diagnosis. Eight patients remained completely asymptomatic during follow-up and have been managed conservatively thus far. The remaining patients developed symptoms at a median age of 26 years. Surgical correction was performed in 33 patients (median age at operation 27 years). No residual intra- ventricular gradient was present at the latest follow-up in 91% of operated patients and functional class improved significantly with only 6 patients remaining in NYHA class 2. There was no early or late operative mortality and no patient required re-operation for DCRV during a median follow-up of 8 years. Conclusions: Contemporary adult DCRV patients have good survival prospects and low long-term morbidity. De- spite occasionally presenting with considerable intraventricular gradients we could not identify any case of sud- den death in our unoperated adult sub-population. Although asymptomatic adults may be encountered even with severe obstruction, symptom development is common during adult life. Cardiac surgery in this cohort is in- herently low risk and offers good long-term haemodynamic and functional results justifying early intervention in consideration of the progressive nature of this rare congenital lesion. © 2014 Elsevier Ireland Ltd. All rights reserved. 1. Introduction Double-chambered right ventricle (DCRV) is an uncommon congen- ital heart condition, in which the right ventricle (RV) is progressively separated into two chambers by one or more obstructing muscle bun- dles. This separation, which is most commonly found in association with a small restrictive ventricular septal defect (VSD), results in a high-pressure inlet- and a low-pressure outlet RV portion, reflected by an intraventricular pressure gradient. DCRV is present in 0.6–2.6% of congenital patients undergoing cardiac catheterization [ [1–5]]; one case of DCRV can be found in about 36,000 autopsies [1]. Detailed description of this condition has been available since the 1960s, when it was recognized as a separate entity of right ventricu- lar obstruction [6,7]. However, DCRV is not a uniform disease [8] and the obstructing muscle bundles may manifest at different levels within the right ventricular cavity [9,10]. Although it is generally ac- cepted that there may be a predisposition to develop an obstruction, which then becomes progressive with time, there is currently no consensus regarding the exact pathogenesis of the obstructing mus- cle bundles. Proposed mechanisms include hypertrophy of the mod- erator band taking off abnormally high from the septal surface [1,11, 12], hypertrophy of accessory septoparietal trabeculations [13,14] or displaced/enlarged septomarginal trabeculations [8,15] (reviewed in [16]). The presence of a small VSD and the associated high- pressure jet may act as a trigger for any of these mechanisms. De- pending on the severity of the condition, DCRV can be associated with symptoms, mainly shortness of breath and exercise intolerance. International Journal of Cardiology xxx (2014) xxx–xxx ⁎ Corresponding author at: Division of Adult Congenital and Valvular Heart Disease, Dept. of Cardiovascular Medicine, University Hospital Muenster, Albert-Schweitzer-Str. 33, 48149 Muenster, Germany. Tel.: +49 251 83 46110; fax: +49 251 83 46109. E-mail address: pckahr@gmail.com (P.C. Kahr). IJCA-18078; No of Pages 7 http://dx.doi.org/10.1016/j.ijcard.2014.04.177 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved. Contents lists available at ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard Please cite this article as: Kahr PC, et al, Long-term natural history and postoperative outcome of double-chambered right ventricle—Experience from two tertiary adult congenital heart centres and review of the literature, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.04.177