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European Journal of Echocardiography Advance Access originally published online on May 7, 2008
European Journal of Echocardiography 2008 9(5):739-741; doi:10.1093/ejechocard/jen158
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

A heart within the heart: double-chambered left ventricle

Ole A. Breithardt1,2,*, Dieter Ropers2, Theresa Seeliger2, Axel Schmid3, Johannes von Erffa2, Christoph Garlichs2, Werner G. Daniel2 and Stephan Achenbach2

1 II. Medizinische Klinik, Klinikum Coburg, Academic Teaching Hospital of the University of Wuerzburg, Ketschendorfer Str. 33, DE-96450 Coburg, Germany
2 Medizinische Klinik 2, Department of Cardiology, University-Hospital Erlangen, Germany
3 Department of Radiology, University-Hospital Erlangen, Germany

Received 19 December 2007; accepted after revision 12 April 2008; online publish-ahead-of-print 7 May 2008.

* Corresponding author. Tel: +49 9561 22 33212; fax: +49 9561 226349. E-mail address: olebreithardt{at}gmx.de


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We describe a rare congenital anomaly in a 49-year-old woman who presented with palpitations and slightly reduced exercise capacity. A double-chambered left ventricle was suspected on echocardiography and confirmed by cardiac computed tomography scanning, cardiac magnet resonance imaging, and invasive angiography.

Keywords: Congenital heart disease; Echocardiography; Computed tomography; Cardiac magnetic resonance imaging; Angiography; Cardiac anomaly; GUCH

A 49-year-old woman presented with recurrent episodes of palpitations and mild symptoms of heart failure with slightly reduced exercise capacity which had slowly developed during the last decade. Physical examination was inconspicuous with no audible cardiac murmur and a regular heart rhythm. The ECG showed regular sinus rhythm with normal QRS axis and duration, delayed R-wave progression on the precordial leads, and a notch in the terminal QRS complex of the inferior leads (Figure 1A). Transthoracic echocardiography demonstrated an abnormally configured left ventricle (LV) with preserved overall LV function and two distinct contracting LV chambers, separated by a thick-walled muscular septum (Figure 1B–D, see Supplementary material online, Movies 1–3). A classical ‘heart-shaped’ appearance was seen in the parasternal short-axis view (Figure 1B, see Supplementary material online, Movie 1). The mitral valve was overriding both LV chambers, LV in- and outflow was unobstructed, and the AV plane showed a normal configuration. The right ventricle was enlarged, but otherwise normal in size and function. Colour-Doppler echocardiography demonstrated a small secundum type atrial septal defect (<1 cm by transoesophageal echocardiography). These findings were confirmed by cardiac magnetic resonance imaging (CMR, Figure 2A and B, see Supplementary material online, Movies 6 and 7), cardiac catheterization (Figure 2C and D, see Supplementary material online, Movies 8 and 9) and computed cardiac tomography (Figure 3, see Supplementary material online, Movie 10). Coronary angiography revealed a small left circumflex artery with an anormal origin from the proximal right coronary artery and a retroaortic course. LV end-diastolic pressure was slightly elevated to 18 mmHg. No significant coronary artery disease was found. LV ejection fraction by CMR was 62% with an end-diastolic volume of 126 mL. A small left-to-right shunt in the apical ventricular region due to a restrictive muscular ventricular septal defect with multiple interventricular connections (‘swiss-cheese’ morphology, maximal gradient >60 mmHg) was suspected by echocardiography and confirmed by right-heart catheterization (shunt fraction 18% by oxymetry, see Supplementary material online, Movies 4 and 5), but could not be visualized by LV angiography and computed tomography. An atrioventricular nodal re-entry tachycardia was documented by electrophysiological testing, but the patient refused ablation therapy.


Figure 1
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  		Figure 1 (A) 12-lead electrocardiogramm at admission. (B) Parasternal short-axis view of the basal LV segments with a ‘heart-shaped’ appearance. A thick, septum-like muscle bundle (middle) divides the left ventricle into a round, larger chamber on the patients left side (LV1) and a small, more oval-shaped chamber on the right (LV2), both connected by a common outflow segment (see Supplementary material online, Movie 1). (C) Apical view with the circular shaped LV1 chamber, the smaller LV2 chamber in the middle and parts of the slightly enlarged right ventricle (see Supplementary material online, Movie 2). (D) Parasternal short-axis view at the mid-apical level showing three distinct chambers (see Supplementary material online, Movie 3).

 


Figure 2
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  		Figure 2 (A) CMR horizontal long-axis view showing the divided LV (LV1 and LV2) and the slightly enlarged RV (SSFP Cine-MRI, see Supplementary material online, Movie 6). (B) CMR short-axis view (see Supplementary material online, Movie 7). (C) LV angiography from the right anterior oblique caudal view (see Supplementary material online, Movie 8). (D) LV angiography from the left anterior oblique caudal view (see Supplementary material online, Movie 9).

 


Figure 3
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  		Figure 3 (A) Three-dimensional computed tomography reconstruction (volume rendering technique, apical view) of the three ventricular chambers (see Supplementary material online, Movie 10). (B) Three ventricular chambers demonstrated by transaxial contrast-enhanced CT (slice thickness 3 mm).

 
This rare congenital disorder is best classified as a ‘double-chambered left ventricle’, a term which has been used to describe the subdivision of an LV cavity by an abnormal septum or muscle bundle into two chambers. Only a few cases with variable morphologies have been reported in the literature, most with either a diverticular appearance or small contracting chambers attached to the LV lateral wall or within the apex.1–3


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Supplementary data are available at European Journal of Echocardiography online.


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  1. Gerlis LM, Partridge JB, Fiddler GI, Williams G, Scott O. Two chambered left ventricle. Three new varieties. Br Heart J (1981) 46:278–84.[Abstract/Free Full Text]
  2. Sanz J, Rius T, Kuschnir P, Macaluso F, Fuster V, Poon M. Images in cardiovascular medicine. Double-chambered left ventricle: complete characterization by cardiac magnetic resonance and multidetector-row computed tomography. Circulation (2004) 110:e502–3.[Free Full Text]
  3. Hemmers T, Schwaiger M, Stern H. Double chambered left ventricle in cardiac magnetic resonance imaging. Heart (2006) 92:1401.[Free Full Text]

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This Article
Right arrow Abstract Freely available
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