DOI: 10.1148/radiol.2442041242
(Radiology 2007;244:617-619.)
© RSNA, 2007
Case 119: Double-chambered Right Ventricle1
J. Joost Kardux, MD,
H. Wouter van Es, MD, PhD and
Benno J. W. M. Rensing, MD, PhD
1 From the Departments of Radiology (J.J.K., H.W.v.S.) and Cardiology (B.J.W.M.R.), St Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, the Netherlands. Received July 15, 2004; revision requested September 27; revision received November 19; accepted December 17; final version accepted March 1, 2005.
Correspondence: Address correspondence to J.J.K. (e-mail: j.j.kardux{at}hccnet.nl).
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HISTORY
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A 17-year-old boy was referred to our institution for cardiovascular analysis and therapy. He had no symptoms besides dyspnea on exertion. Auscultation revealed a cardiac murmur. His medical history included a prior episode of endocarditis involving the pulmonary valve after tooth extraction. This was successfully treated with antibiotics.
Electrocardiography showed signs of right ventricular hypertrophy. Laboratory findings were normal. Magnetic resonance (MR) imaging was performed.
Supplemental material: http://radiology.rsnajnls.org/cgi/content/full/244/2/617/DC1
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IMAGING FINDINGS
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MR imaging included T1-weighted black-blood sequences in various directions and cine imaging of the right ventricular outflow tract with balanced fast field-echo sequences. The coronal T1-weighted black-blood sequence revealed marked hypertrophy of the right ventricle wall (Fig 1). The T1-weighted black-blood image of the right ventricular outflow tract showed thickened muscular bands between the thin-walled infundibulum and the inflow segment of the right ventricle. These bands reached inward from the interventricular septum and the anterior free wall and divided the right ventricle into two chambers. The muscular bands created a marked stenosis between the chambers (Fig 2). The muscular bands were also seen on coronal images. The stenosis resulted in a jet phenomenon seen on the balanced fast field-echo right ventricular outflow tract image (Fig 3) because a steep pressure gradient between the inflow tract and the infundibular segment of the right ventricle caused high-velocity blood to enter slower flowing blood in the infundibulum. Although the jet phenomenon was visible on an MR image, it was best appreciated with cine imaging (Movie, http://radiology.rsnajnls.org/cgi/content/full/244/4/617/DC1).
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Figure 1: Coronal T1-weighted black-blood MR image of the heart (repetition time msec/echo time msec, 2000/8.6; flip angle, 90°) shows hypertrophy of the wall of the right ventricle (arrows) and an anomalous muscle band (arrowheads) dividing the right ventricle.
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Figure 2: Sagittal T1-weighted black-blood MR image of the right ventricular outflow tract of the heart (2000/8.6; flip angle, 90°) shows an anomalous muscle band creating two chambers and a stenosis (arrowheads) separating the inflow tract (long arrow) from the outflow tract (twin arrows) of the right ventricle. The pulmonary valve (short arrow) is also visible.
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Figure 3: Sagittal balanced fast field-echo MR image of the right ventricular outflow tract of the heart (4.0/2.0; flip angle, 50°) shows jet phenomenon (arrows) due to the pressure gradient distal to the stenosis.
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The right atrium was dilated as a result of the obstructive lesion in the right ventricle. Pulmonary valve anatomy and pulmonary artery size were normal, as was the left side of the heart. The ventricular septum showed no defect.
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DISCUSSION
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Clinical findings in this patient were rather nonspecific. The patient's medical history included endocarditis involving the pulmonary valve, which is a rare location for this disease (1). MR imaging clearly depicted a double-chambered right ventricle (DCRV) or subinfundibular stenosis. Possible symptoms of DCRV are related to pulmonary obstruction and include dyspnea on exertion, exercise intolerance, chest pain, syncope, and dizziness. These symptoms can also be found in association with other obstructive lesions in the right side of the heart, such as pulmonary valve or infundibular stenosis. Pulmonary valve damage caused by the altered flow dynamics due to DCRV might have caused the previous episode of pulmonary valve endocarditis. This association has been reported in the literature (1).
DCRV is an uncommon anatomic anomaly of the heart (2). It is currently considered a cardiac defect that evolves progressively in postnatal life. It is often associated with a perimembranous septal defect (3). In this patient, no septal defect was found with MR imaging or during surgery.
In most patients, DCRV is diagnosed and treated during childhood, with most patients being younger than 20 years. Occasionally, the lesion manifests in adulthood. The ventricular obstruction tends to progress rapidly, making surgical correction necessary (4–6).
In this patient, echo Doppler ultrasonography (US) depicted an 85 mm Hg pressure gradient over the stenosis. Echocardiography further revealed marked hypertrophy in the right ventricle, a dilated right atrium, and paradoxal movement of the ventricular septum. The latter finding was confirmed with cine MR imaging. Pulmonary valve dimensions and function were normal.
The nature of the obstruction in patients with DCRV is not well defined: Anomalous muscle bands that divide the right ventricle in either a low oblique or a high horizontal position have been described (7). Other authors have suggested that the obstruction might be related to displacement and alteration of the moderator band (8).
Besides MR imaging, transesophageal or transthoracic echocardiography or right ventricular angiography can be used to diagnose DCRV (9–14). The advantages of MR cardiography include multiplanar and cine imaging, which depicts both the anatomic and the functional features of DCRV.
The approach to surgical correction is either right atriotomy or ventriculotomy. In this patient, surgery was performed with a transventricular approach. The anomalous muscle bundles were resected. Postoperative recovery was excellent, although a right bundle-branch block was induced. Echo Doppler US revealed a postoperative decrease in the pressure gradient from 85 mm Hg to 6 mm Hg.
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FOOTNOTES
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Authors stated no financial relationship to disclose.
| Part one of this case appeared 4 months previously and may contain larger images.
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References
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- Lopez-Pardo F, Aguilera A, Villa M, et al. Double-chambered right ventricle associated with mural and pulmonic valve endocarditis: description of a clinical case and review of the literature. Echocardiography 2004;21:171–173.[CrossRef][Medline]
- Peacock TB. Malformations of the human heart. London, England: John Churchill, 1858; 60.
- Pongiglione G, Freedom RM, Cook D, Rowe RD. Mechanism of acquired right ventricular outflow tract obstruction in patients with ventricular septal defect: an angiocardiographic study. Am J Cardiol 1982;50:776–780.[CrossRef][Medline]
- Hachiro Y, Takagi N, Koyanagi T, Morikawa M, Abe T. Repair of double-chambered right ventricle: surgical results and long-term follow-up. Ann Thorac Surg 2001;72:1520–1522.[Abstract/Free Full Text]
- McElhinney DB, Chatterjee KM, Reddy VM. Double-chambered right ventricle presenting in adulthood. Ann Thorac Surg 2000;70:124–127.[Abstract/Free Full Text]
- Oliver JM, Garrido A, Gonzalez A, et al. Rapid progression of midventricular obstruction in adults with double-chambered right ventricle. J Thorac Cardiovasc Surg 2003;126:711–717.[Abstract/Free Full Text]
- Alva C, Ortegon J, Herrera F, et al. Types of obstruction in double-chambered right ventricle: mid-term results. Arch Med Res 2002;33:261–264.[CrossRef][Medline]
- Wong PC, Saunders SP, Jonas RA, et al. Pulmonary valve-moderator and band distance and association with development of double-chambered right ventricle. Am J Cardiol 1991;68:1681–1686.[CrossRef][Medline]
- Sreeram N, de Bruijn D, Hitchcock JF. Double chambered right ventricle: delineation by multiplane transoesophageal echocardiography. Int J Cardiol 1998;66:309–311.[CrossRef][Medline]
- Lascano ME, Schaad MS, Moodie DS, Murphy D. Difficulty in diagnosing double-chambered right ventricle in adults. Am J Cardiol 2001;88:816–819.[CrossRef][Medline]
- Kucher N, Seiler C, Allemann Y, Eberli FR. Double-chambered right ventricle. Circulation 2001;103:E105–E106.[Medline]
- Nakata T, Hattori A, Shimamoto K. Double-chambered right ventricle. Lancet 2004;363:1137.[CrossRef][Medline]
- Kilner PJ, Sievers B, Meyer GP, Ho SY. Double-chambered right ventricle or subinfundibular stenosis assessed by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2002;4:373–379.[CrossRef][Medline]
- Hoffman P, Wojcik AW, Rozanski J, et al. The role of echocardiography in diagnosing double chambered right ventricle in adults. Heart 2004;90:789–793.[Abstract/Free Full Text]
Congratulations to the 29 individuals and six resident groups that submitted the most likely diagnosis (double-chambered right ventricle) for Diagnosis Please, Case 119. The names and locations of the individuals and resident groups, as submitted, are as follows:
Individual responses
- Skip Michael Alderson, MD, Abington, Pa
- Anil Kumar Attili, MBBS, Ann Arbor, Mich
- Douglas Charles Brown, MD, Virginia Beach, Va
- Marc G. De Baets, MD, Agra, Switzerland
- Johannes F.K. De Villiers, MBChB, MMed, Gisborne, New Zealand
- Mustafa Kemal Demir, MD, Istanbul, Turkey
- Thaworn Dendumrongsup, MD, Songkla, Thailand
- Seyed A. Emamian, MD, PhD, Rockville, Md
- Akira Fujikawa, MD, Setagaya, Tokyo, Japan
- Hakan Genchellac, Edirne, Turkey
- Kiriakos Kalampoukas, MD, Halandri, Greece
- Stefanos Lachanis, Athens, Greece
- Mario A. Laguna, MD, Milwaukee, Wis
- David Anthony Lisle, MBBS, Brisbane, Australia
- Patricia Ann Lowry, MD, Chattanooga, Tenn
- Nikolaos Michailidis, Thessaloniki, Greece
- Jose Mondello, MD, Buenos Aires, Argentina
- Klaus Orth, Aachen, Germany
- Matthew P. Shapiro, MD, Charlottesville, Va
- Taro Shimono, MD, Osaka, Sayama, Japan
- Jadranka Stojanovska, MD, Ann Arbor, Mich
- Subramanian Subramanian, MD, New Delhi, India
- Norio Takahashi, MD, Fukui, Japan
- Osman Temizoz, Edirne, Turkey
- Philippe Thoma, Brussels, Belgium
- Eleni Vafeiadou, Thessaloniki, Greece
- Satoru Yoshida, Muroran, Japan
- Joe Yut, Olathe, Kan
- Bas Zonneveld, MD, Deventer, Netherlands
Resident group responses
- Baylor University Medical Center Radiology Residents, Dallas, Tex
- Hospital Sírio Libanês Radiology Residents, São Paulo, Brazil
- Prince of Songkla University Radiology Residents, Hat Yai, Songkla, Thailand
- Trakya University School of Medicine Radiology Residents, Edirne, Turkey
- University of Pennsylvania Radiology Residents, Philadelphia, Pa
- Virginia Commonwealth University Radiology Residents, Richmond, Va
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HISTORY
IMAGING FINDINGS
