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Year : 2017  |  Volume : 27  |  Issue : 2  |  Page : 77

Double-chambered right ventricle: The importance of a long-term follow-up

Department of Clinical and Experimental Medicine, Cardiology Unit, University of Messina, Messina, Italy

Date of Web Publication31-Mar-2017

Correspondence Address:
Lilia Oreto
Department of Clinical and Experimental Medicine, Cardiology Unit, University Hospital, Via Consolare Valeria 100, Messina
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcecho.jcecho_14_17

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How to cite this article:
Oreto L. Double-chambered right ventricle: The importance of a long-term follow-up. J Cardiovasc Echography 2017;27:77

How to cite this URL:
Oreto L. Double-chambered right ventricle: The importance of a long-term follow-up. J Cardiovasc Echography [serial online] 2017 [cited 2021 Oct 22];27:77. Available from: https://www.jcecho.org/text.asp?2017/27/2/77/203555

Dear Sir,

Two cases of double-chambered right ventricle (DCRV) in adult patients have been reported in previous issues of the Journal of Cardiovascular Echography.[1],[2]

In DCRV a progressive muscular obstruction divides the right ventricle into two portions. Hypertrophy of the muscular bands separates the proximal high-pressure inlet chamber from the distal low-pressure infundibular chamber.

Obstruction is usually progressive with a wide variability of progression rates, and age at presentation may be equally variable.

There are three main forms of DCRV:[3] isolated, with associated ventricular septal defect, and with associated subaortic stenosis. A ventricular septal defect is present in >50%–75% of cases and is usually perimembranous, creating a communication between the left ventricle and the proximal high-pressure chamber of the right ventricle. Most of the times the ventricular septal defect is small and tends to a spontaneous closure. When septal defect is due to malalignement, there is a higher tendency toward left ventricular outflow tract obstruction, which is usually due to a discrete subaortic fibromuscular stenosis.

Echocardiography can identify and well characterize DCRV, provided that all available projections are used, including subcostal views.[4] Whenever right ventricular hypertrophy is found in an adult patient, a careful examination of the pulmonary and subpulmonary region is advisable. Parasternal short-axis view may not be sufficient for a clear evaluation of the right midventricular region, and even if aliasing is seen with color Doppler, it is not possible to correctly align the ultrasound beam through an infundibular stenosis, which would be too close to the transducer. Therefore, measurement of the intracavitary gradient would be clearly underestimated. The subcostal right oblique view provides the best point of observation for the right ventricle, with optimal visualization of the inlet and infundibular portions. Besides, the ultrasound beam can be correctly aligned through the midventricular region, with a reliable estimation of the peak velocity.

When a right midventricular obstruction is found, a warning sign should switch on in the operator's mind, and a careful evaluation of the membranous ventricular septum and the subaortic region should be carried out. Particularly, in the case by Barik,[1] there is no reported septal defect, but in the video of the left heart angiography, there is clearly a small ventricular septal defect, filling with contrast.

Moreover, a discrete subaortic fibromuscular ridge may potentially develop even many years after the DCRV is diagnosed.

Therefore, the importance of a long-term follow-up is evident, considering that: (1) a small percentage of ventricular septal defect operated in infancy may develop a DCRV after many years, (2) operated DCRV itself may relapse and progress, and (3) associated left ventricular outflow obstruction may appear with late onset.

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  References Top

Barik R. Role of echocardiograghy in treating a case of double chamber right ventricle with delayed presentation. J Cardiovasc Echography 2017;27:10-3.  Back to cited text no. 1
  [Full text]  
Chellappan S, Sahu B, Sathe YC. Isolated double-chambered right ventricle with intact interventricular septum. J Cardiovasc Echography 2016;26:127-30.  Back to cited text no. 2
  [Full text]  
Freedom RM, Yoo SJ, Mikailian H, Williams WG. The Natural and Modified History of Congenital Heart Disease. New York, USA.: Wiley-Blackwell; 2004.  Back to cited text no. 3
Lai WW, Mertens LL, Cohen MS, Geva T. Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult. New York, USA: Wiley-Blackwell; 2009.  Back to cited text no. 4


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