---
title: Double-chambered right ventricle
description: What double-chambered right ventricle is, why muscle bands divide the cavity of the right ventricle into two chambers, how the defect is found and which treatment approaches clinical guidelines describe.
type: disease
aliases:
  - DCRV
  - Double-chambered RV
  - Sub-infundibular stenosis
  - Subinfundibular stenosis
sources:
  - tier: guideline
    org: esc
    title: 2020 ESC Guidelines for the management of adult congenital heart disease
    year: 2020
    doi: 10.1093/eurheartj/ehaa554
    url: https://doi.org/10.1093/eurheartj/ehaa554
  - tier: reference
    org: statpearls
    title: Double-Chambered Right Ventricle
    year: 2024
    url: https://www.ncbi.nlm.nih.gov/books/NBK546625/
  - tier: guideline
    org: minzdrav-rf
    title: Дефект межжелудочковой перегородки
    year: 2024
    url: https://cr.minzdrav.gov.ru/view-cr/806_1
sources_checked: 2026-10-14
review: null
---

Double-chambered right ventricle is a congenital heart defect in which a narrowing in the middle of the cavity of the <Structure id="right-ventricle">right ventricle</Structure> divides it into two chambers: one nearer the inlet, with high pressure, and one further downstream, near the outlet, with low pressure.<Cite n={2} /> The narrowing is formed by large, thickened muscle bands or ridges inside the ventricle.<Cite n={1} />

It is one form of obstruction in the path of blood from the right ventricle to the pulmonary artery. Such an obstruction can lie at different levels: below the outflow tract of the ventricle (this is double-chambered right ventricle), in the outflow tract itself, at the <Structure id="pulmonary-valve">pulmonary valve</Structure> or above it.<Cite n={1} /> When the valve itself is narrowed, that is a different disease: [pulmonary valve stenosis](../pulmonary-valve-stenosis/).<Cite n={1} />

<Diagram name="double-chambered-right-ventricle">
  <p slot="normal">
    The diagram shows systole, when the right ventricle contracts. Normally there is no narrowing
    in the path of blood from the right ventricle to the pulmonary artery: not inside the ventricle,
    not in its outflow tract and not at the pulmonary valve.<Cite n={1} />
  </p>
  <p slot="disease">
    In double-chambered right ventricle, thickened muscle bands narrow the cavity in its
    middle.<Cite n={[1, 2]} /> In the diagram, below them are the inlet and apex of the ventricle,
    with high pressure and a thickened wall; above them is the outflow tract, with low pressure and
    a wall that is not thickened.<Cite n={1} /> The narrowing lies inside the ventricle, not at the
    valve, so the pulmonary valve in the diagram is drawn as normal.<Cite n={1} /> A ventricular septal
    defect is often present as well; it is not shown in the diagram.<Cite n={[1, 2]} />
  </p>
</Diagram>

## Why it happens

<Patient>

It is a congenital defect, but cases in which it appears after birth have also been described.<Cite n={2} /> Most often it occurs together with a [ventricular septal defect](../ventricular-septal-defect/) (VSD): according to StatPearls, many people with double-chambered right ventricle, up to 75%, have a defect in the membranous part of the septum.<Cite n={[1, 2]} />

According to the ESC guidelines, in people with a VSD, most often a perimembranous one, double-chambered right ventricle can develop over time; a possible cause is damage to the inner lining of the right ventricle by the fast jet of blood that passes through the defect.<Cite n={1} /> StatPearls describes such cases in some people with a small VSD: muscle structures inside the right ventricle gradually thicken.<Cite n={2} />

The defect can also occur together with other congenital defects, for example [tetralogy of Fallot](../tetralogy-of-fallot/), [Ebstein anomaly](../ebstein-anomaly/), [transposition of the great arteries](../transposition-of-the-great-arteries/), [atrial septal defect](../atrial-septal-defect/) and [pulmonary valve stenosis](../pulmonary-valve-stenosis/).<Cite n={2} />

The narrowing gets worse over time.<Cite n={1} />

</Patient>

<Student>

The ESC places double-chambered RV at the sub-infundibular level of right ventricular outflow tract obstruction (RVOTO): narrowing between prominent, hypertrophied muscle bands or ridges separates the hypertrophied, high-pressure inlet and apical portions of the RV from the low-pressure, non-hypertrophied infundibular portion, which is itself non-obstructive.<Cite n={1} /> At the infundibular and, to some extent, the sub-infundibular level, the obstruction tends to be dynamic: the orifice narrows in systole.<Cite n={1} />

StatPearls describes two types by the tissue causing the mid-ventricular obstruction: in type 1, anomalous muscle bundles cross the RV; in type 2, the obstruction is caused by hypertrophy of the parietal and septal muscles.<Cite n={2} /> An acquired form can develop after birth through progressive hypertrophy of the crista supraventricularis or other muscle structures of the RV in some patients with small, restrictive VSDs.<Cite n={2} /> The most common associated lesion is a membranous VSD; it can also occur with [double outlet right ventricle](../double-outlet-right-ventricle/), ruptured sinus of Valsalva aneurysm, quadricuspid aortic valve, persistent left superior vena cava and other defects.<Cite n={2} />

In the ESC classification, double-chambered RV (repaired or unrepaired) is a congenital heart defect of moderate complexity.<Cite n={1} />

</Student>

<HeartModel highlight={['right-ventricle', 'tricuspid-valve', 'pulmonary-valve']} />

The 3D model shows a normal heart, without muscle bands. The highlight marks the right ventricle: in this defect the narrowing lies in its middle part, between the inlet, where blood enters through the <Structure id="tricuspid-valve">tricuspid valve</Structure>, and the outflow tract leading to the pulmonary valve.<Cite n={[1, 2]} />

## Symptoms

<Patient>

According to the ESC guidelines, adults with double-chambered right ventricle may have no symptoms.<Cite n={1} /> When there are symptoms, they are shortness of breath, chest discomfort, dizziness or fainting during physical exertion.<Cite n={1} />

As with any heart condition, call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK) for fainting, chest pain or severe shortness of breath: this is the site's general safety rule, repeated at the bottom of every page.

</Patient>

<Student>

The ESC describes these manifestations together for the sub-infundibular and infundibular levels of RVOTO in adults and notes that the degree of obstruction is progressive over time.<Cite n={1} />

</Student>

## How it is diagnosed

<Patient>

When the outflow from the right ventricle is narrowed, the doctor may hear a harsh heart murmur while the ventricles contract.<Cite n={1} />

The main test is an ultrasound scan of the heart ([echocardiography](../../diagnostic/echocardiography/)): it shows the size, shape and function of the right ventricle and the exact level of the narrowing, as well as the pulmonary valve and the pulmonary artery.<Cite n={1} /> In people with a VSD, echocardiography specifically checks for double-chambered right ventricle, both when the diagnosis is made and later during follow-up.<Cite n={[1, 3]} />

Cardiac MRI and CT often give important additional information: they help find the level of the narrowing, including inside the ventricle, and measure the volumes of the right ventricle.<Cite n={1} /> Sometimes cardiac catheterisation is needed to confirm the extent, severity and level of the narrowing.<Cite n={1} />

</Patient>

<Student>

In RVOTO the ESC describes a harsh systolic murmur across the obstruction and wide splitting of the second heart sound.<Cite n={1} /> For quantifying RV size, volumes and ejection fraction, cardiac MRI is more reliable than echocardiography.<Cite n={1} />

According to the ESC, with normal RV function and normal flow, RVOTO is mild when the peak gradient is below 36 mm Hg, moderate at 36–64 mm Hg and severe when the gradient is above 64 mm Hg.<Cite n={1} /> The ESC notes that flow velocity correlates well with the pressure gradient only in discrete stenosis, for example isolated valvular stenosis.<Cite n={1} /> With a long narrowing or several stenoses in series, the Bernoulli equation overestimates the gradient; RV pressure is then estimated more reliably from the tricuspid regurgitation velocity.<Cite n={1} /> The gradient reflects the severity of the obstruction only when RV systolic function is good.<Cite n={1} />

In its echocardiography protocol for VSD, the Russian Ministry of Health guideline states that double-chambered RV must be excluded when an isolated VSD is diagnosed.<Cite n={3} />

</Student>

## How it is treated

<Patient>

Whether an intervention (surgery or a procedure through a catheter) is needed, and when, is decided by the doctor. The European Society of Cardiology (ESC) gives the same rules for any narrowing in the path of blood from the right ventricle to the pulmonary artery, not only for double-chambered right ventricle. If the narrowing is severe, the ESC recommends an intervention even when there are no symptoms. If the narrowing is less severe, an intervention should be considered when symptoms caused by the narrowing appear or the right ventricle starts to work less well.<Cite n={1} />

If an intervention is needed and the narrowing lies inside the right ventricle, the ESC guidelines name heart surgery for it. Widening a narrowing with a balloon through a catheter is recommended by the ESC for a different defect, when the pulmonary valve itself is narrowed.<Cite n={1} /> Both operations and catheter procedures for congenital defects like these should be done only in centres that specialise in congenital heart disease.<Cite n={1} />

</Patient>

<Student>

The ESC recommendations on intervention in RVOTO apply to all its levels, not only to double-chambered RV. Provided that no valve replacement is required, intervention at any level is recommended regardless of symptoms in severe obstruction (Doppler peak gradient above 64 mm Hg; I C).<Cite n={1} /> With a gradient below 64 mm Hg, intervention should be considered in the presence of one or more of the following: symptoms related to the stenosis; decreasing RV function and/or progressive tricuspid regurgitation to at least moderate; right-to-left shunt through an ASD or VSD (IIa C).<Cite n={1} /> In the text of the treatment section (without a class of recommendation), the ESC states that surgery is recommended for sub-infundibular or infundibular stenosis and hypoplastic pulmonary annulus, for dysplastic pulmonary valves, and for associated lesions requiring surgery, such as severe pulmonary or tricuspid regurgitation; catheter balloon valvotomy is recommended for non-dysplastic valvular and peripheral pulmonary stenosis.<Cite n={1} />

</Student>

<Quiz />