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[Development of double-chambered right ventricle after surgical closure of a ventricular septal defect]
1Département Universitaire de Pédiatrie, CHR Citadelle, Liège, Belgique.
Insights
Double-chambered right ventricle (DCRV) can develop after ventricular septal defect (VSD) repair. This rare condition requires consideration in children with a history of VSD presenting with a systolic murmur.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Defects
Background:
- Double-chambered right ventricle (DCRV) is a rare congenital heart anomaly.
- It is frequently associated with ventricular septal defect (VSD).
Observation:
- A case of DCRV is presented in a child initially treated for perimembranous VSD.
- Post-VSD repair, the child developed moderator band hypertrophy and a significant right midventricular pressure gradient.
- Diagnosis was confirmed invasively before surgical correction of DCRV.
Findings:
- DCRV development can be dissociated from the natural course or surgical repair of VSD.
- A progressive pressure gradient across the right ventricle was observed despite successful VSD repair.
- Surgical correction of DCRV was successful with no restenosis at follow-up.
Implications:
- DCRV should be suspected in children with a history of VSD, even after closure.
- A systolic ejection murmur in such patients warrants further investigation for DCRV.
- This highlights the potential for late-onset or progressive DCRV following VSD treatment.
Unlabelled:
The double-chambered right ventricle (DCRV) is a rare cardiac malformation, often associated with ventricular septal defect (VSD).
Case Report:
The authors describe a case of DCRV in a child who underwent surgical repair of perimembranous VSD at the age of 7 months because of systemic pulmonary artery hypertension and growth retardation. At this time, no pressure gradient was measured within the right ventricle and no muscle proliferation was observed on echocardiography or right ventriculography. Postoperative follow-up revealed hypertrophy of the moderator band accompanied by progressive development of a right midventricular pressure gradient, which reached 60 mm Hg at the age of 3 1/2 years. The child was operated after invasive confirmation of the diagnosis of DCRV and the haemodynamic data. The child is now 6 years old and no restenosis has been observed.
Conclusion:
DCRV is usually associated with VSD, but the course of the two diseases can be dissociated. The diagnosis of DCRV must be considered in any child with a history of VSD, even after surgical or spontaneous closure, when clinical examination reveals a systolic ejection murmur.
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