History

Most patients with double-chambered right ventricle (DCRV) initially present with no symptoms.

The most common reason for referral is the detection of a murmur.

Clinically, patients with double-chambered right ventricle and no ventricular septal defect (VSD) resemble patients with isolated pulmonary valve stenosis.

When a VSD is present, the clinical picture relates to a VSD. Usually, the patient is diagnosed with a VSD or pulmonary outflow tract obstruction and, subsequently, may show signs of progression of the outflow obstruction, such as cyanosis, fatigue, and decreased exercise tolerance.

Rowland et al describe four physiologic groups (see Pathophysiology) with patients presenting usually with left-to-right shunt or tetralogy of Fallot physiology.^[6]

Patients with severe right ventricle (RV) hypertension may present with cyanosis, RV failure, failure to thrive, and fatigue.

Association with other syndromes is well recognized, and double-chambered right ventricle may be found during their workup.

Physical Examination

Most patients are nondysmorphic and acyanotic with normal peripheral examination findings. Auscultation reveals a variable intensity of the second heart sound.

A holosystolic ejection murmur, which peaks in intensity near midsystole, with greatest intensity at mid-left and upper-left precordial areas, characterizes double-chambered right ventricle.

An right ventricle (RV) heave, hepatomegaly, and tachypnea indicate RV hypertension.

Differential Diagnoses

Barik R. Role of echocardiograghy in treating a case of double chamber right ventricle with delayed presentation. J Cardiovasc Echogr. 2017 Jan-Mar. 27(1):10-13. [QxMD MEDLINE Link]. [Full Text].
Restivo A, Cameron AH, Anderson RH, Allwork SP. Divided right ventricle: a review of its anatomical varieties. Pediatr Cardiol. 1984 Jul-Sep. 5(3):197-204. [QxMD MEDLINE Link].
Byrum CJ, Dick M 2nd, Behrendt DM, et al. Excitation of the double chamber right ventricle: electrophysiologic and anatomic correlation. Am J Cardiol. 1982 Apr 1. 49(5):1254-8. [QxMD MEDLINE Link].
Hubail ZJ, Ramaciotti C. Spatial relationship between the ventricular septal defect and the anomalous muscle bundle in a double-chambered right ventricle. Congenit Heart Dis. 2007 Nov. 2(6):421-3. [QxMD MEDLINE Link].
Vogel M, Smallhorn JF, Freedom RM, et al. An echocardiographic study of the association of ventricular septal defect and right ventricular muscle bundles with a fixed subaortic abnormality. Am J Cardiol. 1988 Apr 1. 61(10):857-60. [QxMD MEDLINE Link].
Rowland TW, Rosenthal A, Castaneda AR. Double-chamber right ventricle: experience with 17 cases. Am Heart J. 1975 Apr. 89(4):455-62. [QxMD MEDLINE Link].
Patibandla S, Kyaw H. Double chambered right ventricle. StatPearls [Internet]. 2022 Jan. [QxMD MEDLINE Link]. [Full Text].
Wong PC, Sanders SP, Jonas RA, et al. Pulmonary valve-moderator band distance and association with development of double-chambered right ventricle. Am J Cardiol. 1991 Dec 15. 68(17):1681-6. [QxMD MEDLINE Link].
Goitein KJ, Neches WH, Park SC, et al. Electrocardiogram in double chamber right ventricle. Am J Cardiol. 1980 Mar. 45(3):604-8. [QxMD MEDLINE Link].
Amano M, Izumi C, Hayama Y, et al. Surgical outcomes and postoperative prognosis beyond 10 years for double-chambered right ventricle. Am J Cardiol. 2015 Nov 1. 116(9):1431-5. [QxMD MEDLINE Link].
Kahr PC, Alonso-Gonzalez R, Kempny A, et al. Long-term natural history and postoperative outcome of double-chambered right ventricle--experience from two tertiary adult congenital heart centres and review of the literature. Int J Cardiol. 2014 Jul 1. 174(3):662-8. [QxMD MEDLINE Link].
Tsuchikane E, Kobayashi T, Kirino M, et al. Percutaneous myocardial ablation in double-chamber right ventricle. Catheter Cardiovasc Interv. 2000 Jan. 49(1):97-101. [QxMD MEDLINE Link].
[Guideline] Maron BJ, Chaitman BR, Ackerman MJ, et al. Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases. Circulation. 2004 Jun 8. 109(22):2807-16. [QxMD MEDLINE Link].
Amedro P, Werner O, Abassi H, et al. Health-related quality of life and physical activity in children with inherited cardiac arrhythmia or inherited cardiomyopathy: the prospective multicentre controlled QUALIMYORYTHM study rationale, design and methods. Health Qual Life Outcomes. 2021 Jul 28. 19(1):187. [QxMD MEDLINE Link]. [Full Text].
Telagh R, Alexi-Meskishvili V, Hetzer R, Lange PE, Berger F, Abdul-Khaliq H. Initial clinical manifestations and mid- and long-term results after surgical repair of double-chambered right ventricle in children and adults. Cardiol Young. 2008 Jun. 18(3):268-74. [QxMD MEDLINE Link].
Alva C, Ho SY, Lincoln CR, et al. The nature of the obstructive muscular bundles in double-chambered right ventricle. J Thorac Cardiovasc Surg. 1999 Jun. 117(6):1180-9. [QxMD MEDLINE Link].
Ceyran H, Narin N, Tasdemir K, et al. Double-chambered right ventricle mimicking asymmetric septal hypertrophy. Turk J Pediatr. 2003 Jan-Mar. 45(1):80-2. [QxMD MEDLINE Link].
Freedom RM, Mawson JB, Yoo SJ, Benson LN. The divided right ventricle: anomalous right ventricular muscle bundles and other entities. Congenital Heart Disease: Textbook of Angiocardiography. Williston, VT: Blackwell Futura; 1997. 389-407.
Hoffman P, Wojcik AW, Rozanski J, et al. The role of echocardiography in diagnosing double chambered right ventriclein adults. Heart. 2004 Jul. 90(7):789-93. [QxMD MEDLINE Link]. [Full Text].
Kurosawa H, Becker AE. Surgical anatomy of the atrioventricular conduction bundle in anomalous muscle bundle of the right ventricle with subarterial ventricular septal defect. Pediatr Cardiol. 1985. 6(3):157-60. [QxMD MEDLINE Link].
Kveselis D, Rosenthal A, Ferguson P, et al. Long-term prognosis after repair of double-chamber right ventricle with ventricular septal defect. Am J Cardiol. 1984 Dec 1. 54(10):1292-5. [QxMD MEDLINE Link].
Nagashima M, Tomino T, Satoh H, et al. Double-chambered right ventricle in adulthood. Asian Cardiovasc Thorac Ann. 2005 Jun. 13(2):127-30. [QxMD MEDLINE Link].
Penkoske PA, Duncan N, Collins-Nakai RL. Surgical repair of double-chambered right ventricle with or without ventriculotomy. J Thorac Cardiovasc Surg. 1987 Mar. 93(3):385-93. [QxMD MEDLINE Link].
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 Oct. 50(4):776-80. [QxMD MEDLINE Link].
Puvaneswary M, Indira N, Sreedhar M, Barooah B. Double-chambered right ventricle: magnetic resonance imaging findings. Australas Radiol. 2005 Apr. 49(2):170-4. [QxMD MEDLINE Link].
Vogel M, Freedom RM, Brand A, et al. Ventricular septal defect and subaortic stenosis: an analysis of 41 patients. Am J Cardiol. 1983 Dec 1. 52(10):1258-63. [QxMD MEDLINE Link].

Media Gallery

Double-Chambered Right Ventricle. Electrocardiogram of an 18-month-old boy with double-chambered right ventricle. Note the upright T waves in the right precordial leads.
Double-Chambered Right Ventricle. Right anterior oblique (RAO) angiogram demonstrating proximal and distal chambers of right ventricle (Image courtesy of R.M. Freedom, MD).
Double-Chambered Right Ventricle. Lateral right ventriculography of a patient with double-chambered right ventricle. Large arrow indicates the presence of a fibromuscular obstruction with division of the right ventricle; small arrows outline pulmonary valve stenosis (Image courtesy of R.M. Freedom, MD).
Double-Chambered Right Ventricle. Subcostal right anterior oblique (RAO) echocardiograph view demonstrating right ventricle muscle bundles separating proximal from distal (*) chamber. PV = Pulmonary valve (Image courtesy of J. Smallhorn, MD)
Double-Chambered Right Ventricle. Subcostal right anterior oblique (RAO) echocardiograph view with color Doppler demonstrating ventricular septal defect jet to proximal chamber. (*) = Distal chamber (Image courtesy of J. Smallhorn, MD).