Double-Chambered Right Ventricle Clinical Presentation

Updated: Jan 07, 2016
  • Author: Shubhayan Sanatani, MD; Chief Editor: Stuart Berger, MD  more...
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Presentation

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 4 physiologic groups (see Pathophysiology) with patients presenting usually with left-to-right shunt or tetralogy of Fallot physiology. [5]

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.

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Physical

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 RV heave, hepatomegaly, and tachypnea indicate RV hypertension.

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Causes

No inheritance pattern has been described, and no risk factors for developing the disease have been encountered.

Sporadic cases have been described in patients with Down syndrome and Noonan syndrome.

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