Characteristics of double-chambered right ventricle in adult patients

Abstract

Background/aims: This study evaluated the clinical features of double-chambered right ventricle (DCRV) in adults. Most cases of DCRV are diagnosed and treated during childhood. Consequently, very few reports include cases in which its clinical characteristics are evident in adults.

Methods: We reviewed the clinical data for 10 adult patients (age > or = 18 years) with DCRV.

Results: Electrocardiogram showed right ventricular hypertrophy in 3 DCRV patients. All cases were associated with ventricular septal defect (VSD; 7 for perimembranous, 2 for muscular outlet, and 1 for the subarterial type). Surgical correction was done for 7 DCRV patients all of whom survived operations. Their follow-up echocardiogram showed the pressure gradient in their right ventricle was significantly decreased from 69.4 +/- 17.2 mmHg preoperatively to 10.2 +/- 5.0 mmHg postoperatively (p < 0.05). In the short-term follow-up, there was no significant increase in the pressure gradient in the right ventricle.

Conclusions: There are lots of cases of DCRV that are not diagnosed accurately in adults. In our experience, all DCRV cases had VSD and surgical correction of these cases showed excellent results. Therefore, accurate diagnosis of DCRV is necessary so that DCRV is not overlooked and operations are enabled within an appropriate time.

Keywords: Double outlet right ventricle; Heart defects, congenital; Heart ventricles; Ventricular outflow obstruction.

Figure 1

Right ventricular pressure tracing (patient 9). Thin arrow, infundibular (outflow chamber) systolic pressure of 42 mmHg. Thick arrow, apical (inflow chamber) systolic pressure of 98 mmHg.

Figure 2

Frontal view of the right ventriculogram (patient 7) at end-systole showing a low and oblique muscular obstruction. Anomalous muscle bundle divided the cavity into the two chambers. AMB, abnormal muscle bundle; MPA, main pulmonary artery; PV, pulmonary valve; RV, right ventricle; RVOT, right ventricle outflow tract.

Figure 3

Doppler echocardiographic assessments of changes in the pressure gradient across the abnormal muscle bundles in the right ventricle over time in 7 DCRV patients with the pressure gradient in the right ventricle greater than 20 mmHg (O), or with significant VSD, Qp / Qs ≥ 2.0 (X). DCRV, double-chambered right ventricle; VSD, ventricular septal defect; Op / Qs, pulmonary flow volume / systemic flow volume.

Figure 4

Transthoracic echocardiograph from a modified short axis view at aortic level (patient 9). Color flow Doppler imaging indicates the turbulent flow originating from the left ventricle through the ventricular septal defect to the abnormal thickened mass in the right ventricle, here accelerated in the mosaic pattern.