CARDIOVASCULAR JOURNAL OF AFRICA • Vol 22, No 5, September/October 2011
274
AFRICA
Double-chambered right ventricle: an uncommon
congenital heart disease. Case report and literature
review
BA ANIMASAHUN, EN EKURE, OF NJOKANMA
Abstract
A double-chambered right ventricle (DCRV) is a rare
congenital heart disease and an uncommon cause of conges-
tive cardiac failure. An anomalous muscle band divides the
right ventricle into two cavities, causing variable degrees of
obstruction. Echocardiography is considered a useful method
for the diagnosis of this pathology, especially in children.
An eight-year-old patient with a small ventricular septal
defect (VSD) and double-chambered right ventricle present-
ed with a history of palpitations, easy fatigability and recur-
rent fever. On presentation, she had features of congestive
cardiac failure. A complete diagnosis was initially missed
with transthoracic two-dimensional (2-D) echocardiography
but later obtained based on transthoracic 2-D echocar-
diography with Doppler facility. This was confirmed with
cardiac catheterisation. The patient was referred for surgical
correction, which was successful.
Due to the rarity of this condition and the consequences
of missing the diagnosis, we present this case in order to
highlight the rarity of this congenital heart disease in child-
hood.
Keywords:
double-chamber right ventricle, echocardiography
Submitted 1/6/10, accepted 19/8/10
Cardiovasc J Afr
2011;
22
: 274–277
DOI: 10.5830/CVJA-2010-068
Case report
An eight-year-old female patient presented to the emergency unit
of the Lagos State University Teaching Hospital, Lagos, with a
five-month history of palpitations, easy fatigability, a four-month
history of loss of appetite and weight loss, and two months of
recurrent fever. The father had died two years earlier secondary
to complications of hypertensive heart disease.
On presentation she was acutely ill looking, pale and dysp-
noeic but acyanosed. The respiratory rate was 58 cycles per
minute and her breath sounds were vesicular with reduced
intensity of breath sounds at the lung bases and bilateral basal
crepitations.
The pulse rate was 128 beats per minute, full volume, regu-
lar and bounding, with a blood pressure of 90/70 mmHg. The
precordium was hyperactive with a left parasternal shift and
heaving apex; apex beat was located at the sixth left intercostal
space along the mid-clavicular line. The first heart sound was
normal but there was fixed splitting of the second heart sound
and a loud P2, with gallop rhythm and multiple murmurs (grade
3/6 diastolic murmur, maximum at the left upper sternal border
and a grade 4/6 ejection systolic murmur, maximum at the same
area, with a grade 4/6 pansystolic murmur, maximum at the left
lower sternal border).
The liver was palpably enlarged 10 cm below the costal
margin, firm, smooth and tender. The patient was in Tanner 1
staging of sexual maturity.
An initial assessment of congestive cardiac failure with
pulmonary hypertension secondary to an acyanotic congeni-
tal heart disease, possibly atrial septal defect was made to
rule out rheumatic heart disease. Packed-cell volume was
27%, blood culture did not yield any organisms, and a chest
radiograph showed cardiomegaly with cardiothoracic ratio of
0.65. There were scattered fluffy opacities in the lung field.
Electrocardiography showed sinus tarchycardia, right-axis devia-
tion with widened P wave, tall R waves in V1 and V2, and deep
S waves in V5 and V6, suggestive of right atrial enlargement and
biventricular hypertrophy.
Initial 2-D echocardiography showed situs solitus, atrio-
ventricular and ventriculo-arterial concordance, dilated inferior
vena cava with intact inter-atrial and inter-ventricular septae but
right atrial enlargement and bi-ventricular hypertrophy, and right
ventricular hypertrophy greater than left ventricular hypertrophy
with severe tricuspid and pulmonary regurgitation.
The patient was commenced on antibiotics and anti-heart
failure drugs and was discharged home after one week, with
remarkable clinical improvement. She continued on anti-heart
failure drugs and clinical follow up and was prepared for
re-evaluation in a centre with a colour Doppler echocardiography
facility. A repeat echocardiography (Fig. 1) showed significant
muscle bundles in the right ventricular outflow tract with a
small peri-membranous ventricular septal defect and a severe
double-chambered right ventricle with a right ventricular outlet
tract gradient of 104 mmHg. Echo findings were confirmed with
cardiac catheterisation.
She had transatrial right ventricular muscle excision and relief
of the right ventricular outlet flow obstruction with closure of the
VSD. She is currently being followed up and is doing well.
Discussion
Double-chambered right ventricle is a rare congenital disease.
1,2
It is characterised by aberrant hypertrophied muscular bands that
Department of Paediatrics and Child Health, Lagos State
University College of Medicine, Ikeja, Lagos, Nigeria
BA ANIMASAHUN, MPH (Lag), FWACP (Paed), FMC (Paed),
OF NJOKANMA, FWACP, FMC (Paed)
Department of Paediatrics, University of Lagos College of
Medicine, Idi-Araba, Lagos, Nigeria
EN EKURE, FWACP
