Antenatal diagnosis of Double chambered Left Ventricle -
Post-natal evolution to LV dysfunction.
Abstract : Double chambered left ventricle is an exceedingly
rare congenital anomaly. We report a case diagnosed prenatally at
24 weeks of gestation and its postnatal evolution to left ventricular
dysfunction.
Key words: Double Chambered Left Ventricle; Antenatal diagnosis; Fetal
Echocardiography.
Introduction: Double Chambered Left Ventricle (DCLV) is an
exceedingly rare congenital malformation which there are 2 types
according to the relationship between the main chamber (MC) and the
accessory chamber (AC) of the left ventricle (LV). In type A, MC and AC
are in a superior-inferior arrangement. In type B, the rarer, MC and AC
are parallel to each other and lie side by side [1]. We report a new
case of type B DCLV diagnosed antenatally in whom the post-natal
evolution was characterized by occurrence of LV dysfunction.
Case report: A 38 year-old primigravida woman was referred at
24 weeks of gestation because of abnormal fetal heart on routine
obstetric ultrasound screening. No other anomaly was suspected on
echography.
Fetal echocardiography showed that the left ventricle (LV) was divided
into 2 cavities of equal size that lie side by side (figure 1, video 1).
The main chamber (MC) was in connection with mitral and aortic valves
(figure 2, video 2). The aneurysm-like accessory chambers (AC) was large
and connected to the LV through a wide communication in the lateral
wall. The 2 chambers were separated by muscle bundles. M-mode
echocardiogram demonstrated that the AC contracted synchronously with
the MC and the RV characterizing a DCLV instead of a left ventricular
aneurysm (figure 3). Color doppler showed that filling of AC occurred in
diastole and draining toward MC during systole (Figure 4).
Amniocentesis with CGH-array analysis was normal. The mother gave birth
to a female infant weighting 2.8 kg at 37 weeks of gestation. The
cardiovascular findings suspected prenatally were confirmed after birth
by cross-sectional echocardiography performed on day 1 (figure 5, video
3). The newborn was asymptomatic. Her EKG showed inverted T waves in
leads V3 to V6. There was no intraventricular conduction delay.
During subsequent visits, LV dilated progressively, and LV function
decreased (figure 6, videos 4). There was a mild mitral insufficiency.
It prompted introduction of a treatment with captopril and carvedilol at
2 months of age. At age 8 months the child is still asymptomatic on beta
blockers and ACE inhibitor. Physical examination is normal.