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.