Background:
Left ventricular noncompaction (LVNC) is a congenital condition that results from arrest of the normal compaction process of the myocardium during fetal development. LVNC shows variability in its genetic pattern, pathophysiologic findings, and clinical presentations1.
There are approximately 25 case reports of this condition in pregnancy from 2003-20142.
Case details:
29 year old lady, G2P0M1 was referred to obstetric medicine clinic. She was diagnosed with LVNC at age 7.
She was asymptomatic and had not suffered any complications. She had no family history of cardiomyopathy.
Her examination was unremarkable.
Her cardiac disease was stable during pregnancy. She was commenced on prophylactic anticoagulation due to risk of systemic thromboembolism.
Initial echocardiogram at 22 weeks gestation showed prominent band across the left ventricle isolating the apical third of the ventricle. Echogenic densities were noted in the apical cavity. Her ejection fraction was 60% with evidence of apical akinesis.
Repeat echocardiogram at 32 weeks showed similar findings with the ejection fraction of 63%
She underwent emergency caesarean section at 37 weeks due to concerns of placental insufficiency and breech presentation. She had uneventful recovery with no intra-operative or post-operative complications. She will continue on prophylactic anticoagulation for 6 weeks post partum.
The baby was noted to have a systolic murmur and is awaiting further investigations.
Conclusions:
LVNC has been considered as a rare condition. There is limited evidence in literature about management of LVNC with pregnancy.
Asymptomatic patients have good prognosis3. It has been associated with increased risk of thromboembolism which is further enhanced by pregnancy. Hence, adequate anticoagulation may be warranted but evidence is lacking4. There are 11 case reports till now of peripartum cardiomyopathy associated with LVNC (5-11). Most case reports show severe presentation during pregnancy which could be a publication bias.
Our case highlights a successful uneventful pregnancy outcome in a patient with asymptomatic left ventricular non compaction cardiomyopathy.