Pregnancy in Familial Left Ventricular Noncompaction-Associa
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Left ventricular (LV) noncompaction is a phenotypic variant of LV structure; however, it also can be associated with cardiomyopathies. Pregnancy in the setting of LV dysfunction carries a risk of maternal and fetal morbidity and mortality.

A 27-year-old female patient with a history of familial, left ventricular noncompaction (LVNC)-associated cardiomyopathy presented at 20 weeks’ gestation with palpitations. The patient had a history of a familial, dilated cardiomyopathy initially diagnosed at the age of 8 and later phenotyped as LVNC-associated cardiomyopathy. She was treated with beta-blockers and angiotensin-converting enzyme (ACE) inhibitors and maintained a left ventricular ejection fraction (LVEF) of 52%.

TTE at 22 weeks’ gestation demonstrated a mildly dilated and diffusely hypokinetic left ventricle (LV) (LVEF 47%) with prominent LV trabeculations and a ratio of noncompacted to compacted (NC/C) myocardium of 2.6 in end-systole at the inferior and lateral mid-ventricular level. Cardiac magnetic resonance imaging performed before conception demonstrated mild LV dysfunction (LVEF 49%) with an NC/C myocardium ratio >2.3 in the mid-inferior, apical inferior, and mid-lateral segments, suggestive of LVNC phenotype.

The patient was seen in consultation by the multidisciplinary Cardiovascular Disease and Pregnancy Service at our institution consisting of maternal fetal medicine, cardiac anesthesia, and cardiology. The decision was made for induction of labor at 37 weeks’ gestation with central venous pressure monitoring with cardiac anesthesia present at the time of induction.