Pregnancy Outcomes in Left Ventricular Noncompaction

Left ventricular noncompaction (LVNC) is a rare cardio-myopathy characterized by bilayered appearance of the myocardium, with prominent left ventricular trabeculations, and deep intertrabecular recesses. Specific morphologic diagnostic criteria and categorization as a primary genetic or "unclassified" cardiomyopathy remain controversial. Because LVNC is associated with increased incidence of arrhythmia, embolic events, and heart failure, we sought to determine its pregnancy outcomes given the limited infor-mation regarding obstetric risks in this population. From a retrospective cohort of 339 adults meeting the imaging criteria for LVNC seen at Mayo Clinic, Minnesota between 2000 and 2016, 61 women between the ages of 18 and 40 years were identified. The inclusion criteria included the presence of LVNC based on fulfilling both echocardio-graphic criteria of Chin et al1 and Jenni et al2; 22 patients also underwent cardiac magnetic resonance imaging with confirmation of LVNC by the criteria of Petersen et al.3 The medical charts were individually reviewed, and com-plete obstetric data were available for 45 women (74%). The Institutional Review Board approved the study. Clinical, echocardiographic, and obstetric data are listed in Table 1. Of the 45 patients, 30 had 78 documented preg-nancies, of which 61 were successful. The maternal age at first pregnancy was 27 +/- 4.6 years. Four were diagnosed with LVNC before the first pregnancy and 9 (30%) with LVNC in the peripartum period; the remaining patients were diagnosed with LVNC later in life. Four had concomi-tant simple congenital heart disease (atrial septal defects in 2 and small ventricular septal defect and coarctation of the aorta in 1 each). antepartum complications. Six women (20%) delivered by cesarean section, 4 performed for obstetric and 2 for car- diac indications: 1 delivered at 32 weeks owing to ventric- ular arrhythmias with hemodynamic instability, and the other at 38 weeks in the setting of maternal atrial fibrilla- tion with rapid ventricular response. There were 16 spon- taneous pregnancy losses, and only 1 termination due to concern for increased maternal cardiovascular risk; this patient had 2 pregnancies before LVNC was diagnosed, with the first pregnancy delivering at term and the second resulting in an intrauterine death at 30 weeks. Of note, of all 61 successful pregnancies, 2 children were diagnosed with LVNC.