Non-compaction cardiomyopathy in pregnancy: a case report of spongy myocardium in both mother and foetus and systematic review of literature

PURPOSE: Cardiovascular disease is the main nonobstetric cause of maternal death during pregnancy and is present in 0.5-4% of pregnancies in the western world. While hypertensive disorders remain the most frequent events, occurring in 6-8% of all pregnancies, cardiomyopathies are rare but encompass high complication rates. The aim of this systematic review is to report all data available up to date regarding pregnancies in patients with left ventricular noncompaction (LVNC) cardiomyopathy. METHODS: PubMed, Medline, Cochrane, Scopus and Embase were searched, up to January 2019, using combinations of these terms: left ventricular noncompaction, hypertrabeculation cardiomyopathy, spongy myocardium, spongiform cardiomyopathy and delivery, gestation, pregnancy, cesarean section (CS). After careful selection, 22 articles, reporting a total of 30 cases, including our own were included in the review. RESULTS: Fifteen out of 26 women (58%) were diagnosed with LVNC before pregnancy. Around 56% of women presented with symptoms during pregnancy while 44% were asymptomatic. Heart failure is by far the most common symptom occurring in almost half the cases. Uncommon clinical presentations included a heart attack, a stroke, and pulmonary hypertension. Timing of delivery was reported preterm in 58% of cases and at term in 42%. Eleven women gave birth through vaginal delivery, while 15 (58%) underwent a CS. Our reported case is the first case of a pregnancy where both mother and fetus are affected by LVNC and the fetus is diagnosed prenatally. CONCLUSIONS: LVNC is not a contraindication for pregnancy, but clearly increases the risk of preterm birth and the rate of cesarean section. On the other hand, pregnancy in a LVNC patient exposes her to increased risk of clinical deterioration. Further studies are needed to better characterize the management of pregnancies in women with cardiomyopathies.