Use of elexacaftor/tezacaftor/ivacaftor combination in pregnancy.

INTRODUCTION: Management of cystic fibrosis has recently stepped forward with the introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulators, although data on potential adverse effects are lacking for many categories of patients, such as pregnant women. METHODS: We report one of the first reports on the outcome of pregnancy in a woman treated with Elexacaftor/Tezacaftor/Ivacaftor during the second and third trimester of pregnancy, showing a significant improvement of respiratory status, compared with the first trimester when the medication was discontinued due to unknown and, therefore, potential teratogenic effects. Also, we performed the review of the existing literature on the topic. RESULTS: The course of pregnancy was uneventful, with reference to major obstetric complications, and the patient delivered a healthy neonate. These results were similar to those coming from other short series of pregnant women affected by cystic fibrosis and treated with CFTR modulators during pregnancy. CONCLUSIONS: Thus, despite the lack of evidence on the topic, the use of Elexacaftor/Tezacaftor/Ivacaftor in pregnancy seems to be apparently not associated with major adverse events, thus opening optimistic scenarios in terms of management of these patients.

Severe maternal thrombocytopenia and prenatal invasive procedures: still a grey zone.

Management of severe thrombocytopenia, particularly of ITP, in pregnancy is mainly based on expert consensus and clinical experience while there are no clear indications about the minimum platelet count requested for prenatal diagnosis invasive procedures. Since the lack of specific recommendations we reported our clinical management of a patient suffering from severe thrombocytopenia, undergoing amniocentesis. Due to the anecdotic possibility of maternal and fetal bleeding in case of severe thrombocytopenia, prophylaxis with IVIG or even corticosteroids could be considered as a safer strategy to prevent post-procedural adverse outcomes.

Pregnancy outcome in systemic lupus erythematosus patients: a monocentric cohort analysis.

OBJECTIVE: SLE is an autoimmune disease, mainly affecting women of childbearing age, with possible impact on pregnancy. In this study, we evaluated pregnancy outcomes in all pregnant patients affected by SLE, followed in the context of a rheumatology/gynaecology multi-disciplinary team. METHODS: Since 2008, we evaluated 70 consecutive pregnancies occurring in 50 SLE patients referring to the Lupus Clinic of Sapienza University of Rome; as controls we evaluated 100 consecutive pregnancies in 100 women without autoimmune diseases. RESULTS: By comparing SLE patients and controls, we did not find differences in terms of pregnancy outcomes, except for the occurrence of small for gestational age, which was significantly higher in the SLE group (22.8% vs 11%, P =0.003). Small for gestational age was associated with the positivity for anti-dsDNA, anti-Sm and anti-RNP (P =0.009, P =0.02, P =0.002, respectively). A disease flare was reported in 28 pregnancies (40%) and in 31 puerperium periods (44.3%). Flare during pregnancy was associated with anti-SSA (P =0.02), while puerperium relapse with previous MMF treatment (P =0.01) and haematological flare during pregnancy (P =0.03). CONCLUSION: The present study confirms how pre-gestational counselling and a multi-disciplinary approach could result in positive pregnancy outcomes for SLE patients. The high percentage of disease relapse justifies even more the need for multi-disciplinary management.

Breastfeeding in women affected by systemic lupus erythematosus: Rate, duration and associated factors.

OBJECTIVE: Breastfeeding is a crucial moment for both mothers and child, providing a beneficial effect on child survival, nutrition, development and on maternal health. Despite the prevalent involvement of childbearing women in systemic lupus erythematosus (SLE), breastfeeding is still a neglected topic. The objective of this study was to evaluate breastfeeding frequency, duration and associated factors in SLE women. METHODS: We consecutively enrolled SLE pregnant women reporting demographic, clinical, serological, gynaecological and obstetric data. Breastfeeding experience was evaluated by using a specific questionnaire. Disease activity was assessed before and during pregnancy as well as during postpartum. RESULTS: A total of 57 pregnancies in 43 SLE women were included in the present study. In almost all the pregnancies, mothers planned to breastfeed their child (96.5%) and forty-one (71.9%) actually did breastfeed. The median time of breastfeeding was 3 months (IQR 7). Non-breastfeeding women showed a more frequent caesarean section (p = 0.0001), IUGR occurrence (p = 0.004) and disease relapse (p = 0.0001) after pregnancy. When comparing patients according with breastfeeding duration (cut-off 6 months), we found a significant more frequent smoking habitus (p = 0.02), caesarean section (p = 0.009), and joint involvement during postpartum (p = 0.0001) in women breastfeeding for less than or equal to 6 months, together with higher median BMI (p = 0.0001). Moreover, breastfeeding duration was positively associated with disease duration and hydroxychloroquine (HCQ) treatment during disease history, pregnancy and postpartum. CONCLUSIONS: SLE women didn't show lower breastfeeding rate in comparison with general population but they presented higher prevalence of early discontinuation within three months. Early interruption was positively associated with smoking, BMI, joint involvement; meanwhile disease duration and HCQ treatment during postpartum were positively associated with a longer breastfeeding duration.

Premature ovarian failure in patients affected by systemic lupus erythematosus: a cross-sectional study.

OBJECTIVES: We evaluated age at natural menopause and the prevalence of premature ovarian failure (POF) in a monocentric Caucasian cohort of patients with systemic lupus erythematosus (SLE). METHODS: In this cross-sectional study, we enrolled women affected by SLE compared with healthy controls (HC) to investigate data about natural menopause (amenorrhoea for at least 12 months at ≥40 years) and POF (amenorrhoea for at least 12 months at <40 years). RESULTS: We enrolled 196 SLE (median age 47.0 years, IQR 16.7; median disease duration 132 months, IQR 180) and 90 HC (median age 49.9 years, IQR 15.0). Ninety-four SLE (48.0%) and 26 HC (23.4%) were menopausal: median age at onset was significantly lower in SLE than HC (47 years, IQR 8.0 vs. 50.5 years, IQR 4; p=0.0001). POF was registered in 17% of the SLE, and in none of the HC (p<0.0001). POF was significantly associated with anti-Sm (p=0.0004), anti-RNP (p=0.02), anti-cardiolipin (p=0.0008), lupus anticoagulant (p=0.0002), treatment with cyclophosphamide (p=0.0001), azathioprine (p=0.0001), mycophenolate mofetil (p=0.0001), cyclosporine A (p=0.007). CONCLUSIONS: SLE patients develop menopause at a younger age; moreover, a higher POF frequency was observed in SLE patients in comparison with HC. POF is associated with specific SLE-related autoantibodies and the use of immunosuppressant drugs, in particular cyclophosphamide.

Treatment with Gonadotropin Releasing Hormone Agonists in Systemic Lupus Erythematosus Patients Receiving Cyclophosphamide: A Long-term Follow-up Study.

BACKGROUND: Cyclophosphamide treatment has been associated with ovarian function impairment. Co-treatment with gonadotropin-releasing hormone-analogue (GnRH-a) seems to be able to prevent this complication. However, even though data are available on neoplastic patients, limited data have been published on systemic lupus erythematosus (SLE) women cohorts. OBJECTIVES: To evaluate GnRH-a efficacy on ovarian function preservation in SLE women receiving cyclophosphamide treatment. METHODS: The authors performed a retrospective study including SLE women requiring cyclophosphamide treatment and compared those treated with and without GnRH-a (case and controls, respectively). All patients were evaluated before cyclophosphamide treatment and every 3 months in the following years. Ovarian function was evaluated using hormonal profiles. RESULTS: The study comprised 33 SLE cyclophosphamide-treated women: 18 co-treated with triptorelin and 15 controls. The mean follow-up was 8.1 ± 5.1 years (range 4-11). Premature ovarian failure (POF) prevalence was significantly lower in SLE women treated by cyclophosphamide plus triptorelin compared to controls (11.1% vs. 33.3%, P = 0.0002). The occurrence of POF was significantly associated with higher age at the time of cyclophosphamide treatment (P = 0.008). Only patients in the GnRH-a treated group had successful pregnancies. CONCLUSIONS: The study provides information about the efficacy of co-treatment with GnRH-a in SLE women receiving cyclophosphamide, as demonstrated by the lower POF incidence compared to untreated subjects, based on long-term follow-up. These results reinforce the use of GnRH-a for fertility preservation in premenopausal SLE patients treated by cyclophosphamide.

New-onset ulcerative colitis in pregnancy associated to toxic megacolon and sudden fetal decompensation: Case report and literature review.

Ulcerative colitis (UC) is a chronic inflammatory disease rarely arising during gestation. Because the available information is based on case reports or small retrospective studies, diagnosis may be difficult and treatment is still controversial. A case of toxic megacolon developing in late pregnancy associated to a sudden fetal decompensation is described. Diagnostic and clinical topics of acute UC onset in pregnancy are debated.A primipara, 34 years old, 33/0 weeks of gestation, was admitted with a diagnosis of preterm labor, associated to acute bloody diarrhea (up to 10 daily motions) and cramping abdominal pain. A diagnosis of new-onset early-stage UC was made by sigmoidoscopy. An intensive care regimen including hydrocortisone, antibiotics and parenteral nutrition was immediately started. Magnetic resonance imaging of maternal abdomen, fostered by the worsening patient conditions, evidenced dilatation of the entire colon and a severely hampered of fetal muscular tone.Toxic megacolon complicated by superimposed Clostridium difficile infection was associated to a sudden fetal decompensation diagnosed by chance during maternal abdominal magnetic resonance imaging. An emergency cesarean section was mandatory. According to a senior surgeon's decision, total colectomy was not immediately performed following cesarean section with reference to the absence of colonic perforation. We obtained a good short-term maternal outcome and an uncomplicated neonatal course. Counseling of those patients must be focused on timely and multidisciplinary intervention in order to improve the course of maternal disease and to prevent fetal distress.

Hypertension and early menopause after the use of assisted reproductive technologies in women aged 43 years or older: Long-term follow-up study.

AIM: The aim of this study was to investigate the long-term consequences to women's health and the onset of menopause in healthy women of advanced reproductive age who conceived by assisted reproductive technologies (ART). METHODS: Healthy women who conceived by ART (72) and controls (80) were selected among 320 women ≥ 43 years, who delivered between January 2010 and December 2011 in the Department of Gynecological and Obstetrical Sciences and Urological Sciences of "Sapienza" University of Rome. Body mass index (BMI), systolic blood pressure (SBP), diastolic blood pressure (DBP), and presence of hypertension and diabetes were analyzed at three days, six months, and three years after delivery. The onset of menopause was analyzed after three years. RESULTS: In the ART group, SBP, DBP and hypertension were higher at three days, six months, and three years after delivery. Menopausal age was significantly lower. CONCLUSION: The impact of ART in healthy women of advanced reproductive age in the years after delivery is not limited to the possible development of cardiovascular risk factors, such as hypertension, but can also influence the age of onset of menopause.

Epirubicin: a new entry in the list of fetal cardiotoxic drugs? Intrauterine death of one fetus in a twin pregnancy. Case report and review of literature.

BACKGROUND: Current knowledge indicate that epirubicin administration in late pregnancy is almost devoid of any fetal cardiotoxicity. We report a twin pregnancy complicated by breast cancer in which epirubicin administration was causatively linked to the death of one twin who was small for gestational age (SGA) and in a condition of oligohydramnios and determined the onset of a transient cardiotoxicity of the surviving fetus/newborn. CASE PRESENTATION: A 38-year-old caucasic woman with a dichorionic twin pregnancy was referred to our center at 20 and 1/7 weeks for a suspected breast cancer, later confirmed by the histopathology report. At 31 and 3/7 weeks, after the second chemotherapy cycle, ultrasound examination evidenced the demise of one twin while cardiac examination revealed a monophasic diastolic ventricular filling, i.e. a diastolic dysfunction of the surviving fetus who was delivered the following day due to the occurrence of grade II placental abruption. The role of epirubicin cardiotoxicity in the death of the first twin was supported by post-mortem cardiac and placental examination and by the absence of structural or genomic abnormalities that may indicate an alternative etiology of fetal demise. The occurrence of epirubicin cardiotoxicity in the surviving newborn was confirmed by the report of high levels of troponin and transient left ventricular septal hypokinesia. CONCLUSION: Based on our findings we suggest that epirubicin administration in pregnancy should be preceded by the screening of some fetal conditions like SGA and oligohydramnios that may increase its cardiotoxicity and that, during treatment, the diastolic function of the fetal right ventricle should be specifically monitored by a pediatric cardiologist; also, epirubicin and desamethasone for lung maturation should not be closely administered since placental effects of glucocorticoids may increase epirubicin toxicity.

Menopausal symptoms after the discontinuation of long-term hormone replacement therapy in women under 60: a 3-year follow-up.

BACKGROUND: Prescriptions for hormone replacement therapy (HRT) declined following the publication of the Women's Health Initiative study. The number of women who experience recurrence of menopausal symptoms after discontinuation of long-term HRT (LT-HRT), the length of time these symptoms last and the preferred alternative treatments remain unknown. METHODS: This prospective 3-year follow-up study analyses the prevalence and intensity of menopausal symptoms that occur in young postmenopausal women who discontinued LT-HRT. Symptoms were evaluated using the Menopause Rating Scale. RESULTS: Women (254) who discontinued LT-HRT (mean use: 6.9 ± 2.3 years) were recruited. Mean age at menopause was 48.1 ± 3.4 years. Mean age at discontinuation was 56.8 ± 3.7 years. 23% of the women were lost to follow-up. Of the remaining 196 women, 93% experienced a recurrence of menopausal symptoms within the first year, 25% resumed low-dose HRT, 62% used vaginal estrogens, 54% used phytoestrogens, and 2% used alternative therapies. A decrease in symptom prevalence and intensity was observed during the 3-year follow-up. CONCLUSIONS: Symptoms re-appeared in a significant proportion of patients within the first year after discontinuation of LT-HRT. However, after 3 years, the majority of these women were asymptomatic. Patients who discontinue LT-HRT may require a more detailed follow-up immediately after the discontinuation of treatment.

Dysregulation of diurnal salivary cortisol production is associated with spontaneous preterm delivery: a pilot study.

OBJECTIVES: The purpose of this study was to evaluate whether the diurnal fluctuation of salivary cortisol and its overall diurnal secretion are associated with the length of gestation in patients who were admitted to the hospital with an assigned diagnosis of possible preterm labor (PL) at a gestational age of 28-33 weeks. METHODS: In 22 patients, maternal saliva samples were collected for a cortisol assay 4 times per day (8 AM, 12 AM, 4 PM and 8 PM) on day 4 and day 6 after antenatal corticosteroid prophylaxis to prevent neonatal respiratory distress syndrome. RESULTS: Eight patients who ultimately delivered before term (32.6 ± 1.7 gestation weeks) showed an inverted fluctuation of salivary cortisol on both days 4 and 6, morning cortisol levels being significantly lower than evening levels. In contrast, in 14 patients who delivered at term (39.5 ± 0.6 gestation weeks), the physiological diurnal fluctuation of salivary cortisol was maintained. In addition, a distinctive feature of women delivering before term was a significantly hampered salivary cortisol diurnal production measured on day 6. CONCLUSIONS: Corticoadrenal activity is dysregulated and anticipates very preterm delivery in women with an assigned diagnosis of possible PL.

Removal versus retention of cervical cerclage with preterm prelabor rupture of membranes: Systematic review and meta-analysis.

OBJECTIVE: To evaluate maternal and perinatal outcomes of removal versus retention of cervical cerclage after premature preterm rupture of membranes (pPROM). STUDY DESIGN: Medline, Embase and Cochrane databases were searched electronically on February 2023 utilizing combinations of the relevant medical subject heading (MeSH) terms, keywords, and word variants that were considered suitable for the topic. Either prospective or retrospective trials were considered suitable for the inclusion. The coprimary outcome of this study were pregnancy latency >7 days from pPROM and pregnancy latency >48 h from pPROM. Random effect head to-head meta-analyses were performed to directly compare each outcome, expressing the results as summary odds ratio (OR) for dichotomous outcomes and as mean difference (MD) for continuous outcomes, plus relative 95% confidence interval (CI). Quality assessment of the included studies was performed using the Newcastle-Ottawa Scale. RESULTS: Six studies involving a total of 377 women (169 in the "removal" and 208 in the "retention" group) were included. The rate of pregnancy prolongation >48 h was significantly lower in the removal compared to retention group (OR 0.15, 95% CI 0.07-0.31; p < 0.0001), as well as the rate of pregnancy prolongation >7 days (OR 0.30 95% CI 0.11-0.83; p = 0.02) and pregnancy latency expressed in days (MD -2.84 days, 95% CI -5.40 to -0.29; p = 0.03). The rate of chorioamnionitis was significantly lower in the removal compared to the retention group (OR 0.57 95% CI 0.34-0.96p = 0.03) as was the rate of Apgar score < 7 at 5 min (OR 0.22 95% CI 0.08-0.56; p = 0.002). There was no difference between removal and retention groups for all the other maternal and perinatal outcomes. CONCLUSIONS: The decision whether to remove or retain cerclage in case of pPROM should balance the prematurity-related risks with that of infectious complications, thus highlighting the need for tailored management based on gestational age at occurrence of pPROM.

Maternal C-reactive protein at hospital admission is a simple predictor of funisitis in preterm premature rupture of membranes.

AIM: To analyze the prognostic value of maternal serum C-reactive protein (CRP) in predicting funisitis in patients with preterm premature rupture of membranes (pPROM). METHODS: 66 patients (gestational age 24-33 weeks) hospitalized 1-12 h after pPROM were enrolled. White blood cell count (WBC), platelet count (PLT) and plasma concentration of CRP were assessed every 3 days. Histological evidence of chorioamnionitis and funisitis was obtained post-partum. Receiver operating characteristic (ROC) curves were employed to evaluate the role of maternal CRP in predicting funisitis. RESULTS: Funisitis was found in 24 patients (36.3%); 42 patients (63.7%) without funisitis were considered as controls. PLT and WBC at admission and before delivery did not show significant differences and were not statistically different between the two groups. Patients with funisitis had significantly higher CRP levels both at admission to hospital and 24- 48 h before delivery. ROC curve analysis showed that CRP at admission (area under the curve: 0.671, p = 0.021) and before delivery (area under the curve: 0.737, p = 0.001) are predictive of funisitis. CONCLUSIONS: High maternal serum CRP levels (>20,000 µg/l) in pPROM patients at admission to hospital may be an early marker which indicates, with a good diagnostic performance, the presence of funisitis.

Pregnancy and post-partum outcomes of obese women after bariatric surgery: A case-control study.

INTRODUCTION: Bariatric surgery (BS) is known to reduce several obesity-related complications during pregnancy, but there is concern that it may increase the risk of maternal-fetal morbidity because of the malabsorption. This study aimed to investigate the impact of restrictive BS on several pregnancy outcomes in comparison with different grades of obesity. MATERIALS AND METHODS: A single-center retrospective case-control study. All primiparous singleton pregnant women who underwent BS between the previous 1-5 years or with body mass index (BMI) ≥ 30 kg/m2 delivering in our center were included. Obstetric and perinatal outcomes were analyzed and compared between the two groups. RESULTS: Overall, 90 women were included: 30 underwent BS and 60 were obese. The mean pre-pregnancy BMI was 31.0 ± 4.2 kg/m2 in the BS group and 38.1 ± 4.3 kg/m2 in the control group (p < 0.001). The obese group experienced higher rate of fetal macrosomia (25% vs 6.7%; p = 0.049), gestational hypertension (23.3% vs 3.3%; p = 0.04), preeclampsia (23.3% vs 0%; p = 0.04), gestational diabetes (33.3% vs 6.7%; p = 0.01), and cesarean section (68.3% vs 20%; p < 0.0001). The BS group showed higher frequency of small for gestational age (SGA) (46.7% vs 18.3%; p = 0.006), late preterm delivery (PTD) (33.3% vs 10%; p = 0.009), cholestasis (13.3% vs 1.7%; p = 0.049). Breastfeeding ≥ 6 months was higher among BS mothers (36.7% vs 11.7%; p = 0.007). CONCLUSIONS: Our findings support the positive impact of BS on several obstetric outcomes, at the expense of a higher frequency of SGA and PTD. BS mothers more frequently achieved the recommended goal of breastfeeding for 6 months compared to obese women.

Clinical management of hypertensive disorders in postpartum women.

Hypertensive disorders of pregnancy (HDP) could persist post-partum, or appear for the first time after delivery and could require a pharmacological treatment. It was found no evidence in literature about which therapy should be used in puerperal hypertension. The aim of this review is to determine the most effective therapy and best in terms of risk-benefit ratio for the treatment of high postpartum systemic arterial blood pressure in women with pregnancy-induced hypertension (PIH) or in those with de-novo diagnosis of hypertension in the puerperium. Cochrane Database of Systematic Reviews (CDSR), Cochrane Central Register of Controlled Trials (CCRCT), Embase, Medline, and PubMed were searched. The main inclusion criterion was articles regarding postnatal women with hypertension, therapeutic treatment for the management of hypertension compared with placebo or no therapy, with the exclusion of preeclampsia/eclampsia. Twenty-three studies were included. This review highlights significant evidence gaps, demonstrating that further comparative research is required, particularly to clarify postpartum antihypertensive selection. In conclusion, there is insufficient evidence to recommend a particular therapy or model of care, but calcium channel blockers, beta-blockers, alpha-blockers and angiotensin-converting enzyme inhibitors (ACEIs) appeared variably effective.

SARS-CoV-2 vertical transmission in a twin-pregnant woman: a case report.

SARS-CoV-2 has affected millions of people around the world in recent years. Among susceptible patients, pregnant women seem to be prone to serious complications. The possibility of SARS-CoV-2 vertical transmission represents one of the most debated topics in the literature, providing inconclusive results. We present a case of a confirmed vertical transmission in a monochorial diamniotic twin pregnancy complicated by a selective intrauterine growth restriction and gestational diabetes mellitus. The analysis of different biological specimens identifies the presence of the SARS-CoV-2 genome in the umbilical cord blood of both twins, and the placental histologic examination confirmed indirect signs of viral infection, supporting the hypothesis that a transplacental infection can occur. Despite the devastating impact that SARS-CoV-2 has worldwide, neonatal infections have been infrequently reported, but they can occur under certain biologic conditions. Deep knowledge of the biological mechanisms underlying the risk of SARS-CoV-2 vertical transmission might be useful to understand the pathophysiological bases and the possible long-term implication of a mother-to-child vertical transmission.

Stillbirth and fetal capillary infection by SARS-CoV-2.

We presented the case of stillbirth in a paucisymptomatic mother affected by SARS-CoV-2. At gross examination, the placenta showed a diffuse marble appearance and a focal hemorrhagic area. Multiple areas of hemorrhagic or ischemic necrosis with central and peripheral villous infarctions and thrombosis of several maternal and fetal vessels with luminal fibrin and platelet deposition were observed. All lesions seemed to be synchronous. Virus particles were identified within the cytoplasm of endothelial cells using electron microscopy, whereas SARS-CoV-2 RNA was detected in the placental tissue using real-time reverse transcription-polymerase chain reaction. Here, fetal vascular malperfusion was associated with infection; in fact, electron microscopy images showed that marked SARS-CoV-2 endotheliotropism involved the intravillous fetal capillaries. Furthermore, we confirmed that syncytiotrophoblast is the major target cell type for SARS-CoV-2 infection of the placenta. In conclusion, the possible consequences of the action of the placentotropic SARS-CoV-2 included the occurrence of vertical transmission, as reported in the literature, and/or stillbirth: the latter possibility may be triggered by a hampered maternal and/or fetal perfusion of the placenta. The diffuse thrombosis and subsequent ischemia of fetal capillaries induced by COVID-19 cannot be predicted by standard clinical surveillance.

Cabergoline as an adjuvant to standard heart failure treatment in peripartum cardiomyopathy: A case report and review of the literature.

INTRODUCTION: Peripartum cardiomyopathy (PPCM) is a rare and idiopathic form of dilated cardiomyopathy presenting late in pregnancy or early postpartum. Since the 16-kDa fragment of prolactin has been identified as a key factor in the pathophysiology of PPCM, prolactin inhibitors have been used as an adjuvant to standard heart failure treatment. Although bromocriptine is the current first choice, promising results have been reported with cabergoline, albeit scant. CASE PRESENTATION: We presented the case of a 41-year-old woman who received a diagnosis of PPCM one week after delivery and was successfully treated with cabergoline, finally experiencing a complete recovery. CONCLUSION: The case adds to the scant evidence supporting the use of cabergoline in PPCM patients. We argue that the favorable pharmacokinetic and metabolic profiles of this drug should prompt its consideration as a valid alternative prolactin inhibitor in these critical patients.

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.