2021-12-232021-12-232011-03Özkan, H. vd. (2011). "Severe fetal valproate syndrome: Combination of complex cardiac defect, multicystic dysplastic kidney, and trigonocephaly". Journal of Maternal-Fetal and Neonatal Medicine, 24(3), 521-524.1476-70581476-4954https://doi.org/10.3109/14767058.2010.501120https://www.tandfonline.com/doi/full/10.3109/14767058.2010.501120http://hdl.handle.net/11452/23507Valproic acid (VPA) is a teratogenic drug used in pregnant women for the treatment of epilepsy and mood disorders. Fetal valproate syndrome (FVS) is characterized by a number of abnormalities associated with VPA exposure in utero including neural tube defects, congenital heart defects, limb defects, genitourinary defects, brain, eye and respiratory anomalies, and abdominal wall defects. Complex cardiac defect and trigonocephaly have rarely been reported and multicystic dysplastic kidney has never been detected in FVS. We here report a female infant who was born to a mother with a history of low-dose VPA monotherapy (250 mg/day) during pregnancy and who had presented with a combination of unilateral multicystic dysplastic kidney, multicomplex cardiac defect including severe coarctation of aorta, Ebstein anomaly, secundum atrial septal defect, mesocardia along with trigonocephaly due to metopic craniosynostosis, typical facial appearance and limb defects. To our knowledge, this is the first case presented with multicystic dysplastic kidney, complex cardiac defect, trigonocephaly and other limb and facial defects because of exposure to very low-dose VPA monotherapy (250 mg/day) in utero. We conclude that VPA must be used very cautiously in pregnant women even as monotherapy and in low doses to prevent major congenital defects.eninfo:eu-repo/semantics/closedAccessObstetrics & gynecologyAntiepileptic drugsSodium valproatePregnancyExposureEpilepsyTeratogenicityAbnormalitiesMalformationsRegisterAplasiaAbnormalities, drug-inducedAbnormalities, multipleCraniosynostosesFatal outcomeFemaleHeart defects, congenitalHumansInfant, newbornLimb deformities, congenitalMulticystic dysplastic kidneyPregnancyPrenatal exposure delayed effectsSeverity of illness indexValproic acidYoung adultArticle0002869930000282-s2.0-7975150424152152424321291342Obstetrics & gynecologyAnticonvulsants; Valproic Acid; LamotrigineValproic acidAbsenceAdultApgar scoreArachnodactylyArticleArtificial ventilationBirth weightBody heightBrain malformationCamptodactylyCardiomegalyCase reportClinical featureCongenital heart malformationCraniofacial synostosisDrug induced diseaseEbstein anomalyEndotracheal intubationFaciesFemaleFetal valproate syndromeFetus echographyFrontal bossingFunnel chestHead circumferenceHeart atrium septum defectHeart right ventricle hypertrophyHigh arched palateHumanHydrocephalusInfantKidney hypoplasiaLimb defectLow drug doseLow set earMesocardiaMicrophthalmiaMouth malformationMulticystic dysplastic kidneyMuscle hypotoniaNeonatal respiratory distress syndromePrenatal drug exposurePriority journalPulmonary valve stenosisRespiratory failureTeratogenicityTrigonocephaly