title: Postnatal Outcome of Fetal Left Ventricular Hypertrabeculation/Noncompaction.
abstract: Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology. Prenatal diagnosis of LVHT can be established by fetal echocardiography. A review of 106 published cases showed that 46 cases with prenatally diagnosed LVHT were alive 0.5-120 months after birth. Since the course of cases with prenatally LVHT after publication is unknown, we aimed to collect follow-up-information. Information regarding vital status, cardiac and extracardiac morbidity was gathered by contacting the authors of the 46 cases. Fourteen of the 28 authors answered and gave information about 18 cases (six females, seven males, five gender-unknown, age 18 months to 10 years, mean follow-up 60 months). No differences were found between the 18 cases with follow-up and the 28 cases without follow-up regarding age, gender, cardiac or extracardiac comorbidities, and interventions. Three of the 18 cases had died subsequently from heart failure, osteosarcoma, and enterocolitis, respectively. Mutations or chromosomal abnormalities were found in six of the seven examined patients, extracardiac abnormalities in nine patients. Three patients received a pacemaker because of complete AV block, and two patients underwent heart transplantation. Cardiac surgical or interventional procedures were carried out in four patients. None suffered from malignant arrhythmias or had a cardioverter-defibrillator implanted. Based on the limited information, there are indications that cases with fetal diagnosis of LVHT have a continuing morbidity and mortality, even if they receive appropriate care. Since fetal LVHT is frequently associated with genetic abnormalities, further research about survival and underlying genetic causes is needed.
authors.affiliation: Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030, Vienna, Austria. claudia.stoellberger@chello.at.|, Steingasse 31/18, 1030, Vienna, Austria. claudia.stoellberger@chello.at.
authors.forename: Claudia
authors.initials: C
authors.lastname: Stöllberger
authors.affiliation: Institut für Demographie der Österr, Akademie der Wissenschaften, Vienna, Austria.
authors.forename: Christian
authors.initials: C
authors.lastname: Wegner
authors.affiliation: Department of Pediatric Cardiology, University Hospital, Vrije Universiteit Brussel (VUB), 101 Laarbeeklaan, 1090, Brussels, Belgium.
authors.forename: Abraham
authors.initials: A
authors.lastname: Benatar
authors.affiliation: Cardiology Department, Penn State Hershey Children's Hospital, 500 University Drive, P.O. Box 850, Hershey, PA, 17033-0850, USA.
authors.forename: Thomas K
authors.initials: TK
authors.lastname: Chin
authors.affiliation: Reference Center for Fetal Cardiology, Perinatology and Perinatal Cardiology Clinic, 2nd Department of Obstetrics and Gynecology, Medical University of Warsaw, ul. Karowa 2, 00-315, Warszawa, Poland.
authors.forename: Joanna
authors.initials: J
authors.lastname: Dangel
authors.affiliation: Department of Clinical Genetics, Erasmus University Medical Centre, Rotterdam, The Netherlands.
authors.forename: Danielle
authors.initials: D
authors.lastname: Majoor-Krakauer
authors.affiliation: Division of Cardiology, Department of Pediatrics, McMaster University, 1280 Main Street West, HSC 3A, Hamilton, ON, L8S 4K1, Canada.
authors.forename: Tapas K
authors.initials: TK
authors.lastname: Mondal
authors.affiliation: Division of Pediatric Cardiology, Masonic Children's Hospital Heart Center, University of Minnesota, 2450, Riverside Ave, Minnesota, MN, 55454, USA.
authors.forename: Shanthi
authors.initials: S
authors.lastname: Sivanandam
authors.affiliation: Divisions of Pediatric Cardiology, Stanford University, Stanford, CA, USA.|University of California San Francisco, 750 Welch Road - Suite 305, Palo Alto, CA, 94304, USA.
authors.forename: Norman H
authors.initials: NH
authors.lastname: Silverman
authors.affiliation: Department of Clinical Genetics, Erasmus University Medical Centre, Rotterdam, The Netherlands.
authors.forename: Jaap
authors.initials: J
authors.lastname: van Waning
authors.affiliation: Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030, Vienna, Austria.
authors.forename: Josef
authors.initials: J
authors.lastname: Finsterer
mesh_terms: D001145:Arrhythmias, Cardiac; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006330:Heart Defects, Congenital; D006801:Humans; D007223:Infant; D008297:Male; D009468:Neuromuscular Diseases
keywords: Cardiomyopathy; Echocardiography; Genetics