Paul Böckenhoff, Astrid Hellmund, Ingo Gottschalk, Christoph Berg, Ulrike Herberg, Annegret Geipel, Ulrich Gembruch
{"title":"Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV).","authors":"Paul Böckenhoff, Astrid Hellmund, Ingo Gottschalk, Christoph Berg, Ulrike Herberg, Annegret Geipel, Ulrich Gembruch","doi":"10.1055/a-1866-4538","DOIUrl":null,"url":null,"abstract":"<p><strong>Purpose: </strong>To assess the spectrum of associated cardiac anomalies, the intrauterine course, and postnatal outcome of fetuses with double inlet ventricle (DIV).</p><p><strong>Methods: </strong>Retrospective analysis of prenatal ultrasound of 35 patients with DIV diagnosed between 2003 and 2021 in two tertiary referral centers in Germany. All fetuses underwent fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports.</p><p><strong>Results: </strong>33 cases of DIV were correctly diagnosed prenatally. 24 fetuses (72.7%) had a double inlet ventricle with dominant left (DILV), 7 (21.2%) with dominant right ventricular morphology (DIRV), and 2 cases (6%) with indeterminate morphology (DIIV). 4 (16.6%) were Holmes hearts. 5 of the 7 fetuses (71.4%) with DIRV had a double outlet right ventricle (DORV). Malposition of the great arteries was present in 84.8%. Chromosomal abnormalities were absent. Termination of pregnancy was performed in 8 cases (24.2%). 24 fetuses (72.7%) were live-born. 5 (20.8%) were female and 19 (79.2%) were male. The median gestational age at birth was 38+2.5 weeks. All but one child received univentricular palliation. The median follow-up time was 5.83 years with an adjusted survival rate of 91.6% (22 of 24 live-born children). There was one case of Fontan failure at 15.7 years.</p><p><strong>Conclusion: </strong>DIV remains a major cardiac malformation although both prenatal diagnostics and cardiac surgery have improved over the years. The course of pregnancy is commonly uneventful. All children need univentricular palliation. The children are slightly physically limited, develop a normal intellect, and attend school regularly.</p>","PeriodicalId":49400,"journal":{"name":"Ultraschall in Der Medizin","volume":null,"pages":null},"PeriodicalIF":3.1000,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/69/5d/10-1055-a-1866-4538.PMC10575714.pdf","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Ultraschall in Der Medizin","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1055/a-1866-4538","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2022/7/1 0:00:00","PubModel":"Epub","JCR":"Q1","JCRName":"ACOUSTICS","Score":null,"Total":0}
引用次数: 0
Abstract
Purpose: To assess the spectrum of associated cardiac anomalies, the intrauterine course, and postnatal outcome of fetuses with double inlet ventricle (DIV).
Methods: Retrospective analysis of prenatal ultrasound of 35 patients with DIV diagnosed between 2003 and 2021 in two tertiary referral centers in Germany. All fetuses underwent fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports.
Results: 33 cases of DIV were correctly diagnosed prenatally. 24 fetuses (72.7%) had a double inlet ventricle with dominant left (DILV), 7 (21.2%) with dominant right ventricular morphology (DIRV), and 2 cases (6%) with indeterminate morphology (DIIV). 4 (16.6%) were Holmes hearts. 5 of the 7 fetuses (71.4%) with DIRV had a double outlet right ventricle (DORV). Malposition of the great arteries was present in 84.8%. Chromosomal abnormalities were absent. Termination of pregnancy was performed in 8 cases (24.2%). 24 fetuses (72.7%) were live-born. 5 (20.8%) were female and 19 (79.2%) were male. The median gestational age at birth was 38+2.5 weeks. All but one child received univentricular palliation. The median follow-up time was 5.83 years with an adjusted survival rate of 91.6% (22 of 24 live-born children). There was one case of Fontan failure at 15.7 years.
Conclusion: DIV remains a major cardiac malformation although both prenatal diagnostics and cardiac surgery have improved over the years. The course of pregnancy is commonly uneventful. All children need univentricular palliation. The children are slightly physically limited, develop a normal intellect, and attend school regularly.
期刊介绍:
Ultraschall in der Medizin / European Journal of Ultrasound publishes scientific papers and contributions from a variety of disciplines on the diagnostic and therapeutic applications of ultrasound with an emphasis on clinical application. Technical papers with a physiological theme as well as the interaction between ultrasound and biological systems might also occasionally be considered for peer review and publication, provided that the translational relevance is high and the link with clinical applications is tight. The editors and the publishers reserve the right to publish selected articles online only. Authors are welcome to submit supplementary video material. Letters and comments are also accepted, promoting a vivid exchange of opinions and scientific discussions.