title: Right ventricular outflow tract obstruction in complicated monochorionic twin pregnancy.
abstract: OBJECTIVE
  Severe right ventricular outflow tract obstruction (RVOTO) is a potential complication in recipient twins of twin-to-twin transfusion syndrome (TTTS) that requires postnatal follow-up or treatment. We aimed to evaluate pregnancy characteristics of neonates with RVOTO from complicated monochorionic twin pregnancies, determine the incidence of RVOTO in TTTS cases and construct a prediction model for its development.
  
  
  METHODS
  This was an observational cohort study of all complicated monochorionic twin pregnancies with a postnatal diagnosis of RVOTO examined at our center. Cases were referred for evaluation of the need for fetal therapy or intervention because of TTTS, selective intrauterine growth restriction (sIUGR) or multiple congenital malformations in one of the twins. Ultrasound data were retrieved from our monochorionic twin database. Among liveborn TTTS recipients treated prenatally with laser therapy, those with RVOTO were compared with those without RVOTO (controls). We describe four additional cases with RVOTO that were not TTTS recipients.
  
  
  RESULTS
  A total of 485 twin pregnancies received laser therapy for TTTS during the study period. RVOTO was diagnosed in 3% (11/368) of liveborn TTTS recipients, of whom two showed mild Ebstein's anomaly. Before laser therapy, pericardial effusion was seen in 45% (5/11) of RVOTO cases (P < 0.01) and abnormal A-wave in the ductus venosus (DV) in 73% (8/11) (P = 0.03), significantly higher proportions than in controls. Mean gestational age at laser therapy was 17 + 3 weeks in RVOTO cases compared with 20 + 3 weeks in controls (P = 0.03). A prediction model for RVOTO was constructed incorporating these three significant variables. One TTTS donor had RVOTO after the development of transient hydrops following laser therapy. Three larger twins in pregnancies complicated by sIUGR developed RVOTO, the onset of which was detectable early in the second trimester.
  
  
  CONCLUSIONS
  RVOTO occurs in TTTS recipient twins but can also develop in TTTS donors and larger twins of pregnancies complicated by sIUGR. Abnormal flow in the DV, pericardial effusion and early gestational age at onset of TTTS are predictors of RVOTO in TTTS recipients, which suggests increased vulnerability to hemodynamic imbalances in the fetal heart in early pregnancy. These findings could guide diagnostic follow-up protocols after TTTS treatment. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.
authors.affiliation: Division of Fetal Medicine, Department of Obstetrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: S J
authors.initials: SJ
authors.lastname: Eschbach
authors.affiliation: Division of Fetal Medicine, Department of Obstetrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: L S T M
authors.initials: LSTM
authors.lastname: Boons
authors.affiliation: Department of Medical Statistics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: E
authors.initials: E
authors.lastname: Van Zwet
authors.affiliation: Division of Fetal Medicine, Department of Obstetrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: J M
authors.initials: JM
authors.lastname: Middeldorp
authors.affiliation: Division of Fetal Medicine, Department of Obstetrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: F J C M
authors.initials: FJCM
authors.lastname: Klumper
authors.affiliation: Division of Neonatology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: E
authors.initials: E
authors.lastname: Lopriore
authors.affiliation: Division of Fetal Medicine, Department of Obstetrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: A K K
authors.initials: AKK
authors.lastname: Teunissen
authors.affiliation: Division of Neonatology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: M E
authors.initials: ME
authors.lastname: Rijlaarsdam
authors.affiliation: Division of Fetal Medicine, Department of Obstetrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: D
authors.initials: D
authors.lastname: Oepkes
authors.affiliation: Division of Neonatology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: A D J
authors.initials: ADJ
authors.lastname: Ten Harkel
authors.affiliation: Division of Fetal Medicine, Department of Obstetrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: M C
authors.initials: MC
authors.lastname: Haak
mesh_terms: D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005330:Fetofetal Transfusion / Q000150:complications / Q000000981:diagnostic imaging*; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012372:ROC Curve; D012680:Sensitivity and Specificity; D014427:Twins; D014694:Ventricular Outflow Obstruction / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology
keywords: monochorionic twin pregnancy; pulmonary atresia; pulmonary stenosis; right ventricular outflow tract obstruction; selective fetal growth restriction; twin-to-twin transfusion syndrome