View Fetal HLHS RAS algorithm (2) flipbook.
1 Risk stratification in fetuses with HLHS for suspected restrictive/intact atrial septum • Measure PV Doppler F/R VTI ratio • Careful assessment for anomalous venous return (e.g.,TAPVR) HIGH INTERMEDIATE LOW PV F/R VTI ≤ 3 PV F/R VTI > 5PV F/R VTI >3, ≤ 5 • Fetal MRI lung evaluation • MHO testing • Consider fetal MRI lung evaluation • If TAPVR, same pathway as Intermediate risk pathway Risk for RAS/IAS: Overall risk: HIGHEST risk, urgent postnatal intervention predicted, minimal survival HIGH risk, urgent postnatal intervention predicted INTERMEDIATE risk, urgent intervention NOT predicted, may need non-urgent atrial septal intervention LOW risk, consider other risks: • ≥ moderate TR • RV dysfunction • Syndrome/genetic Dx (e.g., XO) “Nutmeg” lung (+) “Nutmeg” lung (-) VTI F/R decreases with MHO, or “Nutmeg” lung (+) VTI F/R no change or increases with MHO, or “Nutmeg” lung (-) No TAPVR LOC: LOC P (palliative), 2nd opinion? LOC 4 LOC 3 LOC 2 Candidate for fetal intervention (see next page)
Children’s Healthcare of Atlanta | Emory University Texas Children’s inclusion criteria for fetal atrial septoplasty 2 • Fetus gestation >28 weeks but <32 weeks • Ultrasound evidence of restrictive or intact atrial septum • Adequate left atrial size • Pulmonary vein forward/reverse VTI ratio </= 3; and/or either to-fro or low diastolic pulmonary vein spectral Doppler pattern • Negative rapid FISH (fluorescence In Situ Hybridization) via amniocentesis • Point of contact at TCH: Shaine Morris, Betul Yilmaz • shainem@bcm.edu • byilmaz@bcm.edu
Children’s Healthcare of Atlanta | Emory University References 3 • Michelfelder EC, Gomez CA, Border W, Gottliebson W, Franklin C. Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle. Circulation 2005; 112: 2974 – 2979 • Divanovic A, Hor K, Cnota JF, Hirsch R, Kinsel-Ziter M, Michelfelder EC. Prediction and Perinatal Management of Severely Restrictive Atrial Septum in Fetuses With Critical Left Heart Obstruction: Clinical Experience Using Pulmonary Venous Doppler Analysis. Journal of Thoracic and Cardiovascular Surgery 2011 Apr;141(4):988-94. • Mardy, C.; Kaplinski, M.; Peng, L.; Blumenfeld, Y.J.; Kwiatkowski, D.M.; Tacy, T.A.; Maskatia, S.A. Maternal Hyperoxygenation Testing in Fetuses with Hypoplastic Left-Heart Syndrome: Association with Postnatal Atrial Septal Restriction. Fetal Diagn. Ther. 2021, 48, 678–689