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Tricuspid Valve: Tricuspid Atresia, Ebstein, TV Dysplasia James C. Huhta, M.D. Perinatal Cardiology Thursday Nov. 3, 2016 11:30 AM Take great care of the patient® CV Profile 10-point score NORMAL -1 POINT -2 POINTS Hydrops None (2 pts) Ascites or Pleural effusion or Pericardial effusion Skin edema Venous Doppler (Umbilical Vein) (Ductus Venosus) DV (2 pts) UV DV UV pulsations Heart Size (Heart/Chest Area) < 0.35 (2 pts) 0.35 - 0.50 >0.50 or <0.20 Cardiac Function Normal TV & MV RV/LV S.F. > 0.28 Biphasic diastolic filling (2 pts) Holosystolic TR or RV/LV S.F. < 0.28 Holosystolic MR or TR dP/dt < 400 or Monophasic filling Arterial Doppler (Umbilical artery) UA (Normal) (2 pts) UA (AEDV) UA (REDV) Tricuspid Valve Regurgitation What it is. What it is not. Grading Use in the CV profile score TR Holosystolic TR Grades mild, mod, severe Trace TR (nonholosystolic < 70 ms duration) False positive from valve closure sound-always Confirm with pulsed Doppler Respondek M, Kammermeier M, Ludomirsky A, Weil SR, Huhta JC: The prevalence and clinical significance of fetal tricuspid valve regurgitation with normal heart anatomy. Am J Obstet Gynecol 171:1265-70, 1994. Tricuspid Valve Regurgitation CW Doppler-dP/dt dP/dt Normal >1000 mmHg/s Abnormal = <600 mmHg/s Tulzer et al. J Matern Fetal Invest 1:15-18, 1991 Tricuspid Atresia

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  • Tricuspid Valve: Tricuspid Atresia, Ebstein, TV Dysplasia

    James C. Huhta, M.D. Perinatal Cardiology

    Thursday Nov. 3, 2016 11:30 AM

    Take great care of the patient®

    CV Profile 10-point score

    NORMAL -1 POINT -2 POINTS

    Hydrops None (2 pts)

    Ascites or Pleural effusion or Pericardial effusion

    Skin edema

    Venous Doppler (Umbilical Vein) (Ductus Venosus)

    DV (2 pts) UV

    DV UV pulsations

    Heart Size (Heart/Chest Area)

    < 0.35 (2 pts)

    0.35 - 0.50

    >0.50 or 0.28 Biphasic diastolic filling (2 pts)

    Holosystolic TR or RV/LV S.F. < 0.28

    Holosystolic MR or TR dP/dt < 400 or Monophasic filling

    Arterial Doppler (Umbilical artery)

    UA (Normal) (2 pts)

    UA (AEDV)

    UA (REDV)

    Tricuspid Valve Regurgitation

    What it is. What it is not.

    Grading Use in the CV profile score

    TR

    Holosystolic TR Grades mild, mod, severe

    Trace TR (nonholosystolic < 70 ms duration)

    False positive from valve closure sound-always Confirm with pulsed Doppler

    Respondek M, Kammermeier M, Ludomirsky A, Weil SR, Huhta JC: The prevalence and clinical significance of fetal tricuspid valve regurgitation with normal heart anatomy.

    Am J Obstet Gynecol 171:1265-70, 1994.

    Tricuspid Valve Regurgitation CW Doppler-dP/dt

    dP/dt – Normal –  >1000 mmHg/s – Abnormal = – 

  • Tricuspid Atresia Tricuspid Atresia

    Tricuspid Atresia Tricuspid Atresia

    TA with atrial restriction

    Use venous Doppler

    Ductus venosus

    Fetal Congestive Heart Failure Abnormal Venous Doppler

    Gudmundsson S, Huhta JC, Wood DC, Tulzer G, Cohen AW,

    Weiner S: Venous Doppler ultrasonography in the fetus with non-immune hydrops. Am J Ob

    Gyn 164:33-37, 1991

  • Tricuspid Atresia Tricuspid Atresia

    Tricuspid atresia with TGA Tricuspid Atresia

    Tricuspid Atresia Severe PS/PA

    Fetal CVP Score - 146 fetuses Congenital Heart Disease Perinatal Mortality

    16% 12%17%

    67%

    100% 100%

    0%

    10%

    20%

    30%

    40%

    50%

    60%

    70%

    80%

    90%

    100%

    10 (n=95) 9 (n=25) 8 (n=18) 7 (n=3) 6 (n=1) 5 (n=4)

    Wieczorek A, Hernandez-Robles J, Ewing L, Leshko J. Luther S, Huhta J. Prediction of outcome of fetal congenital heart disease using a cardiovascular profile score.

    Ultrasound Obstet Gynecol. 2008 Feb 5 31(3):284-288

  • PA - IS

    RA

    RV

    XXXXXXXXXXXXXX

    Fetal PA-IS

    Ebstein Ebstein

    Ebstein Ebstein Low origin of TR

  • Ebstein xxxxxxxx

    TVD

    Tricuspid Valve Dysplasia - •  Frequently fatal in utero •  Frequent atrial flutter •  Cardiomegaly causing lung

    hypoplasia

    TV dysplasia

    •  Clapping hands •  Severe TR •  Often fatal in utero

    TVD

    17 week fetus with valve leak TV dysplasia

  • TV dysplasia TR with TVD

    TVD

    •  Ebstein versus TVD

    TVD/Ebstein with Functional PA

    Perinatal Cardiology

    Cardiology for the fetus, child, and mother

    TV dysplasia

  • TV dysplasia

    TV dysplasia TVD

    R heart defects

    CVP score Higher aortic velocity Higher RV pressure estimate