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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
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