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