1. CV Profile Scoring and Assessment
James C. Huhta, M.D.
Perinatal Cardiology
JHM-All Children’s Hospital
5th Phoenix Fetal Cardiology Symposium
Wed. April 23, 2014, 1:30-2:00 PM

2. Perinatal Cardiology Cardiology for the fetus, child, and mother

3. Faculty Disclosure Information In the past 12 months, I have no relevant financial relationships with the manufacturer(s) of any commercial product(s) and/or provider(s) of commercial services discussed in this CME activity. I serve as co-PI of a study of Edoxaban for Daiichi-Sankyo. I do not intend to discuss an unapproved/investigative use of a commercial product/device in my presentation.

4. Fetal congestive heart failure CV profile Score
Hydrops-– a measure of capillary permeability and/or elevated capillary venous pressure and/or hypoproteinemia
Venous Doppler-– a measure of central venous pressure, and/or RV diastolic function
Heart size-– a measure of remodeling of the heart in response to increased preload, afterload or anemia
Heart function - heterogenous measure of afterload and/or anular dilation (tricuspid valve regurgitation), (ventricular shortening), extreme diastolic filling abnormality (monophasic filling), and dP/dt estimate
Arterial Doppler-a measure of placental resistance and/or combined cardiac stroke volume at falling outputs

5. CV Profile 10-point score
NORMAL -1 POINT 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
>0.50 or <0.20
Cardiac
Function
Normal TV & MV
RV/LV S.F. > 0.28
Biphasic diastolic filling
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)
UA (AEDV)
UA (REDV)

6. Future Research Disease – specific CVP Score
Prospective trial of digoxin in fetal CHF
Comparison with Biophysical Profile Score
First Trimester CVP Score
Mouse embryo CVP Score

7. Future Research Disease – specific CVP Score
Prospective trial of digoxin in fetal CHF
Comparison with Biophysical Profile Score
First Trimester CVP Score
Mouse embryo CVP Score

9. Heart to chest area ratio

10. Valve regurgitation

11. CVP score range-last exam 3-10 6 died-Median CVP score 6 versus 7
Hofstaetter C, Hansmann M, Eik-Nes SH, Huhta JC, Luther SL, A cardiovascular profile score in the surveillance of fetal hydrops, J Matern Fetal Neonatal Med, 2006, 19(7):407-13
100 hydropic fetuses
CVP score range-last exam 3-10
6 died-Median CVP score 6 versus 7

12. Detection of CHD-Disproportion

14. 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

15. Perinatal Management Salvage of HLHSRA LA

16. Perinatal Management Cardiomyopathy

17. Fetal Valve Regurgitation

18. Tricuspid regurgitation dP/dt

19. Perinatal Management Cardiomyopathy
Myocarditis
Genetic syndromes
Inherited defects
Consider transplantation
as a neonate

20. Fetal CHF with CHD Examples
CHD with increasing heart size in utero
Tet absent valve syndrome
Pulmonary atresia with collaterals
Ebstein’s malformation
Critical AS
L isomerism with CHB

21. Fetal CVP Score - 146 fetuses Congenital Heart Disease Perinatal Mortality
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 Feb 5 31(3):

22. For 5 minute Apgar score <=6
Sensitivity
Specificity
PPV
For Mortality
0.25
0.98
0.88
Sensitivity
Specificity
PPV
For 5 minute Apgar score <=6
0.22
0.98
0.75

23. 33 weeks
33 weeks gestation

24. Diagnosis of Fetal CHF in IUGR

25. IUGR – longitudinal observations (≤ 32 weeks)6
Umbilical artery
Ductus venosus
Standard deviation
Short term variation
Middle cerebral a. -6
-35
-28
-21
-14 -7
Hecher, Ultrasound Obstet Gynecol 2001;18:564-70
Days before delivery

26. Validation of CVP score IUGR-Makikallio et al.
Eight out of 75 neonates died before discharge or had severe CP (n=2)
Delivery at earlier gestational age 28 (range 24-35) weeks vs. 35 (range 26-40) weeks, p<0.001
Lower fetal CVP scores 4 (range 2-6) vs. 9 (range 5-10), p<0.001)
All fetal subset scores of CVP except umbilical artery evaluation were lower (p<0.001) in the group with neonatal death.

27. Validation of CVP score-IUGR
Neonates with 5-minute Apgar scores < 7 had lower CVP scores than with scores > 7 (6 (2-10) vs. 9 (5-10), p<0.001)
Umbilical artery NT-proANP levels of newborns with CVP score < 6 were greater (5208 ( ) pmol/L) than the levels of neonates with CVP exceeding 6 (1626 ( ) pmol/L), p=0.0001).
All NT-proANP values of newborns with CVP score <6 were above the 95th percentile NT-proANP value in normal pregnancies, while 42 out of 67 (63%) fetuses with CVP > 6 showed NT-proANP concentrations exceeding the 95th percentile value in normal pregnancies
Umbilical artery NT-proANP values correlated inversely and significantly with CVP score values

30. Validation of CVP score- Complete AV Block
We have implemented a strategy that includes the biophysical profile, which assesses fetal well-being, in combination with the cardiovascular profile that assesses cardiac function and the circulation.
Two cases of fetal complete heart block in which early delivery was recommended due to worsening cardiovascular profile scores. Biophysical profile scores were normal. Both babies were successfully treated, despite having risk factors that predicted poor outcomes. We hypothesize that our management protocol initiated intervention before fetal compromise, hydrops, and myocardial damage occurred.
We recommend an evaluation of heart function in addition to an assessment of fetal well-being in fetuses with complete heart block. Early delivery should be considered if there is evidence of distress and/or deteriorating cardiac function.
Donofrio MT, Gullquist SD, Mehta ID, Moskowitz WB.Congenital complete heart block: fetal management protocol, review of the literature, and report of the smallest successful pacemaker implantation. J Perinatol Feb;24(2):112-7.

31. Validation of CVP score- T-T Transfusion