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
Perinatal Cardiology
Cardiology for the fetus, child, and mother
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.
Fetal congestive heart failure
CV profile Score
1. Hydrops-– a measure of capillary permeability and/or
elevated capillary venous pressure and/or
hypoproteinemia
2. Venous Doppler-– a measure of central venous pressure,
and/or RV diastolic function
3. Heart size-– a measure of remodeling of the heart in
response to increased preload, afterload or anemia
4. 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
5. Arterial Doppler-a measure of placental resistance
and/or combined cardiac stroke volume at falling outputs
CV Profile
10-point score
NORMAL
-1 POINT
Hydrops
None
(2 pts)
Ascites or
Pleural effusion or
Pericardial effusion
Venous Doppler
(Umbilical Vein)
(Ductus Venosus)
DV (2 pts)
DV
-2 POINTS
Skin edema
UV pulsations
UV
Heart Size
(Heart/Chest Area)
< 0.35
0.35 - 0.50
>0.50 or <0.20
(2 pts)
Cardiac
Function
Normal TV & MV
RV/LV S.F. > 0.28
Biphasic diastolic
filling
(2 pts)
Arterial Doppler
(Umbilical artery)
UA
(Normal)
(2 pts)
Holosystolic TR or
RV/LV S.F. < 0.28
Holosystolic MR or
TR dP/dt < 400 or
Monophasic filling
UA (REDV)
UA (AEDV)
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
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
Heart to chest area ratio
Valve regurgitation
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
Detection of CHD-Disproportion
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
Perinatal Management
Salvage of HLHS
RA
LA
Perinatal Management
Cardiomyopathy
Fetal Valve Regurgitation
Tricuspid regurgitation dP/dt
50
cm /se c
0
50
100
dP
TR
150
200
250
300
dt
Doppler-Derived Right Ventricular dP/dt
1500
1250
1000
Survivors
dP/dt
750
(m m Hg/s)
Non-Survivors
500
250
0
0
1
Ductal
Constriction
2
NIHF
3
Perinatal Management
Cardiomyopathy
Myocarditis
Genetic syndromes
Inherited defects
Consider transplantation
as a neonate
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
Fetal CVP Score - 146 fetuses
Congenital Heart Disease Perinatal Mortality
100%
100%
100%
6 (n=1)
5 (n=4)
90%
80%
67%
70%
60%
50%
40%
30%
20%
16%
12%
17%
10%
0%
10 (n=95)
9 (n=25)
8 (n=18)
7 (n=3)
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.
Sensitivity
Specificity
PPV
For Mortality
0.25
0.98
Sensitivity Specificity
0.88
PPV
For 5 minute Apgar score <=6
0.22
0.98
0.75
33 weeks
33 weeks gestation
Diagnosis of Fetal CHF in IUGR
IUGR – longitudinal observations (≤ 32 weeks)
Standard deviation
6
Umbilical artery
Ductus venosus
0
Short term variation
Middle cerebral a.
-6
-35
-28
-21
-14
-7
Days before delivery
0
Hecher, Ultrasound Obstet
Gynecol 2001;18:564-70
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 2435) 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.
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 (2850-16030) pmol/L) than the
levels of neonates with CVP exceeding 6 (1626 (402- 9574)
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
Validation of CVP scoreComplete 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. 2004 Feb;24(2):112-7.
Validation of CVP scoreT-T Transfusion