Anomalies of the PV and RV
James C. Huhta, M.D.
Perinatal Cardiology
JHM-All Children’s Hospital
5th Phoenix Fetal Cardiology Symposium
Wed. April 23, 2014, 4-4:30 PM
Fetal PV RV CHD
Data to be Presented:
CHD – PS,
Tet,
Tet abs valve
CHF dx and Rx
Fetal PS
• May not have post-stenotic dilation
• Trace PR may be present
• “Dagger” Doppler pattern
• May increase ductal velocity by transmitted
turbulence
Fetal
PS
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Echocardiography in Fetal
Tetralogy of Fallot
Tetralogy of Fallot comprises 10% of all
congenital heart disease and is the
most common form of cyanotic heart
disease beyond infancy.
Echocardiography in Fetal
Tetralogy of Fallot
The embryology of Tetralogy of Fallot
may be thought of simply as anterior
deviation of the infundibular septum.
This creates the overriding aorta, the
VSD and the narrowing of the RVOT.
Echocardiography in Fetal
Tetralogy of Fallot
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Fetal echocardiography
combines assessment of
the cardiac situs, the
anatomy and the
physiology
Echocardiography in Fetal
Tetralogy of Fallot
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Measurements include
Doppler in the umbilical
artery, middle cerebral
artery, uterine artery and
growth assessment of the
heart and fetus.
Echocardiography in Fetal
Tetralogy of Fallot
. Classic tetralogy of Fallot may be missed if echo
examination of the fetal heart is confined to the
four chamber view as it is usually normal in this
condition.
Echocardiography in Fetal
Tetralogy of Fallot
• Typical findings on fetal echo include:
• a large size perimembranous subaortic VSD, large
overriding aorta (Ao),
• anterior malalignment of conal septum with
subpulmonary narrowing,
• small main pulmonary artery/ confluent branches, and
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• RV outflow velocity of over 1.4 m /s.
Echocardiography in Fetal
Tetralogy of Fallot
• Diagnosis of TOF should prompt referral for
• a thorough anatomic examination by ultrasound,
• amniocentesis for karyotype for chromosomal
anomalies including FISH screen for chromosome
22q11 microdeletion
Echocardiography in Fetal Tetralogy of Fallot
Two Vessel Cord
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Echocardiography in Fetal
Tetralogy of Fallot
• The perinatal outcome of fetal tetralogy of Fallot is
worse than that observed for postnatally identified
tetralogy of Fallot. A possible explanation is the
relatively high incidence of aneuploidy and extracardiac
anomalies in fetal cases
Echocardiography in Fetal
Tetralogy of Fallot
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Follow-up fetal studies should examine;
growth of the pulmonary arteries,
direction of ductal flow,
additional ventricular septal defects,
mitral valve abnormalities.
Tetralogy may also be associated with left atrial isomerism
Development of hydrops fetalis is uncommon in fetal tetralogy.
Congestive heart failure may develop over time
Echocardiography in Fetal
Tetralogy of Fallot
•
Congestive heart failure may develop if there is
significant pulmonary insufficiency (so-called
tetralogy of Fallot with absent pulmonary valve
syndrome), or the presence of a restrictive ventricular
septal defect
Echocardiography in Fetal
Tetralogy of Fallot
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Tetralogy with pulmonary stenosis (58%)
tetralogy with pulmonary atresia (25%),
with absent pulmonary valve syndrome (14%)
with associated atrioventricular septal defect (3%)
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot with Pulmonary atresia
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
MAPCAS
The presence of
aortopulmonary
collateral arteries is a
poor prognostic sign.
Echocardiography in Fetal
Tetralogy of Fallot
MAPCAS
Echocardiography in Fetal
Tetralogy of Fallot with absent Pulmonary Valve
No ductus arteriosus
Massively dilated
pulmonary arteries
Compression of the
bronchi in utero
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot
Echocardiography in Fetal
Tetralogy of Fallot-Absent valve
Echocardiography in Fetal Tetralogy of Fallot
With Absent Pulmonary Valve Syndrome
Echocardiography in Fetal Tetralogy of Fallot
With AV Canal Defect
Case 1-35 weeks
33 Weeks Gestation
33 weeks
33 weeks gestation
35 weeks
33 weeks gestation
Determinants of Outcome in Fetal Pulmonary Valve
Stenosis or Atresia with Intact Ventricular Septum
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Kevin, Fouron, Masaki, Smallhorn, Chaturvedi, Jaeggi - Toronto /
Montreal
Am J Cardiol 2007;99:699-703
Prediction of a non - biventricular outcome:
• TV / MV ratio < 0.7
• RV / LV length ratio < 0.6
• TV inflow duration < 31.5%
• Presence of sinusoids
If 3/4 were
present:
Sensitivity: 100%
Specificity: 75%
Children’s Heart Centre Linz
Fetal Predictors of Postnatal 2V Repair
Salvin et al. Pediatrics 2007 (Boston)
Morphological and functional predictors of eventual
circulation in the fetus with PA/IVS or critical PS
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Gardiner, Belmar, Tulzer et al London/Linz
J Am Coll Cardiol. 2008;51:1299-30
• N = 34 fetuses (15-33 weeks) - 21 liveborn
• < 23 weeks:
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Median TV Z-score > -3.4 and PV Z-score > -1.0
< 26 weeks:
Median TV Z-score > -3.95
26 - 31 weeks:
Median PV Z-score > -2.8 + medTV:MV > 0.71
> 31 weeks:
Median TV Z-score > -3.9 + medTV:MV > 0.59
Children’s Heart Centre Linz
Rational for intervention in
PA/IVS
• decompression of the RV
• promotion right heart growth
• to increase the likelihood of a
biventricular repair postnatally
How to select patients?
• suitable anatomy (membranous
atresia)
• exclusion of large coronary artery
fistulas
• prediction of a univentricular
outcome
RV
RV
RV
Procedure
• technically more challenging than
AS
• small RV
• atretic valve needs to be perforated
Advances in Perinatal Cardiology
10th Fun in the Sun Course
Oct. 23-26, 2014
St. Petersburg, FL
Focus: Fetal Treatment
See www.allkids.org
“Conferences”