Anomalies of the PV and RV

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Anomalies of the PV and RV

• James C. Huhta, M.D.
• Perinatal Cardiology
• JHM-All Childrens Hospital
• 5th Phoenix Fetal Cardiology Symposium
• Wed. April 23, 2014, 4-430 PM

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Fetal PV RV CHD

• Data to be Presented
• CHD PS,
• Tet,
• Tet abs valve
• CHF dx and Rx

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Fetal PS

• May not have post-stenotic dilation
• Trace PR may be present
• Dagger Doppler pattern
• May increase ductal velocity by transmitted
  turbulence

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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.

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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.

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Echocardiography in Fetal Tetralogy of Fallot
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Fetal echocardiography combines assessment of the
cardiac situs, the anatomy and the physiology
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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.
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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.

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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
• RV outflow velocity of over 1.4 m /s.

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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

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Echocardiography in Fetal Tetralogy of FallotTwo
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

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Echocardiography in Fetal Tetralogy of Fallot

• 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

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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

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Echocardiography in Fetal Tetralogy of Fallot

• Tetralogy with pulmonary stenosis (58)
• tetralogy with pulmonary atresia (25),
• with absent pulmonary valve syndrome (14)
• with associated atrioventricular septal defect
  (3)

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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
with Pulmonary atresia
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
MAPCAS The presence of aortopulmonary collateral
arteries is a poor prognostic sign.
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Echocardiography in Fetal Tetralogy of Fallot
MAPCAS
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Echocardiography in Fetal Tetralogy of Fallot
with absent Pulmonary Valve

• No ductus arteriosus
• Massively dilated pulmonary arteries
• Compression of the bronchi in utero

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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of Fallot
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Echocardiography in Fetal Tetralogy of
Fallot-Absent valve
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Echocardiography in Fetal Tetralogy of
FallotWith Absent Pulmonary Valve Syndrome
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Echocardiography in Fetal Tetralogy of
FallotWith AV Canal Defect
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Case 1-35 weeks
33 Weeks Gestation

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33 weeks

33 weeks gestation
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35 weeks

33 weeks gestation
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Determinants of Outcome in Fetal Pulmonary Valve
Stenosis or Atresia with Intact Ventricular Septum

• Kevin, Fouron, Masaki, Smallhorn, Chaturvedi,
  Jaeggi - Toronto / Montreal
• Am J Cardiol 200799699-703

• Prediction of a non - biventricular outcome
• TV / MV ratio lt 0.7
• RV / LV length ratio lt 0.6
• TV inflow duration lt 31.5
• Presence of sinusoids
• Sensitivity 100
• Specificity 75

If 3/4 were present
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Fetal Predictors of Postnatal 2V RepairSalvin et
al. Pediatrics 2007 (Boston)
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Morphological and functional predictors of
eventual circulation in the fetus with PA/IVS or
critical PS

• Gardiner, Belmar, Tulzer et al London/Linz
• J Am Coll Cardiol. 2008511299-30

• N 34 fetuses (15-33 weeks) - 21 liveborn
• lt 23 weeks
• Median TV Z-score gt -3.4 and PV Z-score gt -1.0
• lt 26 weeks
• Median TV Z-score gt -3.95
• 26 - 31 weeks
• Median PV Z-score gt -2.8 medTVMV gt 0.71
• gt 31 weeks
• Median TV Z-score gt -3.9 medTVMV gt 0.59

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Rational for intervention in PA/IVS

• decompression of the RV
• promotion right heart growth
• to increase the likelihood of a biventricular
  repair postnatally

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How to select patients?

• suitable anatomy (membranous atresia)
• exclusion of large coronary artery fistulas
• prediction of a univentricular outcome

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RV
RV
RV
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Procedure

• technically more challenging than AS
• small RV
• atretic valve needs to be perforated

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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