1. 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

2. Fetal PV RV CHD Data to be Presented: CHD – PS, Tet, Tet abs valve CHF dx and Rx

3. Fetal PS May not have post-stenotic dilation Trace PR may be present “Dagger” Doppler pattern May increase ductal velocity by transmitted turbulence

5. Fetal PS xxxxxxxxxxxxxxxxxxxxxxx

7. 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.

8. 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.

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

10. Echocardiography in Fetal Tetralogy of Fallot Measurements include Doppler in the umbilical artery, middle cerebral artery, uterine artery and growth assessment of the heart and fetus. xxxxxxx

11. 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.

12. 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. xxxxxxxxx

13. 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

14. Echocardiography in Fetal Tetralogy of Fallot Two Vessel Cord xxxxxxxxxxx

15. 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

16. 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

17. 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

18. 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%)

19. Echocardiography in Fetal Tetralogy of Fallot

29. Echocardiography in Fetal Tetralogy of Fallot with Pulmonary atresia

30. Echocardiography in Fetal Tetralogy of Fallot

31. MAPCAS The presence of aortopulmonary collateral arteries is a poor prognostic sign.

32. Echocardiography in Fetal Tetralogy of Fallot MAPCAS

33. Echocardiography in Fetal Tetralogy of Fallot with absent Pulmonary Valve No ductus arteriosus Massively dilated pulmonary arteries Compression of the bronchi in utero

34. Echocardiography in Fetal Tetralogy of Fallot

38. Echocardiography in Fetal Tetralogy of Fallot-Absent valve

39. Echocardiography in Fetal Tetralogy of Fallot With Absent Pulmonary Valve Syndrome

40. Echocardiography in Fetal Tetralogy of Fallot With AV Canal Defect

41. Case 1-35 weeks 33 Weeks Gestation

42. 33 weeks 33 weeks gestation

43. 35 weeks 33 weeks gestation

44. Children’s Heart Centre Linz 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 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 Sensitivity: 100% Specificity: 75% If 3/4 were present:

45. Fetal Predictors of Postnatal 2V Repair Salvin et al. Pediatrics 2007 (Boston)

46. Children’s Heart Centre Linz 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. 2008;51:1299-30 N = 34 fetuses (15-33 weeks) - 21 liveborn < 23 weeks: –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

47. Rational for intervention in PA/IVS decompression of the RV promotion right heart growth to increase the likelihood of a biventricular repair postnatally

48. How to select patients? suitable anatomy (membranous atresia) exclusion of large coronary artery fistulas prediction of a univentricular outcome

49. RV

50. Procedure technically more challenging than AS small RV atretic valve needs to be perforated

51. Advances in Perinatal Cardiology 10th Course Advances in Perinatal Cardiology 10th Fun in the Sun Course Focus: Fetal Treatment See www.allkids.orgwww.allkids.org “Conferences ” Oct. 23-26, 2014 St. Petersburg, FL