Heart and Soul Evaluation of the Fetal Heart

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1 Heart and Soul Evaluation of the Fetal Heart Ivana M. Vettraino, M.D., M.B.A. Clinical Associate Professor, Michigan State University College of Human Medicine

2 Objectives Review the embryology of the formation of the heart Compare and contrast fetal versus post-natal circulation List indications for detailed assessment of the fetal heart Review abnormal fetal cardiac anatomy. Discuss the role of the 3 vessel and 3 vessel trachea view

3 Introduction Congenital anomalies are the leading cause of infant death Congenital heart disease (CHD) accounts for 30 to 50 percent of these deaths CHD is the most common congenital disorder in newborns Affect 40,000 infants born in the USA each year Prevalence 6 to 13 per 1000 live births 8 per 1000 live births the average quoted

4 Introduction March of Dimes Data

5 Introduction Critical CHD is present in approximately 25 to 50 percent Most are ductal dependent lesions Coarctation of the aorta, interrupted aortic arch, aortic stenosis, pulmonary stenosis, hypoplastic left heart syndrome, transpositions of the great vessels, tetralogy of Fallot Non ductal dependent lesions Total anomalous pulmonary return, truncus arteriosus Risk of morbidity and mortality increases in this group with delayed diagnosis A missed diagnosis is thought to occur in approximatley 1 in 15,000 births

6 EMBRYOLOGY OF THE HEART

7 Congenital Heart Disease Stage of Development Primitive heart tube Looping Wedging/ventricular development Atrial septation Systemic and pulmonary veins Atrioventricular valves Aortic and Pulmonary outflow tracts Aortic arch Associated Anomaly Lethal defects Dextrocardia, Situs inversus totalis, Heterotaxy, Corrected TGA VSDs, hypoplastic ventricles, Double outlet right ventricle, double inlet left ventricle Common atrium, atrial septal defects Bilateral SVCs, Interrupted IVC/azygous vein, Total anomalous pulmonary return Epstein's, atresia of the AV valve Truncus arteriosus, double outlet RV, double inlet LV, TGA, absent DA, DiGeorge syndrome, CATCH 22 Catch 22, interrupted aortic arch, right aortic arch, tetralogy of Fallot, aberrant subclavian veins

8 FETAL AND NEONATAL CIRCULATION

9 The fetal circulation. Fetal Circulation

10 Summary

11 THE FETAL HEART EXAM

12 2007

13 2013

14 General Considerations

15 Changes Since 2007 Color Doppler sonography was optional now required M-Mode optional now required Pulsed Doppler required AV valves Semilunar valves Ductus venosus Cardiac rhythm disturbance

16 Essentially Unchanged Since 2007 Cardiac Biometry Optional But Should Be Considered for Suspected Structural or Functional Anomalies Cardiac Function Assessment Optional But Should Be Considered for Suspected Structural or Functional Cardiac Anomalies Complementary Imaging Strategies Optional 3- and 4-dimensional sonography

17 Indications for Fetal Echocardiogram Maternal Indications Autoimmune antibodies anti-ro (SSA)/anti-La (SSB) Familial inherited disorders 22q11.2 deletion In vitro fertilization Metabolic disease Diabetes mellitus Teratogen exposure Lithium Fetal Indications Abnormal cardiac screen First-degree relative of fetus with congenital heart disease Abnormal heart rate or rhythm Fetal chromosomal anomaly Extracardiac anomaly Hydrops Increased nuchal translucency/fold Monochorionic twins

18 The Basics Determine situs Do not assume that the situs is correct if the stomach and heart are on the same side Evaluate size Fetal heart occupies one third of the area of the fetal chest with an axis ~ 45 degrees to the left Cardiac circumference to chest circumference greater than 0.5 consistent with an enlarged heart

19 The Basics Visualization of fetal heart possible in the first trimester Optimal time to perform cardiac screening is between 18 and 22 weeks gestation Apical four-chamber is main screening view Evaluation of situs Evaluation of size Position Anatomy Function

20 Situs

21 The Basics The position Levocardia Heart located in left chest with apex pointing to the left Dextrocardia Heart located in right chest with apex pointing to the right Mesocardia Heart centrally located with apex pointing anteriorly Abnormalities of position can be associated with other cardiac anomalies

22 Cardiac Axis Obstet Gynecol 1987;70:255.

23

24

25 Cardiac Size

26 The Apical Four Chamber View

27 The Basics Four-chamber view Detect 43% to 96% of fetuses with CHD As a screening tool in general population expected to detect 40% to 50% of cases of CHD Ability to image the fetal heart influenced by gestational age, fetal position, amniotic fluid volume, and maternal body habitus

28 Structures Seen in the 4 Chamber View Atrial and ventricular size Atrial and ventricular septae Atrioventricular size and function Coronary sinus Ventricular function in long axis Semilunar valve function Pulmonary veins

29 Four Chamber Screening View Abnormalities easily missed on four chamber Ventricular septal defects Atrial septal defects Coarctation Tetralogy of Fallot Transposition of the great arteries Double-outlet right ventricle Truncus arteriosus Total anomalous pulmonary venous return

30 The Outflow Tracts Increases detection rate of CHD to 70 to 90% Cardiac anomalies associated with outflow tracts detected in only 6.7 percent of cases Left ventricular outflow tract (LVOT) 45 tilt of transducer from the four chamber view perpendicular to the septum to an oblique view from the fetal left upper quadrant of the abdomen to the right fetal shoulder Right ventricular outflow tract (RVOT) Further rotation in the same direction as noted above and rocking the transducer from the LVOT

31 Four Chamber and Outflows

32 Scanning Planes

33 Fetal Cardiac Scanning Short-axis view Obtained by scanning perpendicular to long axis of the heart Long-axis view Aligned with the left ventricular outflow tract Caval long-axis view Obtained with the imaging plane parallel to the caval connections to the right atrium Ductal view Obtained when the imaging plane is aligned with the right ventricular outflow tract and main pulmonary artery Aortic arch Obtained with the beam aligned from anterior right of fetal chest to posterior left of fetal chest

34 The Outflow Tracts Left Ventricular Outflow Tract (LVOT)

35 The Outflow Tracts Right Ventricular Outflow Tract (RVOT)

36 The Outflow Tracts

37 EXAMPLES

38 The Apical Four Chamber View

39 The Short Axis View RV mpa lpa aao rpa SVC

40 The Aortic Arch

41 Aortic Arch and Inferior Vena Cava

42 The Vena Cavas

43 The Ductal Arch

44 Three Vessel View

45 Four Chamber View

46

47

48

49 Ventricular Septal Defect

50 Ventricular Septal Defect

51 Ventricular Septal Defect

52 Ventricular Septal Defect

53 Atrioventricular Septal Defect

54 The AVSD

55 Ebstein s Anomaly

56 Ebstein s Anomaly

57 Tricuspid Regurgitation

58 Hypoplastic Left Heart

59 Hypoplastic Left Heart

60 HLHS

61 Transposition of the Great Vessels (TGA)

62 TGA Three Vessel View

63 Tetralogy of Fallot Large ventricular septal defect Over-riding aorta Pulmonary stenosis Right ventricular hypertrophy

64 Case

65 Case Liver Bowel

66 Pentalogy of Cantrell Rare form of abdominal wall defect Five Associated Anomalies Midline epigastric abdominal wall defect Defect of the lower sternum Deficiency of the anterior diaphragm Defect in the diaphragmatic pericardium Intra cardiac defects

67 Case 2

68 Case 2

69 Case 2

70 The Cardiac Mass

71 The Cardiac Mass

72 The Cardiac Mass

73 The Cardiac Mass

74 FETAL CARDIAC DYSRHYTHMIAS

75 Cardiac Dysrhythmia Occur in 1 to 3 percent of all pregnancies Detected by auscultation Most are benign Most are ectopic premature atrial contractions 10% of pregnancies complicated by fetal arrhythmias, have a potentially life threatening arrhythmia Tachyarrhythmias (heart rate in excess of 180 beats/min) Supraventricular tachycardia (SVT) Atrial flutter (AFL) Bradyarrhythmias (heart rate less than 100 beats/min) Second-degree atrioventricular (AV) block Complete AV block (CAVB)

76 Premature Atrial Contraction

77 PACs

78 PACs

79 PACs

80 Supraventricular Tachycardia

81 SVT

82 Pericardial Effusion

83 The Sail Sign

84 Mechanical PR Interval

85 Mechanical PR Interval

86 THE FUTURE

87 The Three Vessel View

88 The Three Vessel View

89 The Three Vessel Trachea View

90 The Three Vessel Trachea View

91 The Three Vessel Trachea View The Normal View Three vessels All vessels to the left of the trachea Vessels similar in size Pulmonary artery (PA) anterior of the Aorta (Ao) Continuous PA and Ao Flow toward the spine in PA and Ao

92 The Three Vessel View Blood flow toward the spine in PA and Ao

93 The Three Vessel Trachea View Examples of abnormalities Too Many Vessels PA and AO Not Continuous Vessel to Right of Trachea Ao Anterior to PA Small PA anterograde Flow Small PA Retrograde Flow Small Ao Left superior vena cava, azygous vein Interupted aortic arch Right sided aortic arch Transposition of the great vessels Tetralogy of Fallot Pulmonary Atresia Hypoplastic left heart

94 Summary Planes

95

96

97

98

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