Anomalies of the Cardiac Chambers (4-Chamber View) Alfred Abuhamad, MD. Eastern Virginia Medical School

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1 Anomalies of the Cardiac Chambers (4-Chamber View) Alfred Abuhamad, MD. Eastern Virginia Medical School

2 Abnormal Cardiac Chambers Ventricular septal defect Atrioventricular septal defect Hypoplastic left ventricle Ebstein anomaly Tricuspid atresia Cardiac tumors

3 VENTRICULAR SEPTAL DEFECT Most common of CHD Accounts for 32 % of CHD diagnosed in 1st year of life Incidence of VSDs in newborns is 5 to 50 per 1,000 VSDs are slightly more common in females (~ 56%)

4 VENTRICULAR SEPTAL DEFECT From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition

5 VENTRICULAR SEPTAL DEFECT 70% 8 % 20 % 7 % - Perimembranous - INLET - Muscular - Outlet

6 Ventricular Septum and US Views 4-chamber view LVOT view RVOT view

7 Ventricular Septum LV RV LA RA Inlet

8 Ventricular Septum Inlet

9 Ventricular Septum Muscular

10 Ventricular Septum Muscular From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition

11 Ventricular Septum Muscular

12 Ventricular Septum Muscular Swiss-cheese

13 Ventricular Septum Perimembranous

14 Ventricular Septum Perimembranous

15 VSD and Fetal Syndromes Chromosomal aberrations occur in > 20% of fetuses with isolated VSD; ~ 35% of isolated VSD and normal karyotype are diagnosed with extracardiac anomalies VSD is the most common cardiac lesion in many genetic syndromes Trisomy 13 Trisomy 21 Trisomy 18 Seckel syndrome CHARGE

16 ATRIOVENTRICULAR SEPTAL DEFECT

17 Atrioventricular Septal Defect (5 leaflets) From

18 ATRIOVENTRICULAR SEPTAL DEFECT Accounts for 4-5% of CHD in infants and occurs in 0.19 per 1000 live births. Most common form of CHD diagnosed in the fetus, accounting for 18% of fetal CHD Synonyms include endocardial cushion defect and atrioventricular canal defect AVSD forms Balanced Unbalanced

19 Atrioventricular Septal Defect Ultrasound Findings: Apical 4-chamber view most optimal Color Doppler very helpful Axial 4-chamber view least optimal Look at atrioventricular length ratio

20 Diastole Systole Atrioventricular Septal Defect

21 Atrioventricular Septal Defect From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition

22 ATRIOVENTRICULAR SEPTAL DEFECT Four-chamber view NORMAL Complete AVSD

23 ATRIOVENTRICULAR SEPTAL DEFECT Short axis view NORMAL Complete AVSD

24 ATRIOVENTRICULAR SEPTAL DEFECT 3D/4D En-Face View of Valve

25 Atrioventricular length ratio Normal Heart Complete AVSD Ultrasound Obstet Gynecol 2004; 24: 618

26 ATRIOVENTRICULAR SEPTAL DEFECT RV LV LA RA Unbalanced type

27 ATRIOVENTRICULAR SEPTAL DEFECT 3D/4D Unbalanced type

28 AVSD and Down syndrome AVSD accounts for 40% of congenital heart disease in children with Down syndrome AVSD does NOT change the survival rate in patients with Down syndrome 1) Complete balanced AVSD 2) Partial AVSD 3) Complete Unbalanced AVSD Risk for DS

29 Hypoplastic Left Heart Syndrome

30 Hypoplastic Left Heart Syndrome Spectrum of Malformations Severe hypoplasia of left ventricle and left ventricular outflow tract Obstruction to Systemic Cardiac Output

31 Hypoplastic Left Heart Syndrome Classic Types: Mitral & aortic atresia Aortic atresia (Patent MV) Ultrasound Obstet Gynecol 2000;4:271

32 From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition Hypoplastic Left Heart Syndrome DA AO PA RA RV

33 Hypoplastic Left Heart Syndrome Variants: Critical AS with hypoplastic LV Severe coarctation of Aorta Severely unbalanced A-V canal Ultrasound Obstet Gynecol 2000;4:271

34 1 Ultrasound Obstet Gynecol 2000;4:271 Pediatrics 1988;82:698 Hypoplastic Left Heart Syndrome Incidence: / 1000 live births Karyotypic abnormalities in 5 % Extracardiac anomalies in up to 28% Preponderance in fetuses (7/10) Represents 5 % of all CHD

35 Hypoplastic Left Heart Syndrome Karyotypic Abnormalities Classic HLHS (4-5%) Critical AS (very Low) A-V canal defects (High) Coarctation of Aorta (High)

36 Hypoplastic Left Heart Syndrome Prenatal US follow-up IUGR due to 20 % reduction in combined cardiac output Am J Epidemiol 1996;143:505

37 Normal HLHS From Little Hearts Matter ( with permission

38 Hypoplastic Left Heart Syndrome Four-Chamber View Diminutive, hypertrophic, hypokinetic LV RV apex forming Mitral valve dysplastic, echogenic and stenotic Foramen ovale leaflet from left to right Color Doppler fills RV only LV LA RV RA

39 Hypoplastic Left Heart Syndrome LV RV LV RV RA LA RA LA Normal

40 Hypoplastic left heart syndrome LV RV LV RV RA LA RA LA Aortic & mitral atresia Critical aortic stenosis

41 Hypoplastic left heart syndrome Four-Chamber View

42 Hypoplastic left heart syndrome Left Ventricular Outflow Tract

43 Hypoplastic left heart syndrome Right Ventricular Outflow Tract

44 Hypoplastic Left Heart Syndrome 4-Chamber Color Doppler From: A Practical Guide to Fetal Echocardiography Abuhamad, Chaoui

45 Hypoplastic left heart syndrome 4-Chamber Color Doppler

46 From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition Hypoplastic Left Heart Syndrome Color Doppler Foramen Ovale

47 Hypoplastic Left Heart Syndrome Left-to-right shunt At Foramen Ovale LA

48 Hypoplastic Left Heart Syndrome Three-Vessel Trachea View Two vessels are seen Dilated PA, next to SVC Nonvisible or diminutive aorta Color Doppler shows reverse flow in aortic isthmus

49 From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition Hypoplastic Left Heart Syndrome 3-Vessel Trachea View

50 Hypoplastic left heart syndrome 3 Vessel-Trachea View

51 Hypoplastic Left Heart Syndrome Prenatal Follow-up: Monthly Fetal growth (IUGR) Size inter-atrial communication (PV Doppler) Function of A-V valve - RV Semin Fetal Neonatal Med 2005;10(6):553

52 From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition Hypoplastic Left Heart Syndrome Restriction of flow across foramen

53 Ebstein Anomaly

54 Ebstein Anomaly From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition

55 Ebstein Anomaly From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition

56 Ebstein Anomaly Wide spectrum Minor form to severe form Associated anomalies: Pulmonary stenosis or atresia VSD ASD

57 Ebstein Anomaly Spectrum of Disease

58 Ebstein Anomaly Ultrasound Findings Enlarged heart Dilated RA Attachment of septal leaflet to RV wall (essential for diagnosis) Paradoxical septal movement Holosystolic TR Jet originates deep in RV RV outflow obstruction

59 Ebstein Anomaly

60 Ebstein Anomaly Color Doppler

61 Ebstein Anomaly

62 Ebstein Anomaly

63 Ebstein Anomaly

64 Ebstein Anomaly From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition

65 Tricuspid Atresia

66 Tricuspid Atresia From Practical Guide To Fetal Echocardiography Abuhamad, Chaoui 2 nd Edition

67 Tricuspid Atresia Inlet-type VSD always present Size of RV is related to size of VSD Widely patent foramen ovale or ASD Flow to RV thru VSD in late diastole

68 Tricuspid Atresia Ultrasound Findings Small RV, normal contractility No RV myocardial thickening Thickened echogenic TV Slightly dilated RA Large foramen ovale Malaligned atrial and ventricular septae

69 Tricuspid Atresia

70 Tricuspid Atresia

71 Tricuspid Atresia Early Diastole Late Diastole

72 Tricuspid Atresia

73 Tricuspid Atresia Type Prevalence Great arteries Type % Normal Type % D-Transposition Type 3 Rare CAT, L-Transposition

74 Cardiac Tumors

75 Cardiac Tumors Rhabdomyomas (80-90%) Teratomas Fibromas Myxomas Rhabdomyosarcomas Hamartomas Others

76 Rhabdomyomas

77 Tuberous Sclerosis

78

79 Four-Chamber View Know the anatomy very well Understand various sonographic approaches to the 4-chamber view Learn sonographic findings in common cardiac malformations

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