NASCI 2012 Segmental Analysis

Transcription

1 NASCI 2012 Segmental Analysis Frandics Chan, M.D., Ph.D. Stanford University Medical Center Lucile Packard Department Children s of Radiology Hospital

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3 Menagerie of Congenital Cardiac Lesions 1. Absent pulmonary valve 2. ALCAPA 3. Aortopulmonary window 4. Atrial septal defect 5. Atrioventricular canal (endocardial cushion defect) 6. Bicuspid aortic valve 7. Cervical aortic arch 8. Circumflex aortic arch 9. Cleft mitral valve 10. Coarctation 11. Coronary fistula 12. Complete transposition of the great arteries 13. Congenital pericardial defect 14. Congenitally corrected transposition of the great arteries 15. Cor triatriatum 16. Criss-cross heart

4 Menagerie of Congenital Cardiac Lesions 17. Diverticulum of Kommerell 18. Double aortic arch 19. Double chambered right ventricle 20. Double inlet left ventricle 21. Double orifice mitral valve 22. Double outlet left ventricle 23. Double outlet right ventricle 24. Ebstein anomaly 25. Gerbode defect 26. Hemitruncus 27. Interrupted aortic arch 28. Interrupted IVC with azygos continuation 29. Major aortopulmonary collateral arteries 30. Persistent fifth aortic arch 31. Partial anomalous pulmonary venous return 32. Parachute mitral valve

5 Menagerie of Congenital Cardiac Lesions 33. Patent ductus arteriosus 34. Patent foramen ovale 35. Pulmonary artery sling 36. Pulmonary atresia with intact ventricular septum 37. Pulmonary atresia with VSD 38. Total anomalous pulmonary venous return 39. Tricuspid atresia 40. Truncus arteriosus 41. Uhl anomaly 42. Unroofed coronary sinus 43. Upstair-downstair heart 44. Ventricular septal defect

6 Menagerie of Cardiac Associated Syndromes 1. Alagille syndrome 2. Bland_Garland-White syndrome 3. CHARGE syndrome 4. DiGeorge syndrome 5. Down syndrome 6. Ehlers-Danlos syndrome 7. Eisenmenger syndrome 8. Ellis-van Creveld syndrome 9. Heterotaxy 10. Holt-Oram syndrome 11. Hunter syndrome 12. Hypoplastic left heart syndrome 13. Kartagener syndrome 14. Long QT syndrome 15. Loeys-Dietz syndrome 16. Marfan syndrome 17. Noonan syndrome 18. Rubella syndrome 19. Scimitar syndrome 20. Shone complex 21. Tetralogy of Fallot 22. Turner syndrome 23. VACTERL association 24. Williams syndrome 25. Wolff-Parkinson-White

7 Menagerie of Cardiac Operations 1. Bentall procedure 2. Blalock-Taussig shunt 3. Brock procedure 4. Damus-Kaye-Stansel operation 5. David-Tyrone procedure 6. Double switch procedure 7. Fontan procedure 8. Glenn shunt 9. Ilbawi procedure 10. Jatene procedure 11. LeCompte maneuver 12. Konno procedure 13. Mustard-Senning procedure 14. Norwood procedure 15. Potts shunt 16. Pulmonary artery banding 17. Rashkind septostomy 18. Rastelli procedure 19. Ross procedure 20. Sterling Edwards 21. Transannular patch 22. Unifocalization 23. Waterston shunt

8 Pearl #1 Segmental analysis is an organizational scheme for congenital heart disease based on anatomy.

9 Richard van Praagh Stella van Praagh Boston Children s Hospital Robert H Anderson University College, London

10 Cardiac Segments

11 Cardiac Organogenesis

12 Segmental Approach to CHD Cardiac Development

13 Segmental Analysis Principles: There are three segments: Atrial, Ventricular, and Arterial Each segment contains paired structures Right atrium : Left atrium Right ventricle : Left ventricle Aorta : Pulmonary Trunk

14 Segmental Analysis Task: Identify the relative positions of the paired structures in each segment. Identify connections between segments. Identify anomalies associated with each segment and between segments

15 Cardiac Segments

16 Effects of Segmental Analysis Limits the kinds of cardiac anomaly possible. Example: No malformation connects the atrium to the artery Defines a group of elemental lesions that can form compound anomalies. Example: Tetralogy of Fallot = VSD + PS Corrected TGA = AV Dis + VA Dis Next

17 Pearl #2 In practice, the segmental analysis is an organized check list of cardiac anomalies. Recognized combinations of anomalies yield syndromes.

18 Segmental Analysis Sections Situs Cavae Pulmonary veins Atria Atrioventricular connections Ventricles Ventriculoarterial connections (Coronary arteries) Aorta Pulmonary arteries (Lung / Airways)

19 Segmental Analysis Sections Situs: Abdomen / Chest / Atrial positions Cavae: Systemic veins, cavoatrial connections Pulmonary veins: PV-atrial connections Atria: Interatrial communications, obstructions Atrioventricular connections: AV alignments, Inlet valve status Ventricles: ventricle positions, ventricle status Ventriculoarterial connections: VA alignment, outlet valve status (Coronary arteries): Coronary anatomy, aneurysm, obstruction Aorta: Anatomy, aneursym, obstruction Pulmonary arteries: Anatomy, aneurysm, obstruction (Lung / Airways): Anatomy, airway obstruction, intrinsic lung dz.

20 Segmental Analysis Sections Situs: Situs Inversus Totalis Cavae: Bilateral SVC Pulmonary veins: Partial Anomalous Pulmonary Venous Return Atria: ASD Atrioventricular connections: AV Canal Defect Ventricles: VSD Ventriculoarterial connections: TGA (Coronary arteries): Aberrant Interarterial Coronary Artery Aorta: Coarctation Pulmonary arteries: Absent Branch PA (Lung / Airways): Tracheal Bronchus

21 Segmental Analysis Sections Situs Cavae Pulmonary veins Atria Atrioventricular connections Ventricles Ventriculoarterial connections (Coronary arteries) Aorta Pulmonary arteries (Lung / Airways)

22 Situs Situs Latin: place where something originates Sidedness of the body Solitus (normal) Inversus Ambiguous Right Isomerism Left Isomerism Undefined

23 Body Situs

24 Heterotaxy

25 Pearl #3 Atrial situs, or the positions of the atria, is the defining reference for cardiac situs.

26 Right Atrial Appendage

27 Left Atrial Appendage

28 Atrial Appendages

29 Right-Isomerism

30 Pearl #4 It is possible to have two morphologically right atrium, but there is only one right-sided atrium. Same is true for the left atrium.

31 Thoracic Situs Pulmonary artery / mainstem bronchi relationships Bronchial morphology Number of lobes in each lung

32 Normal PA-Bronchial Relationship RPA LPA

33 Right Isomerism PA Bronchus

34 Normal Bronchi Normal Inversus

35 Isomerism Right Isomerism Left Isomerism

36 Segmental Analysis Sections Situs Cavae Pulmonary veins Atria Atrioventricular connections Ventricles Ventriculoarterial connections (Coronary arteries) Aorta Pulmonary arteries (Lung / Airways)

37 Normal Venae Cavae

38 Bilateral SVC

39 Bilateral SVC R-Isomerism

40 Interrupted IVC Azygos Cont. L-Isomerism

41 Segmental Analysis Sections Situs Cavae Pulmonary veins Atria Atrioventricular connections Ventricles Ventriculoarterial connections (Coronary arteries) Aorta Pulmonary arteries (Lung / Airways)

42 Normal Pulmonary Veins

43 TAPVR Supracardiac Intracardiac

44 TAPVR Infradiaphragmatic Mixed

45 PAPVR Associations Sinus Venosus ASD Scimitar Syndrome Others: ASD, 2% has PAPVR Congenital mitral stenosis DORV, TOF, VSD, PS, Coarc, PDA, AS, Hypoplastic aorta

46 Isolated PAPVR

47 PAPVR

48 Segmental Analysis Sections Situs Cavae Pulmonary veins Atria Atrioventricular connections Ventricles Ventriculoarterial connections (Coronary arteries) Aorta Pulmonary arteries (Lung / Airways)

49 Atrial Segment Atrial positions defined by situs Interatrial communications PFO ASD Atrial size Atrial obstructions Cor Triatriatum (Levo / Dextra) Supra-mitral ring

50 Classification Ostium Secundum Ostium Primum Sinus Venosus -superior -inferior Unroofed Coronary Sinus Braunwald, Heart Disease: A Textbook of Cardiovascular Medicine, 2001.

51 Location Frequency 75% Ostium secundum Ostium primum Sinus venosus Coronary sinus 2% 8% 15%

52 Ostium Secundum Defect

53 Sinus Venosus ASD Q p /Q s = 2.4

54 Superior SVASD + PAPVR

55 Segmental Analysis Sections Situs Cavae Pulmonary veins Atria Atrioventricular connections Ventricles Ventriculoarterial connections (Coronary arteries) Aorta Pulmonary arteries (Lung / Airways)

56 Atrioventricular Lesions Abnormal chamber connections: Tricuspid atresia Congenitally corrected TGA (L-TGA) Double inlet left ventricle Abnormal inlet valve Cleft mitral valve Double orifice mitral valve Atrioventricular canal defect Parachute mitral valve Ebstein s anomaly Tethered tricuspid valve

57 AV Connection Equal Chambers

58 Normal AV Connection

59 AV Canal Defect

60 AV Discordant

61 AV Connection Unequal Chambers

62 Tricuspid Atresia

63 Hypoplastic Left Heart

64 Double Inlet Left Ventricle

65 Segmental Analysis Sections Situs Cavae Pulmonary veins Atria Atrioventricular connections Ventricles Ventriculoarterial connections (Coronary arteries) Aorta Pulmonary arteries (Lung / Airways)

66 Ventricular Segment Ventricular morphology: Left / Right Looping Embryological event at 22 days D-loop: normal places RV right-side of LV L-loop: inversed places RV left-side of LV Hand rule Looping is not related to apex position L-loop is not dextrocardia

67 Pearl #5 There can only be one right ventricle and one left ventricle.

68 Ventricle Assignment Right Ventricle Trabeculation Moderator Band Inlet-outlet valve discontinuity Conal muscle Apical inlet valve Septal papillary muscle Left Ventricle Smooth wall No moderator band Inlet-outlet valve fibrous continuity No conal muscle Basal inlet valve No septal papillary muscle

69 Ventricle Assignment

70 Offset Inlet Valves

71 Embryological Cardiac Loop

72 Hand Rule for Ventricular Loop

73 Ventricular Lesions Small ventricle Poor function Volume overload Pressure overload Shunting Cardiomyopathy

74 Ventricular Septal Defect Most common intracardiac structural defect Types: Perimembranous (70%) Muscular (20%) Supracristal (5%) Inlet, isolated (< 5%) Inlet VSD + primum ASD = AVC defect

75 Supracristal VSD

76 Perimembranous VSD

77 Muscular VSD

78 Segmental Analysis Sections Situs Cavae Pulmonary veins Atria Atrioventricular connections Ventricles Ventriculoarterial connections (Coronary arteries) Aorta Pulmonary arteries (Lung / Airways)

79 Ventriculoarterial connection Concordance normal Discordance TGA RV to Aorta+PA DORV LV to Aorta+PA DOLV, rare Aorta doubly committed TOF Pulmonary atresia PAcVSD, PAIVS Aortic atresia - HLHS

80 Normal Conotruncal Septation

81 Etienne-Louis Arthur Fallot In 1888 described in details the four anatomical abnormalities of TOF, a cause of blue baby syndrome

82 Tetralogy of Fallot

83 Conventional TOF

84 Transposition of the Great Arteries Ao PA RV LV

85 Complete TGA (D-TGA) CTGA Normal

86 Complete TGA (D-TGA) ASD PDA

87 Congenitally Corrected TGA (L-TGA) CCTGA Body RA LA RV LV Lung Normal Body RA LA RV LV Lung

88 Congenital Corrected TGA CCTGA L-TGA LV RV Ao PA CTGA D-TGA RV LV Ao PA

89 Segmental Analysis Sections Situs Cavae Pulmonary veins Atria Atrioventricular connections Ventricles Ventriculoarterial connections (Coronary arteries) Aorta Pulmonary arteries (Lung / Airways)

90 Arterial Segment Great Arteries D-position: normal Aorta to the right of PA L-position: inverted Aorta to the left of PA Refers to position at birth Adult position may change due to chamber enlargement and rotation of the heart.

91 Pearl #6 The letter before TGA refers to D- or L- loop of the ventricle, not the D- or L- relationship of the great arteries.

92 Pearl #6 D-position and L-position do not specify anterior-posterior relation of the great arteries.

93 Arterial Morphology Aorta Coronary arteries (except ALCAPA) Supplies to systemic organs Pulmonary Artery No coronary arteries Characteristic location Supplies to lung exclusively

94 Anomalies of the Aorta Truncus arteriosus Aortopulmonary window Patent ductus arteriosus Interrupted aorta / Coarctation Arch anomalies Vascular rings

95 Truncus Arteriosus Aorta and PA connected to a common truncal valve Uncommon 1% of CHD Classifications Collett and Edwards Van Praagh Large L-to-R shunting Early pulmonary vascular disease and PHTN

96 Truncus Arteriosus Tricuspid Truncal Valve Quad. Truncal Valve Sub-truncal VSD

97 Anomalies of the Pulmonary Arteries Anomalous origins From PA (Pulmonary sling) From systemic arteries (Truncus) Anomalous branching Scimitar syndrome Branch PA stenoses Tetralogy of Fallot Williams syndrome Alagille syndrome

98 Absent Branch Pulmonary Artery

99 Pulmonary Sling

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