Acyanotic and cyanotic shunt lesions 03 2019
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This document discusses the differential diagnosis of acyanotic and cyanotic congenital heart defects. It begins by explaining how congenital heart disease can cause left-to-right shunts, left or right heart obstructions, pump failure, or hypoxemia. It then provides examples of cyanotic defects like tetralogy of Fallot, transposition of the great arteries, and tricuspid atresia. It also discusses physical exam findings, imaging, and treatments for various defects.
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Acyanotic and cyanotic shunt lesions 03 2019
- 1. ABB MD FACC© Acyanotic & Cyanotic Shunt Lesions: Differential Diagnosis A. Bornstein, M.D., F.A.C.C. Assistant Professor of Medicine Weill Cornell Medical College New York, NY
- 2. ABB MD FACC© 1) Large volume left-to-right shunt 2) Left heart inflow obstruction or left heart outflow obstruction 3) Pump failure 4) Hypoxemia Understanding the Pathophysiology of CHD Simplifies the Differential Diagnosis • Symptomatic congenital heart disease relates to:
- 3. ABB MD FACC© Differential Diagnosis of Cyanotic CHD • Tetralogy of Fallot (TOF) • d-Transposition of the great arteries (d-TGA) • Tricuspid valve atresia • Total anomalous pulmonary venous return (TAPVR) • Truncus arteriosus • Pulmonary atresia (with VSD or IVS) • Single ventricle heart (DILV) • Ebstein’s anomaly of the tricuspid valve • T • T • T • T • T • PA (with VSD or IVS) • Single ventricle (DILV) • Ebstein’s anomaly http://www.blaufuss.org/tutorial/index2.html
- 4. ABB MD FACC© Evaluation of the Acyanotic & Cyanotic Shunt Lesions Acyanotic: Shunt absent Shunt present Left to Right Malformation in left heart Malformation in right heart Normal PBF PBF
- 5. ABB MD FACC© Evaluation of the Acyanotic & Cyanotic Shunt Lesions Acyanotic: PBF Left to right shunt RVH: LVH: LVH & RVH:
- 6. ABB MD FACC© Evaluation of the Acyanotic & Cyanotic Shunt Lesions Acyanotic: PBF Left to right shunt RVH: LVH: LVH & RVH: ASD PAPVD
- 7. ABB MD FACC© Atrial Septal Defect: Chest X-Rays & Shunt Size
- 8. ABB MD FACC© EKG Tracings in ASD Types Sinus venosus ASD Ostium secundum ASD Caval ASD Ostium secundum ASD Sinus venosus ASD
- 9. ABB MD FACC© EKG Tracings in ASD Types Ostium primum ASD Ostium primum ASD
- 10. ABB MD FACC© Sinus Venosus ASD Type Associated With PAPVR
- 11. ABB MD FACC© Percutaneous Closure of Secundum ASD
- 12. ABB MD FACC© Evaluation of Acyanotic & Cyanotic Shunt Lesions Acyanotic: PBF Left to right shunt RVH: ASD PAPVD LVH: LVH & RVH: VSD PDA A-P window
- 13. ABB MD FACC© Ventricular Septal Defect Supracristal (subpulmonic) VSD Muscular VSD Membranous VSD
- 14. ABB MD FACC© ABB MD Patent Ductus Arteriosus
- 15. ABB MD FACC© Evaluation of Acyanotic & Cyanotic Shunt Lesions Acyanotic: PBF Left to right shunt RVH: ASD PAPVD LVH: VSD PDA A-P Window LVH & RVH: A-V canal defect VSD (late) or (with mild PS) PDA (late) or (with mild PS)
- 16. ABB MD FACC© ABB MD A-V Canal Defect
- 17. ABB MD FACC© Evaluation of Acyanotic & Cyanotic Shunt Lesions Cyanotic: R to L shunt Bi-directional shunt PBF Normal or PBF (+) Pulmonary hypertension (-) Pulmonary hypertension (+) LV (+) RV1) TGA 2) Truncus 3) TAPVR 4) Taussig-Bing (DORV) 5) Single Ventricle (DILV) 1) Ebstein’s anomaly 2) Tricuspid atresia 3) Pulmonary atresia with IVS 1) TOF 2) Pulmonary atresia with VSD 1) Eisenmenger’s
- 18. ABB MD FACC© CXR in Cyanotic CHD Right aortic arch: TOF Small Heart: TAPVR ‘Snowman in a snowstorm’: TAPVR ‘Boot shaped heart’: TOF, Pulmonary Atresia ‘Egg-on-string’: d-TGA, Tricuspid atresia/TGA
- 19. ABB MD FACC© CXR in Cardiosplenic (Heterotaxy) Syndromes • Dextrocardia or mesocardia in 50% • Abnormal abdominal situs: right sided stomach, malrotation, transverse liver • Usually decreased PBF • Bronchopulmonary symmetry
- 20. ABB MD FACC© ECG in Cyanotic CHD • d-TGA: RVH or normal • TOF, PA/VSD: RVH • TAPVR: RVH • Truncus: LVH and RVH • Cardiosplenic: abnormal P wave, LAD, RVH Abnormal P wave RVH
- 21. ABB MD FACC© ECG in Cyanotic CHD Pulmonary atresia/IVS: absent RV forces; normal axis; atrial enlargement Tricuspid atresia: absent RV forces; LAD; atrial enlargement
- 22. ABB MD FACC© Hypoxemia With Pulmonary Blood Flow ‘Tetralogy physiology’ – Pulmonary stenosis with an upstream R-to-L ‘blow-off’ at ventricular level Same physiology for single ventricle/PS; d-TGA/VSD/PS; pulmonary atresia/VSD ‘Hypoplastic right heart physiology’ – Pulmonary atresia with IVS – Tricuspid atresia – Ebstein’s anomaly with impaired right atrial emptying, with upstream ‘blow-off’ at atrial level
- 23. ABB MD FACC© ‘Tetralogy of Fallot Physiology’
- 24. ABB MD FACC© ‘Hypoplastic Right Heart Physiology’ ASD Pulmonary atresia Hypoplastic RV PDA
- 25. ABB MD FACC© ‘Hypoplastic Right Heart Physiology’ Ebstein’s anomaly
- 26. ABB MD FACC© Hypoxemia With Normal-to- Pulmonary Blood Flow • Ventriculoarterial discordance (d-TGA) – Cyanosis is greatest when ‘mixing’ is least – Mixing’ is best with: Aorta-to-PA shunt at ductal level Left atrial-to-right atrial reciprocal shunt at PFO (patent foramen ovale) VSD
- 27. ABB MD FACC© d-Transposition of the Great Arteries
- 28. ABB MD FACC© Evaluation of Acyanotic & Cyanotic Shunt Lesions Cyanotic: R to L shunt Bi-directional (+) Pulmonary hypertension (-) Pulmonary hypertension (+) LV (+) RV 1) TGA 2) Truncus 3) TAPVR 4) Taussig-Bing (DORV) 5) Single Ventricle (DILV) 1) Ebstein’s anomaly 2) Tricuspid atresia 3) Pulmonary atresia with IVS 1) TOF 2) Pulmonary atresia with VSD 1) Eisenmenger’s PBF Normal or PBF
- 29. ABB MD FACC© Evaluation of Acyanotic & Cyanotic Shunt Lesions Cyanotic: R to L shunt Bi-directional shunt Normal or PBF (-) Pulmonary hypertension (+) RV 1) Tetralogy of Fallot 2) Pulmonary atresia with VSD
- 30. ABB MD FACC© Tetralogy of Fallot 1) VSD (malalignment) 2) Pulmonary stenosis; Infundibular stenosis 3) Overriding aorta 4) RVH
- 31. ABB MD FACC© ABB MD Tetralogy of Fallot • Accounts for 8-10% of congenital heart disease • Most common cyanotic congenital cardiac lesion at all ages; conotruncal anomaly: may be seen with DiGeorge syndrome (22q11 chromosome microdeletion) • ‘Boot-shaped’ heart with decreased PBF on CXR • Untreated, only 50% live 1 year and 10% live 20 years • May be palliated with a shunt
- 32. ABB MD FACC© Tetralogy of Fallot: Embryological Development
- 33. ABB MD FACC© Tetralogy of Fallot • Tetralogy associated anomalies: – 25% right aortic arch – 10% ASD – 10% coronary anomalies
- 34. ABB MD FACC© Anatomy of Tetralogy of Fallot
- 35. ABB MD FACC© Tetralogy of Fallot: Physical Exam • Cyanosis: degree depends on PS • Normal pulses • Increased RV impulse at RSB • Murmur along LSB
- 36. ABB MD FACC© CXR in Tetralogy of Fallot • Coeur-en-sabot: boot-shaped heart • Decreased PBF • Right aortic arch (25-30%) • CHF unlikely
- 37. ABB MD FACC© Tetralogy of Fallot: Catheterization Data
- 38. ABB MD FACC© PS in Tetralogy of Fallot: RV Angiogram
- 39. ABB MD FACC© ABB MD Pulmonic Stenosis in Tetralogy of Fallot Valvar PS Infundibular PS
- 40. ABB MD FACC© ABB MD Hypoxic Spells in Tetralogy of Fallot • Relieved by squatting • Occurs only in childhood
- 41. ABB MD FACC© Palliative Shunt for Tetralogy of Fallot
- 42. ABB MD FACC© ABB MD Definitive Surgical Repair for Tetralogy of Fallot
- 43. ABB MD FACC© ABB MD Long-term Results for Tetralogy of Fallot Repair
- 44. ABB MD FACC© ABB MD Pulmonary Atresia With Intact Ventricular Septum • < 1% of CHD • Pulmonary atresia and tricuspid atresia make up the ‘hypoplastic right heart syndromes’ • Varying degrees of RV hypoplasia • Ductal dependent lesion • May have RV to coronary artery fistulae
- 45. ABB MD FACC© Pulmonary Atresia Valvar pulmonary atresia Muscular pulmonary atresia Solitary arterial trunk
- 46. ABB MD FACC© Pulmonary Atresia With Intact Ventricular Septum • Severe cyanosis dependent on PDA status, with O2 saturation between 50-80%; may have TR murmur ECG with atrial enlargement, decreased RV forces, and QRS axis of 0-90 degrees • Palliated with shunt (+/-) RVOT patch • May eventually get 2 ventricle repair, but more often requires Fontan approach
- 47. ABB MD FACC© Chest X-Ray & EKG in Pulmonary Atresia With Intact Ventricular Septum
- 48. ABB MD FACC© Pulmonary Atresia With Intact Ventricular Septum
- 49. ABB MD FACC© Pulmonary Atresia: Selective RV Angiogram & PA Angiogram
- 50. ABB MD FACC© Pulmonary Atresia: Selective RV Angiogram
- 51. ABB MD FACC© 1) Ebstein’s anomaly 2) Tricuspid atresia 3) Pulmonary atresia with IVS Evaluation of Acyanotic & Cyanotic Shunt Lesions Cyanotic: R to L shunt Bi-directional PBF Normal or PBF (+) Pulmonary hypertension (-) Pulmonary hypertension (+) LV (+) RV 1) TGA 2) Truncus 3) TAPVR 4) Taussig-Bing (DORV) 5) Single Ventricle (DILV) 1) TOF 2) Pulmonary atresia 1) Eisenmenger’s
- 52. ABB MD FACC© Evaluation of Acyanotic & Cyanotic Shunt Lesions Cyanotic: R to L shunt Bi-directional shunt PBF 1) TGA 2) Truncus 3) TAPVR 4) Taussig-Bing (DORV) 5) Single Ventricle (DILV)
- 53. ABB MD FACC© ABB MD d-Transposition of the Great Vessels • 5% of CHD; male to female ratio = 3:1 • Presents with severe cyanosis (pO2 ~ 20-30 mm Hg or O2 saturation ~ 50-70%) in the 1st week of life • Newborns with d-TGA & ASD, VSD, &/or PDA are less cyanotic • Frequently have no murmur • ‘Egg-shaped heart’ on CXR • May require balloon atrial septostomy to palliate • Surgery of choice is arterial switch operation
- 54. ABB MD FACC© ABB MD d-Transposition of the Great Arteries d-TGA stages of development • Anatomic - Anterior aorta - Aorta arises from RV • Physiologic - Aorta receives systemic mixed venous blood - Parallel circuits
- 55. ABB MD FACC© d-TGA: Catheterization Data
- 56. ABB MD FACC© Physical Exam for d-TGA • Cyanosis: moderate to severe with PaO2 = 20-30 mm Hg (O2 saturation 50-80%) • Normal peripheral pulses • Normal to increased RV impulse • Loud single S2 with anterior aorta • Soft 1-2/6 systolic flow murmur at mid-LSB or no murmur
- 57. ABB MD FACC© Chest X-Ray in d-TGA • May be normal in neonate • ‘Egg on a string sign’ 1) Egg-shaped heart in ~ 33% of patients (ovoid heart) 2) Narrow mediastinum and ovoid heart • Increased PBF after several days
- 58. ABB MD FACC© ABB MD d-TGA: Pathology & Selective LV Angiogram
- 59. ABB MD FACC© ABB MD d-TGA: Pathology & Selective LV Angiogram
- 60. ABB MD FACC© ABB MD d-TGA (Transposition) & l-TGA (Congenitally Corrected TGA)
- 61. ABB MD FACC© Balloon Atrial Septostomy for d-TGA
- 62. ABB MD FACC© Atrial Switch for d-TGA: Mustard or Senning Repair Senning procedure: A) Opening of the right atrium. B) A flap of interatrial septum is folded over and sewn to the posterior wall of the left atrium, isolating the pulmonary veins below it. C) The posterior margin of the right atrial opening is sewn to the interatrial septum, in this way, it isolates caval flow from the tricuspid valve. So, caval flow is directed through the created atrial septal defect into the left atrium and thus into the left ventricle and pulmonary artery.
- 63. ABB MD FACC© Arterial Switch (Jatene Procedure) for d-TGA Jatene procedure: A) The coronary arteries are excised from the aorta with a buttonlike margin of tissue. B) Small holes are created in the neoaorta and the coronary arteries are implanted above the valve. C) Ascending aorta and the pulmonary artery are transected above the valves & aorta is replaced posterior to pulmonary artery before anastomosis (Lecompte maneuver). D) Reconstruction of areas of the PA from which the coronary arteries were excised using pericardial patch. In TGA with VSD, VSD is corrected as well. AO: Aorta. PA: Pulmonary artery. TV: Tricuspid valve. MV: Mitral valve.
- 64. ABB MD FACC© ABB MD Arterial Switch Operation for d-TGA
- 65. ABB MD FACC© ABB MD Persistent Truncus Arteriosus
- 66. ABB MD FACC© ABB MD Persistent Truncus Arteriosus • Usually mild cyanosis with early CHF in first few weeks of life • To-and-fro murmur with valve stenosis & insufficiency • Right aortic arch in 30-50% • Interrupted aortic arch in 10-20%
- 67. ABB MD FACC© ABB MD Persistent Truncus Arteriosus • Incidence 0.4% - 2.8% of all CHD anomalies • Common arterial trunk with malalignment VSD immediately beneath truncal valve (frequently an abnormal truncal valve) • 33% of cases are associated 22q11 chromosomal micro-deletion; also associated with DiGeorge syndrome
- 68. ABB MD FACC© ABB MD Persistent Truncus Arteriosus Collet & Edwards Classification • Type I) Short main PA arises from left posterolateral aorta • Type II) Pulmonary arteries arise separately from left posterolateral aorta • Type III) Each pulmonary artery arises from lateral aorta • Type IV) Pulmonary arteries arise from descending aorta (‘pseudotruncus’ more likely TOF with pulmonary atresia)
- 69. ABB MD FACC© ABB MD Persistent Truncus Arteriosus: Cardiac Catheterization Data
- 70. ABB MD FACC© ABB MD Total Anomalous Pulmonary Venous Return • 1% of congenital heart disease • Classified as: – Supracardiac (50%) – Cardiac (20%) – Infracardiac (subdiaphragmatic) (20%) • If unobstructed, presents with mild to moderate cyanosis and CHF at weeks to months • If obstructed (infracardiac), presents early with severe cyanosis • ‘Snowman in a Snowstorm’ or small heart on CXR
- 71. ABB MD FACC© ABB MD TAPVR Types
- 72. ABB MD FACC© ABB MD TAPVR Types 50% 20% 20%
- 73. ABB MD FACC© ABB MD TAPVR (Supradiaphragmatic Type): Cardiac Catheterization Data To SVC in anterior view To left SVC in posterior view
- 74. ABB MD FACC© ABB MD TAPVR (Obstructed Infradiaphragmatic Type): Cardiac Catheterization Data To IVC in anterior view To IVC/HPV in posterior view
- 75. ABB MD FACC© ABB MD TAPVR With Pulmonary Venous Obstruction Cardiac Catheterization-Lingular Injection
- 76. ABB MD FACC© ABB MD Chest X-Ray in TAPVR A) Snowman with supracardiac B) Obstructed infradiaphragmatic A B
- 77. ABB MD FACC© ABB MD Total Anomalous Pulmonary Venous Return
- 78. ABB MD FACC© ABB MD TAPVR With Pulmonary Venous Obstruction Catheterization- Right PA Injection
- 79. ABB MD FACC© ABB MD Total Anomalous Pulmonary Venous Return (Supradiaphragmatic) (Infradiaphragmatic)
- 80. ABB MD FACC© ABB MD Double Outlet Right Ventricle: RAO Projection PAAo RV LVVSD
- 81. ABB MD FACC© ABB MD Double Outlet Right Ventricle
- 82. ABB MD FACC© ABB MD Clinical Characteristics of Single (Common) Ventricle • Definition: (DILV) double inlet left ventricle – MV & TV enter a common ventricle – Usually LV morphology • Intraventricular anatomy: – 80% associated outlet chamber (conus cordis origin?) – 20% no outlet chamber
- 83. ABB MD FACC© ABB MD Single Ventricle
- 84. ABB MD FACC© ABB MD Evaluation of the Acyanotic & Cyanotic Shunt Lesions Cyanotic: R to L shunt Bi-directional (+) Pulmonary hypertension (-) Pulmonary hypertension (+) LV (+) RV 1) TGA 2) Truncus 3) TAPVR 4) Taussig-Bing (DORV) 5) Single Ventricle (DILV) 1) Ebstein’s anomaly 2) Tricuspid atresia 3) Pulmonary atresia with IVS 1) TOF 2) Pulmonary atresia 1) Eisenmenger’s PBF Normal or PBF
- 85. ABB MD FACC© ABB MD Evaluation of Acyanotic & Cyanotic Shunt Lesions Cyanotic: R to L shunt Bi-directional shunt Normal or PBF (-) Pulmonary hypertension (+) LV Ebstein’s anomaly Tricuspid atresia Pulmonary atresia with IVS
- 86. ABB MD FACC© ABB MD Ebstein’s Anomaly: Chest X-Ray
- 87. ABB MD FACC© ABB MD Ebstein’s Anomaly Transesophageal Echocardiogram • Incidence <1% • Anterior leaflet is usually not totally displaced but enlarged, tethered & sail-like • Valve closure is likewise displaced downwards • Treatment: TVR or various types of plastic reconstruction
- 88. ABB MD FACC© ABB MD Ebstein’s Anomaly: Apical Four Chamber View
- 89. ABB MD FACC© ABB MD Tricuspid Valve Atresia • 1-3% of congenital heart disease • Mild to severe cyanosis depending on size of VSD & amount of PS • May be ductal dependent • Palliated with shunt or occasionally PA band • ECG with LAD, LAE & RAE, decreased RV forces • Definitive repair consists of Fontan operation (direct connection of SVC & IVC to PA without intervening RV)
- 90. ABB MD FACC© ABB MD Tricuspid Valve Atresia
- 91. ABB MD FACC© ABB MD Fontan Operation for Tricuspid Atresia
- 92. ABB MD FACC© ABB MD Tricuspid Atresia: 2-D Echocardiogram
- 93. ABB MD FACC© ABB MD Tricuspid Atresia: Electrocardiogram • 1-3% incidence • 66% associated with PS or pulmonary atresia • 30% associated with d-TGA
- 94. ABB MD FACC© ABB MD Tricuspid Atresia: Cardiac Catheterization Data
- 95. ABB MD FACC© ABB MD First Fontan Operation for Tricuspid Atresia 1971
- 96. ABB MD FACC© ABB MD First Fontan Operation for Tricuspid Atresia 1971
- 97. ABB MD FACC© Abb md Original Fontan Repair for Single Ventricle Physiology Atriopulmonary baffle Main pulmonary artery Atrial appendage
- 98. ABB MD FACC© ABB MD Glenn Shunt and Fontan Shunt
- 99. ABB MD FACC© ABB MD Fontan Surgery for Tricuspid Atresia Kaplan-Meier curves for cumulative continued survival of the 197 operative survivors divided into era of surgery: 1973 through 1980 (solid line), 1981 through 1987 (dotted line), 1988 through 1997 (dashed line). There is a statistically significant improvement in continued survival among patients operated on more recently.
- 100. ABB MD FACC© Evaluation of the Acyanotic & Cyanotic Shunt Lesions Cyanotic: R to L shunt Bi-directional PBF Normal or PBF (+) Pulmonary hypertension (-) Pulmonary hypertension (+) LV (+) RV1) TGA 2) Truncus 3) TAPVR 4) Taussig-Bing (DORV) 5) Single Ventricle (DILV) 1) Ebstein’s anomaly 2) Tricuspid atresia 3) Pulmonary atresia with IVS 1) TOF 2) Pulmonary atresia 1) Eisenmenger’s
- 101. ABB MD FACC© Evaluation of Acyanotic & Cyanotic Shunt Lesions Cyanotic: R to L shunt Bi-directional shunt Normal or PBF (+) Pulmonary hypertension 1) Eisenmenger’s
- 102. ABB MD FACC© Eisenmenger’s Syndrome: Chest X-Ray
- 103. ABB MD FACC© Pulmonary Hypertensive Vascular Disease Vascular changes Normal Medial thickening Medial & intimal thickening Morphology changes Grade 0 Grade I Grade II Plexiform lesions form if PA pressure >90 mm Hg
- 104. ABB MD FACC© ABB MD Pulmonary Hypertensive Vascular Disease: Plexiform Lesion forms if PA pressure >90 mm Hg Normal pulmonary arteriole Plexiform lesion Fibrointimal hyperplasia