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Approach to TOF physiology
The document provides a detailed overview of the differential diagnosis and physiological classifications of cyanotic congenital heart disease (CHD), including several specific conditions like tetralogy of Fallot (ToF), transposition of great arteries (TGA), and tricuspid atresia. It discusses the symptoms, presentations, natural history, and physical exam findings associated with each condition, along with their respective diagnostic implications. Emphasis is placed on the anatomical and physiological characteristics, along with the historical context of these congenital defects.
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Approach to TOF physiology
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- 3. Cyanotic CHD CLASSIFICATION Anatomico-pathologicalPhysiological
- 4. Cyanotic CHD withIncreased PBF • (5 Ts and 2 Ss) – TAPVC – Single Ventricle – TGA – Single (Common) – Taussig-Bing Atrium – Tricuspid Atresia – Truncus Arteriosus
- 5. Cyanotic CHD withIncreased PBF • (5 Ts and 2 Ss) Admixture Physiology (Intercirculatory mixing) • PRE-TRICUSPID • Venous level – TAPVC • Atrial level – Single atrium, Tricuspid atresia, HLHS • POST-TRICUSPID • Ventricular level – Single Ventricle • Arterial level – Truncus Transposition Physiology • TGA • Taussig Bing
- 6. Cyanotic CHD withDecreased PBF RV Dominance PAH Acyanotic CHD with ES VSD PDA, AP window ASD Cyanotic CHD with PAH DORV TGA TAPVC Truncus No PAH RVOTO Quiet Precordium Single S2 ± PS murmur TOF DORV WITH PS TGA WITH PS L-TGA WITH PS ASD with PS
- 7. Cyanotic CHD withDecreased PBF LV dominance LV type Apex (Exam/CXR) No Parasternal Heave LAD ± LV dominance on ECG Tricuspid Atresia with PS Pulmonary Atresia with intact IVS Single Ventricle with PS Ebstein’s
- 8. Cyanotic CHD withNear Normal PBF • Pulmonary AV Fistula • Unroofed coronary sinus into LA • Anomalous drainage of vena cava to LA
- 9. CCHD Physiological classification •TOF Physiology • Transposition Physiology • Admixture Physiology • Eisenmenger Physiology • Duct-dependent Physiology – For PBF – Pulmonary atresia – For SBF – Aortic atresia/HLHS
- 10. CCHD Physiological classification •TOF Physiology • Transposition Physiology • Admixture Physiology • Eisenmenger Physiology • Duct-dependent Physiology – For PBF – Pulmonary atresia – For SBF – Aortic atresia/HLHS • Near-normal Physiology – Pulmonary AVFs • Miscellaneous – Ebstein’s – ASD with PS – Unroofed CS into LA
- 11. • Symptom complex– guide to PHYSIOLOGY • Examination – guide to ANATOMY (Physical findings) • Radiology, ECG – add to BOTH
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- 13. TOF PHYSIOLOGY • CyanoticCHD with decreased PBF having 2 key components anatomically – Severe RVOTO – Decreasing PBF – Large VSD – causing equalization of RV and LV pressure with right to left shunt due to outflow obstruction (Acyanotic TOF not included)
- 14. TOF PHYSIOLOGY • CyanoticCHD with decreased PBF having 2 key symptoms Physiologically – History of Spells – History of Squatting – No CHF symptoms
- 15. Cyanotic CHD withDecreased PBF RV Dominance PAH Acyanotic CHD with ES VSD PDA, AP window ASD Cyanotic CHD with PAH DORV TGA TAPVC Truncus No PAH RVOTO Quiet Precordium Single S2 ± PS murmur TOF DORV WITH PS TGA WITH PS L-TGA WITH PS ASD with PS
- 16. Cyanotic CHD withDecreased PBF LV dominance LV type Apex (Exam/CXR) No Parasternal Heave LAD ± LV dominance on ECG Tricuspid Atresia with PS Pulmonary Atresia with intact IVS Single Ventricle with PS Ebstein’s
- 17. Cyanotic CHD withDecreased PBF LV dominance LV type Apex (Exam/CXR) No Parasternal Heave LAD ± LV dominance on ECG Tricuspid Atresia with PS Pulmonary Atresia with intact IVS Single Ventricle with PS Ebstein’s
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- 19. “Tetralogy of Fallot”History • 1671: First reported by Niels Stenson a.k.a Nicholas Steno • 1777; 1784; 1839; 1866; 1872 Similar Case reports
- 20. • 1888: Etienne LouisArthur Fallot – Anatomic diagnosis at bedside – Confirmed at postmortem – Coined term Tetralogie (Fr.)
- 21. • 1894: PierreMarie (French), first used term “Tetralogie de Fallot” • 1924: Maude Abbott, first used term “Tetralogy of Fallot” & “Fallot’s Tetralogy”
- 22. Pathology • Van Praaghcalled TOF “Monology of Stenson” • Central pathology – Underdevelopment/hypoplasia of Subpulmonary infundibulum • Gives rise to 4 components of ‘tetralogy’ – Obstructive RVOT – Large Malaligned VSD – Aortic override – Dominant RV hypertrophy
- 23. Pathology • Van Praaghcalled TOF “Monology of Stenson” • Central pathology – Underdevelopment/hypoplasia of Subpulmonary infundibulum • Gives rise to 4 components of ‘tetralogy’ – Obstructive RVOT – Large Malaligned VSD – Aortic override – Dominant RV hypertrophy
- 24. TOF • 1 in3600 live births • M=F
- 25. Natural History • Survival –66% 1st yr – 50% 3rd yr – 25% 1st decade • Poor survival with PA – 50% 1st yr – 10% 1st decade
- 26. Symptoms/Presentation • Cyanosis – 1-2weeks after birth – More severe the PS, earlier the presentation • Hypercyanotic Spells – 2 months to 2 years of age • Exertional dyspnoea – Older child • Squatting – To alleviate a spell or dyspnoea
- 27. Physical Exam • Physicallyunderdeveloped • Cyanosis (Depending on PBF) • Pulse – NORMAL (irrespective of PS severity) – Wide PP – only in Large MAPCA/Severe AR
- 28. Physical Exam • JVP –NORMAL (Height and waveform) (RAP stays normal unless significant TR present)
- 29. Physical Exam Palpation • RVimpulse – Gentle; like normal neonatal RV; but stays like that even as child grows – 4th LICS – 5th LICS & Subxyphoid (if Sub-Infundibular stenosis)
- 30. Physical Exam Palpation • LVimpulse – ABSENT (Conspicuous feature) – Absent even if MAPCAs present
- 31. Physical Exam Palpation • 2nd/ 3rd LICS – A2 maybe palpable – Left upper ICS (Not right) (due to hypoplastic PA)
- 32. Physical Exam Palpation • Rightsternoclavicular joint pulsation – Right sided Aortic arch • No thrill due to RVOTO – BF goes uninterrupted to dilated Aorta
- 33. Physical Exam Auscultation • Aorticarea (2nd RICS) – Loud aortic EC from aortic root – Maximum in expiration • Pulmonary area – Very delayed and soft P2 – EC and P2 almost inaudible (Bicuspid PV – decreased mobility)
- 34. Physical Exam Auscultation • 3rdLICS – Superficial murmur starting with S1 Duration and intensity decreases with severity of PS • No S4 (RA contraction not forceful, RAP normal) • No S3 (No RVF)
- 35. Physical Exam Auscultation • Continuousmurmurs – In case of Pulmonary atresia/MAPCAs • AR murmur ± • PR murmur ±
- 36. ECG • P wave –Height normal, peaked – Duration short (LA underfilled) • PR – Normal
- 37. ECG • QRS – RAD(Like newborns) – Clockwise depn – Normal duration – No notching – RVH • Tall R in V1 • Sudden transition V1-2 • Q in V5,6 - PBF
- 38. ECG • T wave –Maybe upright/inverted – Deep inversion rare (RVSP never suprasystemic)
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- 40. CXR • Reduced PBFmarkings • Lacy appearance in Pulmonary Atresia • Dilated Asc Ao • Rt Ao arch 20-30%
- 41. CXR • Coer-en-Sabot • Heartresembling a wooden boot – Concave PA bay – Small underfilled LV above a horizontal IVS – Concentric RVH
- 42. CXR • Coer-en-Sabot • Intrauterinelife LV is normal – so boot shape develops after 1-2 mos of birth • TOF-PA – Boot even in neonates if low PBF
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- 44. DORV • Exact incidenceunknown – Less than TOF • Subaortic VSD 40-50% DORV • PS 40-70% Subaortic VSD
- 45. DORV-PS • History • PhysicalExam • CXR Same as TOF
- 46. DORV-PS • Exceptions - Ifrestrictive VSD present (Subaortic stenosis) • Long decrescendo systolic murmur at LPS area (obligatory flow murmur) • ECG - LVH
- 47. DORV-PS • Differentiation from TOF -ECG - PR Prolonged - Counterclockwise depn - RVH but no sudden transition
- 48. D-TGA – VSD– PS
- 49. D-TGA • 1 in2500-5000 live births • M>F 4:1 • VSD is most common communication • PS (LVOTO) present in 15%
- 50. Symptoms/Presentation • Cyanosis – Sincebirth – 1st day of life • Hypercyanotic Spells – May be present occasionally • Squatting – Rare
- 51. Natural History • Mortalityrate in TGA without PS – 30% 1st week – 50% 1st month – 90% 1st year • Better survival with PS – may survive adolescence
- 52. Physical Exam • Birthweight > normal (Contrast to other CHDs) • Deep Cyanosis • Scalp & Arm varicose veins (Systemic volume overload with desaturated blood)
- 53. • Pulse – Fullvolume bounding pulse – Warm extremities • JVP – Elevated RAP with dominant A wave (Systemic volume overload with desaturated blood)
- 54. • Palpation – RVimpulse gentle at birth – Soon after 1 week – Prominent RV impulse (Systemic volume overload with desaturated blood) – Rt sternoclavicular impulse – Rt arch (11-16% in TGA-VSD-PS) Just like TOF
- 55. • Auscultation – Justlike TOF
- 56. ECG • P wave –Tall, Peaked (Hypervolemic RA) • RAD, RVH • T – Usually positive in ALL precordial leads – Taller in right precordial leads • Counterclockwise depn
- 57. CXR • Thymic shadowabsent (after 12 hrs of birth) • Characteristic narrow pedicle (egg on side) ABSENT in severe PS – due to dilated right anterior aorta • CXR like TOF
- 58. L-TGA – VSD– PS
- 59. L-TGA • 1 in13000 live births • M>F 1.5:1 • VSD 80% L-TGA • PS 50% L-TGA 80% of PS a/w VSD
- 60. Symptoms/Presentation • Cyanosis appearanceaccording to PS severity • Spells and squatting uncommon • Older adults – Stokes-Adams & Syncope (High degree AV blocks)
- 61. Natural History • Bettersurvival with VSD-PS – may survive adolescence
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- 63. • Pulse – Normal –Bradycardia/Blocks • JVP – Normal – 1st /2nd /3rd degree AV blocks
- 64. • Palpation – IVSalmost vertical and parallel to left sternal border – Morph. RV – anterior & left position forming apex – Systolic RV Impulse – Morph. LV – posterior & right position behind sternum – LV Impulse non-palpable (even if enlarged) – Ao EC & A2 palpable 2nd LICS
- 65. • Auscultation – Justlike TOF – In case of blocks • Soft S1 – in 1st degree AVB • Variable S1 – High degree AVB
- 66. ECG • AV Blocksmaybe present – CHB – ventricular activation sequence normal; QRS narrow • LAD (d/t LAFB) • RVH
- 67. • Q /q –Present in right precordial leads / absent in left (Septal activation right to left directed) – Present in III, avf (III>avf) / absent in I, aVL (Septal activation superiorly directed) • T – Usually positive in ALL precordial leads • Clockwise depn
- 68. CXR • Thymic shadowabsent • Ao & PA side by side • Ao left and Anterior • Straight left border • Hump shaped heart • RPA and LPA at same level
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- 70. Tricuspid Atresia (TA) •1 in 17000 live births • 90% TA – No TGA • 90% have Restrictive VSD – Physiologically PS • 10% TA – TGA • 90% have no PS – Increased PBF
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- 72. Natural History • TA-NRGA-PS –80% mortality in 1st yr – Already restrictive VSD – decreases in size and closes! (Like a PM-VSD!) – Acquired Pulmonary atresia without embryological collaterals – fatal!
- 73. Physical Exam • LikeTOF • No left precordial bulge – RV underdeveloped
- 74. • Pulse – Normal •JVP – Height increased – A prom – PFO/Restrictive ASD/Decr LV compliance – V prom – MR
- 75. • Palpation – IVSalmost vertical and parallel to left sternal border – Systolic LV Impulse (present even if low PBF) – RV Impulse non-palpable
- 76. • Auscultation – SingleS1 (M1) – Single S2 (A2); P2 soft & delayed – maybe heard – LVS4 if unrestrictive VSD/LVH – Obligatory VSD holosystolic murmur • 3rd-4th LICS • Radiates to 2nd LICS • Duration shortens in closing VSD
- 77. ECG • P wave –Tall, Peaked (RAE) • LAD • LVH with adult precordial progression • Counterclockwise depn
- 78. CXR • RAE –prominent rt. Upper border • Small RV – Flat receding rt. Inferior border • LV apex • Prominent Ao/small PA
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- 80. Single ventricle (SV) •1 in 20000 live births • M>F 2-4:1 • 80% SLV • 10% SRV • 10% Indeterminate • SLV OC inverted (left); Noninverted (right) TGA present (Ao from OC; PA from SLV)
- 81. Symptoms/Presentation • Cyanosis sincebirth • Spells and squatting maybe present
- 82. Natural History • SLV50% mortality before 14 yrs • SRV 50% mortality before 4 yrs
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- 84. • Pulse – Normal •JVP – Normal – V prom – if Rt AV valve regurgitant
- 85. • Palpation – SystolicLV Impulse at apex – 3rd LICS – palpable impulse due to inverted OC maybe present
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- 87. ECG • P wave –Tall, Peaked (RAE) • Inverted OC – RAD, Clockwise depn • Noninverted OC – LAD, Counterclockwise depn • Stereotyped rS or RS complexes V2-V5 • Tall R in V1 despite LVH
- 88. CXR • Non-inverted OC •Doesn’t form left border – just like D-TGA • LV apex • Characteristic waterfall appearance of RPA – NOT present in PS – Low PBF
- 89. CXR • Inverted OC •Form left border – just like L-TGA • Maybe visible as convexity on left • Left straight border • LV apex
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- 91. • Incidence acccordingto age and natural history – Age of presentation? – Survival?
- 92. • PRESENTATION – Timingof cyanosis appearance? – Characteristic spells/squatting?
- 93. • Physical Exam –LV or RV predominent?
- 94. • ECG – LAD/RAD –Clock/Counterclock – LVH/RVH – AV Blocks
- 95. • CXR – Lesshelpful – LV/RV apex – Left Straightening