Compressed tetralogy basic course 4-10-13(1)

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  • Figure 4. Palliative surgical shunts. Whenever there is inadequate pulmonary blood flow, palliative shunts may be of value before definitive correction. The sketch shows the classic subclavian artery–to-PA shunt (Blalock-Taussig), the modified Blalock procedure with the use of a tube of polytetrafluoroethylene, the Waterston shunt between the ascending aorta and right PA, and the Potts shunt between the descending aorta and left PA. See text for discussion.
  • Clinical Diagnoses • Repaired tetralogy of Fallot • Hancock pulmonary valve replacement. • Branch pulmonary arterioplasty • Moderate dilation of the ascending aorta (49 mm). • Mild aortic regurgitation (1998). • Right aortic arch. • Prior left Blalock-Taussig shunt.   Clinical Background 47 year old woman born with tetralogy of Fallot. Left BT shunt at age 5. Repair of tetralogy at age 31. Further surgery at age 41 to place a 29 mm Hancock porcine bioprosthesis in the pulmonary position and to occlude a persistently patent left BT shunt. This woman has had substantial exertional dyspnea for the past 3 years. The basis has not been identified despite an extensive workup. Looks well. Mild pectus carinatum. Moderate RV impulse. Soft systolic murmur from the bioprosthesis. Grade 2 murmur of aortic regurgitation and a grade 2 pansystolic murmur of tricuspid regurgitation. Echo highlights - normal RV size and function; mild to moderate TR; pulmonary bioprosthetic function normal with peak gradient 13 mmHg; estimated RVSP 37 mmHg; ascending aorta aneurysmal at 49 mm (stable for several years). Stage I exercise test showed maximal oxygen uptake 18.7 cc/kg/minute with anaerobic threshold at 66% of that value. Cardiac limitation. MRI highlights - RV mild to moderate dilation with ejection fraction 40% (normal 50%). Ascending aorta 50 mm. LPA small (10 mm) with a probable stenosis. Right PA 17 mm and main PA 21 mm.   CXR Dictation This patient, with a history of repair of tetralogy of Fallot, shows normal heart size, mildly asymmetrical (right more than left) pulmonary blood flow, and a right aortic arch. This suggests preferential flow to the right lung. On this occasion, stenosis of the left pulmonary artery should be suspected. There is an opaque ring-like structure in the right ventricular outflow tract, representing a bioprosthetic pulmonary valve. The sternum shows significant anterior bowing (pectus carinatum), and there is mild kyphosis.
  • Clinical Diagnoses • Repaired tetralogy of Fallot • Hancock pulmonary valve replacement. • Branch pulmonary arterioplasty • Moderate dilation of the ascending aorta (49 mm). • Mild aortic regurgitation (1998). • Right aortic arch. • Prior left Blalock-Taussig shunt.   Clinical Background 47 year old woman born with tetralogy of Fallot. Left BT shunt at age 5. Repair of tetralogy at age 31. Further surgery at age 41 to place a 29 mm Hancock porcine bioprosthesis in the pulmonary position and to occlude a persistently patent left BT shunt. This woman has had substantial exertional dyspnea for the past 3 years. The basis has not been identified despite an extensive workup. Looks well. Mild pectus carinatum. Moderate RV impulse. Soft systolic murmur from the bioprosthesis. Grade 2 murmur of aortic regurgitation and a grade 2 pansystolic murmur of tricuspid regurgitation. Echo highlights - normal RV size and function; mild to moderate TR; pulmonary bioprosthetic function normal with peak gradient 13 mmHg; estimated RVSP 37 mmHg; ascending aorta aneurysmal at 49 mm (stable for several years). Stage I exercise test showed maximal oxygen uptake 18.7 cc/kg/minute with anaerobic threshold at 66% of that value. Cardiac limitation. MRI highlights - RV mild to moderate dilation with ejection fraction 40% (normal 50%). Ascending aorta 50 mm. LPA small (10 mm) with a probable stenosis. Right PA 17 mm and main PA 21 mm.   CXR Dictation This patient, with a history of repair of tetralogy of Fallot, shows normal heart size, mildly asymmetrical (right more than left) pulmonary blood flow, and a right aortic arch. This suggests preferential flow to the right lung. On this occasion, stenosis of the left pulmonary artery should be suspected. There is an opaque ring-like structure in the right ventricular outflow tract, representing a bioprosthetic pulmonary valve. The sternum shows significant anterior bowing (pectus carinatum), and there is mild kyphosis.
  • Plot of QRS duration in 182 patients. Those with syncope and ventricular tachycardia (squares), sudden death (triangles), and the single syncopal patient with atrial flutter (star) are plotted separately.
  • Clinical Diagnoses • Repaired tetralogy of Fallot. • Prior left Blalock-Taussig shunt. • Right aortic arch.   Clinical Background 45 year old woman born with tetralogy. Left BT shunt age 5. Tetralogy repair age 11. Ligation of reopened left BT shunt age 21. Asymptomatic. Two healthy children. On exam left radial pulse missing. Grade 2 ESM at the base and grade 2 murmur of low pressure pulmonary regurgitation. Echo highlights - both ventricles show normal size and function. Mitral and tricuspid valves normal. RVSP 29 mmHg. No other abnormality.   CXR Dictation This patient, with repaired tetralogy of Fallot, has mild cardiomegaly, normal pulmonary vascularity, and a right aortic arch. The right upper heart border shows long segment bulging, which suggests dilatation of the ascending aorta. There is scoliosis. Some deformities are seen in the left upper thoracic cage due to a previous left thoracotomy at a time of the Blalock-Taussig shunt.
  • Clinical Diagnoses • Severe residual pulmonary regurgitation. • Ligated ductus arteriosus. • Prior left Blalock-Taussig shunt. • Right aortic arch. • Prior TIA associated with a patent foramen ovale.   Clinical Background 32 year old man. Born with tetralogy. Left BT shunt age 3. Tetralogy repair age 8. Possible TIA age 29 thought possibly due to a paradoxic embolus through a PFO. BP 120/70. Moderate RV lift. Grade 2 ESM at the base. Grade 2 murmur of low pressure pulmonary regurgitation. Echo highlights - left heart normal; moderate to severe pulmonary regurgitation; severe RV enlargement with some hypokinesis; bubble study positive for right to left shunt at atrial level. No arrhythmias. Pulmonary valve implantation will probably be needed.   CXR Dictation This patient, with a history of repaired tetralogy of Fallot using a transannular patch, has only mild cardiomegaly and normal pulmonary vascularity. There is no bulging of the right ventricular outflow tract to suggest an outflow tract patch aneurysm. There is a right aortic arch.
  • Clinical Diagnoses • Repaired tetralogy of Fallot. • Previous tricuspid valve repair. • Residual mild tricuspid stenosis. • Residual moderately severe tricuspid regurgitation. • Residual moderate pulmonary regurgitation. • Chronic atrial fibrillation. • Suture closure of ASD.   Clinical Background This 43 year old man was born with tetralogy of Fallot. He had a primary repair at age 18. He was reoperated at age 27. At that time, LV-RA and LV-RV communications were patched, the tricuspid valve repaired, and residual RV outflow obstruction resected. He did well until he developed paroxysmal atrial fibrillation which eventually became chronic. This, combined with a concurrent embolic event, led to his being on long-term Coumadin. He is asymptomatic from a cardiac standpoint. His main finding is JVP elevation to 10 cm ASA with a slow Y descent. There is a grade 2 low pitched diastolic murmur in the mid left sternal border and a grade 2 pansystolic murmur at the left lower sternal border. Both increase on inspiration. He is on Atenolol and Coumadin. Highlights of the echo: moderate pulmonary regurgitation, RVSP 37 mmHg, a moderately dilated RV with mild hypokinesis, moderate tricuspid regurgitation, and mild tricuspid stenosis with an estimated valve area of 1.7 cm 2 . Both atria are dilated. The left heart is otherwise normal.   CXR Dictation This patient, with repaired tetralogy of Fallot, has moderate cardiomegaly and normal pulmonary vascularity. Cardiomegaly in patients after tetralogy of Fallot repair usually suggests pulmonary regurgitation and/or tricuspid valve regurgitation. In this case, tricuspid stenosis probably has a role in causing right atrial prominence. There is increased kyphosis of the spine.
  • Clinical Diagnoses • Repaired tetralogy of Fallot. • Bioprosthetic pulmonary valve replacement. • Mechanical aortic valve replacement. • Ligation of ductus arteriosus. • Prior Pott's shunt.   Clinical Background 30 year old woman. Began with pulmonary atresia form of tetralogy. PDA ligation age 2. Tetralogy repair including porcine valve age 9. Bioprosthetic aortic valve replacement age 12. St. Jude mechanical aortic valve replacement age 17. Removal of Hancock porcine pulmonary valve age 20. Reinsertion of #27 Hancock porcine valve age 28. No important cardiac symptoms. NYHA Class I-II. Regular treadmill exercise program. Works as assistant bank manager. JVP normal. Grade 2/6 short systolic murmur ULSB. Normal mechanical aortic valve sounds. Echo shows peak pulmonary valve gradient of 15 mmHg. Ascending aorta dilated at 45 mm. No other abnormalities.   CXR Dictation This patient, with repaired tetralogy of Fallot, has mild cardiomegaly, normal pulmonary vascularity and a left aortic arch. An opaque ring of the bioprosthetic pulmonary valve is seen at the expected site of the pulmonary valve. The mechanical aortic valve is faintly visualized at the expected site of the aortic valve. The pulmonary vascularity is normal.
  • Compressed tetralogy basic course 4-10-13(1)

    1. 1. Adult Congenital Heart Disease Basic Teaching Course Tetralogy of Fallot Dr. Gary Webb Cincinnati
    2. 2. Adult Congenital Heart Disease Basic Teaching Course Tetralogy of Fallot Dr. Gary Webb Cincinnati
    3. 3. ISACHDCongenitalHeartDiseaseBasicCourse DDx Cyanotic Congenital Heart Disese 5Ts •Tetralogy. •Transposition. •Tricuspid atresia. •Truncus arteriosus. •Total anomalous pulmonary venous drainage. 2Es •Ebstein anomaly. •Eisenmenger syndrome.
    4. 4. ISACHDCongenitalHeartDiseaseBasicCourse Components of Tetralogy
    5. 5. ISACHDCongenitalHeartDiseaseBasicCourse Pulmonary Atresia Form of Tetralogy
    6. 6. ISACHDCongenitalHeartDiseaseBasicCourse Associated Anomalies in Tetralogy • Right aortic arch (25%). • Consider 22Q11 deletion. • Coronary artery anomalies (5%). • Left anterior descending arises from the proximal right coronary and crosses the right ventricular outflow tract. • Complete AV septal defect.
    7. 7. ISACHDCongenitalHeartDiseaseBasicCourse The Essence of Tetralogy
    8. 8. ISACHDCongenitalHeartDiseaseBasicCourse Pathophysiology of Tetralogy • The degree of cyanosis reflects the severity of RVOT obstruction. • Right to left shunting across the VSD. • Unrepaired patients are prone to “spells”. These are acute drops in arterial saturation due to dynamic RVOT obstruction. The child may squat.
    9. 9. ISACHDCongenitalHeartDiseaseBasicCourse Natural History of Tetralogy • Hypoxia tends to progress early in life. • Survival to adult life is rare without palliation or correction.
    10. 10. ISACHDCongenitalHeartDiseaseBasicCourse Figure 4. Palliative surgical shunts. Bashore T M Circulation 2007;115:1933-1947 Copyright © American Heart Association
    11. 11. ISACHDCongenitalHeartDiseaseBasicCourse
    12. 12. ISACHDCongenitalHeartDiseaseBasicCourse
    13. 13. ISACHDCongenitalHeartDiseaseBasicCourse
    14. 14. ISACHDCongenitalHeartDiseaseBasicCourse Physical Findings in Repaired Tetralogy • Right ventricular lift. • Low-pitched diastolic murmur. • Systolic ejection murmur. • Pansystolic murmur. • High-pitched diastolic murmur.
    15. 15. ISACHDCongenitalHeartDiseaseBasicCourse ECG in Repaired Tetralogy
    16. 16. ISACHDCongenitalHeartDiseaseBasicCourse CXR in Repaired Tetralogy DO112
    17. 17. ISACHDCongenitalHeartDiseaseBasicCourse CXR in Repaired Tetralogy DO112
    18. 18. ISACHDCongenitalHeartDiseaseBasicCourse Echo Findings in Repaired Tetralogy • Moderate to severe pulmonary regurgitation (usual). • RVOT obstruction (unusual). • VSD patch leak (usually small). • RV dilation (due to the PR). • RV systolic dysfunction (late finding). • Significant TR (when RV failing). • Ascending aortic dilation (15%).
    19. 19. ISACHDCongenitalHeartDiseaseBasicCourse MRI in Repaired Tetralogy • Ventricular volumes. • Stroke volumes. • Ejection fractions. • Pulmonary regurgitant fraction.
    20. 20. ISACHDCongenitalHeartDiseaseBasicCourse Davlouros 2001Davlouros 2001 Forward flow in PA Regurgitant flow in PA
    21. 21. ISACHDCongenitalHeartDiseaseBasicCourse MRI in Repaired Tetralogy • Ventricular volumes. • Stroke volumes. • Ejection fractions. • Pulmonary regurgitant fraction. • Pulmonary arterial morphology. • Aortic dilation. • Coronary anomalies. • Aortopulmonary collateral arteries.
    22. 22. ISACHDCongenitalHeartDiseaseBasicCourse Arrhythmias in Repaired Tetralogy • SCD is an issue. • SCD occurs in patients with: • Severe PR. • Ventricular dysfunction. • Past history of cardiac arrhythmias. • ICDs placed for primary and secondary prevention. • Primary prevention if SCD rate estimated at ≥ 4% per year
    23. 23. ISACHDCongenitalHeartDiseaseBasicCourse ICDs in Repaired Tetralogy • Primary prevention in high risk patients. • Left ventricular dysfunction. • Inducible VT. • QRS duration ≥ 180 msecs.
    24. 24. ISACHDCongenitalHeartDiseaseBasicCourse SCD in Congenital Heart Defects • Repaired tetralogy. • Mustard/Senning repairs. • Aortic stenosis. • Aortic coarctation. • Eisenmenger syndrome. • CCTGA. • Ebstein anomaly.
    25. 25. ISACHDCongenitalHeartDiseaseBasicCourse Sustained VT in Repaired Tetralogy • Occurs in about 14% of patients. • Almost always in the setting of severe PR. • Mechano-electric relationships: • Dilated right ventricle. • Irritable right ventricle. • Treatment needed for both the electrical and hemodynamic problems.
    26. 26. ISACHDCongenitalHeartDiseaseBasicCourse Atrial Flutter in Repaired Tetralogy • Occurs in about 20% of patients. • Associated with: • Severe PR. • RV systolic dysfunction. • Moderate-severe TR.
    27. 27. ISACHDCongenitalHeartDiseaseBasicCourse Reintervention After Tetralogy Repair • VSD with > 1.5/1.0 shunt. • RVOT obstruction with an RV systolic pressure > 2/3 systemic. • Severe PR with: • Exercise intolerance; • Sustained arrhythmias; • Severe RV dilation. • Severe aortic regurgitation.
    28. 28. ISACHDCongenitalHeartDiseaseBasicCourse Reintervention After Tetralogy Repair
    29. 29. ISACHDCongenitalHeartDiseaseBasicCourse Reintervention After Tetralogy Repair
    30. 30. ISACHDCongenitalHeartDiseaseBasicCourse Pregnancy After Tetralogy Repair
    31. 31. ISACHDCongenitalHeartDiseaseBasicCourse Closing Remarks re Tetralogy Repair Patients
    32. 32. ISACHDCongenitalHeartDiseaseBasicCourse Tetralogy of Fallot Dr. Gary Webb Cincinnati
    33. 33. ISACHDCongenitalHeartDiseaseBasicCourse Tetralogy of Fallot Dr. Gary Webb Cincinnati
    34. 34. ISACHDCongenitalHeartDiseaseBasicCourse Nollert JACC 1997Nollert JACC 1997 Munich Tetralogy Long-Term Survival
    35. 35. ISACHDCongenitalHeartDiseaseBasicCourse Brickner M et al. N Engl J Med 2000;342:334-342
    36. 36. ISACHDCongenitalHeartDiseaseBasicCourse Source: http://www.nhlbi.nih.gov/health/dci/Diseases/tof/tof_what.html
    37. 37. ISACHDCongenitalHeartDiseaseBasicCourse Tetralogy of Fallot - Apitz, Christian; Webb, Gary D; Redington, Andrew N. Lancet, 10/2009, Volume 374, Issue 9699, pp. 1462 - 1471
    38. 38. ISACHDCongenitalHeartDiseaseBasicCourse PVR and the RV – Toronto 2 Therrien AJC 2005 
    39. 39. ISACHDCongenitalHeartDiseaseBasicCourse Plot of QRS duration in 182 patients. Gatzoulis M A et al. Circulation 1995;92:231-237 Copyright © American Heart Association
    40. 40. ISACHDCongenitalHeartDiseaseBasicCourse From: Becker et al – Fallot’s Tetralogy A Morphometric and Geometric Study. American Journal of Cardiology, Vol 35, p. 402-412, 1974
    41. 41. ISACHDCongenitalHeartDiseaseBasicCourse Segmental duplications: an 'expanding' role in genomic instability and disease. Beverly S. Emanuel & Tamim H. Shaikh Nature Reviews Genetics 2, 791-800 (October 2001)
    42. 42. ISACHDCongenitalHeartDiseaseBasicCourse Park: Pediatric Cardiology for Practitioners, 4th ed., Copyright © 2002 Mosby, Inc..
    43. 43. ISACHDCongenitalHeartDiseaseBasicCourse Park: Pediatric Cardiology for Practitioners, 4th ed., Copyright © 2002 Mosby, Inc..
    44. 44. ISACHDCongenitalHeartDiseaseBasicCourse RVOT Obstruction in Tetralogy • The dominant site of obstruction is subvalvar. • The pulmonary valve is often stenotic as well. • Pulmonary arterial abnormalities. • In some cases the outflow tract is atretic (pulmonary atresia). This form is often associated with aortopulmonary collateral arteries.
    45. 45. ISACHDCongenitalHeartDiseaseBasicCourse Park: Pediatric Cardiology for Practitioners, 4th ed., Copyright © 2002 Mosby, Inc..
    46. 46. ISACHDCongenitalHeartDiseaseBasicCourse Tex Heart Inst J. 1994; 21(4): 272–279. wikipedia
    47. 47. ISACHDCongenitalHeartDiseaseBasicCourse
    48. 48. ISACHDCongenitalHeartDiseaseBasicCourse
    49. 49. ISACHDCongenitalHeartDiseaseBasicCourse Born et al. Visual Analysis of Cardiac 4D MRI Blood Flow Using Line Predicates. IEEE Transactions and Computer Graphics. Vol 19, No 6, June 2013.
    50. 50. ISACHDCongenitalHeartDiseaseBasicCourse Born et al. Visual Analysis of Cardiac 4D MRI Blood Flow Using Line Predicates. IEEE Transactions and Computer Graphics. Vol 19, No 6, June 2013.
    51. 51. ISACHDCongenitalHeartDiseaseBasicCourse Copyright © The American College of Cardiology. All rights reserved. From: Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late? J Am Coll Cardiol. 2000;36(5):1670-1675. doi:10.1016/S0735-1097(00)00930-X Right ventricular (RV) end-systolic volume at rest. Pre-PVR = before pulmonary valve replacement, Post-PVR = after pulmonary valve replacement. Figure Legend:
    52. 52. ISACHDCongenitalHeartDiseaseBasicCourse Date of download: 4/24/2013 Copyright © The American College of Cardiology. All rights reserved. From: Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late? J Am Coll Cardiol. 2000;36(5):1670-1675. doi:10.1016/S0735-1097(00)00930-X Right ventricular (RV) end-diastolic volume at rest. Pre-PVR = before pulmonary valve replacement, Post-PVR = after pulmonary valve replacement. Figure Legend:
    53. 53. ISACHDCongenitalHeartDiseaseBasicCourse Palliative Surgery for Tetralogy • Seldom done any more. • The purpose is to increase pulmonary blood flow and improve oxygenation. • Types: • Blalock/Taussig/Thomas shunt. • Waterston shunt. • Potts shunt. • Central shunt.
    54. 54. ISACHDCongenitalHeartDiseaseBasicCourse Repair of Tetralogy • Alleviation of RVOT obstruction. • Patch closure of VSD. • Variable amount of RVOT and pulmonary arterial patching. • Surgical approach through right atrium and the pulmonary artery. • A few patients need a valved conduit from RV to PA.
    55. 55. ISACHDCongenitalHeartDiseaseBasicCourse Course after Tetralogy Repair
    56. 56. ISACHDCongenitalHeartDiseaseBasicCourse Of patients surviving tetralogy repair, > 90% will live at least 25 years. We have some data beyond that, but treatment keeps changing, and so must our analysis..
    57. 57. ISACHDCongenitalHeartDiseaseBasicCourse Pulmonary regurgitation is overwhelmingly the dominant issue after tetralogy repair!!
    58. 58. ISACHDCongenitalHeartDiseaseBasicCourse Echocardiography is not reliable in assessing the adult right ventricle. MRI is best, with CT angiography a close second when needed.
    59. 59. ISACHDCongenitalHeartDiseaseBasicCourse Ventricular volumes should be indexed. Should they be indexed to BSA or to height?
    60. 60. ISACHDCongenitalHeartDiseaseBasicCourse We need to learn what aspect of right ventricular size and function should drive our decision-making: •RV diastolic volume index? •RV systolic volume index? •RV ejection fraction? •Absolute values or changes? I believe diastolic volume index is most important.
    61. 61. ISACHDCongenitalHeartDiseaseBasicCourse The severity of PR is assessed best by MR, and quite well by echo in skilled hands.
    62. 62. ISACHDCongenitalHeartDiseaseBasicCourse The indications for and impact of pulmonary valve replacement are becoming clearer.
    63. 63. ISACHDCongenitalHeartDiseaseBasicCourse The probability of SCD after TOF repair is ~0.06% annually for the first 10 years, and ~0.2% annually thereafter.
    64. 64. ISACHDCongenitalHeartDiseaseBasicCourse Most repaired TOF patients who die suddenly have had sustained arrhythmias or heart failure.
    65. 65. ISACHDCongenitalHeartDiseaseBasicCourse Repaired TOF patients with sustained VT should have both the arrhythmia and the underlying hemodynamic problems treated.
    66. 66. ISACHDCongenitalHeartDiseaseBasicCourse
    67. 67. ISACHDCongenitalHeartDiseaseBasicCourse Els Pieper’s Suggestion • Text level 2 • Text level 3 • Text level 3 • Text level 2 • Text level 2
    68. 68. ISACHDCongenitalHeartDiseaseBasicCourse Michigan Tetralogy Long-Term Survival 162 survivors Followed 15-26 years (mean 20.2) Late deaths = 9 25 year survival = 94.4% Rosenthal ATS 1984
    69. 69. ISACHDCongenitalHeartDiseaseBasicCourse Hickey EJCTS 2009 Toronto Tetralogy Late Outcomes Inception cohort 1181 children born before 1984 repaired HSC. Follow-up 2003-2006 1965 group outcomes – 1 year survival 72% – 40 year survival 64% 1985 group outcomes – 1 year survival
    70. 70. ISACHDCongenitalHeartDiseaseBasicCourse Hickey EJCTS 2009
    71. 71. ISACHDCongenitalHeartDiseaseBasicCourse Hickey EJCTS 2009 Instantaneous hazard for death: 0.25% annually at 10 years 0.37% 20 0.49% 30 0.59% 40
    72. 72. ISACHDCongenitalHeartDiseaseBasicCourse Munich Tetralogy Long-Term Survival 490 patients surviving at least 1 year Surgery 1958-1977 Follow-up mean 25.3 ± 5.8 yrs Survival – 10 years 97% – 20 years 94% – 30 years 89% Nollert JACC 1997
    73. 73. ISACHDCongenitalHeartDiseaseBasicCourse Repaired Tetralogy and PR #1 Most repaired TOF patients have substantial PR PR is the dominant issue in their surveillance PR is well tolerated by most for at least 20 years In late follow-up to date, a significant minority will die or develop complications
    74. 74. ISACHDCongenitalHeartDiseaseBasicCourse Repaired Tetralogy and PR #2 Most of the deaths and complications occur in patients with substantial PR PR can be eliminated (for a time) by PVR PVR is a low-risk procedure in skilled hands PVR will require re-intervention at a predictable rate over time
    75. 75. ISACHDCongenitalHeartDiseaseBasicCourse Repaired Tetralogy and PR #3 Patients who will not need PVR are those without “laminar PR”, & perhaps with a PR regurgitant fraction of < 20%, or a PR pressure half-time > 100 msec. The other patients should be considered “at risk” of needing PVR at some time
    76. 76. ISACHDCongenitalHeartDiseaseBasicCourse Repaired Tetralogy and PR #4 Indications for the timing of PVR are becoming clearer for patients with moderate-severe PR, and include: – Attributable symptoms – Sustained arrhythmias – Severe RV dilation (?RVEDVi > 150-170 cc/m2 ) – Modulating factors (QRS ≥180 msec, LV dysfunction, inducible VT)
    77. 77. ISACHDCongenitalHeartDiseaseBasicCourse What Can MRI Give Us? RV end-diastolic volume (& index) RV end-systolic volume (& index) RVEF PR fraction (main & branch PAs) LV volumes & LVEF Other data
    78. 78. ISACHDCongenitalHeartDiseaseBasicCourse MRI is Best for the RV
    79. 79. ISACHDCongenitalHeartDiseaseBasicCourse Knauth Heart 2008 Z-score 7 = 172 cc/m2 women & 185 cc/m2 in men
    80. 80. ISACHDCongenitalHeartDiseaseBasicCourse Pressure Half-time is Very Good Silversides JASE 2003 Mild PR
    81. 81. ISACHDCongenitalHeartDiseaseBasicCourse Pressure Half-time is Very Good Silversides JASE 2003 Severe PR
    82. 82. ISACHDCongenitalHeartDiseaseBasicCourse JACC 36; 1670-75; 2000
    83. 83. ISACHDCongenitalHeartDiseaseBasicCourse PVR and the RV – Toronto 1 Conclusion: – RVEF did not improve after PVR Therrien JACC 2000
    84. 84. ISACHDCongenitalHeartDiseaseBasicCourse PVR and the RV - Leiden Vliegen et al JACC 2002
    85. 85. ISACHDCongenitalHeartDiseaseBasicCourse PVR and the RV - Leiden Vliegen et al JACC 2002   
    86. 86. ISACHDCongenitalHeartDiseaseBasicCourse EHJ 2005
    87. 87. ISACHDCongenitalHeartDiseaseBasicCourse The Melody Valve Coats EJCTS 2005
    88. 88. ISACHDCongenitalHeartDiseaseBasicCourse The Melody Valve
    89. 89. ISACHDCongenitalHeartDiseaseBasicCourse Incidence of SCD in Congenital Heart Disease Population-based study 3,600 patients < 19 years Surgery for common CHD Between 1958-1996 41 SCD in total 1/1,118patient-years FU Event rate was lesion specific Silka JACC 1998Silka JACC 1998
    90. 90. ISACHDCongenitalHeartDiseaseBasicCourse Khairy Circulation 117: 363-370; 2008
    91. 91. ISACHDCongenitalHeartDiseaseBasicCourse Khairy Circulation 117: 363-370; 2008
    92. 92. ISACHDCongenitalHeartDiseaseBasicCourse ECG in Repaired Tetralogy
    93. 93. ISACHDCongenitalHeartDiseaseBasicCourse Components of Tetralogy • Ventricular septal defect. • Pulmonary stenosis (right ventricular outflow tract obstruction). • Overriding of the aorta (located above the VSD and partially over the right ventricle). • Right ventricular hypertrophy.

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