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.
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.
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
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.
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.
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.
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.
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
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. 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. 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. 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)
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
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.
Editor's Notes
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.
