Complication | Comments |
Chronic pulmonary regurgitation | - The degree of PR depends in part on the surgical repair.
- Repairs that include a transannular patch result in obligate severe PR.
- Approximately 25 to 35% of patients require PVR by age 25.
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Residual RVOT obstruction | - Branch pulmonary artery stenosis (most frequently LPA stenosis in the region of the ductal insertion) is a common cause.
- Other causes include hypertrophied subvalvar muscle, annular hypoplasia, pulmonary valve stenosis, and supravalvar pulmonary stenosis.
- Mild obstruction is usually well tolerated, but severe obstruction may require reoperation or catheter-based intervention.
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RV dysfunction | - Occurs largely as a consequence of RV dilation from chronic PR.
- The goal of PVR is to improve RV remodeling before severe RV dysfunction develops.
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Aortic root dilation | - Aortic root dilation is seen in 30 to 50% of adult patients with repaired TOF and is usually well tolerated. It can lead to malcoaptation of the aortic valve. Aortic dissection is a rare complication.
- Moderate to severe aortic regurgitation occurs in approximately 3 to 5% of adults with repaired TOF.
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Endocarditis | Factors predisposing to risk of IE among patients with repaired TOF: - Prosthetic valves: Among patients with surgical or transcatheter bioprosthetic pulmonary valves, the risk of IE is highest among those with bovine jugular vein valves.
- Residual defects at or adjacent to the site of prosthetic material.
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ATs | - The prevalence of ATs among adult patients with repaired TOF is approximately 20%; the risk increases considerably after age 45 years.
- Of note, most ATs in this population are wide-complex (with right bundle branch block) regardless of their specific mechanism.
- Risk factors for ATs include multiple prior cardiac operations and older age at repair.
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Ventricular arrhythmias | - The prevalence of ventricular arrhythmias among adult patients with repaired TOF is approximately 10 to 15%.
- Risk factors for ventricular tachycardia include multiple prior cardiac operations, PR, ventricular dysfunction, prolonged QRS duration, and older age at repair.
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SCD | - The risk of SCD in adults with repaired TOF is approximately 2 to 7% over 10 years.
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