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Complete Transposition of the Great Arteries - Follow up Le Kim Tuyen, MD Transposition of the Great Arteries Repaired by the Mustard or Senning Procedures • 15%, will develop overt heart failure in late follow-up • More than mild systemic tricuspid regurgitation may be present in 10–40% • Atrial flutter occurs in 20% of patients by age 20, and evidence of sinus node dysfunction is seen in about 50% in the adult age group Transposition of the Great Arteries Repaired by the Mustard or Senning Procedures (cont.) • Shortened life expectancy takes the form of 70– 80% survival at 20- to 30-year follow-up. • Regular echocardiography in skilled hands can qualitatively assess systemic right ventricular function, the degree of systemic tricuspid regurgitation, the presence or absence of subpulmonary left ventricular outflow tract obstruction, as well as baffle leaks or obstruction. Transposition of the Great Arteries Repaired by the Mustard or Senning Procedures (cont.) • because echocardiography may not accurately assess right ventricular systolic function or acoustic access may be limited, periodic MRI examinations may be of great value. • If the patient has a pacemaker or other contraindication to MRI, computerized tomographic angiography may also be very helpful in assessing the systemic right ventricle. Transposition of the Great Arteries Repaired by the Mustard or Senning Procedures (cont.) Transposition of the Great Arteries Repaired by the Mustard or Senning Procedures (cont.) Transposition of the Great Arteries Repaired by Arterial Switch • The anatomic pulmonary valve must serve in the systemic circulation lifelong, and there are accumulating data on progressive neoaortic valve dysfunction and root dilation during the second and third decades of follow-up. • Following the Lecompte maneuver both pulmonary arteries are anterior to the ascending aorta; posterior tension can cause further distortion of the neoaortic root and/or cause supravalvar pulmonary stenosis. Transposition of the Great Arteries Repaired by Arterial Switch (cont.) 11.7.1. Problems and Pitfalls The following are potential problems and pitfalls related to adults with d-TGA: ● Antiarrhythmic therapy, which might aggravate sinus node dysfunction in patients after atrial baffle operation, must be used cautiously. ● A detailed assessment of the atrial baffle for leak and obstruction must be undertaken before endocardial pacemaker implantation. ● There is potential for endocardial pacing leads to exacerbate obstruction in the atrial baffle. ● The absence of typical symptoms of coronary ischemia does not preclude the presence of important ostial coronary artery disease in patients with prior ASO. 11.8.1. Medical Therapy The role of medical treatment (eg, ACE inhibitors and beta blockers) to prevent or treat ventricular dysfunction has only been studied in small numbers, and its benefit is controversial The role of ACE inhibitors and beta blockers remains uncertain, and beta blockers may precipitate complete AV block in patients with preexisting sinus node dysfunction. Therapy for heart failure now incorporates medications directed at the renin-angiotensin-aldosterone system. 11.8.2. Recommendations for Interventional Catheterization for Adults With DextroTransposition of the Great Arteries CLASS IIa 1. Interventional catheterization of the adult with d-TGA can be performed in centers with expertise in the catheterization and management of ACHD patients. (Level of Evidence: C) 2. For adults with d-TGA after atrial baffle procedure (Mustard or Senning), interventional catheterization can be beneficial to assist in the following: a. Occlusion of baffle leak. (Level of Evidence: B) b. Dilation or stenting of superior vena cava or inferior vena cava pathway obstruction. (Level of Evidence: B) c. Dilation or stenting of pulmonary venous pathway obstruction.(Level of Evidence: B) 11.8.2. Recommendations for Interventional Catheterization for Adults With DextroTransposition of the Great Arteries (cont.) 3. For adults with d-TGA after ASO, interventional catheterization can be beneficial to assist in dilation or stenting of supravalvular and branch pulmonary artery stenosis. (Level of Evidence: B) 4. For adults with d-TGA, VSD, and PS, after Rastelli-type repair, interventional catheterization can be beneficial to assist in the following: a. Dilation with or without stent implantation of conduit obstruction (RV pressure greater than 50% of systemic levels, or peak-to-peak gradient greater than 30 mm Hg; these indications may be lessened in the setting of RV dysfunction). (Level of Evidence: C) b. Device closure of residual VSD. (Level of Evidence: C) 11.8.3. Recommendations for Surgical Interventions 11.8.3. Recommendations for Surgical Interventions 11.8.3. Recommendations for Surgical Interventions 11.8.3. Recommendations for Surgical Interventions
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