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THE ADULT WITH REPAIRED COARCTATION OF THE AORTA

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PATIENT STORY

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A 48-year-old woman with coarctation of the aorta, ventricular septal defect (VSD), and bicuspid aortic stenosis was followed with progressive aortic root dilation and severe aortic regurgitation. She also had moderate aortic valvular stenosis and underwent aortic valve and root replacement with a 22-mm homograft and reimplantation of the coronary arteries in 2002 at 38 years of age. This operation was complicated by right brachial artery occlusion requiring surgical bypass graft. She also developed third-degree heart block and underwent dual-chamber transvenous pacemaker placement. In the distant past, she had undergone initial repair of her coarctation and VSD closure at age 14 months in 1965 (coarctation resection with end-to-end anastomosis, required reoperation for residual coarctation in 1973 at 9 years of age). She had developed systemic hypertension and was managed with dual therapy using a beta-blocker and angiotensin-converting enzyme (ACE) inhibitor. She continued to have frequent headaches, fatigue with exertion, bilateral lower extremity pain, and fatigue with ambulation.

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On physical examination, the patient had evidence of right brachial to left femoral pulse delay, the right femoral pulse was only faintly palpable. A 2/6 mid-systolic murmur was auscultated at the right upper sternal border. A soft systolic murmur was heard over the left scapula, no continuous murmurs or diastolic murmurs were appreciated. An S4 gallop was auscultated over the apex.

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A stress echocardiogram in 2009 demonstrated a peak resting instantaneous gradient of 63 mm Hg, mean 32 mm Hg across the coarctation site, increased to PIG of 132 mm Hg with exercise. She had poor exercise capacity, went 6 minutes on a Bruce protocol with right upper extremity (RUE) blood pressure (BP) rising to greater than 200 mm Hg systolic, lower extremity exercise BP could not be measured. The decision was made to proceed with invasive catheterization to further assess anatomy and hemodynamics and to perform palliative intervention if deemed feasible and necessary.

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INTERVENTIONAL CARDIAC CATHETERIZATION: CASE EXPLANATION
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  • The patient was brought to the catheterization laboratory and placed under general anesthesia in expectation of possible trans-catheter intervention. The right femoral pulse was faintly palpable, evaluation with ultrasound suggested an obstructed right femoral artery. The left femoral arterial pulse was palpated and a 6-French (F) sheath was placed under fluoroscopic and ultrasound guidance using the modified Seldinger technique.

  • Hemodynamic evaluation demonstrated a minimal gradient across the aortic homograft, an elevated left ventricular end-diastolic pressure (LVEDP) of 20 mm Hg, and a peak-to-peak gradient of 40 mm Hg across a focal area of re-coarctation in the proximal descending aorta (Figure 14-1).

  • Angiography demonstrated a focal narrowing at the site of prior coarctation repair, the transverse aorta, and descending aorta at the diaphragm measured 17 mm in diameter, the area of stenosis measured 8 mm in minimum diameter. A left femoral angiogram demonstrated complete occlusion of the left femoral ...

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