Table 4 Outcomes of children who underwent surgical intervention for medication-refractory Takayasu Arteritis
Patient | Surgical procedure (s) | Indication (s) | Outcome (s) |
|---|---|---|---|
1 | -Ross procedure | -Severe aortic regurgitation on echocardiogram; diplopia; symptoms of heart failure | -Improvement in symptoms; mild aortic narrowing on MRA ~6 months post-op; annulo-aortic ectasia with enlargement of root and ascending aorta per CTA ~2 years post op; mild aortic regurgitation on echocardiogram ~3 years post-op |
-Pulmonary artery stent placement | -Severe pulmonary artery narrowing | -No significant stenosis of the main or central pulmonary arteries ~1 year and ~2 years post op per CTA; diastolic flow reversal in the proximal pulmonary artery branches suggestive of free pulmonary insufficiency on echocardiogram ~2 years post-op | |
2 | -Replacement of ascending aorta, aortic arch, and proximal descending aorta with elephant trunk procedure in the descending aorta | -Severe lower extremity claudication, saccular aneurysm of the distal arch and aortic isthmus per MRA | -No improvement of lower extremity claudication; stable appearance of graft ~6 months post op |
-Abdominal aortic aneurysm resection, infrarenal graft, and IMA revascularization | -SMA occlusion, infrarenal stenosis per MRA | -Resolution of lower extremity claudciation ~1 year post op; intact infrarenal graft, proximal superior SMA occlusion, reimplanted inferior mesenteric artery appears moderately narrowed at the anastomosis on MRA ~ 1 year post op | |
3 | -Right renal artery angioplasty | -Severe hyptertension; headache; severe right renal artery stenosis per MRA | -Improvement in hypertension and headaches; right renal artery stenosis per MRA and ultrasound ~2 months post op; widely patent right renal artery ~4 years post op after medicinal treatment with methotrexate |
4 | -Bilateral renal artery angioplasty | -Malignant hypertension requiring IV anti-hypertensive drip | -Initial improvement of blood pressure, but 9 days later developed worsening hypertension and fatigue |
-Right nephrectomy and left kidney aorto-renal bypass with the left saphenous vein | -Severe hypertension again requiring IV anti-hypertensive drip and fatigue | -Improved hypertension; Mild stenosis of left renal artery 3 days post op per renal ultrasound; MRA 5 days post op with left renal artery occlusion in its mid segment with patent bypass graft | |
5 | -Renal artery angioplasty | -Renal arteries and SMA occlusion per CTA | -Improved hypertension |
-Right and left renal arteries and SMA bypass | -Severe hypertension again requiring IV anti-hypertensive drip; bilateral severe renal artery stenosis on CTA | -Further improvement of hypertension; MRA ~1 week post op with patent SMA graft with occlusion at ostium, patent right renal artery, patent left renal artery bypass with occlusion at ostium; ~1 year post op CTA with patent left renal artery bypass, mild stenosis of right renal artery, SMA occluded but asymptomatic | |
6 | -Left renal artery stent | -Severe left renal artery stenosis per CTA; hypertenstion | -Improvement in hypertension; MRA ~6 months post-op with patent bilateral renal arteries; patent on MRA ~3 years post op |
7 | -Right nephrectomy | -Severe renal artery stenosis; hypertension | -Improvement of hypertension |
8 | -Replacement of aortic arch and ascending aorta | -Aneurysmal dilatation of the ascending thoracic aorta, complete occlusion of the proximal left subclavian artery per MRA | -2 days post op CTA showed intact graft; MRA ~1 year post op with stable aortic dimensions; MRA ~3 years post op with thickening to the aortic arch and interval development of thickening and dilation of the descending aorta; patient has remained on infliximab since surgery |