Print this pageSevere Hypertension In A Teenager With Williams's Syndrome
Brian Nolan, Anthony DiScipio, Richard Powell
Dartmouth Hitchcock Medical Center, Lebanon, NH
A 17 year old female with Williams's syndrome presented 4 years previously with difficult to control hypertension on 4 medications. At that time she was referred to Interventional Radiology and found to have a thoracoabdominal coarctation and bilateral renal artery stenosis for which she underwent bilateral renal stent placement with improvement in her blood pressure. Her hypertension however recurred and she subsequently developed severe left ventricular hypertrophy. Repeat arteriography demonstrated a mild supravalvular aortic stenosis, coarctation of her thoracic aorta beginning at the level of the left subclavian artery extending to the para-renal aorta and bilateral in-stent recurrent renal artery stenosis. Spiral 3-D computed tomography scans confirmed these findings and showed the lumenal diameter of the para-renal aorta to be 2-3 mm. Physical examination she was an obese young female with absent femoral and pedal pulses. To treat this, the patient underwent ascending aorta to infrarenal aortic bypass using a 16 mm tube graft that was tunneled inferior to the heart, through the left lateral diaghram, and the left retroperitoneum. Bilateral renal bypasses were performed using a 12X7 bifurcated Dacron graft originating from the aortic tube graft. This procedure was performed through a combined median sternotomy and midline abdominal incision. Her postoperative course was complicated by a right jugular deep vein thrombosis and pulmonary embolism that required coumadin. One year post-operatively her blood pressure is well controlled on 2 medications and she has palpable femoral pulses. Repeat arteriography at 4 months demonstrated that all grafts were patent. Williams's syndrome patients are known to have aortic and visceral arterial stenosis. This case is unique in the extensive nature of her aortic stenosis. This is the first reported case of ascending aorta to infrarenal aortic bypass to treat such a condition.