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Unexpected Aortic Events In Asymptomatic Turner Syndrome Patients
Milan Jaiswal, BA, Sara Mansoorshahi, BS, Siddharth Prakash, MD, PhD, Rana Afifi, MD.
UTHealth Houston McGovern Medical School, Houston, TX, USA.
DEMOGRAPHICS:Case 1: A 51-year-old female Turner Syndrome (TS), thoracic aortic aneurysm, bicuspid aortic valve, and history of coarctation status-post repair.Case 2: A 70-year-old female with TS, bicuspid aortic valve, and history of coarctation status-post repair.
HISTORY:Case 1:Patient was seen in clinic for routine follow-up. She had been active and healthy her entire adult life and was asymptomatic. Routine surveillance imaging detected no aortic changes over the previous five years. A routine MRI incidentally detected a Type A aortic dissection of the ascending aorta with a maximum diameter of 3.6 cm. Patient was asymptomatic and summoned to the hospital. Case 2:Patient was seen in clinic for cardiovascular evaluation. She had been active and healthy her entire adult life and was asymptomatic. Routine surveillance imaging detected no aortic changes over the previous four years. The next MRA showed a new dissection and pseudoaneurysm involving the distal aortic arch and proximal descending thoracic aorta.
PLAN:Case 1:Patient underwent uncomplicated replacement of the ascending aorta and transverse arch. On post-op day 6, she developed atrial fibrillation with rapid ventricular response and acute decompensation due to pulmonary edema. After rhythm control and diuresis, she was discharged on post-op day 15 with anticoagulation and had recovered to baseline status at one-month follow-up.Case 2:Patient underwent elective thoracic endovascular aortic repair. Completion angiography detected a Type A dissection that was managed using a Gore extension cuff stent graft. The intraoperative TEE showed a new peri-aortic intramural hematoma. Due to her fragile pulmonary status and hemodynamic stability, the lesion was observed closely. After imaging was stable, she was discharged on post-op day 9 with amiodarone, metoprolol, and furosemide. The following month, CTA revealed new dissections in the descending thoracic aorta and proximal to the extension graft, which are being closely monitored (Figure 1).
DISCUSSION:These cases feature individuals with TS who presented for acute aortic pathologies decades after their initial cardiovascular diagnoses. Both patients denied cardiovascular symptoms, even as they developed rapidly evolving aortic lesions. These cases illustrate how lifelong cardiovascular surveillance of TS patients can save lives because aortic disease, especially in individuals with left-sided obstructive lesions, can escalate suddenly.
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