Clinical Characteristics and Long-Term Outcomes of Midaortic Syndrome.

BACKGROUND: Midaortic syndrome (MAS) is a rare congenital or acquired condition marked by segmental or diffuse stenosis of the distal thoracic and/or abdominal aorta and its branches. The optimal approach to medical or interventional management of MAS and long-term outcomes in adults are not well defined. We reviewed MAS cases to characterize the natural history of aortic disease, identify prognostic factors, and evaluate the durability of invasive interventions. METHODS: We conducted a retrospective review of patients with MAS who presented to Memorial Hermann Hospital and Baylor College of Medicine between 1997 and 2018. We categorized cases according to demographic and clinical manifestations, etiologies, the extent of aortic involvement, interventions, and vascular outcomes. RESULTS: We identified a cohort of 13 patients with MAS. The etiology of MAS was identified in 6 cases, including genetic syndromes (neurofibromatosis type 1 (2/13), Williams syndrome (1/13), fibromuscular dysplasia (2/13), and Takayasu arteritis (1/13)). Mean age at first documented clinical event was 25.2 (2-67) years, but cases with genetic etiologies presented significantly younger (18.2 years). The most common primary anatomic site was the suprarenal and infrarenal aorta (zones 5-8). Extra-aortic locations involved the renal (4/13), celiac (3/13), and superior mesenteric (3/13) arteries. Clinical manifestations included hypertension (13/13), claudication (9/13), and postprandial abdominal pain (5/13). All patients with available follow-up data underwent at least one surgical or endovascular intervention (range: 1-8). Postoperative complications included renal failure requiring postdischarge hemodialysis and respiratory failure. There were no deaths in long-term follow-up. CONCLUSIONS: MAS is a complex vasculopathy with substantial variability in clinical presentation and anatomic distribution. Extensive disease frequently requires multiple invasive interventions and results in refractory hypertension, which may predict subsequent clinical events. A multidisciplinary approach with long-term monitoring is essential for preservation of end-organ function and quality of life in this debilitating disease.

A 23-year experience with the reversed elephant trunk technique for staged repair of extensive thoracic aortic aneurysm.

OBJECTIVE: The reversed elephant trunk technique permits staged repair of extensive thoracic aortic aneurysm in patients whose distal (ie, descending thoracic and thoracoabdominal) aorta is symptomatic or disproportionately large compared with their proximal aorta (ie, ascending aorta and transverse aortic arch). We present our 23-year experience with the reversed elephant trunk approach. METHODS: Between 1994 and 2017, 94 patients (median age 62 [46-69] years) underwent stage 1 reversed elephant trunk repair of the distal aorta. Fifty-three patients (56%) had aortic dissection, and 31 patients (33%) had heritable thoracic aortic disease. Eighty-eight operations (94%) were Crawford extent I or II thoracoabdominal aortic repairs. Twenty-seven patients (29%) underwent subsequent stage 2 repair of the proximal aorta; 14 patients (52%) required redo median sternotomy. The median time between the stage 1 and 2 operations was 18.8 (4.8-69.3) months. RESULTS: The operative mortality was 10% (9/94) for stage 1 repairs and 4% (1/27) for stage 2 repairs; 1 patient with heritable thoracic aortic disease died after stage 1 repair (1/31, 3%), and 1 patient died after stage 2 repair (1/13, 8%). Two patients (2%) had ruptures after stage 1 repair; 1 resulted in death, and 1 precipitated emergency stage 2 repair. In total, 36 patients (38%) who survived stage 1 repair died before stage 2 reversed elephant trunk completion repair could be performed. CONCLUSIONS: Managing extensive aortic aneurysm with the 2-stage reversed elephant trunk technique yields acceptable short-term outcomes. This technique is useful for the reversed elephant trunk in patients who require distal aortic repair before proximal repair and is particularly effective in patients with heritable thoracic aortic disease. The low number of patients returning for completion repair is concerning. Rigorous surveillance is needed.