Total Arch Replacement with Stented Elephant Trunk Technique for Syphilitic Thoracic Aortic Aneurysm
Syphilitic aortic aneurysm, a tertiary manifestation of untreated Treponema pallidum infection, has become exceedingly rare in the modern antibiotic era. Historically, cardiovascular syphilis manifested 10–30 years after primary infection, characterized by obliterative endarteritis of the vasa vasorum, leading to medial necrosis, fibrosis, and aneurysm formation. These aneurysms predominantly involve the tubular ascending aorta, aortic arch, and descending thoracic aorta, sparing the aortic root and sinuses of Valsalva. Despite its rarity, syphilitic thoracic aortic aneurysms carry a high mortality risk, necessitating timely surgical intervention. This article details the application of total arch replacement combined with the stented elephant trunk technique in three patients with syphilitic aortic aneurysms involving the aortic arch and proximal descending aorta, highlighting the procedural rationale, technical execution, and clinical outcomes.
Clinical Presentation and Diagnostic Evaluation
Three male patients (aged 53, 64, and 67 years) presented with symptoms of hoarseness or progressive dyspnea, attributed to mechanical compression by large saccular thoracic aortic aneurysms. All patients had a history of hypertension but no prior diagnosis or symptoms of syphilis. Preoperative computed tomographic angiography (CTA) revealed aneurysmal involvement of the aortic arch and proximal descending aorta, with ascending aortic ulcers observed in two cases [Figure 1A, 1B]. The aneurysms measured 6.5 cm, 7.2 cm, and 5.8 cm in maximal diameter, respectively, causing compression of adjacent structures: the recurrent laryngeal nerve (hoarseness), trachea, and left lung (dyspnea). Cardiac evaluations ruled out aortic root dilation, aortic valve insufficiency, and coronary artery disease.
Serological testing confirmed syphilitic infection: rapid plasma reagin (RPR) titers were 1:64 in all cases, and fluorescent treponemal antibody absorption tests were positive. A diagnosis of tertiary syphilis with cardiovascular involvement was established. Preoperative management included a two-week course of intravenous penicillin to target active infection, supplemented with oral prednisone to mitigate potential Jarisch-Herxheimer reactions.
Surgical Technique
The surgical approach prioritized complete resection of diseased aortic segments and prevention of residual aneurysm progression. Total arch replacement with a tetrafurcate graft and stented elephant trunk implantation was performed via median sternotomy under cardiopulmonary bypass (CPB) and selective cerebral perfusion (SCP).
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Cannulation and Perfusion Strategy:
The right axillary artery was cannulated for CPB and SCP, while right atrial cannulation facilitated venous return. CPB was initiated with systemic cooling to a nasopharyngeal temperature of 27°C before establishing circulatory arrest. -
Aortic Resection and Arch Reconstruction:
The ascending aorta and aortic arch were extensively resected, preserving the aortic root and sinotubular junction. The distal aortic arch was transected proximal to the aneurysm, and the tetrafurcate graft (28–30 mm diameter) was anastomosed to the ascending aorta. In cases where the aneurysm involved the left subclavian or left common carotid arteries, these vessels were reconstructed using graft branches. -
Stented Elephant Trunk Deployment:
A 10-cm stented graft (MicroPort Medical Co Ltd, Shanghai, China) was inserted into the descending aorta through the open distal arch. The stent graft, constrained during insertion, was deployed by releasing circumferential sutures, ensuring its distal end anchored in a normal aortic segment. The proximal stent edge, reinforced with a 1-cm polyester graft extension, was sutured to the tetrafurcate graft, creating a seamless transition [Figure 1C, 1D]. -
Pathological Findings:
Histopathological examination of resected aortic tissue revealed lymphoplasmacytic infiltration and medial destruction, consistent with syphilitic aortitis.
Postoperative Outcomes
All patients recovered without major complications. There were no instances of stroke, spinal cord ischemia (paraparesis), visceral malperfusion, or lower extremity dysfunction. Postoperative CTA confirmed complete aneurysm exclusion, with no evidence of endoleak or residual flow into the sac [Figure 1E]. Follow-up serological testing guided additional antibiotic therapy, though no disease recurrence was observed during a median follow-up period of 18 months.
Discussion
The stented elephant trunk technique, originally developed for type A aortic dissection, was adapted here to address the unique challenges of syphilitic aneurysms. Key advantages include:
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Comprehensive Disease Eradication:
By replacing the ascending aorta and arch while excluding the descending aneurysm, this approach eliminates pathological aortic segments prone to rupture. Traditional open repair risks excessive bleeding due to friable, inflamed tissues; the stented elephant trunk circumvents this by avoiding direct aneurysm incision. -
Simplified Staging and Reduced Risk:
Conventional elephant trunk techniques require a second-stage procedure to address the descending aorta, during which patients remain at risk for aneurysm rupture. The integrated stented graft provides immediate stabilization, negating the need for interval surgery. -
Hybrid Approach Limitations:
Endovascular repair, while minimally invasive, is contraindicated in syphilitic aneurysms involving the ascending aorta or arch due to pathological proximal landing zones. Hybrid procedures (e.g., debranching with stent grafting) also risk retrograde dissection and spinal ischemia. -
Durability and Anatomic Suitability:
The rigid stent graft resists compression, ensuring long-term patency. Its design accommodates tortuous anatomy, with the 10-cm length providing sufficient overlap in the descending aorta.
Conclusion
Total arch replacement with the stented elephant trunk technique offers a definitive, single-stage solution for syphilitic thoracic aortic aneurysms involving the arch and proximal descending aorta. The procedure combines the durability of open surgery with the stability of endovascular exclusion, addressing the inherent weaknesses of inflamed aortic tissues. Preoperative antibiotic therapy and meticulous surgical planning are critical to optimizing outcomes in this rare but life-threatening condition.
doi: 10.1097/CM9.0000000000000993
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