Abstract
Background
Frozen elephant trunk (FET) implantation is an established technique for acute Type A aortic dissection (ATAAD), promoting true lumen expansion and aortic remodeling. However, in patients with preoperative false lumen perfusion, FET carries a significant risk of postoperative false lumen thrombosis that may acutely obstruct branch vessel flow and trigger malperfusion syndrome.
Case presentation
A 74-year-old hypertensive female presented with acute chest pain. Computed tomography angiography confirmed ATAAD with false lumen perfusion supplying all visceral arteries. Emergency ascending aorta replacement, total arch replacement, and FET implantation were performed. The patient was discharged on postoperative day twelve with therapeutic aspirin. Six days post-discharge, she developed fatal visceral malperfusion syndrome secondary to superior mesenteric artery thrombosis originating from false lumen occlusion.
Conclusions
FET implantation in ATAAD patients with false lumen perfusion may precipitate catastrophic thrombosis despite antiplatelet. Surgical strategy selection between traditional elephant trunk and FET techniques must rigorously evaluate individual anatomy and perfusion patterns.
Supplementary Information
The online version contains supplementary material available at 10.1186/s13019-025-03812-0.
Keywords: Type A aortic dissection, Malperfusion syndrome, Frozen elephant trunk stent
Background
Delayed intestinal ischemia following central aortic repair for acute type A aortic dissection (ATAAD) is exceedingly rare, with an incidence of 1% to 3%.1–3 We present a rare case of a 74-year-old female diagnosed with ATAAD, who unfortunately passed away postoperatively due to visceral malperfusion syndrome, manifested by thrombosis within the false lumen of the superior mesenteric artery, resulting in bowel ischemia and infarction.
Case presentation
A 74-year-old female patient with a history of hypertension was admitted to the emergency department with a sudden onset of chest pain lasting 10 hours. Aortic computed tomography angiography (CTA) revealed Type A aortic dissection, with the primary intimal tear on the right lateral aspect of the ascending aorta, extending to the iliac arteries. The celiac trunk (Fig. 1A), superior mesenteric artery (Fig. 2A), left renal artery, and inferior mesenteric artery were all perfused by the false lumen (Supplementary material). Plasma lactate levels were mildly elevated, measuring 1.65 mmol/L. Echocardiography suggested mild to moderate aortic valve regurgitation.
Fig. 1.
(A) Pre-operative, (B) Day 7 post-operative, and (C) Day 18 post-operative of the celiac trunk. Arrows indicate the opening of the celiac trunk
Fig. 2.
(A) Pre-operative, (B) Day 7 post-operative, and (C) Day 18 post-operative of the superior mesenteric artery. Arrows indicate the opening of the superior mesenteric artery
The patient urgently underwent ascending aorta replacement, total arch replacement, and frozen elephant trunk (FET) implantation in the operating room. Intraoperative inspection revealed a maximum ascending aortic diameter of 5.0 cm, and the primary entry tear was identified on the anterior aspect of the ascending aorta. After hypothermic cardiopulmonary bypass, the affected ascending aorta and aortic arch were resected. Upon cooling to 28 °C, unilateral antegrade cerebral perfusion was initiated via the brachiocephalic trunk. Subsequently, a 28 × 100 mm FET was implanted, and the 28-mm four-branch graft was anastomosed to the corresponding great vessels. Cardiopulmonary bypass time was 151 min, and aortic cross-clamp time was 97 min. Postoperatively, the patient recovered well and extubated on the second day. Throughout the postoperative period, peripheral blood pressure was strictly controlled within the range of 110–130/60–80 mmHg. A follow-up CTA on postoperative day seven showed thrombosis at the false lumen supply of the celiac trunk, causing ostium narrowing (Fig. 1B), while the superior mesenteric artery and inferior mesenteric artery showed patent blood flow through the false lumen (Fig. 2B, Supplementary material). The patient was discharged on postoperative day twelve with a prescription for aspirin 100 mg daily for antiplatelet.
Six days after discharge, the patient presented with sudden abdominal pain, profuse sweating, and persistent nausea. She was admitted to our emergency department. The repeat CTA confirmed thrombosis at the origin of the celiac trunk and occlusion of the superior mesenteric artery (Figs. 1C and 2C, and Supplementary material). Laboratory tests revealed severe metabolic acidosis accompanied by a lactate level of 21.6 mmol/L and a D-dimer level exceeding 20 mg/L FEU. Besides, a mild elevation in both direct and indirect bilirubin was also observed. The remaining cardiac and digestive enzymes were within normal limits. Emergency percutaneous balloon angioplasty of the superior mesenteric artery was attempted; however, the patient’s condition continued to deteriorate, with severe visceral malperfusion syndrome unrelieved, ultimately leading to death due to circulatory failure.
Discussion
This case report presents a challenging scenario of ATAAD with false lumen perfusion. The use of frozen elephant trunk (FET) stent graft is controversial in such cases. While FET implantation facilitates true lumen opening, enhances distal true lumen perfusion, and stimulates proximal descending aortic remodeling,4 the risks of thrombosis and visceral ischemia are significant, especially when the false lumen perfuses vital organs. Our patient’s outcome highlights the potential complications and warrants further evaluation of this technique in patients with false lumen perfusion.
In our reported ATAAD case, the celiac trunk, superior mesenteric artery, left renal artery, and inferior mesenteric artery were perfused by the false lumen preoperatively. Post-FET implantation, the true lumen was reestablished and the false lumen gradually thrombosed. Thrombosis at the false lumen ostium of the celiac trunk caused severe stenosis at discharge. Despite aspirin therapy, thrombosis developed at the superior mesenteric artery on post-discharge day six, leading to malperfusion syndrome. The patient succumbed to refractory circulatory failure due to progressive multi-organ dysfunction secondary to persistent malperfusion syndrome, despite aggressive resuscitation efforts.
We posit that the FET stent may not be appropriate for ATAAD patients whose distal organs are predominantly perfused by the false lumen. Thrombosis within the false lumen may lead to acute obstruction of blood flow, potentially inducing malperfusion syndrome. While the FET stent graft offers a one-stage repair solution, it may not be suitable for all patients, particularly those with significant false lumen perfusion. Therefore, the risk of thrombosis and consequent visceral ischemia constitutes a considerable concern. In patients with visceral dependence on false lumen perfusion, FET implantation and subsequent true lumen expansion may precipitate false lumen thrombosis. This thrombus can potentially occlude the origins of visceral arteries, thereby inducing visceral ischemia. To preserve visceral perfusion and mitigate this thrombotic risk, the traditional elephant trunk technique is recommended. This technique, which involves opening the intimal membrane of dissection and implanting a soft elephant trunk, is designed to maintain patency of both lumens and thereby ensure perfusion to distal organs. In conclusion, the choice between the conventional elephant trunk and FET techniques should be guided by the patient’s specific anatomy, comorbidities, and surgical team expertise.
Supplementary Information
Below is the link to the electronic supplementary material.
Supplementary Material 1: Video 1 Computed tomography angiography of the abdominal aorta obtained preoperatively, postoperatively, and during the readmission.
Acknowledgements
None.
Abbreviations
- ATAAD
Acute type A aortic dissection
- CTA
Computed tomography angiography
- FET
Frozen elephant trunk
Author contributions
HZ and LG carried out the studies, participated in collecting data, and drafted the manuscript. HZ and HG participated in its design. All authors read and approved the final manuscript.
Funding
None.
Data availability
The datasets used during the current study are available from the corresponding author on reasonable request.
Declarations
Consent for publication
Not applicable.
Competing interests
The authors declare no competing interests.
Footnotes
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Huai-Yu Zhao and Li-Xi Gan contributed equally to this work.
References
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Supplementary Material 1: Video 1 Computed tomography angiography of the abdominal aorta obtained preoperatively, postoperatively, and during the readmission.
Data Availability Statement
The datasets used during the current study are available from the corresponding author on reasonable request.