Key result
Urgent bovine pericardial patch repair successfully resolves giant LVOT pseudoaneurysm, allowing pregnancy continuation.
Why the study?
Loeys-Dietz syndrome is associated with aortic aneurysms and increased surgical complications, and recognizing LVOT pseudoaneurysm after aortic root surgery is challenging but critical due to rupture risk.
Case Report (n=1)
This case illustrates the critical need for multimodality imaging and shorter surveillance intervals to detect early, life-threatening postoperative pseudoaneurysms in high-risk patients with connective tissue disorders like Loeys-Dietz syndrome.
May support surgical repair to enable pregnancy continuation in Loeys-Dietz syndrome; leaves open optimal surveillance strategies.
Background: Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder associated with aortic aneurysms and increased surgical complications. Recognizing left ventricular outflow tract (LVOT) pseudoaneurysm after aortic root surgery is challenging but is essential given rupture risk. Case summary: A 20-year-old woman with genetically confirmed LDS (TGFBR2-related) and history of elective valve-sparing aortic root replacement for a 4.3 cm aortic aneurysm presented with progressive substernal chest pain and dyspnoea. Transthoracic echocardiography revealed a giant LVOT pseudoaneurysm and mildly reduced left ventricular systolic function. Chest CT angiography confirmed the diagnosis and defined its extent. She underwent urgent surgical repair using a bovine pericardial patch with intraoperative finding of suture-line dehiscence at the aorto-mitral intervalvular fibrosa. Four weeks after redo cardiac surgery, she was found to be 7 weeks pregnant and, after counselling regarding her extremely high-risk status, opted to continue the pregnancy. Discussion: This case illustrates the complexities of surgical intervention in LDS, where tissue fragility and comorbidities increase perioperative risk and complicate management. Close postoperative TTE may enable early detection of LVOT pseudoaneurysm after aortic surgery. A low threshold for chest CTA is key for distinguishing true from pseudoaneurysm. Shorter surveillance intervals may be warranted in high-risk patients with risk factors such as tissue fragility, complicated postoperative course, or chest wall deformity.
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Rice et al. (2026) conducted a case report in Loeys-Dietz syndrome with giant left ventricular outflow tract pseudoaneurysm (n=1). Urgent surgical repair with bovine pericardial patch was evaluated on Resolution of pseudoaneurysm and clinical recovery. Urgent surgical repair using a bovine pericardial patch successfully resolved the giant LVOT pseudoaneurysm, allowing the patient to achieve functional improvement and continue her pregnancy.
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