A Case Report of Giant Left Ventricular Outflow Tract Pseudoaneurysm in a Pregnant Woman with Loeys-Dietz Syndrome: A Management Conundrum

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ID: 321196
2026
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Abstract
Abstract 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 Loeys-Dietz syndrome (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 dyspnea. Transthoracic echocardiography revealed a giant left ventricular outflow tract (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 seven weeks pregnant and, after counseling 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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Authors James Rice, Esosa Odigie-Okon
Journal European Heart Journal - Case Reports
Year 2026
DOI
10.1093/ehjcr/ytag490
URL
https://doi.org/10.1093/ehjcr/ytag490
Keywords Keywords not found

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