日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Transthoracic echocardiography (TTE) is essential for detecting macrovascular lesions such as aortic dilatation, aortic regurgitation, and mitral valve deviation in Marfan syndrome. However, visualizing the aortic valve can be challenging due to scoliosis or other thoracic abnormalities. We report a case involving a 15-year-old female patient with severe postoperative aortic valve stenosis that was undiagnosed by TTE prior to her emergency admission. She had undergone aortic root replacement via the Bentall procedure using a bioprosthetic valve at the age of 11. Although she was followed up with TTE in the outpatient department, her scoliosis complicated the imaging process. The patient presented to the emergency department with a respiratory disorder of one-week duration and subsequently underwent emergency reoperation for severe aortic stenosis caused by calcification of the bioprosthetic valve. In patients with Marfan syndrome, scoliosis may result in poor TTE imaging results, making additional evaluations with magnetic resonance imaging, computed tomography, or transesophageal echocardiography necessary. Moreover, patients with Marfan syndrome require close follow-up because elastin-derived proteins may contribute to the calcification of the bioprosthetic valve, resulting in early valve dysfunction.