Tokuno Shota, Takeuchi Toshiharu, Nakagawa Naoki
Department of Internal Medicine, Division of Cardiology and Nephrology, Asahikawa Medical University, Asahikawa, Hokkaido, Japan.
Catheter Cardiovasc Interv. 2025 Aug;106(2):1173-1176. doi: 10.1002/ccd.31658. Epub 2025 Jun 4.
A 24-year-old male with Marfan syndrome was admitted following an episode of ventricular fibrillation (VF) during exercise. He had previously undergone multiple aortic surgical procedures for symptomatic aortic regurgitation and an ascending aortic false aneurysm. Ultimately, Bentall procedure and total arch replacement were performed, with a saphenous vein graft (SVG) anastomosed to the right coronary artery. Severe stenosis at the SVG ostium was identified, and ischemia in the inferior wall was suspected to be contributing to VF. Revascularization of the SVG stenosis by percutaneous coronary intervention (PCI) was deemed necessary despite its challenges. After confirming that a 4 Fr Judkins right (JR)-4 guiding catheter via the right femoral artery was suitable, a 6 Fr JR-4 guiding catheter was successfully engaged with the SVG. The SVG stenosis was crossed using a 0.014-in. guidewire, and drug-eluting stent implantation was successfully performed without complications. The patient's left ventricular ejection fraction improved, and no further ventricular arrhythmic events occurred after PCI. Marfan syndrome inherently carries a risk of fatal arrhythmia, and we emphasize that therapeutic management of myocardial ischemia is crucial, even in challenging PCI cases.
一名24岁患有马凡综合征的男性在运动期间发生室颤(VF)后入院。他此前因有症状的主动脉反流和升主动脉假性动脉瘤接受了多次主动脉外科手术。最终,实施了Bentall手术和全弓置换术,并用大隐静脉移植物(SVG)与右冠状动脉吻合。发现SVG开口处严重狭窄,怀疑下壁缺血是导致室颤的原因。尽管存在挑战,但经皮冠状动脉介入治疗(PCI)对SVG狭窄进行血运重建被认为是必要的。确认经右股动脉置入4 Fr Judkins右(JR)-4引导导管合适后,成功将6 Fr JR-4引导导管与SVG对接。使用0.014英寸导丝穿过SVG狭窄部位,成功进行了药物洗脱支架植入,无并发症发生。患者的左心室射血分数提高,PCI术后未再发生室性心律失常事件。马凡综合征本身就有致命性心律失常的风险,我们强调即使在具有挑战性的PCI病例中,心肌缺血的治疗管理也至关重要。