Zhou Ruihai, Yeung Michael, Sharma Mahesh S
Division of Cardiology, Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7075, USA.
Department of Cardiothoracic Surgery, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7075, USA.
Eur Heart J Case Rep. 2022 Jul 23;6(8):ytac308. doi: 10.1093/ehjcr/ytac308. eCollection 2022 Aug.
Ruptured sinus of Valsalva (SOV) is a rare cardiac anomaly with poor prognosis if untreated. Early diagnosis with accurate delineation of its anatomy is critical for timely treatment and choice of surgical vs. percutaneous intervention. Here we report a case of fistulous rupture of SOV; the preoperative multimodality studies including echocardiography, cardiac magnetic resonance and cardiac catheterization provided teaching and learning points.
A 48-year-old man with history of heart murmur and hypertension presented with a 5-day history of shortness of breath and peripheral oedema. He was diagnosed with rapid atrial flutter. The transthoracic and transesophageal echocardiography showed severe biventricular systolic dysfunction with a left-to-right shunt from ruptured SOV. The colour Doppler by transthoracic and transesophageal echocardiography and cardiac magnetic resonance revealed a swaying shunt flow exiting in direction to the right atrium (RA) and basal right ventricle (RV) during systole and diastole with no myocardial scaring. The left and right heart catheterization showed elevated right-sided pressures, pulmonary capillary wedge pressure, and left ventricular end-diastolic pressure. There was no difference in O2 saturation between venae cavae and RA but a misleading step-up in O2 saturation between RA and RV. Owing to rupture anatomy with uncertainty, the patient underwent surgical intervention. The ruptured SOV tunnelled through the base of tricuspid annulus to the RA very close to the basal RV.
Even with multimodality studies it can still be challenging to delineate the anatomy of a ruptured SOV without uncertainty preoperatively.
瓦氏窦瘤破裂（SOV）是一种罕见的心脏异常疾病，若不治疗，预后较差。早期诊断并准确描绘其解剖结构对于及时治疗以及选择手术或经皮介入治疗至关重要。在此，我们报告一例SOV瘘管破裂病例；术前的多模态研究，包括超声心动图、心脏磁共振成像和心导管检查，提供了教学和学习要点。
一名48岁男性，有心脏杂音和高血压病史，出现气短和外周水肿5天。他被诊断为快速心房扑动。经胸和经食管超声心动图显示严重的双心室收缩功能障碍，伴有因SOV破裂导致的左向右分流。经胸和经食管超声心动图及心脏磁共振成像的彩色多普勒显示，在收缩期和舒张期，有一股摇曳的分流血流朝着右心房（RA）和右心室基底部（RV）方向流出，无心肌瘢痕形成。左右心导管检查显示右侧压力、肺毛细血管楔压和左心室舒张末期压力升高。腔静脉和RA之间的氧饱和度无差异，但RA和RV之间的氧饱和度有误导性的升高。由于破裂解剖结构存在不确定性，该患者接受了手术干预。破裂的SOV通过三尖瓣环基部通向非常靠近RV基底部的RA。
即使进行多模态研究，术前明确描绘破裂SOV的解剖结构仍可能具有挑战性。
