Murphy Seán P, Sultana Sadia, Zern Emily K, Tower-Rader Albree, Churchill Jessica L, Stefanescu Schmidt Ada C, Huang Sihong, Learn Christopher P, Churchill Timothy W, DeFaria Yeh Doreen, Yucel Evin
Division of Cardiology (S.P.M., A.T.-R., A.C.S.S., S.H., C.P.L., T.W.C., D.F.Y., E.Y.), Massachusetts General Hospital, Boston.
Division of Radiology (S.P.M., S.S., A.T.-R.), Massachusetts General Hospital, Boston.
Circ Cardiovasc Imaging. 2025 Feb;18(2):e017126. doi: 10.1161/CIRCIMAGING.124.017126. Epub 2025 Jan 13.
Disorders of the pulmonic valve (PV) receive considerably less attention than other forms of valvular heart disease. Due to the dramatically improved survival of children with congenital heart disease over the last 5 decades, there has been a steady increase in the prevalence of adults with congenital heart disease, which necessitates that clinicians become familiar with the anatomy and the evaluation of right ventricular outflow tract and PV anomalies. A multimodality imaging approach using echocardiography, cardiac computed tomography, and magnetic resonance imaging is essential for a comprehensive evaluation of the anatomy and function of the right ventricular outflow tract, PV, and supravalvular region. As clinical presentation is often insidious with nonspecific symptoms, yet morbidity and mortality associated with severe untreated PV disease are significant, a high index of suspicion coupled with appropriate use of imaging techniques is critical in facilitating timely diagnosis and treatment. In this review, we aim to present a comprehensive approach to the diagnosis of PV disease and associated right ventricular outflow tract or supravalvular pulmonary stenosis, including optimal use of multimodality imaging to facilitate timely diagnosis, optimize therapeutic strategies, enhance postprocedural surveillance, and ultimately improve patient outcomes.
肺动脉瓣(PV)疾病受到的关注远少于其他形式的瓣膜性心脏病。由于在过去50年中先天性心脏病患儿的存活率显著提高,患有先天性心脏病的成年人患病率稳步上升,这就要求临床医生熟悉右心室流出道和PV异常的解剖结构及评估方法。使用超声心动图、心脏计算机断层扫描和磁共振成像的多模态成像方法对于全面评估右心室流出道、PV和瓣上区域的解剖结构和功能至关重要。由于临床表现往往隐匿且症状不具特异性,但未经治疗的严重PV疾病相关的发病率和死亡率却很高,因此高度的怀疑指数加上适当使用成像技术对于促进及时诊断和治疗至关重要。在本综述中,我们旨在介绍一种诊断PV疾病及相关右心室流出道或瓣上肺动脉狭窄的综合方法,包括最佳使用多模态成像以促进及时诊断、优化治疗策略、加强术后监测,并最终改善患者预后。