Kuźma Jacek, Buczyński Michał, Emam Mohamed Sameh, Mądry Wojciech, Zarlenga Magdalena, Gudziewski Olivia, Bramnik Daria, Kuśmierczyk Mariusz
Department of Cardiothoracic Surgery and Transplantology, Medical University of Warsaw, Warsaw, Poland.
Department of Cardiothoracic Surgery and Transplantology, Medical University of Warsaw, Warsaw, Poland.
JACC Case Rep. 2025 May 21;30(11):103308. doi: 10.1016/j.jaccas.2025.103308. Epub 2025 Mar 12.
Among the conotruncal abnormalities, double-outlet left ventricle (DOLV) has been considered an embryologic conundrum, and no more than 200 cases have been described since 1975. DOLV refers to anomalous ventriculoarterial connections with the great arteries arising entirely or predominantly from the left ventricle. It represents a heterogenous group of heart defects considering embryology, morphology, and hemodynamics. DOLV can be detected prenatally or postnatally by transthoracic echocardiography.
This study presents a case series of 3 newborns with DOLV and a leftward anterior aorta. The neonates showed symptoms of hypoxia and cardiac compromise after birth and required emergency interventional and surgical procedures.
The presented cases contribute interesting insights into the variability of DOLV, and they show the importance of multimodality imaging with echocardiography, computed tomography, and cardiac catheterization.
Complex heart defects coexisting with DOLV require precise visualization before surgical or interventional procedures (eg, a single ventricular vs a biventricular solution).
在圆锥动脉干畸形中,双出口左心室(DOLV)一直被认为是一个胚胎学难题,自1975年以来报道的病例不超过200例。DOLV是指大动脉全部或主要起源于左心室的异常心室动脉连接。从胚胎学、形态学和血流动力学角度来看,它代表了一组异质性的心脏缺陷。DOLV可通过经胸超声心动图在产前或产后检测到。
本研究报告了3例患有DOLV且主动脉位于左前方的新生儿病例系列。这些新生儿出生后出现缺氧和心脏功能受损的症状,需要紧急介入和外科手术。
所呈现的病例为DOLV的变异性提供了有趣的见解,并显示了超声心动图、计算机断层扫描和心导管检查等多模态成像的重要性。
与DOLV并存的复杂心脏缺陷在进行外科或介入手术前(例如,单心室或双心室解决方案)需要精确的可视化。