Yabur-Espitia Mirna, Muñoz-Castellanos Luis, Espinola-Zabaleta Nilda, Enríquez-Rodríguez Eduardo, Alcantar-Fernández Ana Cecilia
Instituto Nacional de Cardiología Ignacio Chávez.
Arch Cardiol Mex. 2010 Apr-Jun;80(2):108-12.
Uhl's anomaly is an uncommon malformation of the right ventricle, which is characterized by the absence of the myocardium in the right ventricular free wall, while the tricuspid and pulmonary valves are morphologically normal. The aim of this work was to present a clinical case of a patient with Uhl's anomaly, to describe the echocardiographic findings and to perform an anatomoechocardiographic correlation with a corresponding specimen. This is a case report of a 33 year old man with suspicion of atrial septal defect, who was in functional class II of the NYHA and presented signs of right ventricular failure. The echocardiogram showed dilation of the right cavities, the free wall of the right ventricle was thin and smooth, and the tricuspid and pulmonary valves were normal, which prompted the diagnosis of Uhl's anomaly. Also, an anatomoechocardiographic correlation was done with an equivalent specimen of the Embryology Department from the Instituto Nacional de Cardiologia Ignacio Chavez's collection.
Uhl's anomaly is an extremely rare entity. The clinical findings and echocardiography are the main tools for its diagnosis. The anatomoechocardiographic correlation was precise and allowed a better understanding of this anomaly."
乌尔氏畸形是一种罕见的右心室畸形，其特征是右心室游离壁无心肌，而三尖瓣和肺动脉瓣形态正常。本研究的目的是报告一例乌尔氏畸形患者的临床病例，描述超声心动图表现，并将解剖超声心动图与相应标本进行对照。这是一例33岁男性病例报告，怀疑患有房间隔缺损，纽约心脏协会（NYHA）心功能分级为II级，并有右心室衰竭体征。超声心动图显示右心腔扩张，右心室游离壁薄且光滑，三尖瓣和肺动脉瓣正常，提示诊断为乌尔氏畸形。此外，还将解剖超声心动图与墨西哥国家心脏病学研究所伊格纳西奥·查韦斯研究所胚胎学系收藏的一个等效标本进行了对照。
乌尔氏畸形是一种极其罕见的疾病。临床发现和超声心动图是诊断该病的主要工具。解剖超声心动图对照准确，有助于更好地理解这种畸形。
