柳叶刀病例：足样心

【足样心】19岁女,因进行性呼吸困难求诊.查体发现心衰体征.超声心动图发现心室弥漫性小梁形成,提示左心室致密化不全(图A.B).左心室射血分数中度下降,肺动脉收缩压估计约65 mm Hg.心血管磁共振成像证实为左心室致密化不全(图C).增强左心室造影如图D.该病罕见,临床特征包括心衰,血栓形成和心律不齐

A 19-year-old woman was admitted to our department with progressive dyspnoea. Physical examination showed signs and symptoms of heart failure. Echocardiography showed diffuse trabeculation of the left ventricle involving the entire apex, the mid-ventricular segments (figure A, video 1), and contrast penetration into the intertrabecular recesses (figure B, video 2) suggestive of left ventricular non-compaction. The left ventricular ejection fraction was moderately decreased and the systolic pulmonary artery pressure estimated by tricuspid regurgitation was roughly 65 mm Hg. Cardiovascular magnetic resonance imaging showed a diastolic non-compacted to compacted layer ratio of 2·4 (figure C), meeting the current diagnostic criteria of left ventricular non-compaction. Contrast left ventriculography showed a characteristic appearance of multiple broad trabeculations of the left ventricle that had a roughly parallel orientation (figure D, video 3). Left ventricular non-compaction is a rare disorder characterised by thickened left ventricular wall with deep intertrabecular recesses and can be associated with neuromuscular disease. Its main clinical manifestations are heart failure, thromboembolic events, and arrhythmias.