先天性肌性室间隔瘤：4例报告并文献复习

Congenital muscular ventricular septal aneurysm: report of four cases and review of the literature.

作者信息

Nguyen Thieu P, Srivastava Shubhika, Ko H Helen, Lai Wyman W

机构信息

Division of Pediatric Cardiology, Department of Pediatrics, The Mount Sinai Medical Center, New York, NY 10029, USA.

出版信息

Pediatr Cardiol. 2008 Jan;29(1):40-4. doi: 10.1007/s00246-007-9048-4. Epub 2007 Sep 5.

DOI:10.1007/s00246-007-9048-4

PMID:17786376

Abstract

We describe a rare association between congenital muscular ventricular septal aneurysm (MuVSA) and right heart lesions, with emphasis on echocardiographic diagnosis, clinical presentation, and outcome. Our four cases of MuVSA occurred in combination with either pulmonary atresia with intact ventricular septum (PAIVS; three patients) or absent pulmonary valve syndrome with severe tricuspid stenosis (one patient). MuVSA and right heart lesions were detected in utero in three patients. The three patients with PAIVS and MuVSA survived with no significant morbidity from the septal aneurysm at 3- to 5-year follow-up. The presence of MuVSA did not negatively affect the outcome of our patients.

摘要

我们描述了先天性肌性室间隔瘤（MuVSA）与右心病变之间的罕见关联，重点在于超声心动图诊断、临床表现及预后。我们的4例MuVSA患者合并了室间隔完整的肺动脉闭锁（PAIVS；3例患者）或伴有严重三尖瓣狭窄的肺动脉瓣缺如综合征（1例患者）。3例患者在子宫内就检测到了MuVSA和右心病变。3例合并PAIVS和MuVSA的患者在3至5年的随访中存活，室间隔瘤未导致明显发病。MuVSA的存在并未对我们患者的预后产生负面影响。