Pourmoghaddas Ali, Zavar Reihaneh, Behjati Mohaddeseh
Cardiology, Isfahan University of Medical Sciences, Isfahan, Iran.
Heart Failure Research Center, Cardiovascular Research Center, Cardiovascular Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran.
Case Rep Cardiol. 2014;2014:325257. doi: 10.1155/2014/325257. Epub 2014 Nov 26.
Background. Noncompaction/hypertrabeculation left ventricle (NCM/HVM) is most commonly reported in one or more segments of left ventricle and sometimes both ventricles. In this case, we present noncompaction of all segments of right and left ventricle, in a young man with mental retardation. Case Presentation. A 19-year-old male was referred to us with sudden dyspnea at rest and chest discomfort. He was a known case of mental retardation. He was born full term with birth weight = 1250 grams. On physical examination. A systolic murmur (II/VI) at left sternal border was heard. ECG showed increased voltage in precordial lead and deep ST segment depression. Chest X-ray (CXR) was within normal limits. Transthoracic echocardiography showed situs solitus, D loop, normal connection of great vessels, noncompaction LV at all segments (noncompaction/compaction = 2.5/0.5) with moderate systolic dysfunction (LVEF = 40%), diastolic dysfunction grade II, normal RV size with mild systolic dysfunction and hypertrabeculation, mild tricuspid regurgitation (TR), and normal pulmonary artery systolic pressure. After injection of agitated saline some bubbles were passed from right to left through patent foramen oval (PFO). Conclusions. Extensive sinusoid formation and trabeculation of RV and nearby all LV segments and its association with mental retardation suggest presence of strong genetic background.
背景。非致密化/过度小梁化左心室（NCM/HVM）最常报道于左心室的一个或多个节段，有时也见于两个心室。在此病例中，我们报告了一名患有智力障碍的年轻男性左右心室所有节段均出现非致密化。病例介绍。一名19岁男性因静息时突发呼吸困难和胸部不适前来就诊。他是一名已知的智力障碍患者。他足月出生，出生体重1250克。体格检查时，在左胸骨缘听到收缩期杂音（II/VI级）。心电图显示胸前导联电压增高及ST段深度压低。胸部X线（CXR）正常。经胸超声心动图显示心房正位、D袢、大血管连接正常，左心室所有节段均有非致密化（非致密化/致密化=2.5/0.5），伴有中度收缩功能障碍（左心室射血分数[LVEF]=40%），舒张功能障碍II级，右心室大小正常，伴有轻度收缩功能障碍和小梁化，轻度三尖瓣反流（TR），肺动脉收缩压正常。注射振摇生理盐水后，一些气泡经卵圆孔未闭（PFO）从右向左通过。结论。右心室及几乎所有左心室节段广泛的窦状隙形成和小梁化及其与智力障碍的关联提示存在强大的遗传背景。
