Loria Valentina, Colizzi Christian, Vaccarella Marcello, Franceschi Francesco, Aspromonte Nadia
Institute of Cardiology, Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Rome, Italy.
Institute of Cardiac Surgery, Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Rome, Italy.
Cardiology. 2019;143(3-4):100-104. doi: 10.1159/000500904. Epub 2019 Sep 11.
A 57-year-old woman presented to the Emergency Department with symptoms of worsening heart failure (HF). She had a past medical history of breast cancer treated with surgery and chemotherapy with anthracyclines and no family history of cardiomyopathy (CMP). In the last year, she received a diagnosis of HF with normal coronary arteries, during hospitalization for acute onset of dyspnea and was treated with medical therapy. After several months, few days before admission to our hospital, an echocardiography (ECHO) showed features of left ventricular noncompaction (LVNC), not described in previous ECHO and further confirmed by cardiac magnetic resonance. This case highlights the current uncertainties regarding the pathogenesis of LVNC and the clinical challenge of cardiologists facing LVNC morphology to decide if they are observing a genetic CMP, a phenotype overlapping with dilated or hypertrophic CMP, or a variant of the left ventricular (LV) wall anatomy. No consensus exists among scientific communities regarding diagnostic criteria of LVNC and in most cases; the key element in the diagnostic decision is not the LVNC by itself, but the associated LV dilation and/or dysfunction, hypertrophy, arrhythmias, and embolic events.
一名57岁女性因心力衰竭（HF）症状加重就诊于急诊科。她既往有乳腺癌病史，接受过手术及蒽环类药物化疗，无心肌病（CMP）家族史。去年，她因急性呼吸困难住院期间被诊断为冠状动脉正常的HF，并接受了药物治疗。数月后，在入住我院前几天，超声心动图（ECHO）显示出左心室心肌致密化不全（LVNC）的特征，此前的ECHO未描述过该特征，心脏磁共振进一步证实了这一点。该病例凸显了目前关于LVNC发病机制的不确定性，以及心脏病专家面对LVNC形态时所面临的临床挑战，即判断他们所观察到的是遗传性CMP、与扩张型或肥厚型CMP重叠的表型，还是左心室（LV）壁解剖结构的变异。科学界对于LVNC的诊断标准尚未达成共识，在大多数情况下，诊断决策的关键因素并非LVNC本身，而是与之相关的LV扩张和/或功能障碍、肥厚、心律失常及栓塞事件。
