Duckheim Martin, Bigalke Boris, Artunc Ferruh, Gawaz Meinrad, Schreieck Jürgen
Medizinische Klinik, Universitätsklinikum Tübingen.
Dtsch Med Wochenschr. 2014 Oct;139(42):2136. doi: 10.1055/s-0034-1387241. Epub 2014 Oct 7.
We report on a case of a 31-year-old patient who was admitted to hospital with acute cardiac failure. At physical examination we found peripheral edema and wet rales.
Cardiac ultrasound revealed severely impaired left and right ventricular function and prominent trabeculation in the right ventricle, but also in the apex of the left ventricle. This was confirmed by cardiac magnetic resonance imaging. Both pulmonary embolism and coronary heart disease were excluded by thoracic computed tomography and coronary angiography.
DIAGNOSIS, TREATMENT AND COURSE: These findings were in line with the diagnosis of non-compaction cardiomyopathy resulting in cardiac failure. We introduced diuretic therapy which leads to weight loss and release of dyspnoea. Due to prevention of embolic events an anticoagulative therapy was started. In light of rhythmogenic vulnerability, a subcutaneous implantable cardioverter defibrillator (ICD) system was implanted. Afterwards the patient was discharged in good general health.
Non-compaction cardiomyopathy (NCCM) is a hereditary non-ischaemic cardiac disease which occurs due to the failure of myocardial compaction during embryogenesis. Common manifestation is the triad of heart failure, rhythmogenic vulnerability and systemic embolic events. First-line imaging technique for diagnosing NCCM is cardiac ultrasound. The prognosis depends on severity of heart failure, ventricular arrhythmia and the incidence of embolic events.
我们报告一例31岁因急性心力衰竭入院的患者。体格检查发现外周水肿及湿啰音。
心脏超声显示左、右心室功能严重受损，右心室及左心室心尖部可见明显的肌小梁。心脏磁共振成像证实了这一情况。胸部计算机断层扫描及冠状动脉造影排除了肺栓塞和冠心病。
诊断、治疗及病程：这些发现符合导致心力衰竭的心肌致密化不全心肌病的诊断。我们采用了利尿剂治疗，使患者体重减轻并缓解了呼吸困难。由于要预防栓塞事件，开始了抗凝治疗。鉴于存在致心律失常易感性，植入了皮下植入式心脏复律除颤器（ICD）系统。之后患者健康状况良好出院。
心肌致密化不全心肌病（NCCM）是一种遗传性非缺血性心脏病，由于胚胎发育过程中心肌致密化失败而发生。常见表现为心力衰竭、致心律失常易感性和全身性栓塞事件三联征。诊断NCCM的一线影像学技术是心脏超声。预后取决于心力衰竭的严重程度、室性心律失常及栓塞事件的发生率。
