Raza Munis, Chalfoun Nagib, Wissam Abdallah, Hashmi Hamza, McNamara Richard
Grand Rapids Medical Education Partners/Michigan State University Internal Medicine Residency, Grand Rapids, Michigan.
Frederik Meijer Heart and Vascular Institute, Spectrum Health, Grand Rapids, Michigan.
Glob Cardiol Sci Pract. 2018 Mar 14;2018(1):9. doi: 10.21542/gcsp.2018.9.
Hypertrophic cardiomyopathy (HCM) is characterized by increased left ventricular wall thickness in the absence of any other identifiable cause of thickness. It predisposes the patient to increased risk of sudden cardiac death (SCD) due to fatal arrhythmias. Approximately 2% of the HCM patients have left ventricular apical aneurysm. CMR imaging is better in identifying this apical aneurysm as compared to echocardiogram. This apical aneurysm, which can be akinetic or dyskinetic, increases the risk of disease-related adverse events as compared to general HCM. These adverse disease-related events include SCD, thromboembolism, and symptoms of heart failure. We report a rare case of hypertrophic cardiomyopathy in association with Williams-Beuren Syndrome. On CMR imaging, patient was found to have a large apical aneurysm and mid-ventricular obstruction with underlying thrombus. He was started on oral anticoagulation, and ICD was recommended.
肥厚型心肌病（HCM）的特征是左心室壁厚度增加，且不存在任何其他可识别的导致心室壁增厚的原因。它使患者因致命性心律失常而发生心源性猝死（SCD）的风险增加。约2%的HCM患者有左心室心尖部室壁瘤。与超声心动图相比，心脏磁共振成像（CMR）在识别这种心尖部室壁瘤方面表现更佳。这种心尖部室壁瘤可表现为运动减弱或运动障碍，与一般的HCM相比，它会增加疾病相关不良事件的风险。这些与疾病相关的不良事件包括SCD、血栓栓塞和心力衰竭症状。我们报告一例罕见的肥厚型心肌病合并威廉斯-贝伦综合征的病例。在CMR成像检查中，发现该患者有一个巨大的心尖部室壁瘤和心室中部梗阻，并伴有潜在血栓形成。患者开始接受口服抗凝治疗，并建议植入植入式心律转复除颤器（ICD）。
