肥厚型心肌病临床进展为限制性生理学病例

M. J. Nisarga, Anupama V. Hegde, Vishwanath Krishnamurthy, H. Rahul
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摘要

肥厚型心肌病(HCM)的定义是:左心室(LV)肥厚≥15 毫米(若有家族史则为 13 毫米),原因不明,且缺乏导致左心室壁增厚的可识别因素,包括压力超负荷、浸润性或贮积性疾病。限制性生理学是一种心室充盈模式,心肌僵硬度增加导致心室压力升高,引起舒张功能障碍。一名 60 岁的男子因持续 2 天呼吸困难和双下肢肿胀而被送往医院。患者已知患有 HCM、缺血性心脏病(IHD),单支血管病变--动脉口 D1 70% 狭窄、心房颤动(AF)、射频消融术后状态、中度左心室收缩功能障碍(射血分数 [EF] - 30%),曾因心力衰竭反复入院。患者出现低血压和双下肢浮肿,全身检查显示双侧心悸。患者被诊断为急性失代偿性心力衰竭、EF值降低的心力衰竭、HCM、陈旧性心肌缺血和心率受控的房颤。连续的二维超声心动图显示,HCM 已发展为限制性生理学。患者病情恶化,计划进行心脏移植,并使用桥接左心室辅助装置治疗。这种转变通常没有报道,相关文献也很少;因此,本病例被报道出来。
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Case of Hypertrophic Cardiomyopathy with Clinical Progression to Restrictive Physiology
Hypertrophic cardiomyopathy (HCM) is defined as an unexplained left ventricular (LV) hypertrophy ≥15 mm (13 mm if family history present) occurring in the absence of identifiable factor that may account for LV wall thickening, including pressure overload and infiltrative or storage disorder. Restrictive physiology is a pattern of ventricular filling with increased myocardial stiffness leading to elevation of ventricular pressure, causing diastolic dysfunction. A 60-year-old man was brought to the hospital with progressive worsening breathlessness for 2 days and bilateral lower limb swelling. The patient was a known case of HCM, ischemic heart disease (IHD) with single-vessel disease – ostial D1 70% stenosis, atrial fibrillation (AF), status post radiofrequency ablation, moderate LV systolic dysfunction (ejection fraction [EF] – 30%), with recurrent admission due to heart failure. The patient presented with hypotension and bilateral lower limb swelling, and systemic examination showed bilateral crepitations. The patient was diagnosed with acute decompensated heart failure, heart failure with reduced EF, HCM, old IHD, and AF with controlled ventricular rate. Serial two-dimensional echocardiography showed the progression of HCM to restrictive physiology. The patient deteriorated and was planned for a heart transplant with bridging LV assist device therapy. The transition is not usually reported, and literature related to this is scarce; hence, this case is being reported.
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