轻链 (AL) 心脏 Amyloidosis 呈现为心力衰竭与减少的喷射分数
Luis E Santiago1,2, Ali Tariq Alvi1,2, Veniamin Melnychuk1,2
1Internal Medicine, HCA Florida Northwest Hospital, Margate, USA.
Cureus
|April 1, 2024
概括
系统性amyloidosis是一种蛋白质错折的疾病,可以导致心力衰竭. 这一案例突出了心力衰竭的罕见表现,最初的症状是减少喷射分数.
科学领域:
- 心脏病学 心脏病学
- 腎臟病學 (nephrology) 是一種醫學專業.
- 在瘤学瘤学.
背景情况:
- 系统性粉症涉及到细胞外蛋白质的错误折叠,导致器官功能障碍.
- 常见的亚型是轻链 Amyloidosis 和 transthyretin 的 Amyloidosis.
- 心脏参与往往导致限制性心肌病和心力衰竭与保存的喷射分数.
研究的目的:
- 为了呈现一种不寻常的全身性氨基粉症病例.
- 描述患有新发性心力衰竭的患者,其主要表现为减少喷射分数.
- 为了强调在无法解释的心力衰竭中考虑氨基粉症的重要性.
主要方法:
- 一个53岁的女性患者的病例报告.
- 新发性心力衰竭的临床表现与减少的喷射分数.
- 对潜在原因的评估,包括粉样性粉症.
主要成果:
- 患者呈现出心力衰竭,排气分数减少和腹功能障碍.
- 没有先前的史或粉样性粉症的症状.
- 这种表现对于心脏粉样性粉症是不典型的,这通常表现为保存的喷射分数.
结论:
- 系统性氨基粉症可以不典型地呈现为心力衰竭,并作为最初的症状减少喷射分数.
- 早期诊断amyloidosis对于及时管理和改善结果至关重要.
- 这一案例强调了在心力衰竭患者中需要广泛的差异诊断的必要性.
相关概念视频
Pathophysiology of Heart Failure
1.6K
Heart failure (HF) is a progressive syndrome involving ventricles that leads to inadequate cardiac output. It can be classified based on location and output or ejection fraction. Ejection fraction (EF) is an essential measurement in the diagnosis and surveillance of HF. Reduced EF corresponds to systolic heart failure (HFrEF). However, HF with preserved ejection fraction (HFpEF) is becoming increasingly prevalent. Also known as diastolic HF, this form of HF is related to aging. The...
1.6K
Imbalances in Cardiac Output
1.4K
The heart's primary function is to pump blood throughout the body, maintaining a balance between blood sent out (cardiac output) and blood returning (venous return). If this balance is disrupted, it can result in congestive heart failure (CHF), a severe condition where the heart becomes an inefficient pump, leading to inadequate blood circulation.
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send...
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send...
1.4K
Heart Failure Drugs: Inotropic Agents
578
Positive inotropic agents are commonly used as the first line of treatment for heart failure. One such agent is digoxin, derived from the genus Digitalis, which has been known for centuries but effectively utilized since 1785. However, these cardiac glycosides can have potentially toxic effects due to their mechanism of action, which involves inhibiting Na+/K+-ATPase and increasing contractility. Digoxin is absorbed orally and distributed in various tissues, including the CNS. It has a long...
578
Heart Failure Drugs: Inhibitors of Renin-Angiotensin System
424
The activation of the sympathetic nervous system and the renin-angiotensin-aldosterone system (RAAS) contributes to cardiac remodeling, and inhibiting the RAAS is a pharmacological target in heart failure management. As a result, neurohumoral modulation is a crucial treatment principle for managing heart failure. This approach involves using medications like ACE inhibitors (ACEIs), angiotensin receptor blockers (ARBs), β-blockers, mineralocorticoid receptor antagonists (MRAs), and neutral...
424
Heart Failure Drugs: Diuretics
370
Heart failure and kidney perfusion are interconnected in a complex way. Reduced renal perfusion and venous congestion are two significant factors that contribute to renal dysfunction in heart failure. The kidneys, primarily responsible for fluid balance in the body, are adversely affected due to compromised cardiac output and increased venous pressure. In response to reduced renal perfusion, the kidneys activate neurohumoral mechanisms to restore balance. However, these mechanisms can be...
370


