*For medical professionals for reference
ATTR-CM diagnostic delay phenomenon is generally common.The key to the diagnostic rate
The rotor-like glands of the heart starch sample myocardial disease (ATTR-CM) is a common type of cardiac starch (CA) [1) [1].Truminerin (TTR) is synthesized by the liver, and is a tetraphinis under normal circumstances. When it is unbuttoned into a monomer and folded into starch-like substances, it is deposited in the myocardial timing of the myocardial degeneration, and the final progress is [2].According to whether genetic mutations can be divided into mutation (ATTRM) and wild types.Due to the lack of specificity of ATTR-CM clinical manifestations and lack of understanding of ATTR-CM by clinicians, the phenomenon of misunderstanding, misdiagnosis and diagnosis delay is common.Therefore, it is urgent to improve the clinicians attention to ATTR-CM, and the early diagnosis of standardized diseases is urgent.The medical community specially invited Professor Peng Daoquan of Xiangya Second Hospital of Central South University to introduce the clinical manifestations and diagnostic points of ATTR-CM, and share viewpoints and experiences to improve the clinicians early diagnosis of ATTR-CM.
ATTR-CM: Multi-system affected systemic diseases
Professor Peng Daoquan pointed out that ATTR-CM is a systemic disease that affects multi-system, and its clinical manifestations often often appearLack of specificity.In clinical practice, it may not only cause heart -related symptoms, but also affect a variety of tissue organs outside the heart, showing diverse symptoms.Among them, the heart is mainly manifested in heart failure and arrhythmia [3]. Patients may have shortness of breath and right heart failure after activity. With the further deposition of TTR, the left ventricular ejection score (LVEF) can be reduced.Arrhythmia is mainly atrial fibrillation and conduction block.Due to decreased heart output and arrhythmia, it can cause syncope and sudden cardiac death.
In addition, patients with ATTR-CM are often accompanied by position hypotension.The aortic valve stenosis can be one of the performances of ATTRWT-CM. For elderly patients with severe aortic aortic stenosis with low flow rate and low pressure steps, the possibility of ATTRWT-CM should be considered.
Starch -like substances can also be deposited from tissue organs deposited outside the heart to cause various clinical manifestations.Professor Peng Daoquan said that patients peripheral symptoms often appear earlier than heart symptoms.For example, the common outside heart manifestations of ATTR-CM patients have multiple peripheral sensory motor neuropathy, which is manifested as neurological pain and/or sensory abnormalities that first occurred from lower limbs, from distant to proximal.walk.Demanguaries of autonomic nerve are also more common. It is manifested as sweat disorder, constipation and diarrhea alternation, orthodox hypotension, and urine retention.Eye and central nervous systems are often involved.In addition, when starch -like substances are deposited in lumbar spinal canal and wriste tunnels, lumbar spinal tube stenosis and bilateral wrist tunnel syndrome can be caused.When TTR is deposited in the kidneys, proteinuria and/or renal insufficiency of different degrees of proteinuria occur [3].Professor Peng Daoquan particularly emphasized that due to the different clinical manifestations of patients with ATTR-CM, they often first diagnosed in neurology, spine surgery and orthopedics, instead of cardiology.Therefore, for patients suspected of ATTR-CM, clinicians need to maintain a high degree of alertness, and comprehensively consider the patients symptoms, signs, and examination results in order to make accurate diagnosis as soon as possible.
Improve ATTR-CM diagnosis rate: Start from identifying the "Warning"
Because the symptoms of ATTR-CM lack specificity, the diagnosis delay of most patients is caused. According to statistics, patients with ATTR-CM have been delayed from symptoms to diagnosis for 3 to 5 years.Therefore, the identification of ATTR-CM suspected patients is very important for clarifying the diagnosis and improving patients prognosis.How to increase the diagnosis of clinicians to ATTR-CM?Professor Peng Daoquan pointed out that patients with "warning" with an ATTR-CM "warning" should be particularly alert to the possibility of ATTR-CM.
These "warning signs" include: elderly heart failure (LVEF ≥ 40%), the left ventricle has no expansion of the left ventricular hypertrophy; the ultrasonic heart dynamic icon icon is hypertrophy and electrocardiogramQRS -free high voltage performance; muscle calcium protein continues to rise at low level; elderly people with low voltage difference, low -rate speed aortic valve narrowing, accompanied by right ventricular hypertrophy;Inhibitors and/or β-blockers; multiple , especially accompanied by autonomic nerve function abnormalities (unknown cause diarrhea and constipation, ascending hypotension, urinary retention, urinary incontinence, etc.); Family peripheral neuropathy; the elderlys bilateral wrist tunnel syndrome and/or lumbar spinal stenosis and repeated eyes white internal barrier [3].
Professor Peng Daoquan also pointed out that on the ultrasonic heart dynamic diagram, patients with ATTR-CM are usually manifested as a reduction in the vertical vertical strain (GLS) of the left ventricular myocardialis, and the overall vertical strain absolute value & lt; 15%[15%[15%[4].The vertical strain of the left ventricular base section and the middle section decreases significantly, while the heart tip is relatively mild. It is called the signs of the "tip of the heart". This is the specific manifestation of ATTR-CM different from other reasons.In addition, strong myocardial particle-like echo and diastolic loss are also common ultrasound performance of ATTR-CM.
In terms of biomarkers, ATTR-CM patients also have some characteristic performance.For example, the increasing proportion of BNP levels (BNP) levels is a significant sign, that is, although the patients LVEF is in a normal range, the BNP level is extremely elevated.In addition, patients may have continuously increased myoscin protein.The change of these biomarkers provides important clues to the diagnosis of ATTR-CM.
ATTR-CM Diagnostic Experience Sharing: Strategy and Process
Early diagnosis is the concept of the disease in the heart of the doctor.In particular, when patients with heart failure as the main manifestation and diastolic function are obviously decreased, the disease should be highly suspected.Next, combined with the "warning signs" mentioned earlier, the ECG shows that the limbs lead QRS low voltage, the ventricular hypertrophy, and the QRS voltage is not high. Consider the diagnosis of ATTR-CM.
In the diagnostic process, Professor Peng Daoquan pointed out that once the patient suspects that the patient is ATTR-CM, the examination of electrocardiography, ultrasonic cardiac percussion and cardiac magnetic resonance need to be further suspected.Ultrasonic heart movement and cardiac magnetic resonance play a vital role in the diagnosis of ATTR-CM. If these tests can clarify the diagnosis of amyloid-like degeneration, then the next step is to determine what kind of starch-like substances are.
Next, light chain screening should be performed to detect the detection of protein electrophoresis, immune fixed electricity, and free light chain.Weapon (such as heart, kidney).If the monoclonal immunoglobulin detection is not abnormal, the 99TCM-PYP nuclein can be used, and the positive person can diagnose ATTR-CM.Finally, in order to determine whether it is a wild or mutant, genetic testing is required.The genetic testing is simple and fast.If the genetic test results show that there is a mutation, that is, the ATTRM-CM; if there is no mutation, it is ATTRWT-CM.Through these steps, clinicians can clearly diagnose ATTR-CM and its type.
Summary
Attr-CM clinical manifestations and lack of specificity, often leading to diagnostic delays.Clinicians need to maintain a high degree of alertness when facing patients suspected of ATTR-CM, and comprehensively consider the patients symptoms, signs and examination results.Early diagnosis is crucial to the prognosis of patients with ATTR-CM, and identifying "warning signs" is the key to improving the diagnostic rate of ATTR-CM.Clinicians should be proficient in the diagnostic strategies and processes of ATTR-CM, and clearly diagnose ATTR-CM and types through electrocardiogram, ultrasonic heart-motion diagrams, cardiac magnetic resonance and follow-up examinations to provide patients with timely and effective treatment.
Expert Introduction
Professor Peng Daoquan
Professor of Xiangya Hospital of Central South University, first -level chief physician, doctoral supervisor
Director of the Institute of Blood Lipid and atherosclerosis of Zhongnan University, Deputy Director of Cardiovascular Department of Cardiovascular Department
Standing Committee member of the Professional Committee of Cardiac Corporation Prevention and Control of the Chinese Association of Prevention Medicine, Cardiovascular Diseases in Hunan Province
Deputy Chairman of the Association, Vice President of Cardiovascular Branch of the Hunan Medical Association, Deputy Chairman of the Hunan Provincial Association of Prevention Medicine Cardiac Prevention and Control Professional Committee
Calgary University and Cleveland Clinical Center for 4 years
Host more than 10 scientific research topics such as the American Cardiac Society (1) and the National Natural Science Foundation (6).
More than 80 SCI papers in Nat Med, EUR Heart J and other magazines
Won 2 items of the US Patent Innovation Award
5 items of scientific and technological achievements such as the Ministry of Education, the Ministry of Health, the Provincial Science and Technology Commission
References:
[1] .sperry bw, et al. Subtype-Specific Interactions and Prognosis In Cardiac Amy Loidosis.J aM Heart Assoc. 2016 Mar 24; 5 (3): E002877.
[2] .rapezzi C, et al. Transthyretin-Relands and the Heart: A Clinical Overview Then, thenNat Rev Cardiol. 2010 Jul; 7 (7): 398-408.
[3].Editorial Committee. Rotating methamphetamine cardiac starch-like diagnosis and treatment of Chinese expert consensus. China Cardiovascular Disease Magazine, 2021, 49 (04): 324-332.
[4]. Chinese Physician Association Ultrasonic Physician Branch Cardiac ultrasonic professional committee. Cardiac starch-like ultrasonic ultrasound cardiac inspection of Chinese expert consensus (2023) [J]. China ultrasound imaging magazine, 2023,32 (12): 1021-1029.
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Approval Code: CN-146700, Expired Date: 2025-11-4
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