PRIMARY AMYLOIDOSIS INVOLVONG THE HEART: CLINICAL OBSERVATION

2021 ◽  
pp. 60-72
Author(s):  
Irina V. Opalinskaya ◽  
Galina V. Danilova ◽  
Natalya Yu. Isaeva ◽  
Ekaterina S. Petrova ◽  
Elena I. Busalaeva ◽  
...  
Keyword(s):  
Heart Failure ◽  
Clinical Observation ◽  
Diastolic Heart Failure ◽  
Clinical Manifestations ◽  
Restrictive Cardiomyopathy ◽  
Target Organ ◽  
Systemic Amyloidosis ◽  
Primary Amyloidosis ◽  
Medicinal Products ◽  
Wide Range

Primary amyloidosis is a disease with a complex and not fully understood pathogenesis, which is characterized by a wide range of clinical manifestations. Light chain amyloidosis is the most common form of systemic amyloidosis. At this, the heart is the dominant target organ in systemic amyloidosis. Cardiac amyloidosis (amyloid cardiomyopathy) is most often manifested by diastolic heart failure resulting from restrictive cardiomyopathy. Therapy of amyloid cardiopathy includes optimal treatment of heart failure and chemotherapy. To reduce the symptoms of heart failure, diuretics are the main means, since other pathogenetic agents cannot be used due to hypotension and a possible decrease in cardiac output. With the introduction of new medicinal products into clinical practice, such as the proteosome inhibitor Bortezomib, the prognosis for patients has improved. However, amyloidosis remains a difficult disease to diagnose and treat.

A clinical case of systemic amyloidosis in a cardiologists’s practice

The Clinician ◽  
2019 ◽  
Vol 13 (1-2) ◽  
pp. 72-79
Author(s):  
V. G. Okorokov ◽  
O. V. Evsina ◽  
V. A. Fomina ◽  
G. O. Ivanova ◽  
E. S. Soldatov ◽  
...  
Keyword(s):  
Clinical Case ◽  
Clinical Observation ◽  
Restrictive Cardiomyopathy ◽  
Systemic Amyloidosis ◽  
Immunohistochemical Method ◽  
Shortness Of Breath ◽  
Normochromic Anemia ◽  
High Level ◽  
Histological Verification

The aim of the work was to study the clinical case of systemic amyloidosis.Materials and methods. Patient Ch., 63 уears old, have admitted to the regional cardiological dispensary with complaints of short stabbing pains in the heart, without reaction to nitroglycerin, interruptions in the work of the heart, mainly at night, shortness of breath with little exertion, weakness, swelling of the legs and feet in September 2018. Sick from April 2016, when was dyspnea on exertion. The high level of creatinine, normochromic anemia have detected. In June 2016 chronic pyelonephritis was diagnosed. In August 2018, a right-sided hydrothorax was diagnosed, a pleural puncture was performed.Results. Laboratory and instrumental research methods were performed. Based on anamnesis data (normochromic anemia, proteinuria, increased creatinine in the blood), clinical picture, data of instrumental studies (restrictive cardiomyopathy, bilateral hydrothorax) clinical diagnosis was made: systemic amyloidosis the kidneys and heart with chronic kidney disease C5 and chronic heart failure. Histological evidence of amyloid and determination of the variant of amyloidosis by immunohistochemical method was recommended. But the patient died before the study. The histological verification of the diagnosis was carried out at the autopsy.Conclusion. This clinical observation is an example of late diagnosis of systemic amyloidosis and postmortal verification of the diagnosis, which is associated with the nonspecific symptoms and the rapidly progressing course of the disease. This case is interesting because amyloidosis proceeded under the guise of other diseases, quickly led to the development of severe heart and renal failure and to the death of the patient before verifying the disease.

scholarly journals Successful management of diastolic heart failure in a patient with restrictive cardiomyopathy following an anterior communicating artery aneurysm clipping

2017 ◽  
Vol 04 (02) ◽  
pp. 120-123
Author(s):  
Rajasekar Arumugam ◽  
Georgene Singh ◽  
Krishnaprabhu Raju ◽  
Ramamani Mariappan
Keyword(s):  
Heart Failure ◽  
Diastolic Heart Failure ◽  
Artery Aneurysm ◽  
Restrictive Cardiomyopathy ◽  
Anterior Communicating Artery ◽  
Aneurysmal Subarachnoid Haemorrhage ◽  
Successful Management ◽  
Aneurysm Clipping ◽  
Anterior Communicating Artery Aneurysm ◽  
Neurosurgical Patients

AbstractPerioperative management of neurosurgical patients with an underlying myocardial dysfunction poses a unique challenge to the neuroanaesthesiologist and the neurointensivist. Sudden catecholamine surge during the aneurysmal subarachnoid haemorrhage (SAH) can cause severe cardiac dysfunction such as myocardial ischaemia and cardiomyopathy. SAH in a patient with restrictive cardiomyopathy could be hazardous to the myocardium leading to severe cardiac morbidity. We report the successful management of an acute post-operative diastolic heart failure with a milrinone infusion in a patient with restrictive cardiomyopathy, following anterior communicating artery aneurysm clipping. We have discussed the ‘dual beneficial’ role of milrinone on the heart and the brain in this clinical setting. In addition, the importance of peri-operative utilisation of transoesophageal echocardiography and transthoracic echocardiography for the successful management of diastolic heart failure is highlighted.

scholarly journals Serum levels of cardiotrophin-1 in patients with obstructive hypertrophic cardiomyopathy and in patients with severe left ventricular dysfunction

2021 ◽  
Vol 36 (2) ◽  
pp. 70-75
Author(s):  
O. N. Ogurkova ◽  
E. N. Pavlyukova ◽  
T. E. Suslova
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Blood Serum ◽  
Diastolic Heart Failure ◽  
Left Ventricular ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
Lv Dysfunction ◽  
Wide Range ◽  
Obstructive Hypertrophic Cardiomyopathy

Relevance. Cardiotrophin-1 (CT-1) is a member of interleukin-6 (IL-6) superfamily and is associated with cardiovascular pathology. The production of CT-1 increases in response to myocardial wall stretching and increase in its rigidity and is also modulated by a wide range of neurohormones and peptides, which allows to monitor CT-1 as a marker of biomechanical stress. However, the prognostic significance of CT-1 in patients with diastolic heart failure with hypertrophic cardiomyopathy (HCM) remains poorly understood.Objective. To study the blood serum cardiotrophin-1 contents and their relationships with NT-proBNP in patients with obstructive hypertrophic cardiomyopathy and in patients with severe left ventricular (LV) dysfunction.Material and Methods. The study comprised a total of 76 patients with obstructive HCM and 31 patients with severe LV dysfunction. The group of patients with HCM comprised patients with obstructive form; the group of patients with severe LV dysfunction included patients with the third type of post-infarction LV remodeling and ejection fraction (EF) of less than 30%. The determination of cardiotrophin-1 and highly sensitive C-reactive protein was carried out by the enzyme immunoassay. The study of NT-proBNP content in blood serum was performed by multiplex immunoassay using the FLEXMAP 3D Luminex Corporation system.Results. The content of cardiotrophin-1 in the blood serum of patients with obstructive HCM was higher than in the group of patients with severe LV dysfunction. The study of NT-proBNP concentrations in the blood serum showed increases in the content in both groups of patients. The median concentrations of NT-proBNP and C-reactive protein in patients with severe LV dysfunction were increased compared to the median concentration in patients with obstructive HCM.Conclusion. The study showed an increase in cardiotrophin-1 content in the blood serum in patients with obstructive HCM with chronic diastolic heart failure. The increase in cardiotrophin-1 content was directly associated with the increase in NTproBNP level in patients with obstructive HCM with chronic diastolic heart failure.

scholarly journals Loeffler Endocarditis: A Unique Presentation of Right-Sided Heart Failure Due to Eosinophil-Induced Endomyocardial Fibrosis

2017 ◽  
Vol 10 ◽  
pp. 117954761772364 ◽  
Author(s):  
Amit Alam ◽  
Shankar Thampi ◽  
Shahryar G Saba ◽  
Rita Jermyn
Keyword(s):  
Heart Failure ◽  
Surgical Intervention ◽  
Diastolic Heart Failure ◽  
Treatment Options ◽  
Hypereosinophilic Syndrome ◽  
Restrictive Cardiomyopathy ◽  
Endomyocardial Fibrosis ◽  
History Of ◽  
The Right ◽  
Loeffler Endocarditis

Loeffler endocarditis is a rare restrictive cardiomyopathy caused by abnormal endomyocardial infiltration of eosinophils, with subsequent tissue damage from degranulation, eventually leading to fibrosis. Although an uncommon entity, it is still a disease with significant morbidity and mortality. Often identified only at late stages, treatment options are limited once fibrosis occurs, usually requiring heart failure medications or surgical intervention. We present a unique case of a woman with remote history of hypereosinophilic syndrome, attributed to treatment of rheumatoid arthritis with infliximab, who presented with symptoms of heart failure refractory to medical management and was found to have Loeffler endocarditis. The severe progression of the disease required surgical intervention with endocardial stripping to treat the right-sided diastolic heart failure.

scholarly journals Clinical experience of observation and tactics of management of a patient with ectodermal angydrotic dysplasia

2021 ◽  
Vol 24 (2) ◽  
pp. 179-186
Author(s):  
Elena Y. Startseva ◽  
Olga V. Bobrova ◽  
Olga I. Letyaeva ◽  
Oleg R. Ziganshin ◽  
Ksenia K. Zakomoldina
Keyword(s):  
Nervous System ◽  
Autosomal Recessive ◽  
Clinical Observation ◽  
Autosomal Dominant ◽  
Clinical Manifestations ◽  
Ectodermal Dysplasias ◽  
Wide Range ◽  
Epidermal Growth ◽  
Mode Of Inheritance ◽  
Autosomal Recessive Manner

The concept of ectodermal dysplasias covers a group of rare hereditary developmental anomalies that have a variety of phenotypic variants, but are characterized by common signs of underdevelopment or abnormal formation of organs and tissues derived from the ectodermal layer (skin and its derivatives - nails, hair, teeth, nervous system and sensory organs) ... Approximately 25% of ectodermal dysplasias known to date are inherited in an autosomal dominant or autosomal recessive manner; in other cases, the mode of inheritance is unclear. The syndrome is characterized by a wide range of clinical manifestations and may include additional symptoms of damage to other ectodermal, mesodermal, and endodermal structures. Ectodermal anomalies are a manifestation of disturbances in spatial-temporal coordination during the development of the epidermis. They involve genes such as EGF (epidermal growth factor), ED1 (ectodisplasin), EDAR (anhydrotic receptor ectodysplasin 1) and others that regulate the activity of genes involved in epidermal morphogenesis by activating or suppressing transcription factors (in particular, pb3; Koster). So far, only about 20% of genes have been identified that are responsible for about 200 ectodermal dysplasias of various symptoms and severity. This article describes the clinical observation of a patient with a rare disease - ectodermal anhydrotic dysplasia. The literature data on the clinical features of the course of this dermatosis, as well as the features of the course in this patient are presented.

scholarly journals Generalized sarcoidosis and severe thrombocytopenic purpura: clinical observation and literature review

2020 ◽  
Vol 1 (2) ◽  
pp. 6-10
Author(s):  
L.A. Ponomareva ◽  
◽  
L.A. Panchenko ◽  
A.B. Ponomarev ◽  
E.N. Popova ◽  
...  
Keyword(s):  
Nervous System ◽  
Literature Review ◽  
Clinical Observation ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Thrombocytopenic Purpura ◽  
Hematologic Disorders ◽  
Autoimmune Thrombocytopenic Purpura ◽  
Wide Range ◽  
Systemic Sarcoidosis

Sarcoidosis is a disease of an unknown etiology and is characterized by a wide range of clinical manifestations due to granulomatous damage of the lungs and other organs. Hematologic disorders in sarcoidosis are represented by different variants of pancytopenia, while autoimmune thrombocytopenic purpura is rare. Hemorrhagic syndrome with critical thrombocytopenia in blood of a patient with systemic sarcoidosis (damage to the lungs, nervous system, skin) coincided with an episode of a viral infection with symptoms of COVID-19 and the detection of an increased titer of antibodies to SARS-CoV-2 in the blood. In our paper we described the clinical features of the disease, medical treatment. The issues of managing patients with sarcoidosis during a pandemic time are discussed.

scholarly journals Impaired relaxation is the main manifestation in transgenic mice expressing a restrictive cardiomyopathy mutation, R193H, in cardiac TnI

2008 ◽  
Vol 294 (6) ◽  
pp. H2604-H2613 ◽  
Author(s):  
Jianfeng Du ◽  
Jing Liu ◽  
Han-Zhong Feng ◽  
M. M. Hossain ◽  
Nariman Gobara ◽  
...  
Keyword(s):  
Heart Failure ◽  
Transgenic Mice ◽  
Cardiac Function ◽  
Diastolic Dysfunction ◽  
Troponin I ◽  
Negative Impact ◽  
Diastolic Heart Failure ◽  
Critical Role ◽  
Restrictive Cardiomyopathy ◽  
Contraction And Relaxation

Transgenic mice were generated to express a restrictive cardiomyopathy (RCM) human cardiac troponin I (cTnI) R192H mutation in the heart (cTnI193His mice). The objective of this study was to assess cardiac function during the development of diastolic dysfunction and to gain insight into the pathophysiological impact of the RCM cTnI mutation. Cardiac function and pathophysiological changes were monitored in cTnI193His mice and wild-type littermates for a period of 12 mo. It progressed gradually from abnormal relaxation to diastolic dysfunction characterized with high-resolution echocardiography by a reversed E-to-A ratio, increased deceleration time, and prolonged isovolumetric relaxation time. At the age of 12 mo, cardiac output in cTnI193His mice was significantly declined, and some transgenic mice showed congestive heart failure. The negative impact of cTnI193His on ventricular contraction and relaxation was further demonstrated in isolated mouse working heart preparations. The main morphological change in cTnI193His myocytes was shortened cell length. Dobutamine stimulation increased heart rate in cTnI193His mice but did not improve CO. The cTnI193His mice had a phenotype similar to that in human RCM patients carrying the cTnI mutation characterized morphologically by enlarged atria and restricted ventricles and functionally by diastolic dysfunction and diastolic heart failure. The results demonstrate a critical role of the COOH-terminal domain of cTnI in the diastolic function of cardiac muscle.

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