Introduction and purpose. Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular (LV) wall thickening not associated with increased afterload (hypertension and aortic stenosis), is usually caused by mutations in sarcomeric protein genes, and is inherited in an autosomal dominant manner. Unlike HCM, myocardial hypertrophy in its phenocopies is associated with the accumulation of substances such as amyloid, glycogen, etc. in the myocardium. The aim of our work was to analyze the features of the diagnosis and course of HCM in real clinical practice. Material and methods. A retrospective analysis of medical records of 80 patients (56.3 % of men) discharged with a diagnosis of HCM from a multidisciplinary hospital in Moscow in the period from 2007 to 2021 was carried out. The diagnosis of HCM in all patients was established on the basis of echocardiography data. The median age (25th and 75th percentiles are indicated in brackets) was 57 (48.5; 63) years. The duration of hospitalization was 8 (6; 12.5) days. Results. The reason for hospitalization was angina syndrome in 35 %, suspicion of acute coronary syndrome in 16.3 %, paroxysmal atrial fibrillation (AF) in 11.3 %, decompensation of chronic heart failure in 11.3 %, syncope in 7.5 % %, hypertensive crisis in 3.8 %, coronary angiography in 3.8 %, pacemaker implantation in 2.5 %, consultation with an arrhythmologist in 2.5 %, implantation of a cardioverter-defibrillator in 1.2 %, medical examination to resolve the issue of fitness for military service in 1.2 %, acute cerebrovascular accident in 1.2 %, hypotension in 1.2 %, drug bradycardia in 1.2 % of patients. Before hospitalization, a history of myocardial infarction was diagnosed in 15 %, arterial hypertension — in 53.8 %, chronic heart failure — in 77.6 %, chronic kidney disease — in 21.3 % of patients. Prior to the analyzed hospitalization, a history of myocardial infarction was diagnosed in 15 %, arterial hypertension in 53.8 %, chronic heart failure in 77.6 %, chronic kidney disease in 21.3 % of patients. LV wall thickness ≥1.5 cm was detected in 91.2 %, symmetrical form of hypertrophy — 22.1 %, apical — 5.2 %, papillary muscle hypertrophy — 1.3 %, interventricular septum — 71.4 % of patients. Permanent obstruction of the LV outflow tract (LVOTO) was detected in 62.8 % (9.0 % of patients had a history of septal reduction), transient LVOTO — in 1.3 %, non-obstructive HCM — in 35.9 %. The ejection fraction (EF) of the LV (according to Simpson) was 63 (55-70) %, CHF with reduced LV EF <40 % was detected in 3.8 %, with a moderately reduced LV EF (40-49 %) — in 5 %, with preserved LV EF — in 68.8 % of patients. Anterior systolic movement of the anterior leaflet of the mitral valve occurred in 47.5 %, prolapse of the anterior leaflet of the mitral valve was described in 7.14 %. Mitral regurgitation was registered in 75 % of patients. 45 % of patients with HCM suffered from AF: permanent 15 %, paroxysmal 23.8 %, persistent 6.2 % of patients. During hospitalization, ventricular tachycardia was registered in 7.5 %, supraventricular tachycardia — 3.8 %, conduction disturbances were noted in 36.3 % of patients, of which atrioventricular block in 6.3 %, blockade of the right bundle branch block in 21.3 %, left bundle branch block in 15 %, and Wolff-Parkinson-White syndrome in 1.3 % . Implantation of a pacemaker in history was in 5 %, including in connection with MorgagniAdams-Stokes attacks — in 3.8 % of patients. During a median follow-up of 87 (interquartile range 45–131.5) months, 13.8 % of patients with HCM died. In deceased patients, LVOTO was significantly more common (in the dead 100 %, in the living 58.2 %, p = 0.006) and AF (in the dead 72.7 %, in the living 40.6 %, p = 0.047). Genetic testing and exclusion of HCM phenocopies was not performed during hospitalization and was not recommended for any patient. Conclusion. In real clinical practice, in most cases, only phenotypic diagnosis of HCM is carried out according to echocardiography, and screening for genetic mutations and HCM phenocopies is not performed. It is necessary to widely introduce genetic testing and screening for HCM phenocopies for the timely diagnosis of pathology that requires the appointment of specific pathogenetic therapy to improve the prognosis of patients

Тромбоз воротной вены – это частичная или полная окклюзия кровотока по воротной вене, возникающая вследствие наличия в ее просвете тромба. Этиология тромбоза воротной вены зачастую многофакторная, возможна комбинация локальных и системных факторов. Необходимо проводить широкий диагностический поиск с целью выявления причин тромбоза, включая болезни печени, злокачественные новообразования, инфекционные заболевания и другие. Клиническая картина тромбоза воротной вены может варьировать от малосимптомных проявлений до острого течения. Хронический тромбоз воротной вены может проявляться также неспецифическими симптомами. Известно, что COVID-19 ассоциируется с усилением свертываемости крови и развитием тромбоэмболических осложнений. Коронавирус тяжелого острого респираторного синдрома 2 (SARS-Cov-2) рассматривается как триггерный фактор, запускающий в организме пациента механизмы реактивации других вирусных инфекций, в частности герпетической группы. В данной статье представлен клинический случай заболевания COVID-19, осложнившегося тромбозом воротной вены у пациента с ВЭБ/ЦМВ-инфекцией

Portal vein thrombosis is a partial or complete occlusion of blood flow through the portal vein, resulting from the presence of a thrombus in its lumen. The etiology of portal vein thrombosis is often multifactorial, a combination of local and systemic factors being possible. It is necessary to conduct a wide diagnostic search in order to identify the causes of thrombosis, including liver diseases, malignant neoplasms, infectious diseases, etc. The clinical picture of portal vein thrombosis can vary from low-symptomatic manifestations to an acute course. Chronic portal vein thrombosis can also manifest with nonspecific symptoms. It is known that COVID-19 is associated with increased blood clotting and the development of thromboembolic complications. Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is considered as a trigger factor for the reactivation mechanisms of other viral infections in the patient's body, in particular the herpes group. This article presents a clinical case of COVID-19, complicated by portal vein thrombosis in a patient with EBV/CMV infection.

Amyloidosis is a heterogeneous group of the diseases caused by the extracellular deposition of amyloid in various organs including a cardiovascular system. Aim . To study the cardiovascular damage in patients with systemic amyloidosis. Material and methods . A retrospective assessment of 38 cases of systemic amyloidosis was carried out. Diagnosis was verified by biopsy in 57,9% (27,3% of these cases were then confirmed by autopsy) and autopsy in 42,1% of patients. Congo red stain and polarized-light microscopy were used. The systemic nature of lesion was diagnosed in the positive biopsies of two or more localizations or in the positive biopsy of one localization in the combination with the clinical picture of amyloidosis at least one organ. Median (interquartile range) of age was 72,5 (64-84) years (women, 65,8%; men, 34,2%). Results . In the medical history, 81,6% of patients with systemic amyloidosis had hypertension, 52,6% — coronary artery disease, 34,2% — myocardial infarction, 36,8% — type 2 diabetes, 44,7% — atrial fibrillation, 26,3% — conduction disorders. Also 13,2% of patients had an implanted pacemaker. According to the electrocardiography, 42,3% of patients had a low QRS voltage. According to echocardiography, left ventricular (LV) hypertrophy was detected in 94,7% of patients. In addition, 31,6% of patients had signs of granular sparkling myocardial appearance, 20% — impaired local LV contractility, 77,8% — increased pulmonary artery reassure, 28,6% — LV diastolic dysfunction, 14,3% — normal diastolic function. LV ejection fraction (LVEF) was 55 (44,5-55)%. LVEF 50% — in 65% of patients. Heart failure was indicated in 71,1% of patients with systemic amyloidosis, chronic kidney disease (CKD) — in 95% of patients. The estimated glomerular filtration rate (CKD-EPI) was 21,2 (13,1-40) ml/min/1,73 m 2 . Proteinuria (1,1 (0,5-3,0) g/l) was detected in 88,9% of patients. Nephrotic syndrome occurred in 27,8% of patients. Conclusion . Patients with cardiovascular lesions of unclear etiology, especially in the presence of LV wall thickening, preserved LVEF, stage 4-5 CKD and proteinuria, require screening for systemic amyloidosis.

Amyloidosis is a heterogeneous group of the diseases caused by the extracellular deposition of amyloid in various organs including a cardiovascular system.Aim. To study the cardiovascular damage in patients with systemic amyloidosis.Material and methods. A retrospective assessment of 38 cases of systemic amyloidosis was carried out. Diagnosis was verified by biopsy in 57,9% (27,3% of these cases were then confirmed by autopsy) and autopsy in 42,1% of patients. Congo red stain and polarized-light microscopy were used. The systemic nature of lesion was diagnosed in the positive biopsies of two or more localizations or in the positive biopsy of one localization in the combination with the clinical picture of amyloidosis at least one organ. Median (interquartile range) of age was 72,5 (64-84) years (women, 65,8%; men, 34,2%).Results. In the medical history, 81,6% of patients with systemic amyloidosis had hypertension, 52,6% — coronary artery disease, 34,2% — myocardial infarction, 36,8% — type 2 diabetes, 44,7% — atrial fibrillation, 26,3% — conduction disorders. Also 13,2% of patients had an implanted pacemaker. According to the electrocardiography, 42,3% of patients had a low QRS voltage. According to echocardiography, left ventricular (LV) hypertrophy was detected in 94,7% of patients. In addition, 31,6% of patients had signs of granular sparkling myocardial appearance, 20% — impaired local LV contractility, 77,8% — increased pulmonary artery reassure, 28,6% — LV diastolic dysfunction, 14,3% — normal diastolic function. LV ejection fraction (LVEF) was 55 (44,5-55)%. LVEF <40% was detected in 15%, 4050% — in 20%, >50% — in 65% of patients. Heart failure was indicated in 71,1% of patients with systemic amyloidosis, chronic kidney disease (CKD) — in 95% of patients. The estimated glomerular filtration rate (CKD-EPI) was 21,2 (13,1-40) ml/min/1,73 m2. Proteinuria (1,1 (0,5-3,0) g/l) was detected in 88,9% of patients. Nephrotic syndrome occurred in 27,8% of patients.Conclusion. Patients with cardiovascular lesions of unclear etiology, especially in the presence of LV wall thickening, preserved LVEF, stage 4-5 CKD and proteinuria, require screening for systemic amyloidosis.

This article presents the clinical features and treatment options for autoimmune thrombocytopenic purpura associated with autoimmune hemolytic anemia — Fisher-Evans syndrome. Patient P., aged 68 years, was admitted to the hospital by an ambulance team with a referral diagnosis of acute pancreatitis. Hemorrhagic and anemic syndromes were the main clinical signs. Physical examination revealed a strip-formed hemorrhagic rash in the area of inguinal folds, the anterior surface of thighs and lower legs. In the course of differential diagnostics, Fisher-Evans syndrome was diagnosed. Initial oral and pulse therapy with prednisolone was not effective. The patient received platelet transfusions regularly. When eltrombopag was included in therapy, there was an improvement in the patient’s condition, as well as a tendency to a rising level of platelets. On the 35th day, the patient was discharged

The article presents the clinical features and treatment options for autoimmune thrombocytopenic purpura associated with autoimmune hemolytic anemia in the context of Fisher-Evans syndrome. Patient P. was admitted to the emergency department by an ambulance team with a direct diagnosis of acute pancreatitis. Leading clinical syndromes were hemorrhagic and anemic syndromes. A physical examination also revealed a strip-formed hemorrhagic rash in the area of inguinal folds, the anterior surface of the thighs and lower legs. During the differential diagnostic search, the diagnosis of Fisher-Evans syndrome was established. Initial oral and pulse therapy with prednisone was not effective. The patient received platelet transfusions regularly. When eltrombopag was included in therapy, there was an improvement in the patient’s condition, as well as a tendency to increase the level of platelets. On the 35th day, the patient was discharged from the hospital.We examined various clinical variants of thrombocytopenia, I meet in real clinical practice.