Primary Data on ATTR-Amyloidosis Prevalence Among Elderly Patients With Left Ventricular Hypertrophy in Russia.

AIM: To estimate the prevalence of amyloid cardiomyopathy (CM) caused by transthyretin amyloidosis (ATTR) and immunoglobulin light chain (AL) amyloidosis among patients aged >65 years with interventricular septal (IVS) hypertrophy of ≥14 mm. MATERIAL AND METHODS: From January through August 2023, 60 patients (mean age 7.2±7.3 years, 34 (56.67%) men) were enrolled. Patients meeting the inclusion criteria underwent an echocardiographic study with determining the myocardial longitudinal strain, myocardial scintigraphy with 99mTc-pyrfotech, myocardial single-photon emission computed tomography, measurement of N-terminal fragment of brain natriuretic peptide and troponin I, and the immunochemical study of serum and urine proteins with measurement of free light chains. In the presence of grades 2 and 3 radiopharmaceutical uptake according to scintigraphy, a molecular genetic study was performed for differential diagnosis of wild-type transthyretin amyloidosis (wtATTR) and hereditary/variant (hATTR) ATTR-CM. RESULTS: According to data of myocardial scintigraphy with 99mTc-pyrfotech, grade 3 uptake in the absence of monoclonal secretion was detected in 5 (8.3%) cases and grade 2 radiotracer uptake in the absence of monoclonal secretion was detected in 6 (10%) patients. Myeloma complicated by AL amyloidosis and primary AL amyloidosis were found in 5 (8.3%) patients. CONCLUSION: Among patients aged ≥65 years with IVS hypertrophy ≥14 mm, amyloid CM was detected in 20% of cases (12 patients), including 5 cases (8.3%) of AL amyloidosis and 7 cases (11.7%) of ATTR amyloidosis.

Retinal Phenotyping of Ferrochelatase Mutant Mice Reveals Protoporphyrin Accumulation and Reduced Neovascular Response.

Purpose: Heme depletion, through inhibition of ferrochelatase (FECH), blocks retinal and choroidal neovascularization. Both pharmacologic FECH inhibition and a partial loss-of-function Fech mutation (Fechm1Pas) are associated with decreased neovascularization. However, the ocular physiology of Fechm1Pas mice under basal conditions has not been characterized. Here, we aimed to characterize the retinal phenotype of Fechm1Pas mice. Methods: We monitored retinal vasculature at postnatal day 17, 2 months, and 6 months in Fechm1Pas homozygotes, heterozygotes, and their wild-type littermates. We characterized Fech substrate protoporphyrin (PPIX) fluorescence in the eye (excitation = 403 nm, emission = 628 nm), retinal function by electroretinogram, visual acuity by optomotor reflex, and retinal morphology by optical coherence tomography and histology. We stained vasculature using isolectin B4 and fluorescein angiography. We determined endothelial sprouting of retinal and choroidal tissue ex vivo and bioenergetics of retinal punches using a Seahorse flux analyzer. Results: Fundi, retinal vasculature, venous width, and arterial tortuosity showed no aberrations. However, VEGF-induced retinal and choroidal sprouting was decreased in Fechm1Pas mutants. Homozygous Fechm1Pas mice had pronounced buildup of PPIX in the posterior eye with no damage to visual function, bioenergetics, and integrity of retinal layers. Conclusions: Even with a buildup of PPIX in the retinal vessels in Fechm1Pas homozygotes, the vasculature remains normal. Notably, stimulus-induced ex vivo angiogenesis was decreased in Fechm1Pas mutants, consistent with reduced pathologic angiogenesis seen previously in neovascular animal models. Our findings indicate that Fechm1Pas mice are a useful model for studying the effects of heme deficiency on neovascularization due to Fech blockade.

[Cardiac damage in sarcoidosis: clinical features and impact of steroid therapy].

Twenty-seven sarcoidosis patients with signs of cardiac damage were examined to study the clinical features of cardiac sarcoidosis and the impact of systemic glucocorticosteroid (GCS) therapy on its course. ECG and echoECG changes were observed in 21 (78%) and 17 (63%) patients, respectively; abnormal perfusion was seen in 25 (93%) patients at single-photon emission myocardial computed tomography using 99mTc-MIBI. Three clusters (clinical types) of patients were identified, which differed in the pattern and degree of cardiac disorders. The use of systemic GCSs in 13 of 20 patients resulted in myocardial perfusion recovery and clinical improvement, the degree and duration of which depended on what cluster it belonged to. Seven GCS-untreated patients had progressive perfusion disorders with subsequent or synchronous clinical deterioration (p < 0.05). The degree and duration of the positive effect of a GCS differed depending on the clinical type of cardiac damage.

[Prediction of results of antiarrhythmic drug therapy in patients with malignant ventricular tachyarrhythmias, based on the prognostic value of left ventricular contractility parameters].

In 44 patients (3 women and 41 men, mean age 54 +/- 11 years) with malignant ventricular tachyarrhythmias (MVT) we assessed dependence of results of testing of antiarrhythmic drugs and efficacy of their long term use for prevention of recurrences of MVT on topography of derangement of local left ventricular (LV) contractility. Regional LV contractility was assessed with transthoracic echocardiography and radionuclide ventriculography (RNV). Testing of antiarrhythmic drugs was performed under control of repetitive intracardiac electrophysiological studies. Duration of follow-up was 28 (13 - 61) months. According to ROC-analysis most precise markers of positive results of drug testing were values of local ejection fraction (EF) in apical LV segment (10th segment on RNV) above 55%. Signs predisposing to absence of MVT recurrences during long term use of antiarrhythmic drugs were lack of mitral regurgitation (above I degree) according to echocardiography data, values of local EF in segment of lateral LV wall (4th segment on RNV) exceeding 42%, or value of LV end diastolic volume less than 365 ml according to RNV data. Parameters of local LV contractility are most precise markers of results of the use of antiarrhythmic drugs in patients with MVT, their diagnostic value is hair than that of global LVEF. Efficacy of antiarrhythmic drugs at electrophysiologic testing and long term follow-up are associated with different parameters of local LV contractility.

[Clinical significance of myocardial lesion in antiphospholipid syndrome].

Antiphospholipin syndrome (APLS) is one of the most frequent reasons for arterial and venous thromboses. Primary and secondary APLS can lead to thrombotic process in coronary arteries. The biggest diagnostic difficulties appear in cases of small coronary vasal involvement leading to diffuse myocardial lesion. Perfusion myocardial scintigraphy (MS) allows specification of the character of myocardial changes. Revealing of myocardial changes by means of MS makes it possible to start timely anticoagulative therapy, which significantly improves prognosis and life quality.

[Diagnostics and treatment of the functional autonomy of the thyroid gland].

Functional autonomy of the thyroid gland (FATG) remains an important problem due to iodine deficiency over the whole territory of Russian Federation and the fact that iodine prophylaxis has not been conducted for a long period of time. Since iodine deficiency affects the thyroid gland (TG) as a whole, the formation of nodules is not limited to a single lobe, but leads to the development of multinodular goiter, the main feature of which is morphological and functional heterogeneity of TG tissue. Active cell proliferation in the iodine deficiency related goiter is associated with an elevated risk of somatic mutations, including activating mutations leading to thyrocyte functional autonomy. Diagnosis is often late due to the absence of any specific complaints in most patients. Hence, the pathology is often an accidental finding and is mostly revealed in patients with cardial diseases. For a long time, TG scintigraphy has been the only method allowing the diagnosis of FATG, while other techniques, such as ultrasonography and the measurement of the hormone levels in blood, only make it possible to assume the presence of this pathology. Nowadays, fine needle biopsy plays an important role since it gives a possibility to obtain direct cell-level findings concerning the morphological structure of TG nodules and, in some cases, the functional condition of cells. Of late, great importance to clinical practice has been attached to new developments in FATG diagnostics, immunomorphological (immunocytochemical and immunohistochemical) techniques in particular. Most probably, new techniques of FATG verification will be based upon the study of TSHR as the specific marker of this pathology, and it will be possible to use immunocytochemical assay as an additional diagnostic method for the verification of autonomic nodular formations as early as right after the puncture.

[Single-photon emission computed tomography in diagnosis of left ventricular post-infarction aneurysm]. / Odnofotonnaia émissionnaia tomografiia miokarda v diagnostike postinfarktnoi anevrizmy levogo zheludochka.

AIM: To assess value of single-photon emission computed tomography with (99m)Tc-sestamibi ((99m)Tc MIBI SPECT) for detection of post-infarction left ventricular aneurysm in patients with coronary artery disease. MATERIAL: Fifty nine patients were operated upon because of coronary heart disease. In 27 patients with post-infarction left ventricular aneurysm modified Dor operation (aneurysmectomy with endoventricular circular patch plasty reconstruction) was performed. In 24 patients with extended cardiosclerosis direct myocardial revascularization was conducted. RESULTS AND CONCLUSION: Comparison of (99m)Tc MIBI SPECT data obtained at rest and during dobutamine stress test revealed some diagnostic features characteristic of patients with left ventricular post-infarction aneurysm. Quantitative and qualitative assessment of viability of myocardium in basal and "borderline" zones influenced not only the volume of revascularization, it was important for preliminary determination of post-infarction left ventricular aneurysm resection level, selection of type of plasty, prognostication of complications in immediate and remote postoperative periods.

[The place of multispiral computed tomography in diagnostics of ischemic heart disease.]. / Mesto mul'tispiral'noi komp'iuternoi tomografii v diagnostike ishemicheskoi bolezni serdtsa.

Multispiral computed tomography (CT) of the heart with detection of coronary artery calcification as well as clinical examination, stress tests, single photon emission computer tomography at rest and during stress, and/or stress echocardiography were used in the study of 56 patients with and 26 without ischemic heart disease (mean age 58+/-1 and 55+/-1.5 years, respectively). In 44 patients multispiral CT was performed with intravenous contrast injection and 27 patients were subjected to coronary angiography. Specificity, sensitivity of multispiral CT for detection of hemodynamically significant (>50%) stenoses compared with angiography and for diagnosis of structural changes of the myocardium were 92, 90%, and 89, 91%, respectively. Calcium index was significantly higher in patients with than without ischemic heart disease (253+/-28 and 3+/-1.5 U, respectively) and could be a significant diagnostic criterion of ischemic heart disease.

[Complex radiological diagnosis of pulmonary thromboembolism]. / Kompleksnaia luchevaia diagnostika tromboémbolii legochnoi arterii.

Three hundred and twelve patients (210 males and 102 females) aged 47 to 69 years who had suspected pulmonary thromboembolism (PTE) were examined. All the patients received hospital treatment in the clinics of I.M. Sechenov Moscow Medical Academy for different diseases, such as coronary heart disease, chronic venous insufficiency, hypertensive disease, and postoperative condition. PTE was ascertained in 209 patients. The examination used a "Hi Speed CT/i" spiral computer tomograph (General Electric) and an "Aquilion" computer tomograph (Toshiba), a "Millennium" gamma-chamber, and a "Sequoia" high-resolution ultrasound apparatus (Acuson). An algorithm of examination of patients with suspected PTE has been developed.

[The cardiovascular system of patients with lymphogranulomatosis in long-time remission]. / Sostoianie serdechno-sosudistoi sistemy u bol'nykh limfogranulematozom v stadii dlitel'noi remissii.

AIM: To study the cardiovascular system in patients with lymphogranulomatosis (LGM) at prolonged remission. MATERIALS AND MATERIALS: 78 patients received radiation and chemotherapy for lymphogranulomatosis in 1973 to 1995. All the patients underwent electro- and echocardiography (EchoCG), 40 patients had radioisotopic ventriculography (RVG). RESULTS: EchoCG revealed nonspecific changes in the ST segment in 19 (24.3%) patients. Systolic function was decreased vto 46% only in 1 patient. RVG showed a significant reduction in ejection fraction in the experimental group as compared to the control one (55.5 +/- 9.5 and 68.4 +/- 8.0, respectively; p < 0.05). CONCLUSION: Cardiac disorders revealed in patients with LGM at prolonged remission are mainly insignificant. The risk for cardiac events is not a guide for reducing radiation doses and the intensity of chemotherapy.

[Gated 99mTC-MIBI SPECT in assessment of left ventricle global contractility]. / Sinkhronizirovannaia tomostsintigrafiia miokarda s 99TC-MIBI v otsenke obshchei sokratitel'noi funktsii levogo zheludochka.

The complex radiodiagnostic study (synchronized 99mTc-MIBI SPECT and equilibrum ventriculography) has been made in 30 pts. The opportunity of use synchronized tomoscintigraphy for an estimation of a global left ventricle ejection fraction was shown.

[Efficiency of antiarrhythmic therapy and resistance to it in patients with malignant ventricular tachyarrhythmias: formulation of prognosis]. / Effektivnost' antiaritmicheskoi terapii i refrakternost' k nei u bol'nykh so zlokachestvennymi zheludochkovymi takhiaritmiiami: postroenie prognoza.

AIM: To study feasibility of predicting results of drug antiarrhythmic therapy in patients with malignant ventricular arrhythmias (MVA) basing on the results of clinical and device examinations. MATERIALS AND METHODS: 136 patients with documented MVA entered the study. 100 patients were in the retrospective analysis group, 36 patients comprised the study group. All the patients underwent physical examination, resting ECG, chest x-ray, radionuclide ventriculography. Intracardiac electrophysiological examination, Holter 24-h ECG monitoring and bicycle exercise provided data for diagnosis of MVA and control over effects of antiarrhythmic drugs. The mathematical model was derived using discriminant analysis. RESULTS: Significant differences were obtained in patients with positive and negative results of drug testing by the number and recurrence time of ventricular tachycardia (VT) for 3 years, survival, cardiac and sudden death, some other parameters. A mathematic model has been designed which allows prognosis of the results of antiarrhythmic therapy (AAT) in patients with MVA. 7 independent predictors of AAT efficiency are shown: left ventricular ejection fraction, duration of P-Q interval, cardiac failure, left ventricular aneurysm, age, number of VT morphologies, insufficiency of aortic valve. Verification of the model on the study group patients showed that prognostic accuracy of the model was 82%. CONCLUSION: The results of the drug tests predict life span of MVA patients. The developed mathematical model allows prediction of AAT results in such patients before the pharmacological test with accuracy 82-87%. The model can help objectivize indications to use of non-pharmacological methods for each patient basing on prognosis of resistance to drug AAT.

[An intensive regimen of supersonic-frequency-current therapy in chronic inflammation of the adnexa uteri]. / Intensivnyi rezhim TNCh-terapii pri khronicheskom vospalenii pridatkov matki.

As shown by clinical, electrophysiological and hormonal examinations of 34 patients with chronic inflammation of the uterine appendages and control subjects, the disease treatment with intensive low-frequency current has no negative effects on cardiovascular and endocrine systems, improves function of the reproductive system. Intensive low-frequency currents are believed to be a useful modality in management of chronic genital inflammation.

[Myocardial tomoscintigraphy with 201Tl in the diagnosis of cardiac involvement in systemic scleroderma]. / Tomostsintigrafiia miokarda s 201Tl v diagnostike porazheniia serdtsa pri sistemnoi sklerodermii.

Myocardial microcirculation was assessed in 23 scleroderma systematica patients using 201Tl tomoscintigraphy at rest (23 patients) and upon dipyridamole vasodilation (6 patients). Most patients were found to have impaired blood supply of the myocardium with sites of myocardiofibrosis. In response to dipyridamole introduction improved myocardial perfusion was registered in 4 out of 6 patients, 1 patient showed signs of transient myocardial ischemia, 1 patient had stable defects of myocardial blood supply. It is suggested that some sclerodermic patients may benefit from vasodilators.

"Silent" myocardial ischaemia in essential hypertension.

During 16-h electrocardiographic monitoring of 29 patients with stage II (WHO) essential hypertension and left ventricular hypertrophy, the episodes of painless "silent" myocardial ischaemia were registered in 12. In these patients a bicycle ergometry test, transoesophageal atrial pacing, isotope ventriculography at rest and during isometric exercise, and coronary angiography in 3 cases, were performed. A comprehensive evaluation of findings revealed the presence of coronary insufficiency not accompanied by pain syndrome. Absence of angiographic signs of coronary stenosis in 3 patients subjected to coronary angiography suggests the presence of relative coronary insufficiency at least in these patients.

[Differential diagnosis of primary vascular pulmonary hypertension]. / K differentsial'noi diagnostike pervichno-sosudistoi legochnoi gipertonii.

The paper is concerned with 18 patients suffering from primary vascular pulmonary hypertension. Of these, 7 patients had the thromboembolic pattern of primary vascular pulmonary hypertension and 11 primary pulmonary hypertension. All the patients underwent comprehensive examinations including routine clinical examinations, x-raying of the chest organs, ECG, ultrasound cardiography, measurement of the pressure in the pulmonary artery by invasive and non-invasive methods. The patients were also subjected to radionuclide studies using radionuclide scintigraphy of the lungs by means of 99mTc microspheres and radionuclide phlebography. Analysis of the clinical picture and of the findings obtained with the majority of instrumental methods including radionuclide scintigraphy did not reveal any appreciable differences between the patients with the thromboembolic pattern of primary vascular pulmonary hypertension and those suffering from primary pulmonary hypertension. Radionuclide phlebography has demonstrated that the patients with the thromboembolic pattern showed the changes in the phlebogram significantly more often than those with primary pulmonary hypertension. It is advisable that radionuclide phlebography should be included into the schedule for examining patients with primary vascular pulmonary hypertension.