RESUMO
LEOPARD syndrome with multiple lentigines (cardiomyopathic lentiginosis) is a rare, genetically predetermined disease with autosomal dominant inheritance. Prevalence of this syndrome is unknown. One of pathognomonic clinical manifestations of this syndrome is the presence of multiple lentiginous pigment spots all over the body. The most common cardiac manifestation (approximately 80%) is myocardial hypertrophy. We presented a rare clinical case of detecting LEOPARD syndrome with multiple lentigines in a 32-year old female patient with major manifestations evident as pronounces morpho-functional alterations, myocardial hypertrophy, and heart rhythm disorders.
Assuntos
Síndrome LEOPARD , Adulto , Feminino , Coração , HumanosRESUMO
Aim of the study was to assess effects of cardiac shock wave therapy (CSWT) in patients with coronary artery disease (CAD) with refractory stable angina pectoris. Seventeen CAD patients with refractory II-IV class angina (3 women and 14 men, mean age 67.4+/-8.6 years) received the course of 9 procedures of CSWT. All patients had I-III New York Heart Association (NYHA) class congestive heart failure. Before and after CSWT medical examination with life quality assessment by means of the Minnesota Living Questionnaire, echocardiography, veloergometry, myocardial perfusion imaging with single-photon emission computed tomography (SPECT) using 99M-Tc-methyl-iodine-benzyl-guanydin (MIBG) and Holter ECG monitoring was performed. The dynamics of pro-angiogenic factors (VEGF, HGF, FGF-) were also measured by ELISA, and of brain natriuretic peptide (Nt-proBNP) by the electrochemoluminescence method. Most patients (80%) had significant life quality (<0.01) and myocardial perfusion improvement. Episodes of angina pectoris and nitrate intake were more than twice decreased. There was a significant increase in exercise tolerance (p<0.01). Holter ECG monitoring showed decreasing of an average heart rate (p<0.02); no worsening of previous cardiac arrhythmias was observed. The significant (p<0.05) decreases in plasma Nt-proBNP and increases in VEGF concentration were revealed after CSWT. CSWT procedures were well tolerated. The results of our study confirm high effectiveness and safety of CSWT in complex treatment of patients with CAD, resistant angina pectoris, including patients after myocardial revascularization and with heart failure.
Assuntos
Isquemia Miocárdica/terapia , Terapia por Ondas Curtas/métodos , Idoso , Angiografia Coronária , Eletrocardiografia Ambulatorial , Feminino , Humanos , Masculino , Isquemia Miocárdica/diagnóstico , Tomografia Computadorizada de Emissão de Fóton Único , Resultado do TratamentoRESUMO
Aim of the study was to assess effect of myocardial revascularization on levels of factors of angiogenesis in early and remote period after intervention. Main group comprised 228 patients with ischemic heart disease (n=228, 194 men, 34 women, mean age 57+/-8.7 years). Coronary bypass surgery was carried out in 29 patients while other 199 were subjected to percutaneous coronary intervention (PCI). Analysis of data was performed in the group as a whole and in 2 subgroups distinguished in dependence on type of invasive treatment. Levels of factors of angiogenesis - vascular endothelial growth factor (VEGF), transforming growth factor beta (TGFbeta), and endostatin - were measured before, in 6 days, and 6 months after invasive treatment. Compared with healthy persons patients with IHD had significantly higher level of VEGF and significantly lower levels of TGFbeta and endostatin. On day 6 after revascularization in the group as a whole level of VEGF insignificantly rose while level of TGFbeta insignificantly decreased. In 6 months after invasive treatment significant lowering of VEGF level and significant increase of TGFbeta was noted. Endostatin level was measured at baseline and in 6 months after invasive treatment. Significant elevation of endostatin level took place after 6 months. Thus PCI and coronary bypass surgery lead to lowering of VEGF level and elevation of levels of TGFbeta in 6 days after intervention. In remote period reverse dynamics was observed: of VEGF level rose and those of TGFbeta and endostatin increased.
Assuntos
Ponte de Artéria Coronária , Circulação Coronária , Isquemia Miocárdica/sangue , Idoso , Indutores da Angiogênese/sangue , Endostatinas/sangue , Feminino , Seguimentos , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Isquemia Miocárdica/fisiopatologia , Isquemia Miocárdica/cirurgia , Receptores de Fatores de Crescimento do Endotélio Vascular/sangue , Fator de Crescimento Transformador beta/sangue , Fator A de Crescimento do Endotélio Vascular/sangueRESUMO
The aim of our investigation was to assess the efficacy of enhanced external counterpulsation (EECP) in ischemic heart disease (IHD) patients depending on previous by-pass surgery. 30 IHD patients (pts) were treated with EECP using standard protocol (35 hours, 1 hour each procedure). All patients were divided into 2 groups. 1-st group (n=15) - pts after by-pass surgery, 2-nd group (n=15) - pts without previous coronary revascularisation. Pts were examined by ECG, echocardiography, bicycle test, radionuclide perfusion scintigraphy (SPECT) before and after EECP treatment. There was significant decreasing of perfusion defect severity for rest SPECT after EECP treatment (64.93+/-13.21 std and 58.53+/-17.28 std, respectively, p<0.01) and decreasing of size of perfusion defects - 26.10+/-15.76% before and 22.63+/-18.54% after EECP (p<0.05). Size and severity of perfusion defects estimated by SPECT at stress conditions didn't change. After EECP treatment the decreasing of perfusion defect severity (p<0.01) was registered in both groups, the decreasing of size of perfusion (p<0.05) - only in the 1-st group. In the 1-st group decreasing of perfusion defect severity and size of perfusion was more intensive. The efficacy of EECP depends on previous coronary revascularization.
Assuntos
Doença da Artéria Coronariana/terapia , Contrapulsação/métodos , Cuidados Pós-Operatórios/métodos , Doença da Artéria Coronariana/diagnóstico , Doença da Artéria Coronariana/fisiopatologia , Ecocardiografia , Eletrocardiografia , Teste de Esforço , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Revascularização Miocárdica , Índice de Gravidade de Doença , Tomografia Computadorizada de Emissão de Fóton Único , Resultado do TratamentoRESUMO
We studied morphological characteristics of platelets and parameters of platelet aggregation in patients with dilation cardiomyopathy. Augmented aggregatory activity of platelets was found in 76% of patients. In blood of majority of patients we found circulating leukocyte-platelet aggregates. This evidenced for development of inflammatory process and could be related to disturbances of blood rheology. In 2 patients examined by virusological method we revealed presence of a virus inside platelets. This phenomenon might serve as one of possible pathological pathways of disease progression at the account of spread of viral infection along vascular bed during thrombus formation.
Assuntos
Cardiomiopatia Dilatada/sangue , Hemostasia/fisiologia , Transtornos Hemostáticos/sangue , Plaquetas/metabolismo , Cardiomiopatia Dilatada/complicações , Progressão da Doença , Feminino , Transtornos Hemostáticos/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Agregação Plaquetária , Fatores de RiscoRESUMO
AIM OF THE WORK: To assess effect of programmed external counterpulsation (ECP) on clinico-functional status, quality of life of patients with chronic heart failure (CHF) of ischemic origin, stable functional class (FC) II-IV angina refractory to drug therapy. MATERIAL AND METHODS: Patients (n=30) with ischemic heart disease (IHD) stable FC II-IV angina (mean age 65.5 +/- 7.9 years) went though complete curative course of ECP which included 35 1-hour procedures 5-6 times a week. Signs of CHF NYHA FC I-II were noted in 18, FC III-IV - in 12 patients. In 8 patients left ventricular (LV) ejection fraction (EF) did not exceed 35%. Methods of investigation used before and after course of ECP included clinical examination, echoCG, VEM-test, perfusion myocardial scintigraphy with 99MTc-MIBI, 24-hour ECG monitoring, assessment of quality of life with the use of Minnesota questionnaire and of dynamics of plasma content of natriuretic peptides (pro-ANP and pro-BNP) measured by immunoenzyme assay. RESULTS: Absolute majority of patients noted improvement of self feeling with significant (p < 0.001) betterment of parameters of quality of life. Number of attacks of angina and nitrate requirement decreased more than two times. Significantly (p < 0.01) rose tolerance to physical work load. In most patients decreases of depth and extent of myocardial perfusion defects were noted. With this positive dynamics of functional status, quality of life and severity of angina was noted irrespective of manifestations of heart failure and degree of suppression of LV contractile function. Significant (p < 0.001) improvement of contractile function of the myocardium according to data of echoCG was noted predominantly in patients with initial LVEF < 35%. According to data of 24-hour ECG monitoring significant (p < 0.01) decrease of average heart rate was also noted. CONCLUSION: The obtained results evidence for high efficacy and safety of ECP in complex treatment of patients with CHF of ischemic origin with resistant to drug therapy angina pectoris.
Assuntos
Contrapulsação/métodos , Insuficiência Cardíaca/terapia , Idoso , Ecocardiografia , Eletrocardiografia , Feminino , Seguimentos , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/fisiopatologia , Humanos , Imunoensaio , Masculino , Peptídeos Natriuréticos/sangue , Qualidade de Vida , Volume Sistólico/fisiologia , Inquéritos e Questionários , Tomografia Computadorizada de Emissão de Fóton Único , Resultado do Tratamento , Função Ventricular Esquerda/fisiologiaRESUMO
Stimulatiion of coronary angiogenesis in patients with IHD appears to be one of perspective directions in contemporary cardiology. Therefore objective assessment of results with the help of instrumental methods of investigation is important. For assessment of the state of coronary vascular bed and myocardial perfusion in the clinic one can use coronarography, single photon emission computer tomography, positron emission tomography, magnetic resonance tomography, and contrast echocardiography. Novel techniques of visualization are elaborated at present with consideration of peculiarities of mechanisms and use of molecular markers of angiogenesis: visualization of integrin avb3, VEGF-receptors, visualization of activity of matrix metalloproteinases.
Assuntos
Circulação Colateral/fisiologia , Circulação Coronária/fisiologia , Isquemia Miocárdica/diagnóstico , Neovascularização Fisiológica/fisiologia , Animais , Angiografia Coronária , Progressão da Doença , Ecocardiografia , Humanos , Imageamento por Ressonância Magnética , Isquemia Miocárdica/fisiopatologia , Prognóstico , Tomografia Computadorizada de Emissão de Fóton ÚnicoRESUMO
AIM: To study interrelation of main clinico-hemodynamic parameters and levels of natriuretic peptides (NUP) in patients with hypertrophic cardiomyopathy (HCMP). MATERIAL AND METHODS: We measured concentrations of N-terminal fragments of precursors of plasma brain and atrial natriuretic peptides (NT-proBNP and NT-proANP) and conducted complex echocardiographical examination in 94 patients (mean age 42.3 +/- 133.8 years) with obstructive (n=43) and nonobstructive (n=51) HCMP. RESULTS: Content of NUP did not depend on patients age, contractile function and dimensions of the left ventricle (LV), moderate and significant correlation between content of NT-proBNP, NT-proANP and type of ventricular filling (r=0.45 and 0.46, respectively), heart failure functional class (r=0.41 and 0.44, respectively), severity of LV hypertrophy (r=0.42 and 0.34, respectively), left atrial dimension (r=0.26 and 0.35, respectively), magnitude of pressure gradient in LV outflow tract (r=0.35 and 0.26, respectively). Degree of mitral regurgitation correlated significantly only with NT -proBNP level (r=0.34). The patients who eventually died (n=6) were characterized by higher plasma content of NUP. CONCLUSIONS: In patients with HCMP elevated plasma content of N-terminal precursors of brain and atrial natriuretic peptides are associated with severity of LV diastolic dysfunction and hypertrophy of LV as well as of hemodynamic and functional disturbances.
Assuntos
Fator Natriurético Atrial/metabolismo , Cardiomiopatia Hipertrófica/metabolismo , Cardiomiopatia Hipertrófica/fisiopatologia , Adulto , Cardiomiopatia Hipertrófica/epidemiologia , Feminino , Humanos , Hipertrofia Ventricular Esquerda/epidemiologia , Hipertrofia Ventricular Esquerda/metabolismo , Hipertrofia Ventricular Esquerda/fisiopatologia , MasculinoRESUMO
The aim of our study was to assess the influence of rosuvastatin on coronary angiogenesis. 30 male patients with chronic coronary heart disease and total cholesterol level > 5.2 mmol/l were treated with rosuvastatin 10 mg daily during 3 months. The serum level of total cholesterol (TC), LDL-cholesterol (LDL-C), HDL-cholesterol (HDL-C), triglycerides (TG) as well as C-reactive protein (CRP) and interleukin-6 (IL-6), vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) were measured initially and in 3 months. There was the significant decreasing of TC, LDL-C and TG concentrations. IL-6 and CRP serum levels were also decreased after rosuvastatin therapy. Three months of treatment resulted to significant decrease of VEGF with no changes of bFGF levels. The correlation was not found between CRP and VEGF levels and between IL-6 and VEGF levels. Also there was no correlation between the degree of decreasing CRP and VEGF, and IL-6 and VEGF. So we have shown significant decreasing of VEGF serum levels on rosuvastatin therapy. It could be possible mechanism of plaque stabilization in patients with coronary heart disease.
Assuntos
Fluorbenzenos/uso terapêutico , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Isquemia Miocárdica/tratamento farmacológico , Neovascularização Fisiológica/efeitos dos fármacos , Pirimidinas/uso terapêutico , Sulfonamidas/uso terapêutico , Idoso , Fatores de Crescimento de Fibroblastos/sangue , Fatores de Crescimento de Fibroblastos/metabolismo , Fluorbenzenos/farmacologia , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/farmacologia , Lipídeos/sangue , Masculino , Pessoa de Meia-Idade , Pirimidinas/farmacologia , Rosuvastatina Cálcica , Sulfonamidas/farmacologia , Resultado do Tratamento , Fator A de Crescimento do Endotélio Vascular/sangue , Fator A de Crescimento do Endotélio Vascular/metabolismoRESUMO
We studied the influence of rosuvastatin therapy and myocardial revascularization on angiogenic growth factors in coronary artery disease (CAD) patients. Two main groups were examined: the first one consisted of patients passed through successful percutaneous coronary intervention (PCI), the second one consisted of patients on 3 months rosuvastatin therapy. Vascular endothelial growth factor (VEGF), VEGF receptor Flt-1 (sVEGF-Rl) and transforming growth factor-beta (TGF-bl) levels were measured in healthy volunteers and CAD patients before and 6 days after myocardial revascularization by PCI. VEGF and basic fibroblast growth factor (bFGF) levels were measured before and 3 month after rosuvastatin therapy, as well as total cholesterol (TC), triglycerides (TG), low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDLC), C-reactive protein (CRP), interleukin 6 (IL-6) and endothelium dependent vasodilation. VEGF levels did not differ, but TGF - b levels were significantly lower in CAD patients compared to healthy subjects (11.0 +/- 4.9 pg/ml and 120.0 +/- 32.4 pg/ml, respectively, p < 0.000l). Myocardial revascularization caused changes in VEGF levels from 192.4 +/- 166.1 pg/ml to 264.7 +/- 226.6 pg/ml (p=0.0066) without significant influence on TGF and VEGF-R1 levels in 6 days. There were positive changes in lipid levels, lowering of CRP and IL-6 concentrations, improvement of endothelial function and decrease of VEGF levels from 382 +/- 249 pg/ml to 297 +/- 220 pg/ml (p=0.006) 3 month after start of rosuvastatin treatment (no changes in bFGF levels were observed). Chronic insufficient myocardial blood supply leads to decreasing of TGF - b levels. The elevation of VEGF after myocardial revascularization reflects transient ischemia and potentially may provoke hemodynamic instability, caused by atherosclerotic plaque neovascularization. Strengthening of statin therapy early after myocardial revascularization may allow to stabilize the atherosclerotic plaque condition, also by means of VEGF lowering.
Assuntos
Anticolesterolemiantes/farmacologia , Anticolesterolemiantes/uso terapêutico , Fluorbenzenos/farmacologia , Fluorbenzenos/uso terapêutico , Isquemia Miocárdica/complicações , Isquemia Miocárdica/tratamento farmacológico , Revascularização Miocárdica/métodos , Neovascularização Patológica/tratamento farmacológico , Pirimidinas/farmacologia , Pirimidinas/uso terapêutico , Sulfonamidas/farmacologia , Sulfonamidas/uso terapêutico , Colesterol/metabolismo , Feminino , Hemodinâmica/efeitos dos fármacos , Humanos , Masculino , Pessoa de Meia-Idade , Neovascularização Patológica/etiologia , Receptores de Fatores de Crescimento do Endotélio Vascular/sangue , Receptores de Fatores de Crescimento do Endotélio Vascular/efeitos dos fármacos , Rosuvastatina Cálcica , Fator de Crescimento Transformador beta1/sangue , Fator de Crescimento Transformador beta1/efeitos dos fármacos , Triglicerídeos/metabolismo , Fator A de Crescimento do Endotélio Vascular/sangue , Fator A de Crescimento do Endotélio Vascular/efeitos dos fármacosRESUMO
AIM: A detailed description of immune status abnormalities of adult patients with heart arrhythmia either idiopathic or in combination with primary heart disease such as chronic myocarditis and dilated cardiomyopathy (DCMP). MATERIAL AND METHODS: Eighty two consecutive patients aged 16-57 years admitted to the L.A. Myasnikov Institute of Clinical Cardiology (Moscow) for heart arrhythmia were studied. Among them 35 patients had idiopathic heart arrhythmia (IHA, group 1) with no evidence of any primary heart disease, while other 47 patients (group 2) had heart arrhythmia combined with primary heart disease (chronic myocarditis or DCMP). In group 1 ventricular arrhythmia was recorded in 27 patients (12 cases with ventricular tachyarrhrythmia ?VTA, 15 cases with ventricular extrasystolia- VE). Supraventricular heart arrhythmia was found in 6 patients (3 cases of constantly recurring supraventriccular tachycardia, 2 cases of paroxysmal and 1 with constant atrial fibrillation). The intermittent atrioventricular block of the second-third degree was recorded in 2 patients. The patients of group 2 were divided into subgroups 2a, 2b and 2c. In subgroup 2a (patients with DCMP without signs of heart failure) ventricular arrhythmia was found in 7 patients (VT ? 5, VE ? 2). Supraventricular arrhythmia was recorded in 7 patients 5 of which had constantly recurring supraventricular tachycardia, 1 ? paroxysmal and 1 constant atrial fibrillation. In subgroup 2b (DCMP patients with obvious signs of heart failure) ventricular arrhythmia was recorded in 12 patients, among them 6 had VT and 6 ? VE, 2 ? constant atrial fibrillation). In subgroup 2c (patients with chronic myocarditis) ventricular arrhythmia was in 7 patients (VT ? 5, VE ? 2), constant atrial fibrillation ? in 2, heart conduction abnormalities ? in 3 patients, atrioventricular block of the first or second degree ? in 2, sick sinus syndrome ? in 1. To verify the diagnosis, all the patients have undergone physical examination, blood cell counts and biochemical tests, urine clinical analysis, ECG and ultrasound heart examination as well as 24h ECG monitoring. On demand, bicycle exercise test or treadmill test, coronaroangiography, endomyocardial biopsy and invasive electrophysiological examination were made. RESULTS: Immune status abnormalities found in patients with heart arrhythmia both idiopathic and combined with primary heart diseases such as chronic myocarditis and DCMO correspond to immune defense response during chronic infection. Activation of different anti-infection defense mechanisms was recorded in patients with idiopathic heart rhythm and conductivity abnormalities. Immune deficiency was found in arrhythmia and conductivity abnormalities combined with primary heart diseases (chronic myocarditis or DCMP). A positive correlation exists between the degree of immune defense failure and reduction of myocardial contractility. CONCLUSION: There exists a characteristic pattern of immune status abnormalities in patients with arrhythmia, both idiopathic or combined with primary heart disease (myocarditis, DCMP). The abnormalities depend on severity of arrhythmia, intensity of inflammatory processes in the myocardium and on the degree of left ventricular contractility dysfunction in patients with primary heart diseases.
Assuntos
Anticorpos Anti-Idiotípicos/imunologia , Arritmias Cardíacas/imunologia , Cardiomiopatia Dilatada/complicações , Imunidade Celular , Imunoglobulina G/imunologia , Miocardite/complicações , Linfócitos T/imunologia , Adolescente , Adulto , Arritmias Cardíacas/etiologia , Relação CD4-CD8 , Cardiomiopatia Dilatada/imunologia , Doença Crônica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miocardite/imunologia , PrognósticoRESUMO
AIM: To examine effects of programmed external contrapulsation (ECP) on the clinico-functional status, quality of life (QOL) in patients with coronary heart disease (CHD), stable angina of FC II-IV resistant to medication. MATERIAL AND METHODS: Eighteen patients with CHD, stable angina of FC III-IV (2 females and 16 males, mean age 63.6 +/- 7.4 years) have completed a course of ECP including 35 one-hour procedures 5-6 times a week for 7 weeks. Ten patients had cardiac failure (NYHA FC II-III). Before and after ECP course the patients were examined using QOL assessment by Minnesota questionnaire, complex echo-cardiography, bicycle exercise test, perfusion myocardial scintigraphy with 99m-Tc-4,2-methoxy-isobutilisonitril, 24-h Holter ECG monitoring, enzyme immunoassay for plasma natriuretic propeptides. RESULTS: Significant subjective QOL improvement (p < 0.01) was noticed by all the patients. Anginal attacks and nitrates doses reduced at least 2-fold. Exercise tolerance rose significantly (p < 0.01), bicycle exercise test was positive in 5 patients, the rest stopped the test after achievement of submaximal heart rate (HR) and fatigue. Most of the patients exhibited improvement of myocardial perfusion. Patients with abnormal myocardial contractility showed a moderate trend (p < 0.03) to an increase in left ventricular ejection fraction. By 24-h ECG, mean HR diminished significantly (p < 0.02). CONCLUSION: ECP is highly effective and safe in combined therapy of CHD, stable angina resistant to drug therapy, in impossibility of myocardial revascularization, including patients with cardiac failure. This manifests in a significant abatement of angina, lower doses of nitrates, improvement of exercise tolerance, quality of life, myocardial perfusion and hemodynamic indices.
Assuntos
Doença das Coronárias/terapia , Contrapulsação/métodos , Angiografia Coronária , Doença das Coronárias/diagnóstico , Doença das Coronárias/fisiopatologia , Ecocardiografia , Eletrocardiografia Ambulatorial , Teste de Esforço , Feminino , Seguimentos , Frequência Cardíaca/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Contração Miocárdica/fisiologia , Projetos Piloto , Volume Sistólico/fisiologia , Resultado do TratamentoRESUMO
Dynamics of fatty acid binding protein (FABP) and pro-brain natriuretic peptide (pro-BNP) levels was studied in patients with ischemic heart disease at rest, during transitory myocardial ischemia, and before and after balloon angioplasty. Forty patients were included: 25 patients with stable angina comprised the study group and 15 patients with acute myocardial infarction (AMI) comprised control group. No significant elevation of FABP was revealed after myocardial revascularization, a tendency was noted to elevation of FABP after transitory ischemia. At the background of stress test pro-BNP level significantly rose. Comparison of FABP levels during first 24 hours of AMI and in patients with IHD both at rest and after veloergometry showed that FABP level in AMI was significantly higher. On day 21 of AMI FABP level became lower and did not differ significantly from this parameter in patients with IHD. Data of this work confirm that search of markers of myocardial ischemia in peripheral blood of patients with IHD is justified, and investigation of their role can allow not only to elevate diagnostic value of stress test, but to assess prognosis and to supplement algorithm of examination of patients with suspected IHD.
Assuntos
Teste de Esforço , Peptídeo Natriurético Encefálico , Angioplastia Coronária com Balão , Proteínas de Ligação a Ácido Graxo , Humanos , Isquemia Miocárdica/diagnóstico , Peptídeo Natriurético Encefálico/sangueAssuntos
Bloqueio de Ramo/diagnóstico por imagem , Cardiomiopatia Dilatada/diagnóstico por imagem , Miocárdio/metabolismo , Tomografia por Emissão de Pósitrons , Adulto , Bloqueio de Ramo/complicações , Bloqueio de Ramo/metabolismo , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/metabolismo , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/metabolismo , Humanos , Masculino , Prognóstico , Compostos Radiofarmacêuticos/farmacocinética , Índice de Gravidade de Doença , Tecnécio Tc 99m Sestamibi/farmacocinéticaRESUMO
One of most widely spread causes of hypertrophic cardiomyopathy (HCMP) is mutation in cardiac beta-myosin heavy chain gene. Data on contribution of this mutation to development of HCMP in Russian patients are very limited. We conducted screening of beta-myosin heavy chain gene for the presence of mutations in 116 patients with confirmed HCMP (probands). DHPLC was used with subsequent sequencing of DNA fragments. Genetic defects of beta-myosin heavy chain were found more than in every 10-th patient. These defects were represented by 13 mutations (Ala729Pro mutation was found twice). Phenotypes of majority of known mutations in Russian population did not differ substantially from their phenotypes in other populations. Six mutations had not been previously described; most of them were associated with especially severe clinical and hemodynamic signs and relatively unfavorable course of the disease. Thus beta-myosin heavy chain gene mutation play important role in etiology of HCMP in patients in Russia.
Assuntos
Cardiomiopatia Hipertrófica/genética , DNA/genética , Mutação , Cadeias Pesadas de Miosina/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Miosinas Cardíacas , Cardiomiopatia Hipertrófica/epidemiologia , Cardiomiopatia Hipertrófica/metabolismo , Cromatografia Líquida de Alta Pressão , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Polimorfismo Genético , Federação Russa/epidemiologiaRESUMO
AIM: To study peculiarities of myocardial perfusion in patients with hypertrophic cardiomyopathy (HCMP) in correlation with clinical and echocardiographic data. MATERIAL AND METHODS: 62 patients with HCMP (23 females and 39 males, mean age 44.4 +/- 11.2 years, the disease duration 13.0 +/- 10.4 years) have undergone ECG, 24-h ECG monitoring, echocardiography, perfusion scintigraphy of the myocardium with 99m-TcMIBI at rest and in combination with bicycle ergometry. The patients were divided into two groups: 35 patients of group 1 had moderate left ventricular hypertrophy (the septal thickness in diastole under 20 mm; 27 patients of group 2 had severe hypertrophy (the thickness was over 20 mm). RESULTS: Dyspnea and syncopal states occurred more frequently in patients from group 2. They also had a higher functional class of heart failure (2.0 +/- 0.8 and 1.2 +/- 0.7 for group 1 and 2, respectively, p < 0.05). Cardiac performance was significantly higher in patients of group 1. The size of the left atrium, left ventricular myocardium mass, the septal thickness and thickness of posterior wall of the left ventricle, gradient of pressure in the outflow tract of the left ventricle proved higher in patients of group 2. Deep stable defects of myocardial perfusion were detected in 5 (15%) patients of group 1 and 10 (37%) patients of group 2. Transient defects of myocardial perfusion were found in 9 (26%) patients of group 1 and 12 (44%) patients of group 2. The index of myocardial ischemia in group 1 patients was significantly lower than in patients of group 2 (3.5 +/- 2.2 and 8.3 +/- 2.5, respectively, p < 0.05). CONCLUSION: Patients with severe hypertrophy of the left ventricle had severe clinical picture, low exercise tolerance, marked hemodynamic changes, more frequent defects of left ventricular perfusion defects compared to patients with moderate hypertrophy of the left ventricular myocardium.
Assuntos
Cardiomegalia/fisiopatologia , Vasos Coronários/fisiopatologia , Fluxo Sanguíneo Regional , Adulto , Cardiomegalia/diagnóstico por imagem , Vasos Coronários/diagnóstico por imagem , Ecocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
The heart is often refereed to as an "beta-adrenergic organ" because beta-adrenergic agonists are powerful stimulants of cardiac contractility. Catecholamines acting through beta-adrenoceptors produce both positive inotropic and chronotropic effects in human heart. It is now generally accepted that in human heart both beta 1- and beta 2-adrenoceptors coexist. beta-Adrenergic transduction system consist of membrane-bound beta-receptors, the effector enzyme adenylyl cyclase and guanine nucleotide-binding transduction (G) proteins. Repeated long-lasting agonist stimulus evokes homologous or heterologous desensitization of transduction system. Chronic heart failure accompanies with decreased responsiveness to beta-adrenoceptor agonists and is thought to exacerbate the loss of cardiac contractility. Depending on the etiology of heart failure abnormalities of the beta-receptor-G protein-adenylyl cyclase system result from a reduced of beta 1-receptors, uncoupling of beta 1- or beta 2-receptors, alteration of G-protein function, or decreased catalytic subunit activity of adenylyl cyclase and enhanced expression of beta-adrenoceptor kinase. The model most widely used is that of circulating lymphocytes that contain a homogeneous population of beta 2-adrenoceptors. The biochemical and pharmacological properties of human lymphocyte beta 2-adrenoceptors are quite comparable to those of heart beta 2-receptors. The analysis of lymphocyte beta 2-adrenoceptor-adenylyl cyclase system can be used as a model for long-term regulation of human cardiac beta 1- and beta 2-adrenoceptors only if serial changes in response to administration of non-selective beta-adrenergic agonists or antagonists are being investigated. This review concentrates on beta-adrenoceptors in human healthy heart and in heart failure and also on lymphocyte beta 2-adrenoceptors and on the changes of these receptors properties under the influence of some cardiotropic drugs.
Assuntos
Insuficiência Cardíaca/fisiopatologia , Coração/fisiologia , Receptores Adrenérgicos beta/fisiologia , Regulação para Baixo/efeitos dos fármacos , Regulação para Baixo/fisiologia , Proteínas de Ligação ao GTP/efeitos dos fármacos , Proteínas de Ligação ao GTP/fisiologia , Coração/efeitos dos fármacos , Insuficiência Cardíaca/tratamento farmacológico , Humanos , Linfócitos/efeitos dos fármacos , Linfócitos/fisiologia , Receptores Adrenérgicos beta/efeitos dos fármacos , Valores de ReferênciaRESUMO
Rhythm and conduction disturbances were compared for 20 patients with dilated cardiomyopathy (group 1) versus 20 patients with chronic myocarditis (group 2). The diagnosis was confirmed at coronary ventriculography and morphological examination of left and right ventricular endomyocardial biopsies. There were minor differences in the parameters studied I, though supraventricular arrhythmia varied more in group 2. The frequency of ventricular arrhythmia appeared higher in group 2 only in circulatory insufficiency stage 1-IIA. Quantitative differences in ventricular and supraventricular arrhythmias between the groups under moderate circulatory insufficiency may result from different morphological changes in the myocardium.
Assuntos
Arritmias Cardíacas/etiologia , Cardiomiopatia Dilatada/complicações , Miocardite/complicações , Adulto , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/fisiopatologia , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/fisiopatologia , Doença Crônica , Diagnóstico Diferencial , Sistema de Condução Cardíaco/fisiopatologia , Frequência Cardíaca , Humanos , Miocardite/diagnóstico , Miocardite/fisiopatologiaRESUMO
Ventricular arrhythmias were analysed in 38 patients with Stages I-IIB heart failure from 24-hour Holter monitoring data obtained before and after digoxin therapy by comparing with the concentrations of catecholamines. There was a direct relationship between the plasma levels of epinephrine and norepinephrine and the severity of ventricular arrhythmias, as well as between the changes in cumulative catecholamine levels and ventricular arrhythmias during digoxin therapy. Virtually in all cases, the antiarrhythmic effect of the drug was accompanied by lower plasma catecholamine concentrations whereas the levels of norepinephrine and epinephrine remained nearly unchanged or increased with the tentatively arrhythmogenic action. The findings may suggest that hypercatecholaminemias are essential in the genesis of ventricular arrhythmias in heart failure. Cardiac glycosides can heterogeneously affect ventricular arrhythmias by modifying the activity of the sympathoadrenal system.