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Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCMP) is a rare myocardial disease with frequent involvement of the endocardium, which is characterized by impaired filling of one or both ventricles with a decrease in their diastolic volume with an unchanged wall thickness. Due to the deterioration of the diastolic properties of the ventricle, after a short period of rapid filling, the further flow of blood into it practically stops, so that the volume of the ventricle during the second half of diastole does not increase significantly. Thus, the filling of the ventricles is carried out almost exclusively in the proto-diastole. Myocardial contractility and systolic function are usually preserved, especially in the early stages. The basis of the disease is common interstitial fibrosis.

The clinical manifestations and nature of cardiohemodynamic disorders in patients with RCMP are very similar to those with constrictive pericarditis, which sometimes presents significant difficulties for differential diagnosis.

RCMP can be idiopathic or due to infiltrative systemic diseases, such as amyloidosis, hemochromatosis, sarcoidosis, etc.

The idiopathic RCMP as a myocardial disease of unknown etiology includes two nosological units - Fibroplastic parietal endocarditis (or endomyocardial disease), Leffler and endomyocardial fibrosis. Recently, the terms "endomyocardial disease with eosinophilia" and "endomyocardial disease without eosinophilia" are also used to refer to these diseases (R. Richardson et al., 1996).

The endomyocardial disease accompanied by hypereosinophilia syndrome was first described by W. Loftier (1930). It occurs mainly in areas with a temperate climate and is combined with a persistent increase in the content of eosinophils in peripheral blood (more than 1.5 • 109 / l for at least 6 months), which is not associated with any known cause or systemic disease. . In the early stages of the disease, eosinophils accumulate in the myocardium, causing damage to it.

Endomyocardial fibrosis affects almost exclusively the inhabitants of the tropics, less often - subtropics and proceeds without eosinophilia. It was first described in 1938 by A. Williams in two Ugandans who suffered from heart failure and mitral regurgitation, who had large foci of fibrosis in the myocardium at the autopsy, which the researcher regarded as the outcome of syphilitic myocarditis. After 8 years, D. Bedford and G. Konstam (1946) noticed that several West African soldiers who died of heart failure showed widespread subendocardial fibrosis, and multiple thrombi were fixed to a thickened endocardium above its foci. The first detailed description of the morphological substrate and the clinical picture of the new disease was made only in 1955 rJ Davies, who gave it the name "endomyocardial fibrosis".

In the 1970s, E. Olsen and S. Spry (1985) drew attention to the similarity of morphological changes in the myocardium in endomyocardial fibrosis among the inhabitants of the tropics and in the late stages of endomyocardial Leffler's disease. In both cases, they are characterized by a combination of myocardial damage with a predominance of fibrotic changes with a pronounced thickening of the endocardium of one or both ventricles, mainly on the flow paths and in the apex area, which causes a significant disruption of their filling. According to the authors, this makes it possible to consider endomyocardial Leffler disease and endomyocardial fibrosis of Davis different variants of the same myocardial disease with the involvement of the endocardium caused by the toxic effects of eosinophils. Such a unitary point of view was subsequently widely spread (W. Roberts and V. Ferrans, 1974; C. Rapazzi et al., 1993, and others), although it is not recognized by all authors.

Pathophysiological mechanisms of hemodynamic disorders.

Disorders of intracardiac hemodynamics in RCMP are typical for diastolic heart failure and are characterized by the development of the so-called restriction syndrome. It is based on a sharp increase in the elastic stiffness of the ventricle, because of which an increase in end-diastolic pressure becomes a necessary condition for maintaining an adequate stroke volume.

Violation of the elastic properties of the ventricle leads to pathological changes in the process of its diastolic filling, which is manifested by a characteristic change in the diastolic pressure curve. It takes the form of a square root (Fig. 33) with a significant decrease in pressure at the beginning of diastole and a subsequent sharp rise with the transition to a plateau. The pressure curve in the atria also changes accordingly, which takes the form of "M" or "W" due to the pronounced pressure drop peaks Y and X, which alternate with high-amplitude waves A and V of the same magnitude. and pulmonary veins. Also systolic pressure in a pulmonary artery increases, often more than 45 mm of mercury. Art., however, pronounced precapillary pulmonary hypertension is not characteristic.

Fig. 33. The curve of diastolic pressure in the left ventricle (in mm Hg. Art.) In patient D.

In addition to increasing the end-diastolic pressure, the suction action of the ventricle in the proto-diastole plays an important role in ensuring adequate filling of the left ventricle and shock release. As the analysis of the nature of the pressure-volume curve during the cardiac cycle shows, in such patients, a decrease in pressure in the left ventricle at the beginning of diastole is accompanied by a distinct increase in diastolic volume, which is not characteristic of healthy individuals. The inclusion of this mechanism, apparently, has an important compensatory importance for maintaining the pumping function of the heart in violation of the ventricular diastolic compliance.

Despite a significant increase in end-diastolic pressure, the systolic function of the ventricles is not significantly impaired, as evidenced by unchanged values ​​of BWW (less than 110 cm3 / m2) and PV (more than 50%). There is no significant hypertrophy of ventricular myocardium.

It should be borne in mind that the hemodynamic syndrome restriction nonspecific idiopathic RCM and marked as constrictive pericarditis, and a number of systemic diseases infarction - infiltrative (amyloidosis, sarcoidosis) neinfiltrativnyh (systemic sclerosis), diseases of the "accumulation" (hemochromatosis, glycogen storage disease, Fabry disease ), as well as in carcinoid, radiation and some types of toxic myocardial damage (adriamycin, anthracycline, etc.), also accompanied by endocardial thickening. Therefore, the diagnosis of idiopathic RCMP necessarily requires a differential diagnosis with these diseases.

Clinic. The main clinical manifestations of RCMP are caused by restriction syndrome and are characterized by venous stagnation of blood along the flow paths to the left and right ventricles, usually with a predominance of stagnation in the great circulation. Patients are usually concerned with severe shortness of breath and weakness at the slightest exertion, associated with exacerbation of diastolic dysfunction during tachycardia. Peripheral edema, hepatomegaly and ascites, often refractory to the therapy, are rapidly increasing. An objective study marked swelling of the cervical veins and an increase in central venous pressure, which is often aggravated by inspiration (sign of Kussmaul). Paradoxical pulse can be detected. Attention is drawn to the discrepancy between the severity of manifestations of heart failure and the lack of cardiomegaly, which, even before performing an instrumental examination, makes it possible to suspect the presence of diastolic myocardial dysfunction. During auscultation of the heart, a protodiastolic, presystolic or summing gallop rhythm and, in a considerable part of cases, also systolic murmur of mitral or tricuspid insufficiency are noted (M. A. Gurevich and M. O. Yankovskaya, 1988; V. S. Moiseev, 1990). The disease is often complicated by atrial fibrillation and ectopic interventricular arrhythmias, as well as thromboembolism in the small and large circulation, the sources of which in most patients are blood clots in the ventricles and in cases of atrial fibrillation — also in the atria (S. Chew et al., 1977; R. Siegel et al., 1984).

Diagnostics. In chest radiography, the size of the heart is not changed or slightly increased. Cardiomegaly can be observed only with the development of hydropericardium. Depending on the location of the lesion (left, right ventricle, or both ventricles), augmentation of the atria and venous congestion in the lungs are expressed in varying degrees.

ECG changes are often noted, but they are not specific. Signs of atrial and ventricular hypertrophy, blockade of the left (more often) or right (less often) Guis bundle legs, nonspecific repolarization disorders, and various heart rhythm disturbances may be recorded (N. Tobias et al. 1992).

When EchoCG dilatation and ventricular hypertrophy are absent, their contractility is not changed. In some patients, the ventricular cavity can be reduced by obliterating the apical segment. Blood clots are often found in the apex. In severe venous congestion, fluid in the pericardial cavity can accumulate in the great circulation. Doppler echocardiography often reveals moderate blood regurgitation through the mitral and (or) tricuspid valves, accompanied by dilatation of the atrial cavities, and sometimes with moderate fibrosis of the valves of the atrioventricular valves (A. Macedo et al. 1995; F. Cetta et al. 1995). Very characteristic, but not specific, to the restrictive type of diastolic dysfunction, is the increase in the maximum rate of early diastolic filling (E), which significantly prevails over that during the atrial systole (A), (E / A> 1.0), and the shortening of the period of slowing down the rate of early filling (R. Tello et al., 1994).

Other non-invasive methods of cardiac imaging, such as computed tomography and magnetic resonance imaging, are mainly used to exclude constrictive pericarditis, which is characterized by thickening of the pericardium, which is not characteristic of RCMP.

Invasive examination — cardiac catheterization with PAH and in some cases with EMB is important for the diagnosis and differential diagnosis of RCMP. The rapid filling of the ventricles in the early period of diastole, with its sharp deceleration in its second half, is reflected in the characteristic change in the shape of the pressure curve in the ventricles and atria (see Fig. 33). Disturbance of the filling of the ventricles is also determined by AKG, which allows documenting their unchanged size and normal EF. Most patients have some degree of regurgitation of blood through atrioventricular valves with varying severity of atrial cavities (J. Bennotti et al., 1980).

With EMB in the early inflammatory stage of endomyocardial Leffler's disease (see below), characteristic eosinophilic infiltrates can be found, and in the late stage of this disease and with endomyocardial fibrosis - more or less common interstitial fibrosis without signs of inflammation. The latter, however, is not a specific sign of idiopathic RCMP and has diagnostic value only in the presence of clinical and hemodynamic criteria for restriction syndrome. The main role of EMB in these diseases is to exclude constrictive pericarditis and RCMP due to systemic and infiltrative myocardial damage.

Differential diagnosis.
Differential diagnosis of RCMP with other cardiomyopathies, differing in fundamentally different pathophysiological mechanisms (Table 21), usually does not cause difficulties. As in the RCMP, the clinical picture of DCMP is dominated by signs of biventricular heart failure, which, however, due to systolic myocardial dysfunction, is accompanied by cardiomegaly, dilatation of all cardiac cavities, mainly ventricles, and their diffuse hypokinesia. These changes in the morphofunctional state of the ventricles, easily detected during echoCG, are fundamentally different from the hemodynamic signs of restriction syndrome inherent to RCMP.

Table 21. Differential diagnosis of CMP

In some patients with hypertrophic cardiomyopathy, signs of venous congestion in the lungs (shortness of breath, changes in radiographs), an increase in end-diastolic pressure in the left ventricle, pressure in the left ventricle, with almost unchanged sizes of the heart and its cavities and preserved left ventricular systolic function. left atrium and "pulmonary capillaries". At the same time, the absence or relatively weak expression of signs of right ventricular insufficiency (except for the terminal stage of the disease), frequent complaints of angina and syncope, signs of marked left ventricular hypertrophy according to ECG and EchoCG, and in the presence of left ventricular outflow tract obstruction, indicate for her echocardiographic signs. The increase in end-diastolic pressure in the left ventricle that develops in such patients is accompanied by a proportional increase in its initial diastolic pressure, which rules out the square root symptom (S. Chew et al., 1977).

Despite the widespread introduction into modern cardiology of modern instrumental methods of research, it remains a very difficult task to recognize idiopathic RCMP from other diseases that cause a violation of the diastolic filling of the ventricles with the development of the clinical-hemodynamic restriction syndrome. In this case, first of all, it is necessary to carry out differential diagnosis of PCMP with constrictive pericarditis.

In a clinical study, in a part of patients with constrictive pericarditis, a history of indications of tuberculosis, chest trauma or acute pericarditis can be found, 80% determine the sign of Kussmaul (increase in central venous pressure during inspiration), approximately 20% have a paradoxical pulse. In contrast to the PCMP, systolic murmur of mitral and (or) tricuspid regurgitation and its signs are not characteristic of the Doppler study. In connection with the inhomogeneity of the lesions of the myocardium and endocardium of the ventricles and their individual sections at RCMP, the values ​​of the end-diastolic pressure in them are usually unequal and differ by more than 5 mm of mercury. Art. (in the left ventricle is usually higher), whereas with constrictive pericarditis they are identical (J. Hirshman, 1978; J. Benotti, 1984). This feature of the RCMP, however, is not strictly required (R. Shabetai, 1990). The diagnosis of constrictive pericarditis can be confirmed without invasive examination, allowing the detection of pericardial calcification sites during chest radiography. Unfortunately, they are detected only in 30-60% of such patients, and therefore the absence of calcifications does not exclude the diagnosis of constrictive pericarditis. More informative are the modern methods of non-invasive pericardial imaging - computed and magnetic resonance imaging. In most cases of constrictive pericarditis (88% according to T. Masui et al., 1992), they make it possible to determine the thickening of the pericardium, which confirms the diagnosis (I. Suchet and T. Horwitz, 1992). In particularly difficult cases, resorted to EMB. Moreover, the absence of morphological changes in myocardial biopsy specimens in a patient with signs of systemic venous congestion, significantly increased central venous pressure and a “small” heart is considered a weighty argument in favor of constrictive pericarditis and requires thoracotomy, even if there is no thickening of the pericardium according to computer and magnetic resonance imaging (N. Spyrou and R. Foale, 1994).

Systemic and infiltrative myocardial diseases that manifest themselves in restriction syndrome are relatively rare. Among them, the differential diagnosis of idiopathic RCMP should be carried out primarily with amyloidosis of the heart. This disease, caused by the deposition of anomalous protein in it, in most cases is part of a systemic lesion with the involvement of other organs in the pathological process - the tongue, intestine, liver, spleen, peripheral nerves, skin. It occurs, as a rule, in persons older than 40 years, more often in men. In most patients, myocardial damage is manifested by diastolic heart failure with all the characteristic clinical and hemodynamic signs of restriction syndrome. Rarely develop systolic heart failure, similar to the picture DCM, and typical angina. On the ECG, there is a decrease in the voltage in the leads from the extremities, which is combined with left ventricular hypertrophy according to echoCG (J. Carroll et al., 1982). Conduction disturbances in the form of sick sinus syndrome, sinoatrial, atrioventricular and intraventricular blockades are very characteristic. Signs of large-focal changes in the myocardium of the left ventricle, caused by the replacement of groups of cardiomyocytes with deposits of anomalous protein or true post-infarction cardiosclerosis due to amyloidosis of the coronary arteries, are often identified. При ЭхоКГ видно увеличение толщины стенок левого и правого желудочков и межжелудочковой перегородки, причиной которого является не истинная гипертрофия кардиомиоцитов, а инфильтрация амилоидом. При этом размеры полости желудочков в большинстве случаев не изменены или уменьшены, а период изоволюмического расслабления удлинен (М. StJohn Sutton и соавт., 1982; Н. Leinonen и S. Pohjola-Sintonen, 1986). При катетеризации сердца из-за повышенного начально-диастолического давления в левом желудочке симптом "квадратного корня" не характерен. Вследствие замедленного наполнения на протяжении всей диастолы обычно не образуется и патологический III тон (С. Chew с соавт., 1977). Диагноз подтверждается при выявлении отложений амилоида в биоптатах слизистой оболочки языка, прямой кишки и, что более надежно, миокарда при окраске ткани конго-рот (J. Benotti, 1984).

Поражение сердца при гемохроматозе, обусловленное отложением железа в сократительном миокарде, проявляется быстро прогрессирующей сердечной недостаточностью - либо преимущественно диастолической с развитием синдрома рестрикции, либо, реже, систолической, сопровождающейся дилатацией желудочков вследствие снижения их сократимости. У части больных отмечаются суправентрикулярные аритмии, неспецифические изменения сегмента ST и зубца Г и патологические зубцы Q на ЭКГ. Характерны нарушения проводимости (М. Fujita с соавт., 1987). При обнаружении характерной триады - пигментации кожи, увеличения печени и сахарного диабета, а также повышения уровня железа в сыворотке крови диагноз гемохроматоза не вызывает сомнений. В неясных случаях следует прибегать к биопсии печени или миокарда.

Поражение сердца при саркоидозе отмечается по секционным данным в 20-30 % случаев этого заболевания, однако в клинике диагностируется еще реже (менее чем в 5 % случаев). Оно характеризуется особым видом воспаления с образованием специфических гранулем, что проявляется картиной диастолической сердечной недостаточности с синдромом рестрикции, реже - систолической сердечной недостаточностью, напоминающей ДКМП. Заболевание часто осложняется различными нарушениями сердечного ритма и проводимости, которые могут служить причиной синкопальных состояний и внезапной смерти. Ввиду отсутствия специфических клинических и инструментальных признаков, распознавание саркоидоза сердца базируется на обнаружении свойственного этому системному заболеванию поражения других органов и тканей (легких, внутригрудных и периферических лимфатических узлов, печени, селезенки, кожи, глаз) с характерной морфологической картиной, а также воспалительными сдвигами в крови. Верифицировать диагноз позволяет, однако, лишь морфогическое исследование ЭМБ.

Следует подчеркнуть, что, как и в случаях других кардиомиопатий, ввиду отсутствия патогномоничных признаков, в том числе морфологических, диагноз идиопатической РКМП ставится только после исключения инфильтративных и системных заболеваний миокарда.

Случаи РКМП, протекающие с гидроперикардом, требуют дифференциальной диагностики с экссудативным перикардитом. Правожелудочковую форму РКМП следует дифференцировать с другими причинами правожелудочковой недостаточности, особенно сопровождающейся преимущественным увеличением правого предсердия, такими, как его миксома и аномалия Эбштейна, а также с первичной легочной гипертензией и некоторыми врожденными пороками сердца — стенозом устья легочной артерии, тетрадой Фалло, дефектом межпредсердной перегородки. С этой целью широко используют данные доплер-ЭхоКГ инвазивного обследования - АКГ и катетеризации сердца.

В целом, как показывает опыт, несмотря на неспецифичность проявлений идиопатической РКМП и ее сходство с целым рядом заболеваний мио-, эндо- и перикарда, тщательный анализ данных клинического и инструментального обследований и, в сложных ситуациях, морфологического изучения биопсийного материала позволяет в большинстве случае поставить правильный диагноз.

Flow. Для течения идиопатической РКМП характерно неуклонное прогрессирование диастолической сердечной недостаточности, которая служит основной причиной смерти таких больных.

Лечение РКМП исключительно симптоматическое и, в целом, мало эффективно. При значительном повышении конечно-диастолического давления в желудочках и клинических признаках застоя в системных и легочных венах определенное облегчение приносят ограничение жидкости и соли и применение диуретиков. К их назначению, однако, следует подходить более осторожно, чем при систолической дисфункции, и использовать меньшие дозы, поскольку при РКМП, как и при констриктивном перикардите, для поддержания адекватного наполнения левого желудочка и ударного выброса требуется повышенное конечно-диастолическое давление. Это касается и периферических вазодилататоров - нитратов и ингибиторов АПФ, бесконтрольное применение которых может снизить наполнение желудочков и сердечный выброс еще больше. Сердечные гликозиды эффективны только в случаях мерцательной аритмии, позволяя уменьшить частоту сокращений желудочков и, тем самым, удлинить диастолическое наполнение. У больных с синусовым ритмом и сохраненной систолической функцией миокарда назначение этих препаратов, как и негликозидных инотропных агентов, нецелесообразно ввиду их неэффективности. Для профилактики и лечения тромбоэмболических осложнений используют непрямые антикоагулянты.

При выраженном утолщении эндокарда и признаках облитерации полости желудочка у части больных удается выполнить хирургическое лечение - эндокардэктомию, подчас приносящую существенное облегчение. При выраженной митральной и трикуспидальной недостаточности определенный гемодинамический эффект оказывает протезирование или пластика клапанов, что, однако, сопряжено с высокой летальностью - 15-25 % (С. Mady с соавт., 1989, R. Nacmth с соавт., 1993, и др.).
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Рестриктивная кардиомиопатия

    Definition Restrictive cardiomyopathy is an infiltrative or fibrous myocardial lesion, which is characterized by rigid, uncompliant ventricular walls, decreased filling and diastolic volume of one or both ventricles with normal or almost unchanged systolic function and wall thickness. The basis of the disease is common interstitial fibrosis.
  2. Restrictive cardiomyopathy.
    This is a primary or secondary myocardial disease, characterized by diastolic dysfunction, lack of dilatation or ventricular hypertrophy, the presence of increased systemic and pulmonary venous pressure. Restrictive cardiomyopathy occupies a special place among all myocardial diseases and is rare. ETIOLOGY. The cause of restrictive cardiomyopathy is considered fibroplastic
  3. Restrictive Cardiomyopathy
    Restrictive CMP is associated with a decrease in the extensibility of the ventricular walls with manifestations of signs of hypodiastolia and symptoms of congestion in the major and minor circulation. Primary myocardial (isolated myocardial damage, similar to DCM). Endomyocardial (endocardial thickening and infiltrative necrotic and infiltrative changes in the myocardium). 1. Hyper-eosinophilic
    According to WHO experts, two diseases are attributed to restrictive cardiomyopathy: endomyocardial fibrosis and Löffler endo-carditis. There is a term that combines both diseases - "Endomyocardial disease". Diseases are found in countries with a hot climate. Endemic foci have been identified in Uganda, Tanzania, Zambia, Mozambique,
  5. Restrictive cardiomyopathy
    Restrictive cardiomyopathy (RCMP) is known in two versions of Fanee, considered as two distinct pathological processes) - endocardial fibrosis and endomyocardial fibroelastosis * eFflera. The pathomorphological picture in two diseases differs little and is characterized by a sharp thickening of the endocardium in combination with ventricular myocardial hypertrophy, the cavities of which
  6. Primary idiopathic restrictive cardiomyopathy
    A rare form of non-infiltrative myocardial disease with restrictive-type diastolic dysfunction. The absence of LVH and systolic dysfunction is characteristic. Often inherited and associated with skeletal myopathy. The disease usually occurs sporadically, but may be inherited in an autosomal dominant manner. The occurrence of a rigid ventricle may be due to pathology.
  7. Cardiomyopathy. Hypertrophic cardiomyopathy (code 142.0)
    The term "cardiomyopathy" refers to a state of unknown etiology, the most important signs of which are cardiomegaly and heart failure; this name excludes heart disease resulting from valve damage, impaired coronary blood flow, and hypertension of the large and small circulation. Etiology. Clinical and anatomical forms. Prospective
  8. Restrictive lung diseases
    Causes of restrictive pulmonary pathology Causes of acute restrictive pathology: • ????? pulmonary edema; • ???? ARDS; •????aspiration; • ???? neurogenic edema; • ????? opioid overdose; • ???? congestive myocardial insufficiency; • ???? pleural effusion; •????pneumothorax; • ???? increase mediastinum; • ???? pneumomediastinum. Chronic lung disease leading to restrictive
  9. Restrictive lung diseases
    Restrictive diseases are characterized by a decrease in lung compliance. Lung volumes are below normal, while the volumetric flow rate on the exhalation is not reduced. Thus, FEV1 and FZHEL are reduced, but the value of the OFVch / FZHEL ratio remains normal. Restrictive diseases include many acute and chronic pathological conditions of the lungs, as well as lesions of the pleura, chest wall, diaphragm and
  10. Obstructive and restrictive lung diseases
    There are two types of diffuse lung lesions. These are obstructive processes, affecting mainly the airways and characterized by an increase in resistance to the passage of air due to partial or complete obstruction at any level (from the trachea to the respiratory bronchioles), and restrictive processes that are associated with a decreased expansion of the lung parenchyma during inhalation and
  11. Restrictive type ODN
    This type of ARF is characterized by a decrease in the surface of the alveolar membrane due to the restrictive or restrictive effect on the lung parenchyma of various factors. They can be due to both changes in the lung parenchyma and extrapulmonary causes: atelectasis of the lung, hydrothorax, pneumothorax, diaphragmatic hernia, systemic lupus erythematosus, fibrosing
  12. Diffuse interstitial (infiltrative and restrictive) lung diseases
    In this section of the chapter, a group of non-communicable diseases is considered, characterized by predominantly diffuse and usually chronic changes that affect mainly the stroma of the lungs, i.e. interstitial tissue of the alveolar walls, consisting of the basement membrane of the endothelium and epithelial cells, collagen fibers, elastic structures, proteoglycans, fibroblasts,
  13. Cardiomyopathy
    ICD Code: 142.0 Dilatation
  14. Cardiomyopathy
    The term "cardiomyopathy" was first used in 1957 to refer to a group of myocardial diseases of unknown etiology. In 1972, the following definition of cardiomyopathy was given: "Cardiomyopathy is an acute, subacute or chronic lesion of the heart muscle of unknown or unclear etiology, often combined with a lesion of the endocardium, and sometimes the pericardium." This definition is accepted.
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