Restrictive cardiomyopathies are heterogeneous group of myocardial disorders characterized by a marked increase in left ventricular myocardial stiffness in their advanced stages. Left ventricular (LV) myocardial stiffness results in an elevated LV filling pressure, left atrium (LA) enlargement, and eventually pulmonary venous congestion, secondary pulmonary hypertension, and often a low cardiac output. In nearly all restrictive cardiomyopathies, there is a progressive worsening of cardiac diastolic function over time. In the early stages of the disease, LV size and ejection fraction are normal, and only mild abnormalities of diastolic function are present, such as impaired LV relaxation. With disease progression, LV ejection fraction typically remains normal but myocardial compliance decreases, which raises LV and LA filling pressures and leads to atrial enlargement. The increase in LA pressure “normalizes” early diastolic filling at the expense of pulmonary venous congestion, and LA hypertrophy becomes an important compensatory mechanism to maintain a normal LV end-diastolic volume and cardiac output. In advanced stages of the disease, “restrictive physiology” is a typical finding. Heart failure symptoms and functional limitations are common. LV size and ejection fraction vary by pathologic cause, but the diastolic abnormalities are generally more severe than systolic dysfunction. The diastolic restrictive physiology is characterized by a pulsed wave Doppler mitral inflow E/A ratio >2.5, a mitral deceleration time of 150 ms, and a markedly increased LA volume index (>50 mL/m2), indicating a very poor prognosis and high 1-year mortality. In contrast to constrictive pericarditis, the hepatic vein diastolic flow reversal in RCM is remarkable during inspiration. Myocardial relaxation is universally impaired, so the mitral annulus E′ velocity obtained from the septal annulus is frequently less than 7 cm/s. However, these typical features are mainly seen in the advanced stages. Cardiac amyloidosis can be quite common finding in elderly patients with aortic stenosis or heart failure with preserved EF. The diagnosis needs an integrated approach, including a high index of suspicion and clinical evaluation considering the extracardiac red flags and histological and imaging findings. In this chapter, we will discuss the echocardiographic features of RCM and the more common types of RCM, including cardiac amyloidosis, sarcoidosis, and Fabry disease.

Echocardiography in Restrictive Cardiomyopathy

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发表日期：

2026年

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Azin Alizadehasl,
Anita Sadeghpour,
Saideh Jamshidi

摘要

Restrictive cardiomyopathies are heterogeneous group of myocardial disorders characterized by a marked increase in left ventricular myocardial stiffness in their advanced stages. Left ventricular (LV) myocardial stiffness results in an elevated LV filling pressure, left atrium (LA) enlargement, and eventually pulmonary venous congestion, secondary pulmonary hypertension, and often a low cardiac output. In nearly all restrictive cardiomyopathies, there is a progressive worsening of cardiac diastolic function over time. In the early stages of the disease, LV size and ejection fraction are normal, and only mild abnormalities of diastolic function are present, such as impaired LV relaxation. With disease progression, LV ejection fraction typically remains normal but myocardial compliance decreases, which raises LV and LA filling pressures and leads to atrial enlargement. The increase in LA pressure “normalizes” early diastolic filling at the expense of pulmonary venous congestion, and LA hypertrophy becomes an important compensatory mechanism to maintain a normal LV end-diastolic volume and cardiac output. In advanced stages of the disease, “restrictive physiology” is a typical finding. Heart failure symptoms and functional limitations are common. LV size and ejection fraction vary by pathologic cause, but the diastolic abnormalities are generally more severe than systolic dysfunction. The diastolic restrictive physiology is characterized by a pulsed wave Doppler mitral inflow E/A ratio >2.5, a mitral deceleration time of 150 ms, and a markedly increased LA volume index (>50 mL/m2), indicating a very poor prognosis and high 1-year mortality. In contrast to constrictive pericarditis, the hepatic vein diastolic flow reversal in RCM is remarkable during inspiration. Myocardial relaxation is universally impaired, so the mitral annulus E′ velocity obtained from the septal annulus is frequently less than 7 cm/s. However, these typical features are mainly seen in the advanced stages. Cardiac amyloidosis can be quite common finding in elderly patients with aortic stenosis or heart failure with preserved EF. The diagnosis needs an integrated approach, including a high index of suspicion and clinical evaluation considering the extracardiac red flags and histological and imaging findings. In this chapter, we will discuss the echocardiographic features of RCM and the more common types of RCM, including cardiac amyloidosis, sarcoidosis, and Fabry disease.