THÔNG TIN TÀI LIỆU
FIG 61.11 Electrocardiographic pattern in restrictive cardiomyopathy A 12-lead electrocardiogram (ECG) from a 1-year-old with familial restrictive cardiomyopathy The ECG shows biatrial enlargement and nonspecific Twave changes Chest Radiograph The chest radiograph typically shows evidence of pulmonary edema as well as cardiomegaly due to significant atrial enlargement Echocardiography Echocardiography reveals biatrial dilation with normal- to small-appearing nonhypertrophied ventricles and nondilated and even small ventricular cavities (Fig 61.12) Patients with a mixed phenotype (HCM/RCM) may have mild hypertrophy of the ventricles FIG 61.12 Echocardiographic pattern in restrictive cardiomyopathy Massively dilated left and right atriums without left ventricular hypertrophy or dilation Diastolic evaluation is consistent with increased myocardial stiffness and increased filling pressures There is increased E-wave velocity, decreased Awave velocity, reversed pulmonary vein A-wave velocity and duration, decreased isovolumic relaxation time, decreased diastolic annular velocities, and increased E/e’ ratios Hepatic vein reversal is common and may be accentuated with inspiration Cardiopulmonary Exercise Testing The role of cardiopulmonary exercise testing in assessing/predicting clinical outcomes in RCM is not well established Exercise testing may help to correlate subjective symptoms with ECG changes at higher heart rates Cardiac Catheterization Cardiac catheterization is integral to decision making and the assessment of disease progression The characteristic finding of elevated end-diastolic pressure is uniformly present Some patients also exhibit a classic early diastolic dip in ventricular pressure followed by a rapid rise and plateau (square root sign) As noted previously, there can be precipitous increases in pulmonary artery pressure and pulmonary vascular resistance, thus routine serial catheterization is common Endomyocardial biopsy is not typically indicated in children, given the typical etiologies and the noninvasive methods available to make a diagnosis Management Symptomatic Therapy Medical therapy is focused on symptom management Diuretics are used to manage systemic and pulmonary venous congestion Fluid management is integral, as overdiuresis may increase symptoms, given the need for adequate preload Rate control may be beneficial in some patients in order to maintain adequate filling times and decrease symptoms Afterload-reducing agents are typically poorly tolerated and should be avoided Although there is limited literature on the true risk of thromboembolism in RCM in children, given the atrial size and predisposition to atrial arrhythmia, systemic anticoagulation with warfarin or antiplatelet agents is common Pacemaker/Defibrillator Given the risk of sudden death, some centers have advocated for the implantation of ICDs for primary prevention There are currently no data on the efficacy of this approach, and given that many centers also proceed with early listing and have relatively short waitlist times, this will likely preclude any assessment of the benefit.367
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