American Society of Echocardiography Recommendations for the Use of Echocardiography in Rheumatic Heart Disease

Abstract

This update by the American Society of Echocardiography (ASE) summarizes the latest recommendations for the use of echocardiography in rheumatic heart disease (RHD)1. It is a media rich publication, with several high quality images and video clips, with associated online continuing medical education (CME) activities related to this article. This paper follows related recent publications including the 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease2, 2021 ESC/EACTS Guidelines for the management of valvular heart disease3, EAE/ASE guidelines on native valvular stenosis4 and regurgitation5, as well as the EACVI/ASE focused update on aortic stenosis6 and BSE guidelines on the assessment of mitral valve disease7. Rheumatic fever (RF) is associated with incompletely treated upper respiratory and skin infections caused by Group A β-hemolytic Streptococcal infections, typically Streptococcus pyogenes related Streptococcal throat (pharyngitis), Scarlet fever, cellulitis, or impetigo8. It is relatively common in developing countries, however with global migration this may occur in any country9. The diagnosis is suggested by a combination of major and minor clinical criteria, including joint and skin involvement, subcutaneous nodules, carditis, involuntary movements, fever, elevated inflammatory markers, and 1st degree heart block on electrocardiography (ECG). Streptococcal infection is confirmed by an elevated antistreptolysin O titer, as well as a positive throat swab or blood culture. Cardiac involvement may result in pericarditis, myocarditis, or valvulitis, and presents with clinical features including dyspnea, orthopnea, palpitations, or a murmur8,10. Molecular mimicry between streptococcal antigens and cardiac proteins in susceptible individuals results in the inappropriate production of antibodies causing inflammation of the valvular endothelium, myocardium, and neighboring cardiac structures, with the classic appearance of Aschoff bodies on microscopy, leading to chronic valvular scarring11. Rheumatic heart disease (RHD) is the commonest cause of valvular heart disease in children and young adults in many parts of the world9. The revised Jones Criteria for the diagnosis of RF recommend the use of echocardiography to detect valvulitis, as clinical features of carditis and auscultation for cardiac murmurs may be associated with a significant false negative rate12. This is important as a prolonged course of antibiotics is recommended for secondary prevention of RF, and to identify patients who may require peri-procedural endocarditis prophylaxis13,14. As RHD primarily affects the mitral and aortic valves, point of care ultrasound (PoCUS) may be a helpful screening tool to detect subclinical valvulitis in resource limited areas, potentially increasing the diagnostic yield, and allowing targeted antibiotic therapy and earlier referral to specialist cardiac centers15–17. In the era of telemedicine, PoCUS images may be easily shared from primary care and acute hospital locations, and reviewed by specialist echocardiographers to improve cardiac triage18. The detection of jugular venous or inferior vena cava distension may suggest intravascular volume overload19, significant heart failure or pulmonary hypertension20,21, or obstructive shock (for example related to a significant pericardial effusion)22. The presence of characteristic B-lines on lung ultrasound may allow early detection of pulmonary edema23–25, allowing differentiation of heart failure with preserved or reduced ejection fraction (HFpEF or HFrEF respectively) according to the Nohria Stevenson classification of cold or warm peripheries, and dry or wet lungs26–28. The addition of ECG can detect arrhythmias such as atrial fibrillation (AF), and simple non-invasive vital sign monitoring may allow identification of a narrow proportional pulse pressure (ratio of pulse pressure to systolic blood pressure)29,30, or elevated shock index (ratio of heart rate to systolic blood pressure)31,32, suggestive of hemodynamically significant lesions. Specific PoCUS appearances associated with cardiac involvement in RF may include mitral or aortic valve thickening and restriction, or a pericardial effusion on two-dimensional (2D) imaging, as well as significant turbulence or regurgitation through the mitral or aortic valves on color flow Doppler (CFD) mapping. Other suggestive features may include identification of impaired ventricular systolic function, and evaluation of the relative size of all four cardiac chambers, to detect left or right ventricular volume overload, and identify post-capillary pulmonary hypertension (PH) with a dilated left atrium and significant tricuspid regurgitation (TR)15–17,33. The performance of an echocardiography begins with identifying significant chamber dilatation, and quantifying left ventricular ejection fraction (LVEF) and PH. The echocardiographic pulmonary to left atrial ratio (EPLAR) divides the peak velocity of the tricuspid regurgitant jet (TR Vmax) by the ratio of the mitral annular tissue Doppler e’ wave to transmitral E wave velocity (E/e’ ratio). This helps to identify patients with or without a pulmonary artery pressure > 25 mmHg, and left atrial hypertension > 15 mmHg, and differentiate pre- and post-capillary PH34. Classical appearances of rheumatic mitral valve disease include valvular thickening, commissural fusion, thickening of the subvalvular apparatus and chordal shortening. Typically mitral stenosis (MS) is associated with diastolic doming and a hockey stick appearance of the anterior mitral valve leaflet (AMVL), and mitral regurgitation (MR) with asymmetric leaflet tethering and reduced coaptation, however MR may also result from chordal rupture with a flail leaflet35,36. The following recommendations are summarized from the ASE guidelines1. A mitral valve area (MVA) < 2.5 cm2 suggests significant MS, and severity is assessed primarily by 2D measurements of thickening (with harmonics switched off), 2D planimetry (with the lowest possible gain settings to avoid underestimation of MVA), as well as Doppler pressure half time (PHT) using the mid-diastolic portion of the waveform, and transmitral mean pressure gradient > 4mmHg. The degree of calcification of the mitral valve (MV) leaflets and commissures is relevant as it may lead to errors in 2D planimetry measurements of MVA, and determines the suitability for percutaneous balloon mitral valvuloplasty (PMBV). In patients with AF, Doppler measurements should be averaged over at least 5 cardiac cycles, preferably using the longest diastolic time intervals. A transmitral gradient using stress echocardiography with supine bicycle exercise > 15mmHg or dobutamine > 18mmHg identifies patients who may benefit from PBMV or surgical intervention. Clinical correlation with symptoms, and other echocardiographic features of HF and PH is advised particularly during pregnancy, as the hyperdynamic state and increased circulating blood volume may exaggerate the transmitral pressure gradient. Whilst the continuity equation and proximal isovelocity surface area (PISA) methods are not routinely recommended in assessing MS severity, these measurements may be useful in cases where other methods yield discordant data. In contrast, the PISA method is recommended in determining effective regurgitant orifice area (EROA) in rheumatic mitral regurgitation (MR), with 3D preferred over 2D PISA measurements, as well as three-dimensional (3D) planimetry of both EROA and vena contracta (VC) area. Severe MR is suggested by a VC ≥ 0.7cm, VC area or EROA ≥ 0.4 cm2, and regurgitant fraction (RF) ≥ 50%. There is an increasing trend towards MV repair in suitable cases, although MV replacement (MVR) may be preferred in cases where calcification, leaflet morphology, or endocarditis precludes repair, or in cases of multiple valve involvement 37–39. Key recommendations regarding rheumatic aortic valve disease are summarized below1. Rheumatic aortic valve involvement results in commissural fusion, and leaflet thickening with systolic doming. Grading of aortic stenosis (AS) severity follows standard guidelines, with severe AS being considered an aortic valve area (AVA) < 1cm2, and a dimensionless index of left ventricular outflow tract (LVOT) to aortic valve (AV) velocity of < 0.256. Three-dimensional (3D) measurement of AVA is preferred over 2D planimetry, as well as the use of the continuity equation, and stress echocardiography Doppler may be appropriate in cases of suspected low flow low gradient AS. Cardiac computerized tomography (CT) is recommended in indeterminate cases. The assessment of rheumatic aortic regurgitation (AR) severity follows standard guidelines, and severe AR is suggested by a VC > 0.6cm, Jet width / LVOT diameter ≥ 65%, LV dilatation, and holo-diastolic flow reversal in the descending thoracic aorta. 3D echocardiography may improve the accuracy of VC area and PISA measurements. Surgical aortic valve replacement (SAVR) is the standard treatment for rheumatic aortic valve disease; however valve repair (valvuloplasty) may be feasible in selected patients40. Alternatively, transcatheter aortic valve implantation (TAVI) has been described in cohorts of patients considered high risk for SAVR41,42. The guidelines provide recommendations on the evaluation of rheumatic involvement of the tricuspid and pulmonary valve, which are less common, and usually occur in conjunction with rheumatic mitral and aortic valve disease1. Often tricuspid regurgitation (TR) is related to annular dilatation from post-capillary PH due to left heart valvular pathology. In addition, specific recommendations are made regarding the assessment of mixed rheumatic valve disease, as well as the use of 2D and 3D transesophageal echocardiography (TEE) to guide percutaneous and surgical valve interventions. In summary, these guidelines provide a comprehensive review of echocardiography in RHD. Detailed summary points are provided for each valve lesion, including specific criteria for grading severity. 1Pandian NG, Kim JK, Arias-Godinez JA, et al. Recommendations for the Use of Echocardiography in the Evaluation of Rheumatic Heart Disease: A Report from the American Society of Echocardiography. 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