ABC | Volume 112, Nº3, March 2019

Updated The Brazilian Society of Cardiology and Brazilian Society of Exercise and Sports Medicine Updated Guidelines for Sports and Exercise Cardiology - 2019 Arq Bras Cardiol. 2019; 112(3):326-368 parasternal cross-section of the long axis. 235,236 It occurs in two forms: the classical form, with diffusely thickened leaflets (≥ 5 mm) with bileaflet prolapse; or the non-classical form, in which there is limited (< 5 mm) or absent thickening and segmental prolapse. Classical prolapse can be further subdivided into symmetrical (when the leaflets meet at a common ring point) and asymmetrical (when one leaflet is moved further towards the atrium). 237 A mitral valve leaflet thickness > 5 mm in the echocardiogram has been associated with an increased risk of SD, stroke and endocarditis in patients with classic prolapse. 236 The etiology can be primary (degenerative disease) or secondary (Marfan syndrome, Ehlers-Danlos syndromes, pseudoxanthoma elasticum). These patients can be identified through auscultation by the presence of a meso-telesystolic click and/or a mitral regurgitation murmur, as well as by chest pain, dyspnea, exercise intolerance, syncope and/or dizziness. The major risks related to mitral-valve prolapse include severe progressive mitral regurgitation requiring valve surgery, infective endocarditis, embolic events, atrial and ventricular tachyarrhythmias and SD (which appears to be associated with structural abnormalities of the mitral valve, as in the classical form), with diffuse thickening, stretching and redundancy, and, in some cases, rupture of chordae tendineae. The prognosis for mitral-valve prolapse is controversial. For example, the Framingham Heart Study 238 described mitral- valve prolapse as a benign entity, but other studies indicate that a subgroup of patients may be at a greater risk of cardiac arrest, which is the most devastating consequence. Thus, the risk for serious adverse events secondary to mitral-valve prolapse remains uncertain. 239,240 SD associated with isolated mitral-valve prolapse is rare among young people, particularly with respect to exercise, and is also rare among professional athletes: its frequency in these groups is no greater than that of the general population. It predominantly occurs in patients over 50 years of age with severe mitral regurgitation and/or systolic dysfunction. However, Basso et al. 241 have shown a growing interest inmitral- valve prolapse, highlighting this entity as a neglected cardiac abnormality that could be associated with severe cardiac events, including SD in youth and adults. Caselli et al. 242 evaluated a large cohort of competitive athletes from 2000 to 2010, finding that mitral-valve prolapse was a relatively common finding, although predominantly benign. In addition, they recommend that detecting moderate-to-severe mitral regurgitation, as well as ventricular arrhythmias, may be useful for identifying athletes with mitral-valve prolapse who are at greater risk. Some individuals with mitral-valve prolapse present a phenotype described as MASS (mitral valve, aorta, skeleton, and skin), which involves connective tissue changes, long limbs, deformity of the thoracic cage, and joint hypermobility). In such patients the risk of progression to aortic dilation/ dissection or SD is greater. The MASS phenotype only applies if the aortic diameter z-score is < 2, the systemic score is ≥ 5 and the patient is at least 20 years old. 243-246 6.3.3.1. Recommendations Athletes with mitral-valve prolapse but no prior syncope (especially if of arrhythmogenic origin), sustained supraventricular or unsustained tachycardia or complex ventricular tachycardia according to 24 h Holter monitoring, severe mitral regurgitation according to an echocardiogram, LV systolic dysfunction (LV ejection fraction < 50%), previous embolic event or family history of SD related to mitral-valve prolapse may participate in any competitive sport. Athletes with mitral-valve prolapse who exhibit any of the above characteristics may participate in competitive sports with low static and dynamic components (class IA). Recommendations for athletes with mitral-valve prolapse and hemodynamic overload secondary to moderate-to-severe mitral regurgitation should be guided by the mitral regurgitation. 6.3.4. Tricuspid Stenosis In isolation, tricuspid stenosis is rare. It is mainly caused by rheumatic disease and is usually associated with mitral stenosis. Although less common, tricuspid stenosis may result from congenital/genetic abnormalities, such as Ebstein’s anomaly, Fabry disease, Whipple disease, or active infective endocarditis. 247 In summary, tricuspid stenosis patients should be considered according to degree of severity (Table 13). 215 6.3.4.1. Recommendations Asymptomatic athletes may participate in any competitive sport. An exercise testing involving at least the activity level achieved during training and competition should be performed. 6.3.5. Tricuspid Regurgitation Tricuspid regurgitation is reported as the most common heart valve disease, affecting up to 85% of the population. 248 It is divided into two categories: primary (or organic) and secondary tricuspid regurgitation, which is the most common form. Namely, only 8% to 10% of tricuspid regurgitation cases are primary, 246 being associated with rheumatic heart disease, pacemaker electrode and defibrillator leads (iatrogenic complications) myxomatous degeneration, myocardial degeneration, tricuspid valve prolapse, infectious diseases (e.g., endocarditis) and congenital heart diseases (e.g., Ebstein’s anomaly). It may also be related to postoperative complications. Physical examination and chest X-ray may help estimate tricuspid regurgitation, but echocardiogram is the gold standard for assessing the mechanism and severity of the condition. Three-dimensional echocardiography is even more sensitive, allowing a simultaneous view of all the leaflets. 249 If RV function cannot be adequately evaluated with this method, CMRI, due to its capacity to quantify RV volumes and ejection fraction, is an important introductory step. 248 If these measures Table 13 – Echocardiographic features of severe tricuspid stenosis Tricuspid valve area ≤ 1.0 cm 2 Mean diastolic gradient right atrium/right ventricle ≥ 5 mmHg Isolated right atrial enlargement Tricuspid pressure half-time ≥ 190 ms 352