Concealed cardiomyopathies in competitive athletes with ventricular arrhythmias and an apparently normal heart: role of cardiac electroanatomical mapping and biopsy

doi:10.1016/j.hrthm.2011.07.021

Heart Rhythm

Volume 8, Issue 12, December 2011, Pages 1915-1922

https://doi.org/10.1016/j.hrthm.2011.07.021 Get rights and content

Background

The diagnosis of subtle structural heart disease in competitive athletes with ventricular arrhythmias (VAs) and an apparently normal heart is challenging. Three-dimensional electroanatomic mapping (EAM) has been demonstrated to reliably identify low-voltage areas that correspond to different cardiomyopathic substrates.

Objective

The purpose of this study was to test whether EAM may help in the diagnosis of concealed cardiomyopathies in athletes with VAs and an apparently normal heart.

Methods

We studied 13 consecutive competitive athletes (12 males, age 30 ± 13 years) who had documentation of VAs within the previous 6 months on 12-lead electrocardiogram (ECG), 24-hour Holter ECG, or ECG exercise testing and who were judged as having a structurally normal heart after a thorough noninvasive evaluation, including signal-averaged ECG, transthoracic echocardiogram, and cardiac magnetic resonance imaging. Depending on the presumed site of VA origin according to 12-lead ECG criteria, patients underwent right or left ventricular EAM and EAM-guided endomyocardial biopsy.

Results

Presenting arrhythmias included sustained ventricular tachycardia (n = 3), multiple episodes of nonsustained ventricular tachycardia (n = 7), and frequent ventricular ectopic beats (>1,000 during 24 hours; n = 3). Three patients had a history of syncope. Twelve (92%) patients had at least one low-voltage region at EAM, which corresponded at EAM-guided endomyocardial biopsy to the histological diagnosis of active myocarditis in seven patients and of arrhythmogenic right ventricular cardiomyopathy in five. In one patient the histological evidence of contraction band necrosis allowed the unmasking of caffeine and ephedrine abuse.

Conclusions

Electroanatomical substrate mapping may help diagnose concealed myocardial diseases in competitive athletes presenting with recent-onset VAs and an apparently normal heart. Further studies are warranted to assess the prognostic implications of such subtle myocardial abnormalities.

Introduction

The clinical assessment of ventricular arrhythmias (VAs) in the setting of an apparently normal heart is a challenging issue,¹ particularly in competitive athletes, in whom a clear association between some forms of structural heart disease and sudden cardiac death has been consistently reported,² thus representing a clinical dilemma regarding their eligibility for sports.³ Distinguishing truly idiopathic VAs from those related to undetected subclinical cardiomyopathies may be difficult4, 5, 6 but is critically important because it implies different prognosis and management strategies.7, 8 The clinically subtle abnormalities of early-stage cardiomyopathies, however, may remain undetected by currently available noninvasive diagnostic techniques, including echocardiography and even cardiac magnetic resonance imaging (cMRI).4, 9, 10 Three-dimensional electroanatomical mapping (EAM) has been demonstrated to reliably identify the pathological substrate underlying VAs in different clinical settings by the detection of myocardial areas with abnormally low voltages,¹¹ which reflect the presence of different cardiomyopathic substrates at endomyocardial biopsy.9, 10, 11

In this study on a consecutive series of competitive athletes with recent-onset VAs and an apparently normal heart, we tested the hypothesis that EAM may help in the differential diagnosis between truly idiopathic and cardiomyopathy-related VAs by the identification of otherwise concealed cardiomyopathic substrates.

Section snippets

Methods

From January 2008 to February 2009 we examined 1,644 athletes at our institution, a national-level referral center for Sports Cardiology. Repetitive VAs (nonsustained and sustained ventricular tachycardia [VT]) were present in 27 (1.6%) subjects, while frequent ventricular premature beats (VPBs) (>1,000/24 hours) were found in 30 (2.1%) subjects. All these subjects underwent a full noninvasive evaluation as reported below, and 17 were judged normal and were considered eligible for invasive

Clinical characteristics

Clinical characteristics and electrophysiological data are summarized in Table 1, Table 2. All patients were actively involved in competitive sports (see Table 1). Two patients (15%) had a family history of premature sudden death (age <40 years) due to ARVC. Spontaneous VAs were documented in all patients, on 12-lead ECG (n = 4), or on 24-hour Holter monitoring (n = 5), or on ECG exercise testing (n = 4). Sustained VT was documented in three (23%) patients, nonsustained VT in seven (54%)

Discussion

The granting of sports eligibility in athletes with VAs with no visible cardiac structural anomalies is a challenging problem, since even subtle structural heart disease in these patients increases the risk of sudden cardiac death by a factor of 2.5 compared with nonathletes.²⁴ Therefore, an early diagnosis of concealed cardiomyopathy in athletes is crucial to promptly ban these subjects from competitive sports.2, 3, 7 In this study, we demonstrate that EAM allows the early recognition of

Conclusions

Our results demonstrate that EAM allows the unmasking of subtle structural heart disease in athletes with VAs and an apparently normal heart, despite a thorough noninvasive evaluation, including cMRI. Further studies are warranted to explore the prognostic implications of such subtle myocardial abnormalities and to assess the optimal management strategies for these patients.

Acknowledgments

The authors thank Gianluca Cionci, MD, for his help in data collection.

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Premature ventricular beats (PVBs) are recorded in a sizeable proportion of athletes during pre-participation screening, especially if the evaluation includes both resting and exercise ECG. While in the majority of cases no underlying heart disease is present, in others PVBs may be the sign of a condition at risk of sudden cardiac death, including cardiomyopathies, congenital, coronary artery, heart valves and ion channels diseases. In this expert opinion document of the Italian Society of Sports Cardiology, we propose a multiparametric interpretation approach to PVBs in athletes and a stepwise diagnostic algorithm. The clinical work-up should include the assessment of the probable site of origin based on the ECG pattern of the ectopic QRS and of the arrhythmia behavior (including the number of different PVB morphologies, complexity, response to exercise and reproducibility), as well as first-line tests such as echocardiography. Based on this initial evaluation, most athletes can be reassured of the benign nature of PVBs and cleared for competition under periodical follow-up. However, when the clinical suspicion is high, further investigations with non-invasive (e.g. cardiac magnetic resonance, cardiac computed tomography, genetic testing) and, in very selected cases, invasive (e.g. endocardial voltage mapping and endomyocardial biopsy) tests should be carried out to rule out a high-risk condition. Importantly, such advanced tests should be performed in centers with a consolidated experience not only in the technique, but also in evaluation of athletes.
Electroanatomic mapping in athletes: Why and when. An expert opinion paper from the Italian Society of Sports Cardiology
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Three-dimensional electroanatomical mapping (EAM) has the potential to identify the pathological substrate underlying ventricular arrhythmias (VAs) in different clinical settings by detecting myocardial areas with abnormally low voltages, which reflect the presence of different cardiomyopathic substrates. In athletes, the added value of EAM may be to enhance the efficacy of third-level diagnostic tests and cardiac magnetic resonance (CMR) in detecting concealed arrhythmogenic cardiomyopathies. Additional benefits of EAM in the athlete include the potential impact on disease risk stratification and the consequent implications for eligibility to competitive sports. This opinion paper of the Italian Society of Sports Cardiology aims to guide general sports medicine physicians and cardiologists on the clinical decision when to eventually perform an EAM study in the athlete, highlighting strengths and weaknesses for each cardiovascular disease at risk of sudden cardiac death during sport. The importance of early (preclinical) diagnosis to prevent the negative effects of exercise on phenotypic expression, disease progression, and worsening of the arrhythmogenic substrate is also addressed.
Ventricular arrhythmias in athletes: Role of a comprehensive diagnostic workup
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Citation Excerpt :
Invasive evaluation included 3-dimensional EAM, electrophysiological study (EPS), and EAM- or cardiac magnetic resonance imaging (cMRI)-guided EMB (Figure 1). The decision to perform invasive diagnostic evaluations was prespecified by institutional protocol as follows: 3-dimensional EAM and EPS were performed in case of diagnostic doubts after noninvasive tests or, in case of diagnostic certainty after noninvasive tests, as a preliminary step to catheter ablation (CA) procedures.1,3–6 EMB was performed in case of persistent diagnostic uncertainty after noninvasive evaluations, EPS, and EAM.6
Ventricular arrhythmias (VAs) represent a critical issue with regard to sports eligibility assessment in athletes. The ideal diagnostic evaluation of competitive and leisure-time athletes with complex VAs has not been clearly defined.
The purpose of this study was to assess the clinical implications of invasive electrophysiological assessments and endomyocardial biopsy (EMB) among athletes with VAs.
We evaluated 227 consecutive athletes who presented to our institutions after being disqualified from participating in sports because of VAs. After noninvasive tests, electrophysiological study (EPS), electroanatomic mapping (EAM), and EAM- or cardiac magnetic resonance imaging–guided EMB was performed, following a prespecified protocol. Sports eligibility status was redefined at 6-month follow-up.
From our sample, 188 athletes (82.8%) underwent EAM and EPS, and 42 (15.2%) underwent EMB. A diagnosis of heart disease could be formulated in 30% of the study population (67/227; 95% confidence interval [CI] 0.24–0.36) after noninvasive tests; in 37% (83/227; 95% CI 31%–43%) after EPS and EAM; and in 45% (102/227; 95% CI 39%–51%) after EMB. In the subset of athletes undergoing EMB, invasive diagnostic workup allowed diagnostic reclassification of half of the athletes (n = 21 [50%]). Reclassification was particularly common among subjects without definitive findings after noninvasive evaluation (n = 23; 87% reclassified). History of syncope, abnormal echocardiogram, presence of late gadolinium enhancement, and abnormal EAM were linked to sports ineligibility at 6-month follow-up.
A comprehensive invasive workup provided additional diagnostic elements and could improve the sports eligibility assessment of athletes presenting with VAs. The extensive invasive evaluation presented could be especially helpful when noninvasive tests show unclear findings.
Special Article - Exercise-induced right ventricular injury or arrhythmogenic cardiomyopathy (ACM): The bright side and the dark side of the moon
2020, Progress in Cardiovascular Diseases
There is still debate on the range of normal physiologic changes of the right ventricle or ventricular (RV) function in athletes. Genetic links to arrhythmogenic cardiomyopathy (ACM) are well-established. There is no current consensus on the importance of extensive exercise and exercise-induced injury to the RV. During the intensive exercise of endurance sports, the cardiac structures adapt to athletic load over time. Some athletes develop RV cardiomyopathy possibly caused by genetic predisposition, whilst others develop arrhythmias from the RV. Endurance sports lead to increased volume and pressure load in both ventricles and increased myocardial mass. The extent of volume increase and changes in myocardial structure contribute to impairment of RV function and pose a challenge in cardiovascular sports medicine. Genetic predisposition to ACM may play an important role in the risk of sudden cardiac death of athletes. In this review, we discuss and evaluate existing results and opinions. Intensive training in competitive dynamic/power and endurance sports leads to specific RV adaptation, but physiological adaptation without genetic predisposition does not necessarily lead to severe complications in endurance sports. Discriminating between physiological adaptation and pathological form of ACM or RV impairment provoked by reinforced exercise presents a challenge to clinical sports cardiologists.
Ventricular Arrhythmias in Myocarditis: Prognostic Role of Electroanatomic Voltage Mapping
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Role of extensive diagnostic workup in young athletes and nonathletes with complex ventricular arrhythmias
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Citation Excerpt :
LV or biventricular EMB through the right femoral vein and femoral artery for access to the RV and LV, respectively, was preferred.15 EMB was guided by low-voltage areas detected by 3D-EAM, as described previously,16 and the local site of EMB as well as possible early complications were monitored by intracardiac echocardiography. Samples for histology and immunohistochemical analysis were promptly fixed in 10% formalin or snap-frozen in liquid nitrogen depending on the antibody to be used.15
Ventricular arrhythmias (VAs) are the most common cause of death in athletes. The differences in the electroanatomic substrate in athletes and nonathletes with complex VA are unknown.
The purpose of this study was to compare the electroanatomic substrate of complex VA in athletes vs nonathletes.
The study prospectively enrolled young athletes and nonathletes with VA. Patients underwent 2-dimensional echocardiography, cardiac magnetic resonance (CMR) imaging, coronary angiography, 3-dimensional electroanatomic mapping (3D-EAM), and 3D-EAM–guided endomyocardial biopsy (EMB). Follow-up included 24-hour electrocardiographic Holter or implantable cardioverter-defibrillator/loop recorder interrogation for VA recurrence.
Thirty-three patients were enrolled: 18 competitive athletes (56%) and 15 nonathletes (44%). Left ventricular and right ventricular (RV) findings by echocardiography and CMR did not show structural disease. Nine athletes (50%) were asymptomatic compared to 1 nonathlete (7%; P <.05). Unifocal origin of VA was reported in 14 athletes (93%) and 17 nonathletes (94%). Athletes showed a larger RV unipolar than bipolar scar (18 ± 17 cm² vs 3 ± 3.8 cm²; P = .04). Diagnostic yield of EMB was 50% in athletes and 40% in nonathletes. Among athletes, the final diagnosis was myocarditis in 2, arrhythmogenic ventricular right cardiomyopathy in 1, and focal replacement fibrosis in 1. Among nonathletes, EMB revealed focal replacement fibrosis in 4 cases. At median follow-up of 18.7 months, Kaplan-Meier curves showed lower VA recurrence in detrained athletes than nonathletes (53% vs 6%; P = .02).
This study showed the need for extensive diagnostic workup in apparently healthy young patients with complex VA in order to characterize concealed cardiomyopathies.

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: The first two authors contributed equally to this work and should be both considered as first authors. C.T. has served as a member of the advisory board of Biosense Webster and has been a consultant for and received lecture fees from St. Jude Medical. A.N. has received consultant fees or honoraria from Biosense Webster, Boston Scientific, Medtronic, Biotronik, and LifeWatch. The other authors declare no significant relationships with industry.

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ClinicalImaging/mappingConcealed cardiomyopathies in competitive athletes with ventricular arrhythmias and an apparently normal heart: role of cardiac electroanatomical mapping and biopsy

Background

Objective

Methods

Results

Conclusions

Introduction

Section snippets

Methods

Clinical characteristics

Discussion

Conclusions

Acknowledgments

Cardiol Clin

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Heart Rhythm

J Am Coll Cardiol

Heart Rhythm

J Am Coll Cardiol

J Am Coll Cardiol

Am Heart J

J Am Coll Cardiol

Am J Med

Clinical
Imaging/mapping
Concealed cardiomyopathies in competitive athletes with ventricular arrhythmias and an apparently normal heart: role of cardiac electroanatomical mapping and biopsy