What are the management and treatment options for an athlete diagnosed with left ventricular hypertrophic cardiomyopathy (LVHC)?

Management of Athletes with Hypertrophic Cardiomyopathy

Athletes with hypertrophic cardiomyopathy should be restricted from high-intensity competitive sports but can safely participate in low-to-moderate intensity recreational activities, with recent evidence showing that competitive athletics in carefully selected patients does not increase sudden cardiac death risk compared to sedentary individuals. 1

Competitive Sports Participation

Absolute Restrictions

• Athletes with HCM must not participate in intense competitive sports regardless of age, sex, race, or presence of other conditions. 1
• High-intensity competitive sports (≥6 METs) including full-court basketball, ice hockey, soccer, racquetball/squash, sprinting, and singles tennis are strongly discouraged (eligibility score 0-1 on 0-5 scale). 1

Evolving Evidence on Competitive Athletics

• Recent prospective registry data from 2024 guidelines demonstrate that competitive athletes with HCM did not experience increased arrhythmic risk compared to moderately active or sedentary individuals over >3 years follow-up. 1
• Among 259 individuals engaging in competitive athletics (including 42 high school/collegiate athletes), no increased sudden cardiac death events occurred during training or competition. 1
• Athletes with ICDs experienced shock rates similar to non-athletic populations, with most shocks occurring outside training/competition and no shock-related injuries or deaths. 1

Permitted Low-Intensity Activities

• Low-intensity competitive sports like golf and bowling are reasonable for patients with HCM (eligibility score 4-5). 1
• Brisk walking, bowling, golf, skating, and snorkeling are probably permitted. 1

Recreational Exercise Guidelines

Recommended Activities (Moderate-to-Low Intensity)

• Moderate-intensity recreational activities (4-6 METs) including biking, jogging, tennis doubles, swimming laps, and treadmill/stationary bicycle are reasonable with eligibility scores of 4-5. 1
• Aerobic exercise is preferable to isometric exercise. 1
• Activities should avoid burst exertion patterns (e.g., sprinting in half-court basketball) in favor of sustained activities like swimming laps or cycling. 1

Activities Requiring Individual Assessment

• Moderate-intensity sports with eligibility scores 2-3 (baseball/softball, hiking, surfing) require clinical assessment on an individual basis. 1
• Sports involving potential traumatic injury (bodybuilding, rock climbing, downhill skiing, free weights, horseback riding) warrant consideration of impaired consciousness risk. 1
• Water-related activities (sailing, surfing, swimming, snorkeling) require assessment of impaired consciousness risk during water exposure. 1
• Scuba diving is strongly discouraged (score 0) due to impaired consciousness risk underwater. 1

Exercise Physiology and Safety Considerations

Cardiovascular Benefits vs. Risks

• Cardiorespiratory fitness reduces all-cause and cardiovascular mortality by 10-20% per improvement level in the general population, and these benefits may outweigh theoretical exercise risks in HCM. 1
• Two-thirds of HCM-related deaths in individuals aged 5-59 occur during routine daily activities (43%) or rest/sleep (24%), not during exercise. 1
• Inactivity is common in HCM patients due to fear of exercise-induced adverse events, but this increases risk of cardiometabolic diseases. 1

Exercise Training Intensity

• Moderate-intensity exercise has been shown safe in the RESET-HCM trial, though cardiorespiratory fitness increases were modest. 1
• High-intensity exercise is more efficacious for increasing cardiorespiratory fitness in chronic disease populations, but current guidelines remain cautious. 1

Environmental and Lifestyle Precautions

Critical Avoidance Measures

• Patients must avoid dehydration and extreme environmental conditions (heat, humidity, cold), particularly those with left ventricular outflow tract obstruction. 1
• Large meals can precipitate chest pain in patients with LVOT obstruction; smaller, more frequent meals are preferable. 1
• Excess alcohol should be avoided, especially in patients with LVOT obstruction. 1

Risk Stratification and Monitoring

Sudden Cardiac Death Risk Assessment

• Sudden death risk stratification and ICD placement decisions should follow standard HCM algorithms independent of sports participation decisions. 1
• Inappropriate ICD utilization solely for sports participation exposes patients unnecessarily to device-related complications. 1

Specialized Evaluation Requirements

• Evaluations and shared decision-making regarding sports participation should be undertaken by professionals with expertise in managing competitive athletes with HCM and repeated at least annually. 1
• Final eligibility decisions for organized sports may involve third parties (team physicians, consultants, institutional leadership). 1

Genotype-Positive, Phenotype-Negative Individuals

• Sudden death in genotype-positive, phenotype-negative individuals is rare, with no arrhythmic events observed in a prospective registry of 126 individuals, including those exercising vigorously. 1
• These individuals are not routinely restricted from competitive sports unless family history indicates high sudden cardiac death risk. 1

Medical Management Considerations

Pharmacologic Therapy

• Beta-blockers are first-line therapy for obstructive HCM (LVOT gradient ≥30 mmHg) and should be continued during exercise participation. 2
• Beta-blocker use is associated with reduced exercise capacity (peak VO2 83% of predicted) and limited peak heart rate response (47% vs 9% in non-users). 3
• Verapamil should be avoided in patients with severe left ventricular dysfunction (ejection fraction <30%) or moderate-to-severe heart failure symptoms, and carries specific warnings for HCM patients with severe LVOT obstruction. 4

Atrial Fibrillation Management

• Anticoagulation with vitamin K antagonists (INR 2.0-3.0) is indicated for any atrial fibrillation (paroxysmal, persistent, or chronic) in HCM patients due to high thromboembolic risk. 1
• Ventricular rate control requires high doses of beta-blockers and non-dihydropyridine calcium channel blockers. 1

Occupational Considerations

Federal Guidelines

• Commercial motor vehicle driving is reasonable if patients have no ICD, no major sudden cardiac death risk factors, and are on guideline-directed medical therapy per Federal Motor Carrier Safety Administration guidelines. 1
• Pilot aircrew may be considered for multicrew flying duties per Federal Aviation Administration guidelines if asymptomatic, low sudden cardiac death risk, and can complete maximal treadmill stress test at 85% peak heart rate. 1

Manual Labor

• Occupations requiring manual labor, heavy lifting, or high physical performance are reasonable after comprehensive clinical evaluation, sudden cardiac death risk stratification, and implementation of guideline-directed medical therapy. 1

Common Clinical Pitfalls

Differential Diagnosis Challenges

• Distinguishing HCM from athlete's heart is critical when maximum wall thickness is 13-15 mm; markers favoring HCM include sarcomeric mutations, family history, unusual/noncontiguous LV hypertrophy pattern, and lack of wall thickness decrease after deconditioning. 1, 5
• Coexistence of HCM with hypertension in older patients requires identification of diagnostic sarcomere mutations or marked LV thickness ≥25 mm and/or LVOT obstruction with systolic anterior motion. 1

Overrestriction vs. Underestimation of Risk

• Historical overrestriction of all physical activity deprives patients of cardiovascular benefits and quality of life improvements. 1, 6
• However, not all athlete types are well-represented in safety studies, requiring cautious interpretation of recent permissive data. 1

Follow-up Requirements

• Close follow-up is essential as 9% of athletes in one cohort required myectomy and 14% received ICDs for primary prevention over 5.6 years. 3
• One athlete with massive LV hypertrophy experienced recurrent ICD shocks for ventricular fibrillation, underwent myectomy, and ceased sports participation. 3