A 20-year-old man comes to the office for a sports physical. The patient has played varsity basketball since high school. He has had no chest pain, shortness of breath, palpitations, or fainting spells. He has no medical history and takes no medications. There is no family history of sudden death.
Blood pressure is 115/75 mm Hg, pulse is 56/min, and BMI is 22 kg/m². The patient is in no distress. The apical precordial impulse is hyperdynamic. There is a normal S1 and S2, and an S4 is heard. He has a 2/6 systolic murmur at the left sternal border that increases with Valsalva maneuver. The lungs are clear to auscultation. The abdomen is soft and nondistended. Peripheral pulses demonstrate a rapid upstroke followed by a decline and secondary rise.
Transthoracic echocardiogram reveals interventricular septal thickness of 1.8 cm (normal: <1.1 cm) and posterior left ventricular wall thickness of 0.9 cm (normal: <1.1 cm) . Left ventricular ejection fraction is 75%. At rest, there is no left ventricular outflow gradient; however, Valsalva maneuver generates a gradient of 30 mm Hg.
Which of the following is the most appropriate recommendation for this patient?

Question

Quizplus · Accepted Answer

This patient's presentation is consistent with hypertrophic cardiomyopathy (HCM), defined as left ventricular wall thickness ≥15 mm (1.5 cm) at any location (eg, interventricular septum in this patient) in the absence of other potential causes (eg, hypertension, valvular heart disease). Patients with HCM may remain asymptomatic, or they may develop dyspnea, orthopnea, palpitations, chest pain, dizziness, or syncope. Exertional myocardial ischemia can lead to sustained ventricular arrhythmia and sudden cardiac death (SCD); HCM is the most common cause of SCD in young athletes. To lower the risk of SCD, patients with probable or definite HCM should not participate in high-intensity competitive sports regardless of the presence of symptoms, degree of wall thickness or left ventricular outflow tract (LVOT) obstruction, or prior treatment. (Choice A) Alcohol septal ablation or surgical septal myectomy is indicated for HCM patients with severe LVOT obstruction (resting or Valsalva gradient >50 mm Hg) and heart failure symptoms that are refractory to optimal medical therapy. This patient is currently asymptomatic; therefore, these procedures are not indicated. (Choice C) An implantable cardioverter defibrillator (ICD) is generally indicated for primary prevention in patients with risk factors for SCD (eg, left ventricular wall thickness >30 mm, family history of SCD due to HCM) and for secondary prevention in patients who survive an episode of sustained ventricular arrhythmia. (Choice D) Medical therapy with negative inotropic agents (eg, beta blockers, nondihydropyridine calcium channel blockers, disopyramide) is recommended in patients with LVOT obstruction and symptoms of heart failure. It is not currently indicated in this asymptomatic patient. (Choice E) Dihydropyridine calcium channel blockers (eg, amlodipine, nifedipine) as well as other vasodilators (eg, ACE inhibitors) and diuretics should be avoided in patients with HCM as they decrease afterload and can worsen LVOT obstruction and symptoms. Educational objective: Patients with probable or definite hypertrophic cardiomyopathy, the most common cause of sudden cardiac death (SCD) in young athletes, should be instructed to avoid competitive sports or high-intensity physical activity. In addition, symptomatic patients should be treated with beta blockers or nondihydropyridine calcium channel blockers; those refractory to medical therapy may require surgical septal myectomy or alcohol septal ablation. Patients at high risk for SCD should receive an implantable cardioverter-defibrillator. __________ References: Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis: a scientific statement from the American Heart Association and American College of Cardiology. (http://www.ncbi.nlm.nih.gov/pubmed/26542657) 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). (http://www.ncbi.nlm.nih.gov/pubmed/25173338)

A 20-Year-Old Man Comes to the Office for a Sports