Working Diagnosis:
Left Ventricular Myocardial Noncompaction, Multifocal Atrial Tachycardia, and Premature Ventricular Contractions

Treatment:
The patient underwent a catheter-based cardiac ablation of the atrial arrhythmogenic foci. She is also treated with oral beta blocker therapy and oral aspirin therapy. She is not currently a candidate for Implantable Cardiac Defibrillator placement.

Outcome:
The patient's palpitations, dyspnea, and fatigue resolved.
The patient was medically disqualified from participation in collegiate athletics due to left ventricular myocardial noncompaction based upon the 36th Bethesda Conference Report recommendations and expert opinion of three cardiologists. The patient is allowed to participate in daily activities, light-duty athletic managerial duties, and Class IA sports. She will be monitored with biannual visits with a cardiologist.
She remains asymptomatic and appropriately active.

Author's Comments:
Left Ventricular Myocardial Noncompaction is cardiomyopathy caused by arrest of compaction of loose fibers of embryological myocardium. Common manifestations include arrhythmias, heart failure, and thromboemboli.
There are familial & sporadic forms. Prevalence is estimated at 0.014% of patients referred for echocardiogram. Mean age at diagnosis is 7yr with a range from 11 months to 22yr. There is a male predominance.
Electrocardiograms are usually abnormal, but nonspecific. Echocardiogram showing greater than 2:1 thickness of noncompacted to compacted myocardium is considered diagnostic by accepted Jenni Criteria.
Treatment includes activity modification, treatment of heart failure, treatment of arrhythmias, beta blocker/Angiotensin Converting Enzyme inhibitor therapy, anticoagulation and monitoring with echocardiography, exercise tolerance testing and ambulatory electrocardiography. Implantable Cardiac Defibrillator placement and heart transplant may be utilized as indicated.
Significant morbidity, mortality and risk of sudden cardiac death exist. Implantable Cardiac Defibrillator placement may prevent sudden cardiac death, but Implantable Cardiac Defibrillator placement is not advised in young patients without symptoms of heart failure due to a lack of study. There is a perceived risk of Implantable Cardiac Defibrillator complications, including myocardial rupture and lead displacement. The natural course of the disease produces death or transplant requirement in 50-60% of patients within 3-6 years of diagnosis.

Editor's Comments:
Re-evaluation of palpitations in patients with a prior workup and benign evaluation is indicated whenever findings suggest a cardiac etiology in athletes. Critical issues can be uncovered on subsequent testing. Although this patient did not have hypertrophic cardiomyopathy a teenager can go from normal to abnormal echo in a matter of years. Dyspnea, new onset of fatigue, decreased exercise tolerance, pedal edema and palpitations can all be worrisome symptoms that may require a re-evaluation. As team physicians we are ultimately responsible for the safety of our athletes. The irregular tachycardia noted on the exam was a clear indication of a critical cardiac condition.

References:
Weiford, B., Subbarao, V., Mulhern, K. (2004). Noncompaction of the Ventricular Myocardium. Circulation, 109, 2965-2971.
Connolly, H. and Jost, C. Isolated left ventricular noncompaction. In: UpToDate, Basow, DS (Ed), UpToDate, Waltham, MA, 2012.
Maron, B., Zipes, D., et al. (2005). 36th Bethesda Conference: Eligibility Recommendations for Competitive Athletes with Cardiovascular Abnormalities. Journal of the American College of Cardiology, Vol. 45, No. 8, 1313-1375.

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