A 15-year-old African-American male was referred for further evaluation for what was interpreted as an abnormal ECG obtained at the time of pre-participation screening. He was active in multiple sports, including basketball, wrestling and weight-lifting. He reported some fatigue and shortness of breath, though not limiting his participation in sports. Upon further questioning, he had experienced occasional chest pressure with activities, lasting approximately five minutes, all over the chest. This would resolve upon rest. His mom, who was present at the exam, says she was unaware of these symptoms, and that no symptoms had been reported by her son's coaches.
This patient's medical history was otherwise unremarkable. Family history was notable for his mother's brother dying suddenly in Honduras at age seven of a "heart problem." Physical exam was unremarkable.
Studies:
ECHO:
LVEDD 5.8 (Z score 2.01) LVESD 4.4 (Z score 3.17)
LVDST 0.6 (Z score -2.5) LVPWT 0.5 (Z score -2.5)
EF 46% by Simpson's, 50% by bullet (or A/L) methods
Normal diastolic indices (E/A, E/E')
Hypertrabeculation of mid-ventricle to apex concerning for LV noncompaction
Holter: rare PACs, PVCs
Stress test (Bruce protocol):
10.5 min duration (15%ile)
Reason for termination: extreme fatigue
Blunted BP response to peak exercise
MVO2 39 mL/kg/min (75%ile)
No ischemic changes or arrhythmias
The correct answer is: C. Recommend light recreational activity
This athlete was diagnosed with left ventricular (LV) noncompaction cardiomyopathy. However, hypertrabeculation of the left ventricle, particularly in black athletes, is increasingly observed as imaging modalities improve. This could be a normal variant and require revision of current diagnostic criteria.1 Cardiac MRI may have been helpful in further defining the structural abnormalities in this patient. However, the cardiomyopathy team has deemed it unnecessary given certainty of the diagnosis. This patient was prescribed an ACE inhibitor and initially restricted from any competitive sports, consistent with Bethesda Conference recommendations, page 1344.2
However, in follow up, it was discovered that he was permitted to play on the high school basketball team. He has remained asymptomatic. Outcomes of participation in sports with LV non-compaction are not known.
References
- Gati S, Chandra N, Bennett RL et al, Increased left ventricular trabeculation in highly trained athletes: do we need more stringent criteria for the diagnosis of left ventricular non-compaction in athletes? Heart 2013;99:401-408.
- Maron BJ, Zipes DP. 36th Bethesda Conference: Eligibility Recommendations for Competitive Athletes With Cardiovascular Abnormalities. J Am Coll Cardiol 2005; 45:1313-1375.