We report 36 patients (Pt), 21 male, mean age 5.8 years with LVNC. EKG and echocardiography were available in all, Holter in 29, MRI in 27, biopsy in 6 and autopsy in 1. Follow-up ranges from 1 to 216 months.
Isolated LVNC was seen in 29 (80%), 6 (17%) had an associated VSD. Family history was present in 6 (17%). At initial presentation, CHF was seen in 19 (52%), dilated cardiomyopathy in 20 (55%), SVT in 5 (14%), VT in 2 (5%), syncope in 2 (5%). The diagnosis was made by echocardiography in all with abnormal trabeculations seen at the apex in 36 (100%), lateral wall in 35 (97%), and apical septum in 17 (47%). Restrictive flow pattern was seen in 14 (39%) and mitral regurgitation (MR) in 31 (86%). MRI correlated with echo findings in all. EKG and Holter showed left ventricular hypertrophy in 14 (39%), biventricular hypertrophy in 8 (22%), WPW in 8 (22 %), SVT in 5 (14%), isolated premature ventricular contractions in 9 (25%), VT in 2 (6%). Six (17%) died, all infants, because of refractory CHF. Treatment helped 30/36 survivors who are now in NYHA I or II.
Conclusion: CHF and arrhythmias were frequent, but thromboembolism was not seen. WPW (22%) and MR (86%) were findings not previously reported with such frequency in children. Mortality in infants from intractable CHF was significant (17%). Anticongestive and arrhythmia therapy seems indicated along with early consideration for transplantation, particularly in infancy, if improvement is not promptly seen.