Abstract
Clinical course and diagnostic studies in 6 infants under 8 weeks with HOCM are presented. One presented with severe CHF, one with severe arrhythmias; both died. In 4, findings were subtle; VSD-like murmurs, mild tachypnea and cardiomegaly. ECGs showed a superior axis in 5 and WPW in 1. Initial M-mode echos were inconclusive. Sector Scans done on 3 were diagnostic in 1, but became more specific at a later age. Cardiac catheterization (CC) was performed at age 1-49 (mean 18) days. Initial resting LV-AO gradient was 50±11 (n=5). Three showed the Brockenbrough response and in 2 no adequate post ectopic beat was recorded. Angiography showed severe septal hypertrophy, hypercontractility, distortion of LV cavity and compression of RV. In 4, the mitral valve (MV) was visualized and showed SAM. In 1 an additional fixed subaortic obstruction was suspected. A second CC in the survivors 18±4.25 mos. later showed a larger gradient, 80±8.17 in 3, and an unchanged gradient in 1. Followup period was 9-54 (mean 23) mos. and all survivors are well on beta blockers. Diagnosis was confirmed at autopsy in 2 and surgery in 1. Conclusion: 1) Prognosis was not related to pressure gradients. 2) Those presented with severe symptoms died. 3) Since physical findings, ECGs and echos in this age are atypical, a high index of suspicion and possibly CC is needed for accurate assessment.
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Nudel, D., Brunson, S., Gootman, N. et al. 180 HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY (HOCM) IN NEW- BORN AND EARLY INFANCY PERIOD. Pediatr Res 15 (Suppl 4), 469 (1981). https://doi.org/10.1203/00006450-198104001-00189
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DOI: https://doi.org/10.1203/00006450-198104001-00189