Neonatal Heart Disease

pp 471-483

Ebstein’s Malformation of the Tricuspid Valve

  • R. M. Freedom
  • , L. N. Benson

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Master Joseph Prescher, a laborer, died in 1864 at the age of 19 years (Prescher being the “benchmark” patient reported in 1866 by Wilhelm Ebstein) and a rare individual can survive to the robust age of 85 years with Ebstein’s anomaly of the tricuspid valve but many are not that fortunate [46, 56, 66, 68]. Newborns presenting with this uncommon anomaly of the tricuspid valve may have massive cardiomegaly and the prognosis for the severely symptomatic newborn with this disorder is poor [8,12, 25, 29, 31, 41, 58, 63, 67, 77]. This anomaly of the tricuspid valve in its neonatal expression is that of displacement, dysplasia, and malattachment of the valve, producing in most tricuspid regurgitation [3, 4, 9, 43, 52, 81, 82].