Hepatic hemangioendothelioma: hemodynamic observations and treatment

The clinical course of five infants with heptic hemangioendothelioma is reviewed. Hemodynamic abnormalities observed at cardiac catheterization included an increased cardiac output (range, 9.2 to 10.5 liters/min/sq m) with over 50% of this output being shunted through the arteriovenous malformation, an elevated right and left end diastolic pressure (8 to 10 mm Hg and 9 to 15 mm Hg respectively), a small systolic pressure gradient across the pulmonary outflow tract (4 to 6 mm Hg), and mild elevation of pulmonary artery pressure (range, 35/16 to 37/12 mm Hg). All four patients treated with prednisone improved while on therapy; three are well one to three years after cessation of therapy. One patient died from unrecognized congestive heart failure following discontinuation of prednisone and another before prednisone was started. We conclude that prednisone in high doses (2 to 4 mg/kg/day) with the addition of digitalis and diuretics for control of congestive heart failure is an effective treatment regimen for infants with hepatic hemangioendothelioma.