LIFE-THREATENING PULMONARY HEMORRHAGE WITH PULMONARY ARTERIOVENOUS-MALFORMATIONS AND HEREDITARY HEMORRHAGIC TELANGIECTASIA

The occurrence of significant pulmonary hemorrhage associated with pul monary arteriovenous malformations (PAVMs) and hereditary hemorrhagic telangiectasia (HHT) and the incidence of PAVMs in family members of p atients with PAVMs and HHT are poorly defined. We reviewed our experie nce in 143 patients with PAVMs and HHT. Eleven (8 percent) of the 143 patients with HHT and PAVMs had a history of either massive hemoptysis or of hemothorax which required hospitalization. One patient died dir ectly related to the pulmonary hemorrhage. There were four men and sev en women. Three of the seven women experienced pulmonary hemorrhage du ring pregnancy. Seven of the 11 families participated in screening for PAVMs. Thirty-six (80 percent) of the 45 screened family members were found to have HHT. Thirteen (36 percent) of the 36 family members wit h HHT were proven to have PAVMs by pulmonary angiography. Pulmonary he morrhage due to spontaneous rupture of the PAVM is a potentially life- threatening complication that should be treated aggressively with tran scatheter embolotherapy. It occurs more frequently than previously rec ognized in patients with PAVMs and HHT. In addition, because of the in creased incidence of PAVMs in family members of patients with HHT and PAVM, screening of family members with HHT is recommended especially i n women of childbearing age.