von Recklinghausen disease complicated by pulmonary hypertension

Two patients with von Recklinghausen disease (neurofibromatosis type 1) wer e admitted to the hospital because of progressive heart failure. Both patie nts had prominent pulmonary hypertension revealed on cardiac catheterizatio n. A lung perfusion scan did not show any gross defect. There were no under lying causes of pulmonary hypertension in either patient, such as chronic l ung disease, congenital or acquired heart disease, deep vein thrombosis, or systemic hypercoagulable states. There may be an unrecognized association between von Recklinghausen disease and pulmonary hypertension.