Pulmonary hemosiderosis in scimitar syndrome after prolonged management of pulmonary hypertension.

OBJECTIVE: To report a fatal outcome from pulmonary hemosiderosis in an infant with scimitar syndrome after prolonged pulmonary vasodilator therapy. DESIGN: Case report. SETTING: A tertiary care pediatric intensive care unit. SUBJECT: An infant with scimitar syndrome. INTERVENTIONS: Treatment included redirection of anomalous right pulmonary venous drainage and closure of atrial septal defect, assisted ventilation via tracheostomy, and protracted nitric oxide and prostacyclin therapy until his death at 1 yr of age. RESULTS: Inhaled nitric oxide (iNO) and/or prostacyclin (PGI(2)) were administered for 6.5 months. Numerous echocardiograms demonstrated good control of pulmonary pressures and no evidence of pulmonary venous obstruction. Repeated attempts to slowly wean from the pulmonary vasodilators resulted in return of pulmonary pressures to systemic levels. Although there was no clinically apparent hemoptysis, pulmonary infiltrates worsened, prompting an open-lung biopsy that revealed pulmonary hemosiderosis. During the last 4 days of the patient's life, the pulmonary hypertensive crises with suprasystemic pressures and pulmonary infiltrates worsened regardless of aggressive vasodilator therapy with iNO, PGI(2), alkalinization, and isoproterenol. Vasodilator therapy was withdrawn and the patient rapidly died. CONCLUSION: We achieved long-term control of pulmonary hypertension with iNO and/or PGI(2) without apparent tachyphylaxis or other major reported side effects. Although pulmonary hypertension was successfully controlled with prolonged iNO and intravenous PGI(2) administration in this patient with scimitar syndrome, the patient died of hypoxemic respiratory failure from pulmonary hemosiderosis. Early evaluation of roentgenographic infiltrates for hemosiderosis and potential lung transplantation in similar patients may be warranted.