Single-lobe lung transplantation for rapidly deteriorating pulmonary venoocclusive disease.

Abstract

Pulmonary venoocclusive disease, classified as a subgroup of pulmonary arterial hypertension, is known to show poor prognosis and lung transplantation is the only possible treatment. Single living-donor lobar lung transplantation is a unique method of treatment, mostly for small children, and size matching is the most important factor to conduct single living-donor lobar lung transplantation safely. We report a successful single living-donor lobar lung transplantation for a 6-year-old girl with pulmonary venoocclusive disease who received the graft from her mother. Preoperatively, the recipient was intubated under deep sedation because of repeated episodes of pulmonary edema due to rapidly deteriorating pulmonary venoocclusive disease.