Bilateral single sesion whole lung lavage under venous-venous ECMO in a case of severe pulmonary alveolar proteinosis: five years follow up

Abstract

Introduction: Pulmonary alveolar proteinosis (PAP) is a rare disease caused by accumulation of proteinaceous material in the alveolar spaces. The most common mechanism implied is the development of antibodies directed to granulocyte macrophage colony stimulating factor (GM-CSF). which inhibits activation of alveolar macrophage, thereby causing accumulation of surfactant. Standard treatment is considered to be whole lung lavage (WLL).

Object: We present the case of a 47 years old woman referred for lung transplantation because of combined lung fibrosis and emphysema. Diagnose was change to respiratory failure secondary to PAP. We performed a single cession bilateral WLL under venous-venous ECMO. After  a 5 years follow up, the patients remains asymptomatic and with normal pulmonary physiology.

Conclusions: The case highlights the importance of careful examination of previous diagnoses, in particular of cases referred for lung transplantation and provides factibility of performing single session bilateral WLL without causing right heart failure.