Novel method of noninvasive ventilation supported therapeutic lavage in pulmonary alveolar proteinosis proves to relieve dyspnea, normalize pulmonary function test results and recover exercise capacity: a short communication

Abstract

Whole lung lavage (WLL) under general anesthesia with a double-lumen endobronchial intubation has remained standard treatment option for pulmonary alveolar proteinosis (PAP) for over fifty years now. To the best of our knowledge, this is the first description of noninvasive ventilation (NIV) as an innovative alternative, which enables safe and effective treatment. NIV support enabled cost-effective lavage of the most affected segments and resulted in restoration and long-term maintenance of exercise capacity and diffusion, without WLL related hypoxaemia, volume overload, intubation, or mechanical ventilation related complications. The study presents all details of performed procedure, including anesthesia, NIV technique and bronchoscopy, therefore this may be easily implemented into clinical practice at other centers conducting PAP treatment. We assume that presented technique of therapeutic lung lavage (TLL) with NIV support may be considered a novel PAP treatment method, however, target population who will benefit the most from such therapy modification must be assessed in large scale prospective trials. (2). This method provides long-lasting beneficial effects. It is usually performed unilaterally, with the patient in a supine position. The lavaged lung is filled up to functional residual capacity (FRC), and cycles of saline instillation and subsequent drainage are repeated, until clear effluent is obtained. Taking into account WLL methodology, it facilitates severe hypoxemia by elicitation of extensive V/Q mismatch due to shunt through the treated lung and respiration conducted by the contralateral lung, which interstitial tissue is often heavily affected by present disease. This mechanism sometimes creates a need for extracorporeal membrane oxygenation ECMO support (3). Moreover, uneven alveolar distribution in the lavaged lung may promote post procedure pulmonary edema which is probably responsible for the demand for prolonged ventilation and oxygen supplementation (4,5). Obviously, due to low incidence of the disease, no gold standard WLL protocols have been developed as yet.The major indications for WLL in centers caring for PAP patients worldwide include radiographic progression, lung function deterioration and hypoxemia (4).WLL, although considered as reasonably safe and efficient if performed in well-experienced specialized centers, has serious limitations, which include post procedure complications, the most common being fever, hypoxemia, wheezing, pneumonia and saline leakage due to malposition of the endotracheal tube (4,6). Also pneumothorax, pleural effusion, cardiac arrest (4) and one case of WLL-associated death (7) were reported. There is a possibility that mortality rate is under reported.Hypoxemia usually occurs during the drainage phase, because blood is again redirected from the ventilated lung to the contralateral lavaged one and a decrease in airway pressure occurs, leading to perfusion of the lung undergoing BAL and thus a fall in partial oxygen pressure PaO 2 (4).Last but no...

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