Pulmonary fibrosis is a progressive interstitial lung disease that leads to architectural distortion of the lungs. It is characterized by fibrosis, honeycombing, and reduced lung volume. Ventilation of patients with pulmonary fibrosis under general anesthesia presents significant challenges due to impaired lung compliance, diffusion abnormalities, and the risk of ventilator-induced lung injury (VILI).

The cornerstone of anesthesia management in patients with pulmonary fibrosis is lung-protective ventilation. This strategy, modeled on protocols used in acute respiratory distress syndrome (ARDS), emphasizes low tidal volumes (4–6 mL/kg of predicted body weight), limiting plateau pressures to ≤30 cm H₂O, and using the lowest possible fraction of inspired oxygen (FiO₂) to achieve acceptable oxygenation (SpO₂ > 90%). These measures help mitigate barotrauma, volutrauma, and oxygen toxicity, all of which may exacerbate the underlying fibrotic pathology (1).

A key aspect of intraoperative management is the judicious use of positive end-expiratory pressure (PEEP). While PEEP typically prevents alveolar collapse and improves oxygenation, in fibrotic lungs, excessive PEEP may lead to overdistension and hemodynamic instability without meaningful improvement in oxygenation. As Yamazaki et al. noted, the fibrotic lung exhibits limited compliance and low recruitability, which makes traditional recruitment strategies less effective and potentially harmful (2). These principles are supported by broader research in acute respiratory distress syndrome (ARDS) physiology, which cautions against excessive ventilatory pressures and volumes, both of which may provoke VILI even in the absence of overt lung inflammation (3).

Permissive hypercapnia is often tolerated to avoid lung overdistension. However, caution is advised when using this approach in patients with coexisting pulmonary hypertension or right ventricular dysfunction, conditions frequently associated with advanced fibrosis. Elevated pulmonary vascular resistance can exacerbate right heart strain during anesthesia, particularly under the influence of mechanical ventilation. Inhaled pulmonary vasodilators such as nitric oxide may be beneficial in select cases, though evidence remains limited.

Preoperative evaluation should include pulmonary function tests (PFTs), arterial blood gases, and high-resolution computed tomography (HRCT). Severely reduced diffusing capacity for carbon monoxide (DLCO) and forced vital capacity (FVC) are indicators of increased perioperative risk. Notably, Kim et al. reported that acute exacerbations and decreased baseline function in idiopathic pulmonary fibrosis are associated with high postoperative mortality, emphasizing the need for cautious patient selection and risk stratification (4). These findings further justify individualized ventilation planning, informed by the predicted postoperative course and patient-specific risk factors.

Postoperatively, early extubation and avoidance of prolonged mechanical ventilation are key goals. Non-invasive modalities, particularly high-flow nasal cannula (HFNC) oxygen therapy, may improve oxygenation while preserving patient autonomy and reducing the risk of VILI. However, patients must be closely monitored for signs of respiratory fatigue because even minor decompensation can lead to catastrophic outcomes given their limited respiratory reserve. Updated guidelines for ARDS management emphasize not only lung-protective tidal volumes, but also careful titration of driving pressures and fluid balance—principles that remain directly relevant to the perioperative care of fibrotic lung disease (5).

Ultimately, ventilation in patients with pulmonary fibrosis who need general anesthesia for their surgery requires an individualized, lung-protective approach that minimizes additional iatrogenic injury. These strategies must be grounded in an understanding of the disease’s pathophysiology, vigilant monitoring, and an awareness of the delicate balance between oxygenation and overdistension in a structurally compromised lung.

References

1. Fernández-Pérez ER, Sprung J, Afessa B, et al. Intraoperative ventilator settings and acute lung injury after elective surgery: a nested case control study. Thorax. 2009;64(2):121-127. doi:10.1136/thx.2008.102228
2. Yamazaki R, Nishiyama O, Gose K, et al. Pneumothorax in patients with idiopathic pulmonary fibrosis: a real-world experience. BMC Pulm Med. 2021;21(1):5. Published 2021 Jan 6. doi:10.1186/s12890-020-01370-w
3. Slutsky AS, Ranieri VM. Ventilator-induced lung injury [published correction appears in N Engl J Med. 2014 Apr 24;370(17):1668-9]. N Engl J Med. 2013;369(22):2126-2136. doi:10.1056/NEJMra1208707
4. Kim DS, Park JH, Park BK, Lee JS, Nicholson AG, Colby T. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J. 2006;27(1):143-150. doi:10.1183/09031936.06.00114004
5. Fan E, Del Sorbo L, Goligher EC, et al. An Official American Thoracic Society/European Society of Intensive Care Medicine/Society of Critical Care Medicine Clinical Practice Guideline: Mechanical Ventilation in Adult Patients with Acute Respiratory Distress Syndrome [published correction appears in Am J Respir Crit Care Med. 2017 Jun 1;195(11):1540. doi: 10.1164/rccm.19511erratum.]. Am J Respir Crit Care Med. 2017;195(9):1253-1263. doi:10.1164/rccm.201703-0548ST

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