Pulmonary Arteriovenous Fistulas

Pulmonary arteriovenous (AV) fistulas are abnormal communications between the arterial and venous pulmonary circulation that result in shunting of blood from right to left without traversing the pulmonary capillary network. This shunt results in a decreased fraction of the pulmonary circulation participating in gas exchange, mixing of oxygenated and deoxygenated blood, and a consequent reduction in arterial oxygen tension (Pa o ₂ ). Many patients with pulmonary AV fistulas are asymptomatic, but some may have associated signs and possible symptoms consistent with chronic hypoxemia ( Box 4-1 ).

Known causes of pulmonary AV fistula formation include congenital malformations ( Box 4-2 ). Patients with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome), an autosomal dominant syndrome most often seen in middle-aged women but sometimes diagnosed in early childhood, are more likely to have multiple fistulas and more severe symptoms.

Patients with pulmonary AV fistula are at risk for rupture, resulting in potentially life-threatening hemothorax and hemoptysis. Thrombus formation within the fistula may also occur, with potential embolization of clot to the brain, resulting in stroke or seizures. Embolization of other organ systems is also possible. If the thrombus becomes infected, septic emboli and potential abscess formation may result.

Surgical intervention in the management of pulmonary AV fistulas becomes necessary when the patient develops more pronounced cardiac symptoms, significant respiratory symptoms, room-air desaturation, or complications such as emboli with central nervous system (CNS) manifestations. Surgical preoperative evaluation requires chest computed tomographic angiography (CTA) or pulmonary arteriography to localize the lesion. Pulmonary lobectomy, segmentectomy, or wedge resection using thoracotomy or video-assisted thoracoscopic surgery (VATS) are the most common procedures. Embolization procedures are becoming the preferred treatment for the majority of patients because embolization is less invasive, is easily repeated, and may be an adjuvant to decrease bleeding and other complications during definitive surgical resection.

Anesthetic evaluation focuses on the degree of shunt and hypoxemia, using ABG analysis. Review of the pulmonary angiogram will reveal the size of the lesion and whether multiple fistulas are present. A significant fistula in the nonoperative lung may compromise arterial oxygenation if one-lung ventilation is required for surgical exposure. Efforts to minimize flow through a pulmonary AV fistula involve avoiding both increased pulmonary vascular resistance (PVR) and elevated levels of positive end-expiratory pressure (PEEP), both of which will increase flow through the low-resistance fistula.

Intraoperative management frequently requires one-lung ventilation to optimize surgical exposure. A double-lumen endotracheal tube (ETT) provides the added benefit of isolating the nonoperative lung and airways from any bleeding, which may occur during a potentially bloody resection. The risk of significant bleeding is decreased if the lesion has been embolized before resection. Large-bore intravenous (IV) access is recommended in the event significant hemorrhage occurs. An arterial catheter is also indicated to monitor oxygenation and guide resuscitative efforts. As mentioned, an important anesthetic goal is to minimize flow through the pulmonary AV fistula. AV fistulas do not have capillary beds and have lower resistance to blood flow than normal pulmonary vasculature. It is important to avoid a general increase in PVR because this will increase flow through the AV fistula. Similarly, minimizing the use of PEEP will minimize increases in PVR and help minimize blood flow through the fistula. Because of the risk of paradoxical emboli passing through the fistula, extra caution must be taken to avoid injection of any air or particulate material into the venous system, because such debris may bypass the pulmonary capillary bed and gain access to systemic arteries, where end-organ embolization can occur ( Box 4-3 ).

Preoperative evaluation should include assessment of neurologic function to rule out prior embolic stroke. Postoperative evaluation should include a neurologic check as well, to look for perioperative CNS embolization.

Wegener’s Granulomatosis

Wegener’s granulomatosis (WG) is a rare disorder characterized by necrotizing giant cell granulomatosis of the upper respiratory tract and lung, widespread necrotizing vasculitis, and focal glomerulonephritis. WG may also affect the cardiovascular, neurologic, and gastrointestinal systems. Although the etiology of WG is unknown, an autoimmune disorder is suspected. A typical patient is in the fourth or fifth decade, and men are twice as likely to have WG as women. Antineutrophil cytoplasmic autoantibody (ANCA) is a serologic marker that can help confirm the diagnosis. Staphylococcus aureus has been implicated as an exacerbating cofactor. Symptoms associated with WG are vague, and diagnosis can be elusive ( Box 4-4 ). Biopsy of a lesion is necessary to make the diagnosis.

If the disease progresses, significant respiratory and renal compromise can occur, as well as hearing and vision loss ( Box 4-5 ). Cardiac involvement is uncommon, although pericarditis, coronary arteritis, valvular involvement, and left ventricular hypertrophy have been reported. Current therapy for WG is often based on disease severity but usually includes cyclophosphamide, corticosteroids, methotrexate, or azathioprine and yields very good results, with long-term remission occurring in the majority of patients. Recent studies have demonstrated possible advantages of antistaphylococcal antibiotics and T-cell inhibitors (leflunomide). Preoperative assessment is directed toward evaluating potential complications of WG, most often renal and pulmonary insufficiency. Blood urea nitrogen (BUN) and creatinine levels will provide adequate insight into the patient’s renal function. A pulmonary flow-volume loop may be indicated if the patient is suspected of having tracheal stenosis and, by providing information about the dynamic changes in tracheal caliber, can supplement static radiographic images. WG may cause either obstructive or restrictive lung disease; the latter can be severe. Spirometry and other PFTs such as formal lung volume measurements can help determine the severity of such disease. Bronchoscopy and neck/chest CT may be necessary to evaluate subglottic stenosis and suggest which EET size can be placed safely.

Several aspects of WG may complicate management of the patient’s airway. A significant amount of granulation tissue is likely to be present in and around the nose and nasopharynx. Insertion of a nasotracheal tube or nasal airway may be impossible, or traumatic with hemorrhage, and is best avoided. Additionally, lesions on the epiglottis or oropharynx may inhibit direct laryngoscopy, despite a normal airway examination. Once the vocal cords have been visualized, the ETT may be difficult to place because of subglottic stenosis and may require multiple laryngoscopies. If the patient is receiving corticosteroids at the time of surgery, stress dosing should be considered (see Box 4-3 ).

In view of these concerns, it is best to proceed with a conservative plan for managing the airway in WG patients, with immediate availability of difficult airway equipment, multiple sizes of ETTs, a videolaryngoscope or fiberoptic bronchoscope, and the means to obtain a surgical airway, as a last resort. If the patient has significant tracheal or bronchial stenosis, care should be taken to prevent air trapping and auto-PEEP by allowing sufficient time for exhalation if the tracheal lesion is below the ETT. (See also Chapter 1 .)

Lymphomatoid Granulomatosis

Lymphomatoid granulomatosis (LYG), also known as angiocentric lymphoma, is a rare lymphoproliferative disease that is angiodestructive and frequently progresses to lymphoma. LYG mimics WG clinically and radiographically, although recent advances have identified LYG as a malignant B-cell lymphoma associated with immunosuppression and Epstein-Barr virus (EBV). Diagnosis requires histologic evaluation of a biopsy specimen. LYG was recently categorized as a lymphoma, although if diagnosed early (grade I angiocentric immunoproliferative lesions), it is considered benign, although premalignant. Typically, it presents in the fifth or sixth decade of life, affecting men twice as often as women. The etiology of LYG is unknown, although its incidence in populations with immune dysfunction, such as human immunodeficiency virus (HIV) patients and organ transplant recipients, is significantly increased compared with the general population. Speculation that LYG resulted from an opportunistic infection has been confirmed through laboratory investigation.

The disease process primarily involves the lungs, although the skin, kidneys, and CNS can also be affected. Signs and symptoms of LYG include an increased risk of pneumonia ( Box 4-6 ). Unlike WG, glomerulonephritis is not part of this clinical picture. LYG is frequently fatal, with 60% to 90% mortality at 5 years, although a small number of patients may undergo spontaneous recovery and complete remission. The cause of death is usually related to extensive destruction of the lungs and resulting respiratory failure.

Corticosteroids and cyclophosphamide are the treatment of choice, resulting in relief of symptoms such as fever, cough, chest pain, weight loss, and sinusitis. If not diagnosed in the premalignant phase, and if the disease has progressed to lymphoma, chemotherapy is necessary. The combination of cyclophosphamide, doxorubicin (hydroxydaunomycin), vincristine (Oncovin), and prednisone (CHOP) is often used. Radiation therapy may be indicated for localized disease. More recently, immunomodulation with interferon alfa-2b and autologous stem cell transplantation have played a role in treatment.

In preparation for anesthesia, evaluation of the patient’s pulmonary function is the primary concern with LYG. Chest radiography may reveal bilateral nodules, cavitations, pleural effusions, or pneumothorax. In the presence of advanced disease, ABG analysis and spirometry help define the extent of the patient’s respiratory compromise and parenchymal destruction. A thorough preoperative neurologic evaluation is advised because of the high incidence of peripheral neuropathy. Toxicities related to any chemotherapeutic agents the patient may have received should also be considered. Toxicity related to the CHOP protocol includes peripheral neuropathy, cardiomyopathy, and myelosuppression.

Churg-Strauss Syndrome

Churg-Strauss syndrome (CSS), also known as allergic granulomatosis, is a rare systemic vasculitis that may affect multiple organ systems, particularly the lungs. Diagnosis requires the presence of at least four of six criteria: bronchospasm, eosinophil count greater than 10%, neuropathy (poly or mono), nonfixed pulmonary infiltrates, paranasal sinus abnormalities, and extravascular eosinophils ( Box 4-7 ). Patients frequently present in the fifth or sixth decade and may have a long-standing history of asthma. Both genders are affected equally. Cardiac involvement occurs later in the course and is the major cause of death. CNS manifestations such as cerebral infarcts, subarachnoid hemorrhage, and optic neuritis are common.

Corticosteroids generally result in dramatic improvement or resolution of CSS symptoms. Cytotoxic therapy should be initiated based on the severity of disease. Patients resistant to corticosteroids may respond to interferon-α treatment. Elective surgery should be postponed if management of bronchospasm has not been optimized. Involvement of other organ systems may necessitate neurologic and renal evaluations. Cardiac evaluation may require testing such as echocardiography to assess myocardial function if the patient has congestive heart failure (CHF) or endocarditis.

Preoperative assessment should include a chest radiograph and PFTs. Chest radiography may reveal multiple small pulmonary nodules or diffuse interstitial disease. Pleural effusions are noted in up to 30% of CSS patients. Spirometry typically demonstrates an obstructive pattern, although restrictive disease may also occur. A decrease in diffusion capacity may be observed from a loss of alveolar capillary surface area. Intraoperative management should include universal asthmatic principles to minimize airway reactivity. If possible, avoidance of airway instrumentation and positive-pressure ventilation (PPV) is desirable. A prolonged expiratory phase may be needed in patients with more advanced obstructive disease if PPV is used, and preoperative spirometry will provide guidance in this area. Nonselective beta-adrenergic blockers should be avoided, if possible, because of the risk of bronchospasm and exacerbation of CHF. If needed for control of ischemic heart disease, selective β ₁ -adrenergic agents, preferably short acting, should be used. Perioperative corticosteroids should be considered because of the risk of adrenal suppression from long-term corticosteroid therapy (see Box 4-3 ).

Primary Pulmonary Hypertension

Primary pulmonary hypertension (PPH) is an idiopathic disease and is a diagnosis of exclusion. The prevalence of PPH is thought to be approximately 1:1 million, with women being twice as likely as men to present with the disease. Some cases appear to be genetically linked. Overall, PPH is more severe and aggressive than secondary pulmonary hypertension. Vascular remodeling, an alteration in pulmonary vascular tone, and a loss of cross-sectional pulmonary arterial area are responsible for the increase in PVR seen in this disease. Dyspnea is the most common presenting symptom, and syncope is a particularly poor prognostic sign ( Box 4-8 ). Right-to-left shunting may occur in the 30% of patients with a patent foramen ovale (PFO). Death typically results from hypoxia, a further increase in pulmonary artery pressure (PAP), and eventually right ventricular (RV) failure.

Historically, treatment for PPH relied on oxygen and calcium channel blockers in an effort to decrease PVR ( Table 4-1 ). In addition, warfarin (Coumadin) is used to reduce the risk of thromboembolism resulting from the enhanced platelet activity seen in PPH. Pulmonary embolism or primary pulmonary vascular thrombosis is poorly tolerated in this patient population. Diuretics and digoxin are also employed when RV failure ensues. More recently, prostaglandins (PGI ₂ , PGE ₁ ; alprostadil) and nitric oxide (NO), alone or in combination, have been used to induce pulmonary vasodilation, with minimal systemic effects. Currently, prostacyclins must be delivered by continuous IV infusion because of their short half-life. NO is delivered by inhalation and requires a tank and delivery system. Phosphodiesterase-5 inhibitors such as sildenafil and dipyridamole potentiate the NO-induced pulmonary vasodilation and can be used separately or in combination. Unfortunately, cost and unwieldy delivery systems have limited the use of these therapies to the short term or the most severe cases. New approaches to delivering PGI ₂ are under development, including the inhaled, subcutaneous, and oral routes. A newer agent, bosentan, an oral endothelin receptor antagonist thought to inhibit smooth muscle vasoconstriction and proliferation, is now approved by the U.S. Food and Drug Administration (FDA) to treat PPH. Adjunctive therapy with bosentan has demonstrated promise when combined with prostacyclin therapy.

Preoperative studies focus on the severity of PPH, degree of hypoxia, and resulting effects on the heart ( Table 4-2 ). ABG analysis elucidates the level of hypoxia and acidemia, both of which exacerbate pulmonary hypertension. A chest radiograph may reveal enlarged main pulmonary arteries or an enlarged heart caused by RV hypertrophy or right atrial dilation. An electrocardiogram (ECG) may also reveal changes consistent with pulmonary hypertension (e.g., right atrial enlargement), as well as the presence of abnormal cardiac rhythm (e.g., atrial fibrillation). Sinus rhythm is essential to adequate RV filling. Preoperative echocardiography is helpful in determining the extent of RV hypertrophy and function, right atrial enlargement, pulmonic or tricuspid valve dysfunction, and patency of the foramen ovale. Pulmonary systolic pressures may be estimated by Doppler techniques. A more accurate but much more invasive method of measuring pulmonary pressures, gauging response to therapies, and detecting a PFO is right-sided heart catheterization. This procedure should be considered only if other studies have not provided an adequate assessment of disease severity and is not typically needed for preanesthetic evaluation. The patient treated with digoxin should have serum potassium and digoxin levels measured.

Cystic Fibrosis

Cystic fibrosis (CF) is an autosomal recessive genetic disease that affects chloride channels. With an incidence of 1 per 2000 to 4500 Caucasians, CF is one of the more common inherited conditions. It results in a significant reduction in life expectancy and quality of life. The responsible gene is found on the long arm of chromosome 7 and codes for a protein known as cystic fibrosis transmembrane (conductance) regulator (CFTR), which functions as a chloride channel. This defect decreases the water content of various secretions throughout the body, resulting in increased viscosity. Diagnosis is based on sweat chloride measurements, genetic testing for the CFTR gene, and clinical symptoms. CF is a universally fatal disease, although advances in therapy have resulted in significant gains in quality of life and longevity. A wide variety of clinical manifestations are seen in CF patients ( Table 4-3 ).

Pulmonary manifestations result from the inability to clear thickened and inspissated mucus from the airways. This causes airway obstruction and impaired defense against bacterial infection, which results in the majority of deaths related to CF. Recurrent bacterial infections result in dilation of the conducting airways, leading to bronchiectasis. Although CF is a chronic progressive disease, the extent of current pulmonary infection fluctuates, creating significant daily variability in a patient’s pulmonary function. Eventually, as the disease progresses, there is destruction of parenchyma and conduction airways. Loss of pulmonary arterial vascular cross-sectional area results in pulmonary hypertension. Chronic hypoxemia also develops.

Patients with more advanced CF may develop spontaneous pneumothorax. The etiology of pneumothorax is unknown but presumably involves rupture of subpleural blebs through the visceral pleura. This becomes more likely in advanced disease. Over a lifetime, the incidence of pneumothorax may be as high as 20% in adult CF patients. Application of PPV can increase the risk of spontaneous pneumothorax. In the event of pneumothorax, surgical pleurodesis is the treatment of choice for CF patients who have a low anesthetic risk; higher-risk patients frequently receive talc pleurodesis as a safer, yet less effective, alternative. Ventilation/perfusion inequality results in hypoxemia. The chronic hypoxia seen in this population causes an increase in PVR and pulmonary hypertension. Loss of pulmonary arterial vascular cross-sectional area also causes increased PVR and pulmonary hypertension, which is exacerbated by chronic hypoxemia. The severity of pulmonary hypertension correlates with the severity of CF. Chronic pulmonary vasoconstriction (from hypoxia) results in a muscularization of the pulmonary arterial vascular tree, which results in cor pulmonale, although the initial enlargement of the right ventricle is considered a beneficial adaptation to the increased resistance to pulmonary blood flow. The only medical therapy effective in treating pulmonary hypertension and improving RV performance in this population is supplemental oxygen. Although lung transplantation has been successful with a 2-year survival of greater than 50%, about 40% of patients do not survive awaiting the transplant due to organ shortage.

The primary gastrointestinal manifestation of CF is malabsorption and steatorrhea caused by pancreatic dysfunction from obstruction of pancreatic ducts with viscous exocrine secretions, usually requiring pancreatic enzyme replacements as well as multivitamins. Malnutrition and deficiencies of fat-soluble vitamins such as vitamin K can increase the patient’s risk of bleeding if this issue is not addressed. Glucose intolerance resulting from pancreatic dysfunction (impaired endocrine function) is also common and may require insulin therapy. CF patients also have an increased incidence of gastroesophageal reflux disease (GERD).

Preparation for anesthesia should focus on evaluation of the CF patient’s pulmonary status. Significant variation in symptoms and disease severity from increased respiratory secretions or infection can be seen in a patient from one day to the next. Surgery should be postponed, if possible, unless the patient is at a baseline level of health. Preoperative testing should include a recent chest radiograph to diagnose pneumothorax, pneumonic processes, or bullous disease. In one series of patients with CF, 16% had an asymptomatic pneumothorax. Thus, chest radiography is essential in these patients. Coagulation studies such as prothrombin time and partial thromboplastin time can provide information regarding coagulopathy resulting from vitamin K deficiency or general malnutrition. Sedating premedications should be given only if absolutely necessary, because of the risk of exacerbating pre-existing respiratory compromise, and only then under close observation with administration of supplemental oxygen to minimize the risk of desaturation. All CF patients should be questioned regarding symptoms consistent with GERD. If present, appropriate premedications and aspiration precautions such as a rapid-sequence induction should be considered, although CF patients may desaturate rapidly when apneic.

Bronchiolitis Obliterans Organizing Pneumonia

Bronchiolitis obliterans organizing pneumonia (BOOP) is an inflammatory lung disease of unknown etiology. It has been associated with bone marrow transplantation, although there is a very low incidence of BOOP in this population; it has not been conclusively determined to be more than an incidental finding. BOOP results from the formation of granulation tissue, which obstructs the lumen of small airways and extends into the alveoli. The formation of the granulation tissue is associated with connective tissue proliferation, fibrinous exudates, and inflammation of alveolar and airway walls. These changes yield a clinical picture that presents as a flulike illness with cough and dyspnea. BOOP shares many characteristics of idiopathic pulmonary fibrosis, with the most significant difference being the reversibility of the fibrinous changes in BOOP as a result of the preservation of lung architecture.

Corticosteroids are often used, although some cases resolve spontaneously. Typically, therapy lasts for 1 year, with resolution of symptoms by the end of the third month of treatment. Symptoms may recur, particularly if the course of corticosteroids is not completed. Other agents such as erythromycin and cyclophosphamide have been used, although their efficacy is not well established. Patients who received cyclophosphamide are at risk of leukopenia and, more rarely, thrombocytopenia or anemia.

Radiologic evaluation is consistent with an organizing pneumonia with patchy consolidation in a diffuse peripheral distribution. Effusions are a rare finding. Spirometry typically demonstrates a restrictive pattern, although it is possible to find an obstructive component. Decreased diffusion capacity and an increased alveolar-arterial oxygen gradient are common. Definitive diagnosis requires lung biopsy, typically performed thoracoscopically. BOOP occurs in 25% to 50% of long-term survivors of lung transplants, and 10% of all lung transplant recipients, indicating a poor prognosis. It is a manifestation of chronic rejection treated, usually unsuccessfully, with steroids and immunosuppressive agents.

Because of the high success rate in treating cases of BOOP unrelated to lung transplant, and because dramatic improvement is typically seen after a few weeks of therapy with prednisone, patients are unlikely to present for surgery with respiratory compromise. These factors also suggest that it may be prudent to defer all but the most emergent surgery in patients just beginning treatment for BOOP. A review of recent radiographs and spirometry, along with a history and physical examination, typically provide enough information as to whether the patient’s pulmonary function has been optimized for elective procedures.

In the event surgery is emergent and cannot be postponed, the primary anesthetic issues relate to ventilator management. As in other restrictive lung diseases, high peak pressures may occur with PPV unless appropriate reductions in tidal volume are made. Rapid arterial hypoxemia can occur with apnea because of a decreased functional residual capacity (FRC). The use of low levels of PEEP will improve FRC and assist in maintaining Pa o ₂ . Continuation of PEEP or continuous positive airway pressure (CPAP) in the postoperative period may be necessary to maintain functional residual capacity ( Box 4-10 ).

Box 4-10

Anesthesia Concerns for Patients with Infiltrative and Interstitial Disease

Bronchiolitis Obliterans Organizing Pneumonia

• 
  

  Assess pulmonary function.

  
  
• 
  

  Tailor anesthetic plan for each patient.

Idiopathic Pulmonary Hemosiderosis

• 
  

  Assess pulmonary function.

  
  
• 
  

  Evaluate for coagulopathy.

  
  
• 
  

  Plan for possible bronchoscopy and pulmonary toilet (use large ETT when possible).

  
  
• 
  

  May require stress-dose steroids.

  
  
• 
  

  Avoid high airway pressures and tidal volumes.

Chronic Eosinophilic Pneumonia

• 
  

  Assess pulmonary function.

  
  
• 
  

  Delay surgery until steroid therapy implemented.

  
  
• 
  

  May need intraoperative bronchodilators.

  
  
• 
  

  Utilize PEEP cautiously and at low levels, if needed at all; minimize intrathoracic pressures to decrease shunt.

Goodpasture’s Syndrome

• 
  

  Assess cardiopulmonary and renal function (BUN/creatinine, urinalysis, ABGs, ECG, echocardiography, spirometry).

  
  
• 
  

  Maintain oxygenation but limit supplemental O ₂ to lowest level consistent with arterial saturation >90%.

  
  
• 
  

  Consider invasive monitors.

  
  
• 
  

  Arterial catheter used for all but the mildest disease; consider TEE or PAC if assessment of volume status or adequacy of cardiac output unclear.

Pulmonary Alveolar Proteinosis

• 
  

  Assess pulmonary function (level of dyspnea, baseline O ₂ saturation, time since last BPL, ABGs, chest radiograph).

  
  
• 
  

  Double-lumen ETT required for BPL.

  
  
• 
  

  Invasive monitoring (PAC, TEE) may facilitate intraoperative management for higher-risk procedures.

Sarcoidosis

• 
  

  Assess all organ system involvement (PFTs, ECG, echocardiography, BUN/creatinine).

  
  
• 
  

  Evaluate airway to rule out lesions by indirect laryngoscopy or CT.

  
  
• 
  

  Possible postoperative ventilatory support.

  
  
• 
  

  Consider invasive monitors.

  
  
• 
  

  May require perioperative stress-dose steroids.

Systemic Lupus Erythematosus

• 
  

  Assess all organ system involvement (chest radiograph, PFTs, ABGs, BUN/creatinine, LFTs).

  
  
• 
  

  Invasive monitors may be indicated if cardiac or pulmonary involvement and for type of surgery (arterial catheters).

  
  
• 
  

  Minimize airway manipulation secondary to risk of inflammation and potential laryngeal involvement.

  
  
• 
  

  Refrain from nitrous oxide use secondary to bone marrow suppression.

  
  
• 
  

  Ensure thorough evaluation of medications:

  
  
  
  
  • 
    

    Echocardiography may be indicated for cardiac function with high-dose cyclophosphamide or hydroxychloroquine.

    
    
  • 
    

    LFTs should be evaluated for hepatotoxicity (azathioprine, methotrexate).

    
    
  • 
    

    May require stress-dose steroids.

    
    
  • 
    

    May require increased doses of neuromuscular blockers if taking azathioprine.

    
    
  • 
    

    Cyclophosphamide may prolong effects of succinylcholine.

  
  

Idiopathic Pulmonary Fibrosis

• 
  

  Assess cardiopulmonary function, (PFTs/spirometry, ECG, echocardiography).

  
  
• 
  

  Evaluate for pulmonary hypertension/cor pulmonale.

  
  
• 
  

  Aspiration precautions should be considered secondary to association with gastroesophageal reflux disease.

Acute Respiratory Distress Syndrome

• 
  

  Assess cardiopulmonary function.

  
  
• 
  

  Lung protective ventilation: low tidal volumes (~6 mL/kg predicted body weight); PEEP to maintain arterial saturation >90%; <30 cm H ₂ O plateau pressures.

  
  
• 
  

  Utilize permissive hypercapnia as needed.

  
  
• 
  

  Consider invasive monitors (arterial/central venous catheters, TEE).

  
  
• 
  

  Provide supportive care, with carefully guided fluid resuscitation.

  
  
• 
  

  Ensure postoperative ventilatory support.

Pulmonary Histiocytosis X

• 
  

  Assess cardiopulmonary function.

  
  
• 
  

  Tailor anesthetic management to progression of disease.

  
  
• 
  

  Give special attention to possible pathologic fractures.

Lymphangioleiomyomatosis

• 
  

  Assess cardiopulmonary function.

  
  
• 
  

  May need enteral/parenteral nutrition perioperatively.

  
  
• 
  

  Consider postoperative ventilation support.

BPL , bronchopulmonary lavage; BUN , blood urea nitrogen; CT , computed tomography; ETT , Endotracheal tube; LFTs , liver function tests; PAC , pulmonary artery catheter; PEEP , positive end-expiratory pressure; TEE , transesophageal echocardiography.

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