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512 PART 4: Pulmonary Disorders

should be considered despite the fact that this finding can result from bronchiectasis), surgery is not advised. For patients with localized
localized bleeding if the blood is extensively aspirated. If uncertainty disease the decision to operate is based on clinical judgment and indi-
remains, bronchoscopy should be performed. Diffuse alveolar hemor- vidual patient assessment, as there are no randomized trials comparing
rhage is suggested by finding an increasing concentration of red blood the outcomes achieved with surgery versus repeated embolization. The
cells in serial samples of bronchoalveolar lavage fluid obtained from a major indications for surgery are (1) recurrence of bleeding after bron-
wedged position. chial arterial embolization, (2) inability to perform the embolization
because of anatomic problems, and (3) multiple bleeding vessels seen on
TREATMENT angiography. Accordingly, with the possible exception of endobronchial
carcinomas that require ablation, BAE should be the initial treatment
■ GENERAL MEASURES of choice for patients with localized disease who are stable enough to

Maintaining adequate oxygenation is the most important initial treatment. attempt the procedure.
Next, underlying coagulopathies should be corrected. The platelet count ■ DIFFUSE ALVEOLAR HEMORRHAGE
should be maintained above 50,000 mm in an actively bleeding patient. Diffuse alveolar hemorrhage (DAH) occurs in conjunction with, and
3
The platelet dysfunction associated with uremia may be treated with may be the initial manifestation of, numerous diseases, conditions,
dialysis or by administration of cryoprecipitate and desmopressin and medications (see Table 57-1). Up to one-third of patients with DAH
(DDAVP). Additionally, DDAVP and cryoprecipitate are standard treat- will not have hemoptysis, but many will have other systemic manifesta-
ments for bleeding patients with von Willebrand disease and hemophilia A. tions of a systemic disease (eg, rash, myalgias, arthralgias, or conjunc-
Factor VIIa has been shown to be effective as a temporizing measure in tivitis). Chest roentgenograms generally show diffuse infiltrates, but
patients with cystic fibrosis and bronchiectasis (likely due to vitamin K localized disease may also be seen. DAH occurs in up to 10% of patients
and coagulation factor deficiencies in this patient population), but with granulomatous vasculitis (formerly called Wegener granuloma-
this intervention has also been associated with pathologic thrombosis tosis), and in up to 33% of patients with microscopic polyangiitis (all
on selected patient populations. In addition, intravenous or topical of whom will have evidence of glomerulonephritis). DAH is also seen
antifibrinolytics have proven beneficial in some case series. Lastly, the in approximately 5% of patients with systemic lupus erythematosus,
prothrombin time and partial thromboplastin time should be corrected and rarely in conjunction with polymyositis, rheumatoid arthritis, and
to near normal. Clotting factor deficiencies are treated with vitamin K, mixed connective tissue disease. Most patients with Goodpasture syn-
or for a more rapid effect, with large volumes of fresh frozen plasma. drome (which is caused by an antibody to the type 4 collagen found in
■ SPECIFIC INTERVENTIONS the basement membranes of alveolar walls and glomeruli) present with

A number of modalities may be used to treat life-threatening hemop- DAH and glomerulonephritis, but up to 10% may have only DAH.
tysis including endobronchial treatment techniques, bronchial arterial Diagnosis of DAH: The diagnosis of DAH is suggested when patients
embolization (BAE), external beam irradiation, and surgical resection. present with hemoptysis, diffuse pulmonary infiltrates, a falling hema-
Endobronchial treatment should begin with serial injection of cold tocrit, and manifestations of systemic disease. However, in those patients
saline lavage to the affected site (50 mL aliquots of normal saline cooled without hemoptysis or diffuse infiltrates the diagnosis becomes more
to 4°C). It may require up to 750 mL for complete effect. If this technique difficult. Bronchoalveolar lavage showing progressively more blood
is unsuccessful topical vasoconstrictive agents (epinephrine 1:20,000) with serial aspiration is considered diagnostic. If DAH is diagnosed
are recommended. Topical antifibrinolytic agents (tranexamic acid) as and serologic tests described above are nondiagnostic, kidney biopsy or
6
well as endoscopic instillation of fibrin/thrombin mixtures have been thoracoscopic lung biopsy is recommended to determine the etiology
used successfully in multiple case series, but have not been explored (Table 57-1). The correct diagnosis is especially important because
in controlled clinical studies for this indication. Balloon tamponade is therapies are often cytotoxic and frequently have long-term side effects.
used for stabilization prior to BAE or surgery. A variety of interventional Furthermore, treatments differ depending on the specific disease
bronchoscopic techniques have been described including laser pho- diagnosed. For example, plasmapheresis is indicated for Goodpasture
tocoagulation, argon plasma coagulation, electrocautery, cryotherapy, syndrome and ANCA-associated vasculitis, but not for lupus vasculitis.
brachytherapy and stent tamponade, though these techniques require Immunofluorescence should be performed on every biopsy sample to
special equipment, highly trained bronchoscopists, and they have not exclude serology-negative Goodpasture syndrome. Despite treatment,
been rigorously studied. 6 over half of patients with DAH resulting from systemic vasculitis or
External beam irradiation has been used successfully in a few patients collagen vascular disease require mechanical ventilation, and mortality
with massive hemoptysis resulting from mycetomas and can also be used ranges from 25% in patients with granulomatous vasculitis to 50% in
for patients with unresectable neoplasms if the rate of bleeding is suf- patients with lupus.
ficiently slow that the course of treatment can be completed.
BAE is the treatment of choice for most patients with life-threatening
hemoptysis resulting from a localized lesion. Though the success rate is
>90% in recent studies due to improvement in technique, recurrence KEY REFERENCES
can occur in 10% to 55% of patients, most commonly in patients with
10
mycetoma, bronchogenic carcinoma or cystic fibrosis. A common reason • Fartoukh M, Khalil A, Louis L, et al. An integrated approach to
for rebleeding is incomplete embolization due to pulmonary artery or diagnosis and management of severe haemoptysis in patients
nonbronchial artery contributions. Multidetector row CT scan prior admitted to the intensive care unit: a case series from a referral
to BAE identifies these additional vascular contributions guiding more centre. Respir Res. 2007;8:11.
complete embolization and results in a decrease in rebleeding events. • Ibrahim WH. Massive haemoptysis: the definition should be
Repeat BAE is often safe and effective although recurrence in myceto- revised. Eur Respir J. 2008;32:1131-1132.
mas and carcinomas should prompt a consideration of surgical resection • Jeudy J, Khan AR, Mohammed TL, et al. ACR Appropriateness
if the patient can tolerate the procedure. Criteria hemoptysis. J Thorac Imaging. 2010;25:W67-W69.
■ SURGICAL VERSUS MEDICAL MANAGEMENT • Khalil A, Fartoukh M, Parrot A, Bazelly B, Marsault C, Carette

When hemoptysis occurs in the setting of diffuse disease (eg, cys- MF. Impact of MDCT angiography on the management of patients
with hemoptysis. AJR Am J Roentgenol. 2010;195:772-778.
tic fibrosis, extensive pulmonary tuberculosis, or other forms of

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