Hemoptysis may be initially classified and triaged as shown in Table 17-1 .

It is often difficult to estimate the amount of blood expectorated by the patient, and pragmatically, it is easier to divide patients into three groups: minor, major (massive), and exsanguinating.

Minor hemoptysis can change into massive hemoptysis ; the dilemma is to predict the future severity of an initial episode of minor grade hemoptysis because catastrophic hemorrhage can occur in otherwise stable patients. Rebleeds may have mortality rate as high as 45% if definitive treatment has not been started.

Mortality is associated with

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  The severity of bleeding:

  
  
  
  
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    9% for hemoptysis of < 1000 mL/24 h

    
    
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    58% for hemoptysis of >1000 mL/24 h

  
  
  
  
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  The rate of blood loss

  
  
  
  
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    71% mortality for a rate of >600 mL in 4 hours.

    
    
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    45% mortality for a rate of 600 mL in 4 to 16 hours.

    
    
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    5% mortality for a rate of 600 mL in 16 to 48 hours.

  
  
  
  
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  Hemorrhage that occurs unexpectedly in stable patients may cause death ; mortality is also associated with rebleeds, and is higher with emergent versus elective surgery and neoplasia.

  
  
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  The mortality rate for conservative treatment of massive hemoptysis (including later rebleeds) is sufficiently high that in all cases, there should be consideration of other treatment modalities (bronchial artery embolization or surgery, or both) for candidates with localized disease.

The definition of massive hemoptysis varies widely among authors: in many series, massive hemoptysis has been defined as starting as low as 200 to 400 mL per 24-hour period and may increase to 1000 mL. Most authors define massive hemoptysis as expectoration of 400 to 600 mL per 24 hours. Others define hemoptysis as one episode of expectoration of at least 200 mL. Massive hemoptysis occurs in only a small percentage of all cases of hemoptysis (1.5%–8%) ; the highest it has ever been in any series of patients with hemoptysis is 14%.

Chronic inflammatory nontuberculosis lung disease is one of the leading causes of massive hemoptysis in the Western World. (See Table 17-1 .) However worldwide, tuberculosis is a leading cause of hemoptysis, although the frequency of massive hemoptysis with this infection is low given the wide prevalence of the disease. It is estimated that 2 billion people are infected by the tuberculosis (TB) bacillus and that 5% to 10% of those infected will develop tuberculosis. Regional referral centers for tuberculosis have higher cluster rates of this as the causative agent for massive hemoptysis.

The causes for hemoptysis are not only geographic but also seasonal in that in trends in hemoptysis of Western societies follow large population seasonal trends of respiratory infections.

Massive hemoptysis is commonly associated with a structural lung abnormality or focus of lung inflammation. Rarely is a hemorrhagic diathesis the primary cause of massive hemoptysis, although it is associated with a higher mortality rate in association with other causes of hemoptysis.

There are many causes of hemoptysis, as shown in Table 17-2 ; however, certain diseases have a higher predilection for massive hemoptysis compared with minor hemoptysis. The common causes of massive hemoptysis in Western societies are as listed in Table 17-2 . Certain disease states (e.g., TB or malignancy) may feature more prominently than others depending on the specialty care and referral pattern for institutional reports.

Hemoptysis in children is uncommon, and is disease and center dependent. The common causes for childhood hemoptysis are

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  Cystic fibrosis

  
  
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  Congenital heart and lung disease

  
  
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  Pneumonia and bronchitis

  
  
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  Tuberculosis

  
  
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  Adenomas

  
  
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  Vasculitis

Bronchial artery flow

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  Arise from the aorta or intercostal arteries and form a plexus in the peribronchial space.

  
  
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  Small penetrating arteries then form a submucosal plexus and supply nutrition and oxygen to the airway support structures including the pulmonary artery vessels (vaso vasorum) but play no role in gas exchange.

Pulmonary arterial flow

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  Account for 99% of the arterial blood supply to the lungs and are solely responsible for gas exchange.

Intersections between the bronchial and arterial blood flow occur as follows:

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  Larger anastomoses between the medium-sized bronchial arteries and the alveolar microvasculature

  
  
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  At the level of the vasa vasorum of the pulmonary arteries, which run parallel to the airways

  
  
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  There are direct connections (anastomosis) between bronchial and pulmonary arteries at the level of the bronchial submucosa), which is physiologic and accounts for a shunt of 5% of cardiac output.

Source of hemoptysis:

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  Bronchial artery in 90% of cases

  
  
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  Pulmonary artery in 5% of cases

  
  
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  Large vessel (aorta or pulmonary artery) fistula to the airway

Bronchial artery hemoptysis is usually associated with bronchial artery occlusion due to:

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  Acute or chronic inflammation

  
  
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  Acquired

  
  
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  Congenital

  
  
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  Reduced or occluded pulmonary artery circulation through the pulmonary arterioles secondary to hypoxic vasoconstriction, thrombosis, or vasculitis

This induces aberrant bronchial artery vessel proliferation, which leads to hypertrophy of these pulmonary vascular anastomoses. It can also induce collateral flow from extrabronchial sources, which creates new vessels that are thin walled and fragile. Under inflammatory conditions and exacerbated by systemic arterial pressure, these vessels can rupture to cause hemoptysis of minor or more often major degree.

Inflammatory vasculitis with vessel wall inflammation and degeneration may also lead to small vessel rupture, leading to alveolar hemorrhage.

Small vessel disease

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  Small vessel disease causing hemoptysis is attributable to dilated, aberrant, weak connections in the vaso vasorum between the bronchial arterial system, which, in turn, transmits systemic pressures to the lower pulmonary arterial system. Increased angiogenic factors, such as vascular endothelial growth factor and angiopoietin 1, are induced by the following:

  
  
  
  
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    Infection (bronchiectasis, tuberculosis, lung abscesses)

    
    
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    Chronic inflammation may cause enlarged ectatic submucosal arteries from the bronchial arterial system. Degeneration of the vessel walls of these ecstatic vessels may rupture spontaneously to cause hemoptysis.

    
    
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    Neoplasia, primary cancers, metastatic disease, or adenomas with hypervascularity (angiogenesis) or vessel wall fragility

    
    
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    Congenital—Arterovenous malformation, sequestrations (intra- or extralobar, or isolated vascular sequestrations) or isolated pulmonary artery agenesis or stenosis with extrapulmonary aberrant systemic blood supply.

    
    
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    Immune complex deposition due to capillary wall inflammation, and necrosis may produce pulmonary alveolar hemorrhage.

    
    
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    Lung infarction

    
    
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    Diffuse alveolar hemorrhage

  
  
  
  
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  Neoplastic

  
  
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  Associated with specific disease entities;

  
  
  
  
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    Renal failure

    
    
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    Coagulopathies

    
    
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    Blood element dyscrasias: aplasias, or leukemias due to invasive aspergillosis or stem cell transplantation

    
    
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    Catamenial hemoptysis with presumed aberrant hypervascularity due to ectopic endometrial tissue in the lung

  
  
  
  
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  Cryptogenic

  
  
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  Iatrogenic—bronchoscopic biopsy of an aberrant submucosal vessel (Dieulafoy’s lesion)

Large vessel disease

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  Acquired major vessel disease:

  
  
  
  
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    Aortic—fistulas to airways:

    
    
    
    
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      Tracheoinomminate artery fistulas following tracheostomy

      
      
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      Aortic graft fistulas to airways

      
      
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      Aortic false aneurysms with erosion into the bronchus

      
      
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      Airway stents with erosions in the aorta

    
    
    
    
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    Pulmonary artery aneurysms:

    
    
    
    
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      Pulmonary artery tear by Swan-Ganz catheter

      
      
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      Penetrating trauma to the lungs

      
      
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      Large vessel vasculidities, such as Behçet’s disease, Takayasu’s disease, neoplasia, or chronic inflammation

      
      
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      Chronic tuberculous pulmonary infection—Rasmussen’s aneurysm

    
    
    
    
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    Malignant invasion with intrapulmonary rupture of either the aorta or the pulmonary artery

  
  

To emergently deal with massive or exsanguinating hemoptysis

To identify those patients whose sentinel bleed may presage a second more massive bleed

To identify patients with massive hemoptysis who would not tolerate resectional surgery either as an initial treatment or as an alternative for other failed therapies

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  An impaired health status (reduced cardiac, pulmonary or overall performance status)

  
  
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  Disease progression (locally advanced or disseminated malignancy)

  
  
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  Siffuse lung bleeds suggesting a systemic problem

Urgency of initiation of investigations based on:

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  Amount of hemoptysis

  
  
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  Cardiopulmonary reserve of the patient

  
  
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  Disease status (large vessel versus small vessel disease)

  
  
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  Possibility of a massive rebleed following a bleed with a previous lesser grade of hemoptysis

Initial investigations:

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  Complete blood count with platelet count

  
  
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  Prothrombin time/partial thromboplastin time

  
  
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  Electrocardiogram

  
  
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  Chest radiograph

  
  
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  Urinalysis (rule out [r/o] hematuria for a vasculitis)

  
  
  
  
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    Consider:

    
    
    
    
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      Cross match blood for possible transfusion

      
      
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      Spirometry and arterial blood gas for early assessment of pulmonary function.

      
      
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      Anti-Gb antibodies for Goodpasture’s syndrome

      
      
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      Erythrocyte sedimentation rate, perinuclear antineutrophil cytoplasmic antibody, or circulating antineutrophil cytoplasmic antibody for vasculitis

    
    

  
  

Subsequent investigations:

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  Computed tomography (CT) of the chest

  
  
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  Multiplanar CT of the chest

  
  
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  Bronchoscopy (see Table 17-3 for comparison of flexible and rigid bronchoscopy for the evaluation of massive hemoptysis ) .

  
  
  
  

  TABLE 17-3 ▪

  
  

  COMPARISON OF FLEXIBLE AND RIGID BRONCHOSCOPY FOR THE EVALUATION OF MASSIVE HEMOPTYSIS

  
  

  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  

  Flexible Bronchoscopy

  Pro : Performed under local anesthesia Ventilation postprocedure not required for poor-risk patients May be easier to obtain procedural time

  Goals of: Localization Identification of etiology of hemoptysis Obtaining culture or pathology specimens Cessation of bleeding with vasoactive agents Insertion of bronchus blockers if lung isolation is necessary

  Con : May be aggravated by bleeding, which Prevents the identification of lesions May overwhelm suction capabilities leading to asphyxiation May induce coughing which can aggravate hemoptysis Removal of clots difficult with flexible bronchoscope

  Rigid Bronchoscopy

  Pro : Performed under general anesthesia, which provides Better airway protection for episodes of massive bleeding Better airway suctioning for clots or fresh bleeding Positive pressure ventilation as counter pressure Cessation of coughing as a cause of hemoptysis More rapid institution of lung isolation with bronchus blockers or double-lumen endotracheal tubes Allows flexible bronchoscopy through the rigid bronchoscope

  Cons : Requires general anesthesia implying: Poor-risk patients may have difficulties with independent pulmonary recovery If extubation is not possible due to lung soilage with blood or poor respiratory mechanics, further pulmonary function testing is prevented, which confounds the ability to assess resections of local lesions Requires ventilation and care that may not be desired for terminal patients

   
  
  
  
  
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  Bronchial artery angiogram (BA)—always consider embolization if BA angiogram is deemed necessary.

  
  
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  Pulmonary artery (PA) angiogram for specific disease states:

  
  
  
  
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    PA aneurysms

    
    
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    Arteriovenous (AV) malformations