Managing Hemoptysis
Richard S. Irwin
Kimberly A. Robinson
Overview
Hemoptysis is defined in Stedman’s Medical Dictionary as “the spitting of blood derived from the lungs or bronchial tubes.” This common symptom may be the primary reason for seeking consultation in approximately 8% to 15% of an average chest clinic population. It elicits great apprehension in the patient and is likely to prompt early medical attention. The basis for this fear is the presumption that the hemoptysis is caused by a serious disease (e.g., cancer) and that it signals impending massive bleeding. The patient may describe an associated burning pain, vague discomfort, or bubbling sensation in the chest and shortness of breath. Hemoptysis may be scant, producing the appearance of streaks of bright red blood in the sputum, or profuse, with expectoration of a large volume of blood.
Massive hemoptysis is defined as the expectoration of 600 mL of blood within 24 to 48 hours and occurs in 3% to 10% of all patients with hemoptysis [1]. Nonmassive hemoptysis produces a quantity smaller than massive hemoptysis and greater than blood streaking. Dark red clots may also be expectorated when blood has been present in the lungs for days.
Pseudohemoptysis, on the other hand, is the expectoration of blood from a source other than the lower respiratory tract. It may cause diagnostic confusion when patients cannot clearly describe the source of their bleeding. Pseudohemoptysis may occur when blood from the oral cavity, nares, pharynx, or tongue drains to the back of the throat and initiates the cough reflex; when blood is aspirated into the lower respiratory tract in patients who have hematemesis; and when the oropharynx is colonized with a red, pigment-producing, aerobic, Gram-negative rod, Serratia marcescens [2]. This colonization may occur in hospitalized or nursing home patients who have received broad-spectrum antimicrobial agents and/or mechanical ventilatory support. Other rare causes of pseudohemoptysis are self-inflicted injuries or other bizarre tactics in the malingering patient seeking hospitalization and rifampin overdose (red man syndrome). The causes and distinguishing features of pseudohemoptysis are listed in Table 53.1.
This chapter deals with managing hemoptysis in the intensive care unit (ICU) in the context of a general discussion of hemoptysis. The management of tracheoartery fistula, traumatic rupture of the pulmonary artery due to balloon flotation catheters, and diffuse intrapulmonary hemorrhage are highlighted.
Etiology
Hemoptysis can be caused by a wide variety of disorders (Table 53.2) [3]. Although the incidences of the causes of hemoptysis have been described in several populations of patients, we are not aware of any study that has reported the most frequent causes of hemoptysis in critically ill patients.
The etiology of hemoptysis is considered here in three general categories: nonmassive, massive, and idiopathic. Patients in the ICU frequently have nonmassive hemoptysis, and the spectrum of the causes of hemoptysis in these patients probably differs little from that reported in major series. Commonly, the causes include trauma (secondary to suctioning), overzealous anticoagulation, and infection. Unlike the general ICU patient, patients with massive hemoptysis are frequently in the ICU because of their hemoptysis and thereby constitute a different subgroup of patients.
Nonmassive Hemoptysis
Although bronchitis, bronchiectasis, pneumonia, lung carcinoma, and tuberculosis have always been among the most common causes of hemoptysis, their incidence has varied depending on the study population and era. For example, in the immunocompromised patient, Pneumocystis jiroveci, fungal disease, Mycobacterium tuberculosis, and Mycobacterium avium intracellulare may be at the top of the differential diagnosis [4,5,6,7,8].
Although bleeding from tracheoartery fistula complicating tracheostomy, rupture of pulmonary artery from a balloon flotation catheter, and diffuse intrapulmonary hemorrhage may be submassive, they are discussed in the following section.
Massive Hemoptysis
The more frequent causes of massive hemoptysis likely to be seen in the ICU are listed in Table 53.3. Virtually all causes of hemoptysis may result in massive hemoptysis, but it is most frequently caused by tuberculosis, bronchiectasis, lung abscess, and lung cancer [5,6]. Infection is also the cause of bleeding from aspergilloma [9] and cystic fibrosis [10]. Idiopathic hemoptysis is less frequent in patients with massive hemoptysis and usually constitutes less than 5% of cases [4].
Table 53.1 Differential Features of Pseudohemoptysis | ||||||||||||||||||||
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Rupture of a pulmonary artery complicates balloon flotation catheterizations in less than 0.2% of cases [11,12]. It is fortunate that it is uncommon because it carries a mortality rate approximating 40% [12]. With the less frequent use of this procedure, this complication will likely become even more rarely seen. Tracheoartery fistula is also an unusual but devastating condition, complicating approximately 0.7% of tracheostomies [13]. Diffuse intrapulmonary hemorrhage, usually due to an immunologically mediated disease, should also be considered in the differential diagnosis of massive hemoptysis in the ICU.
Idiopathic Hemoptysis
Using the systematic diagnostic approach outlined later and in Tables 53.4 and 53.5, the cause of hemoptysis can be found in most instances. In 2% to 32% of patients (average, 12%) [14], the cause cannot be determined. This condition, called idiopathic or essential hemoptysis, is seen most commonly in men between the ages of 30 and 50 years. Prolonged follow-up studies with rare exceptions usually fail to reveal the source of bleeding, even though 10% of patients continue to have occasional episodes of hemoptysis [15]. In a subset of patients, Dieulafoy disease of the bronchus (i.e., an abnormal superficial vessel contiguous to the epithelium of the bronchial mucosa) has been demonstrated at pathologic examination when surgery has been performed for massive bleeding [16].
Pathogenesis
To appreciate fully the pathogenesis of hemoptysis, it is necessary to review briefly the normal anatomy of the nutrient blood supply to the lungs [17]. The bronchial arteries are the chief source of blood of the airways (from mainstem bronchi to terminal bronchioles); the supporting framework of the lung that includes the pleura, intrapulmonary lymphoid tissue; and large branches of the pulmonary vessels and nerves in the hilar regions. The pulmonary arteries supply the pulmonary parenchymal tissue, including the respiratory bronchioles. Communications between these two blood supplies, bronchopulmonary arterial and venous anastomoses, occur near the junction of the terminal and respiratory bronchioles. These anastomoses allow the two blood supplies to complement each other. For instance, if flow through one system is increased or decreased, a reciprocal change occurs in the amount of blood supplied by the other system [18]. Arteriographic studies in patients with active hemoptysis have shown that the systemic circulation (bronchial arteries) is primarily responsible for the bleeding in approximately 92% of cases [19].
The pathogenesis of hemoptysis depends on the type and location of the disease [20]. In general, if the lesion is endobronchial, the bleeding is from the bronchial circulation, and if the lesion is parenchymal, the bleeding is from the pulmonary circulation. Moreover, in chronic diseases, repetitive episodes are most likely due to increased vascularity in the involved area [21].
In bronchogenic carcinoma, hemoptysis results from necrosis of the tumor, with its increased blood supply from bronchial arteries, or from local invasion of a large blood vessel. In bronchial adenomas, bleeding is usually from rupture of the prominent surface vessels. In bronchiectasis, granulation tissue often replaces the normal bronchial wall and, with infection, this area can become irritated and bleed. In acute bronchitis, bleeding results from irritation of the unusually friable and vascular mucosa [20].
The mechanism of hemoptysis in mitral stenosis is controversial, but the most likely explanation is rupture of the dilated varices of the bronchial veins in the submucosa of large bronchi [22] due to pulmonary venous hypertension. Pulmonary venous hypertension may also be responsible for the bleeding in congestive heart failure because it is associated with widening of the capillary anastomoses between bronchial and pulmonary arteries [21].
Hemoptysis in pulmonary embolism may be due to infarction, with necrosis of parenchymal tissue, or due to hemorrhagic consolidation secondary to increased bronchial artery blood flow, which forms collaterals with the pulmonary circulation to bypass the obstructing clot [23].
Table 53.2 Causes of Hemoptysisa | ||
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Table 53.3 Common Causes of Massive Hemoptysis | |
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In tuberculosis, bleeding can occur for a variety of reasons [24]. In the acute parenchymal exudative lesion, scant hemoptysis may result from necrosis of a small branch of a pulmonary artery or vein. In the chronic parenchymal fibroulcerative lesion, massive hemoptysis may result from rupture of a pulmonary artery aneurysm bulging into the lumen of a cavity [25]. The aneurysm occurs from tuberculous involvement of the adventitia and media of the vessel [26]. When a healed and calcified tuberculous lymph node erodes the wall of a bronchus because of pressure necrosis, the patient may cough up blood as well as the calcified node (broncholith). In endobronchial tuberculosis, hemoptysis may result from acute tuberculous ulceration of the bronchial mucosa. In healed and fibrotic parenchymal areas of tuberculosis, bleeding may arise from irritation of granulation tissue in the walls of bronchiectatic airways in the same areas.
Table 53.4 Routine Evaluation of Hemoptysis | ||
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Table 53.5 Special Evaluation of Hemoptysis | ||
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In traumatic rupture of the pulmonary artery by a balloon flotation catheter, risk factors include pulmonary hypertension, distal location of the catheter tip, excessive catheter manipulation in an attempt to obtain a pulmonary artery-occluded pressure measurement, a large catheter loop in the right ventricle, and advanced age [12].
In tracheoartery fistula complicating tracheostomy, bleeding is due to trauma from the tracheostomy cannula or balloon [13]. Bleeding usually is due to rupture of the innominate artery. The fistula can form at three tracheal locations: the stoma, the intratracheal cannula tip, and the balloon. Trauma at the stoma is caused by pressure necrosis, usually because the tracheostomy was created too low (below the fourth tracheal ring); at the cannula tip because of excessive angulation of the cannula; and at the balloon site due to pressure necrosis caused by use of excessive inflation pressures.
Diagnosis
General Considerations
The success rate in determining the cause of hemoptysis is excellent but variable. If one accepts the diagnosis of idiopathic (essential) hemoptysis as a distinct entity [15], the cause of hemoptysis can be determined in nearly 100% of cases [14]. The diagnostic work-up of hemoptysis involves routine (Table 53.4) as well as special evaluations (Table 53.5). Routine evaluations are initially performed in every patient, whereas special studies are ordered only when the clinical setting suggests they are indicated. In general, each category of disease (Table 53.2) has its special studies (Table 53.5).
Routine Evaluation
As in any diagnostic problem, a detailed history and physical examination must be performed. These should be performed in a systematic fashion to rule not only in the common causes of hemoptysis but also in the category of the cause (Table 53.2).
Although the amount of bleeding usually is not indicative of the seriousness of the underlying disease process, a history of the frequency, timing, and duration of hemoptysis may be helpful. For example, repeated episodes of hemoptysis occurring during months to years suggest bronchial adenoma and bronchiectasis [20], whereas small amounts of hemoptysis occurring every day for weeks are more likely to be caused by bronchogenic carcinoma [32], as hemoptysis is generally a late finding in these patients. Hemoptysis that coincides with the menses (catamenial) suggests the rare diagnostic possibility of pulmonary endometriosis [33,34], whereas bleeding associated with sexual intercourse [35] or other forms of exertion suggests passive congestion of the lungs.
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