Haemoptysis: Diagnosis and Treatment

Acute Medicine 2012; 11(1): 39-45 39 Trainee Section

K Hurt & D Bilton

Abstract There are no consensus guidelines available for Haemoptysis is a common symptom in clinical practice, the management of haemoptysis and its treatment which requires further investigation. Fortunately, massive can be challenging, even for experienced physicians. haemoptysis only accounts for a small proportion of these episodes. It is a medical emergency that carries a high Pathophysiology mortality rate. There are no agreed management guidelines. The lungs have a double arterial supply. The This review discusses proposed methods of resuscitation as pulmonary arteries carry deoxygenated blood under well as outlining a diagnostic algorithm and discusses low pressure to the lungs for the purpose of gas treatments including bronchial artery embolization, exchange. The bronchial arteries account for <1% of endobronchial therapy, surgery and medical therapies. the circulation to the lungs and supply oxygenated blood to the lung parenchyma. They are under Keywords systemic pressure, originating from the descending Haemoptysis, bronchoscopy, bronchial artery embolization, aorta, usually between 3rd and 8th thoracic vertebral CT thorax, lung cancer, bronchiectasis, cystic fibrosis. level, most commonly between the 5th and 6th. 6 There is a wide anatomical variation in bronchial Introduction artery anatomy. In around 5-10% of people the Haemoptysis can be defined as the expectoration of anterior medullary artery that supplies the anterior blood from the lower respiratory tract. The amount spinal artery arises from the intercostal bronchial of blood can vary widely, from streaking of the trunk. However a recent report of bronchial artery sputum to rapidly fatal massive haemoptysis. Whilst embolizations suggests that this figure is somewhat haemoptysis in general is a relatively common lower. 7 clinical problem, massive haemoptysis remains Around 90% of haemoptysis will originate from fortunately very rare. The exact incidence is the bronchial arteries and around 5% from the unknown because there is no agreed definition in pulmonary arterial circulation. 8 Mechanisms of bleeding are multi-factorial. In the literature as to what constitutes massive Katharine Hurt haemoptysis. Definitions range from a blood loss of the presence of chronic inflammation and infection MBBS MRCP 100 mls of blood expectorated in 24 hours 1 up to bronchial arteries hypertrophy and proliferate. There Clinical Research Fellow, 1000mls in 24 hours. 2 Most respiratory physicians is also enlargement of the usual systemic to Royal Brompton Hospital would use a definition somewhere between 200- pulmonary artery and pulmonary vein and Imperial College, 600mls/24 hours. It has been suggested that volume communications. These factors lead to increased London expectorated definitions are not useful in clinical blood flow and neovascularization. The resultant Diana Bilton practice and actually it is better to define thin walled vessels are at risk of rupturing into the FRCP MD functionally. 3,4 There are good reasons for this. airway causing haemoptysis. 7,9 Consultant Respiratory Haemoptysis is difficult to quantify, patients may Physician, over report amounts produced and the physiological Case History Royal Brompton Hospital and Imperial College, effects of haemoptysis will depend on the patient’s A 28 year old woman with cystic fibrosis (CF) and severe London clinical status. Death from massive haemoptysis is bronchiectasis presented to her local hospital after a single usually through a process of asphyxiation, rather than episode of haemoptysis. This was estimated, by the patient, Correspondence: exsanguination. 5 to have been around 200mls. She also described increased Katharine Hurt Haemoptysis is a serious symptom that requires shortness of breath, right sided chest pain and dizziness. Department of Cystic further investigation as it may be a sign of underlying Her CF was complicated by diabetes, for which she Fibrosis Royal Brompton Hospital required insulin, and chronic infection with Pseudomonas severe disease. The most common causes vary Sydney Street geographically with tuberculosis (TB) being the aeruginosa; she took a number of regular medications London most common underlying aetiology in the including pancreatic enzymes, vitamins, prophylactic SW3 6NP developing world and elsewhere bronchitis and lung nebulised antibiotics (colomycin) and a nebulised mucolytic. Email: k.hurt@imperial cancer. 2 She was not taking anticoagulant or anti-platelet drugs. .ac.uk

Neoplastic Airway Disease History of presenting complaint It is important to clarify that bleeding has arisen from the Bronchogenic carcinoma Bronchiectasis lower respiratory tract and to exclude haematemesis and Pulmonary metastatic Bronchitis epistaxis as alternative explanations. Attempts to quantify disease the amount of blood should also be made, although this Kaposi’s sarcoma Cystic Fibrosis can be very difficult.

Infection Primary Vascular Disease Associated symptoms and risk factors Bacterial pneumonia Pulmonary AV malformations Any current or past diagnoses of respiratory disease should TB Pulmonary embolism be elicited and a detailed respiratory history should be taken to try and ascertain the underlying diagnosis. Mycetoma Pulmonary hypertension Symptoms of cough, sputum production, fever, chest pain Non tuberculous Congestive cardiac failure and weight loss could suggest acute infection, TB, chronic mycobacterial disease bronchitis, chronic suppurative lung disease or lung cancer Respiratory viral infection Mitral stenosis depending on time course. Risk factors for PE should be considered as well as symptoms of heart failure and valvular Parasitic disease Miscellaneous heart disease. In addition a smoking and travel history Systemic Disease Foreign body inhalation should be taken. Goodpasture’s syndrome Endometriosis A careful drug history, especially use of anticoagulant should be noted, any association with menses for female Wegener’s granulomatosis Amyloidosis patients should be considered and risk factors for HIV Microscopic polyarteritis Pulmonary sequestration infection should be identified. Concurrent systemic upset Systemic lupus Iatrogenic Lung Injury and rash may suggest an underlying vasculitis. erythematosus Clinical examination Coagulopathy In addition to a detailed respiratory examination, general Table 1. Important causes of haemoptysis. examination should involve assessment of nutritional status. Finger nails should be checked for signs of clubbing. What are the possible causes of her Lymph nodes should be examined in the neck, haemoptysis? supraclavicular and axilla regions. The skin and mucous Table 1 lists the most important causes of haemoptysis. membranes should be inspected for signs of bruising, Lung cancer accounts for around 20-30% of cases in pallor, rash, telangiectasia and gingivitis. recent series. Despite extensive investigation, the cause of Full cardiovascular examination should be performed, looking specifically for signs of congestive heart failure, haemoptysis remains unknown in 3-43% of patients. 10-12 In this case the presence of known bronchiectasis pulmonary hypertension, thromboembolic disease and makes this the most likely cause of her haemoptysis, valvular heart disease. although other causes in the list should be considered. How do you investigate and manage non On arrival in the emergency department she was apyrexial, massive haemoptysis? alert and orientated; her heart rate was 88/min with a blood Chest x-ray (CXR) is an important initial investigation. pressure of 100/64mmHg. Her respiratory rate was 18/min and Tumours, consolidation and mycetomas may be obvious. oxygen saturations 97% on air. Cardiovascular and abdominal Bilateral alveolar shadowing may suggest alveolar examination was unremarkable. Respiratory examination revealed haemorrhage and bronchiectasis may be seen. However, coarse crepitations throughout her right lung. CXR will fail to reveal the diagnosis in up to 46% of cases. 14 It is also important to remember that a normal What are the important features of history CXR does not mean that a diagnosis of lung cancer can be and examination when assessing a patient excluded. When investigating haemoptysis up to 24% of with haemoptysis? patients diagnosed with lung cancer had a normal CXR at For the purposes of management the patient needs to be the time of presentation. 15 Depending on the CXR there allocated into one of the following groups based on history are three main investigation routes. 16 and clinical findings. 1. If the CXR demonstrates a mass lesion, the patient should be referred to a respiratory specialist for 1. Minor haemoptysis (which may be investigated and further investigation and treatment, 17 which will treated as an outpatient) usually involve a staging CT scan, bronchoscopy (or 2. Major / massive haemoptysis with clinical stability. percutaneous lung biopsy) and cancer 3. Major / massive haemoptysis with clinical instability multidisciplinary team discussion. Initial assessment should use the ‘ABCDE’ approach in 2. If the CXR demonstrates parenchymal accordance with resuscitation council guidelines. 13 abnormalities referral to a respiratory specialist is recommended. A high resolution CT scan would

be the next investigation of choice, and further mortality rates of up to 38%, 10 although this is probably more specialist investigation and treatment based on falling, with older reviews suggesting higher mortality this result should be initiated. If the history is rates. 19 Despite the high mortality associated with massive suggestive of vasculitis, blood should be sent for haemoptysis there are no agreed consensus guidelines for specific autoantibodies such as anti-neutrophil management and respiratory physicians will adopt varying cytoplasmic antibodies (ANCA) or anti nuclear approaches. 20 antibodies (ANA). If sputum is produced this should be sent for microscopy, culture and acid-fast Resuscitation and airway management bacillus smear and culture. Initial stabilisation using the ABCDE approach should 3. If the chest x-ray is normal and the haemoptysis focus on airway protection, appropriate oxygenation and is low volume the patient should be referred to a adequate volume resuscitation. 13 respiratory specialist as an outpatient. If the The patient should be managed in a high dependency history or examination suggests bacterial area, supported by experienced physicians, nursing staff infection, antibiotics should be given according and allied healthcare professionals. If there is immediate to local policy. 16 If the patient has risk factors for threat to the patient’s airway or evidence of respiratory lung cancer, CT scanning and bronchoscopy may failure the patient should be intubated with a large be arranged following respiratory specialist endotracheal tube (>size 8) to allow for adequate review. 17 This is usually unnecessary for non- suctioning followed by bronchoscopy. 8 smoking patients aged less than 40, particularly If it is known which lung the bleeding originates from, after an isolated episode. 18 If the haemoptysis the patient should be managed bleeding side down to try recurs or persists, CT scanning and and prevent contralateral aspiration. The patient may be bronchoscopy may still be required. able to give a history of bubbling on one side. Single lung ventilation can also be considered to protect the Case Progression contralateral lung. 21 This will require the use of a double Initial blood test revealed a raised white cell count and normal lumen endotracheal tube inserted by an experienced haemoglobin, platelets and clotting. Renal and liver function were operator. 22 If a double lumen tube is not available the also normal patient can undergo selective lung intubation by inserting an endotracheal tube directly over a bronchoscope into the Her initial CXR demonstrated severe bronchiectasis that was left or right main bronchus. However it should be borne in unchanged from previous films. There was no evidence of acute mind that selectively ventilating the right lung may lead to infection, mycetoma or any other cause for the haemoptysis inadvertent occlusion of the right upper lobe due to its (Figure 1). proximal origin, leaving only the lower and middle lobe How should massive haemoptysis be managed? for ventilation. In this situation a Fogarty catheter may be passed into the left main bronchus under bronchoscopic Massive haemoptysis is a medical emergency, which carries guidance and the balloon inflated, occluding the bronchus a high mortality. Recent studies have demonstrated and allowing the trachea to be Intubated and the right lung to be ventilated without risk of aspiration. 23 Thorough suctioning of blood and clots should take place to improve gas exchange. Large bore cannulae should be inserted; blood should be taken for full blood count, clotting studies, liver and renal function, arterial blood gases, inflammatory markers and cross match of at least 6 units of blood. 8 The patient should be volume resuscitated with crystalloid or blood 24 and clotting abnormalities corrected.

What do you do once the patient is stabilized? Once the patient has been haemodynamically stabilized, the airway is secure and the patient has adequate gas exchange the next priority of treatment is to localise and treat the source of bleeding (Figure 2). Transfer to a specialist centre for definitive treatment may be required; early consultation with a thoracic surgical team is advised. If this is the case discussion with a senior physician experienced in patient transfer should be Figure 1. Admission chest X-ray demonstrating widespread consulted for the safest way to do this. bronchiectasis; a port-a-cath is present overlying the lateral aspect of There is no consensus on further investigation. If the the right lung field. patient has been stabilized a CT scan can be performed,

Massive haemoptysis

Admit to HDU/ICU

At risk Airway

Patent Intubate with large ET tube. Consider single lung ventilation

Breathing Respiratory distress

No respiratory distress

Give Oxygen

Circulation Volume resuscitation. Xmatch and correct clotting Crystalloid and blood Reassess

Unstable Stable

Bronchoscopy CT scan

Bronchial Artery Embolization Medical Therapy Endobronchial treatment Failure

Surgery

Figure 2. Treatment algorithm for massive haemoptysis.

which will often identify the source of bleeding and procedures are being considered. Otherwise fibre optic establish the cause. 19 Contrast enhanced multidetector CT bronchoscopy can be used, which identifies the site of provides angiographic studies that have recently been bleeding in up to 93% of cases. 19 shown to be superior to conventional angiography 25 and is important for considering suitability and planning What are the treatments available for bronchial artery embolizations. Bronchoscopy may be massive haemoptysis? required after CT scanning in some circumstances. 23 In the Bronchoscopic treatment for endobronchial lesions case of bronchiectasis and CF, bronchoscopy is not There are a number of therapeutic techniques that can be indicated as endobronchial therapy is unlikely to be performed through a bronchoscope, most of them best beneficial and may delay time prior to bronchial artery performed through a rigid bronchoscope and usually only embolization. 26,27 in specialist centres. Instillation of epinephrine (1:20,000) If the patient with massive haemoptysis is too unstable to an identified point of bleeding can be used. It is effective to undergo CT scanning, bronchoscopy should be in mild to moderate haemoptysis but of doubtful efficacy performed immediately. Rigid bronchoscopy may be in major haemoptysis. 28 Cold saline lavage, 29 fibrinogen required if there is massive bleeding (to allow for rapid compounds combinations 30 and tranexamic acid 31 may also suctioning) or if certain therapeutic bronchoscopic be used, although evidence is limited.

Interventional bronchoscopic techniques include endobronchial balloon tamponade. In this procedure a Fogarty catheter can be used to tamponade using flexible bronchoscopic guidance. 32 Stents have also been used with success. Other methods that have been used but are not routinely available include the endobronchial insertion of a haemostatic mesh and endobronchial sealing with glue. 19 Laser photocoagulation using a Nd-Yag laser was first used in 1982 33 and is potentially a definitive treatment, however, the literature is mixed in reporting it’s success. Eletrocautery, cryotherapy and brachytherapy may play a role in palliation of haemoptysis in the context of lung cancer but there is very little evidence available to support their use.

Bronchial artery embolization (BAE) BAE is now a widely used and successful technique, which provides immediate control of bleeding in most patients (86-99%). 34 Outcomes have gradually improved since the 1970s when the procedure was first performed. 35 During Figure 3. The contrast enhanced CT aorta/thorax demonstrates the procedure the patient undergoes a descending thoracic multiple enlarged bronchial arteries, more evident on the right. aortogram to identify bronchial artery anatomy and bleeding site. The most commonly used embolic material is polyvinyl alcohol. 36 resistant mycetoma. Otherwise, it is indicated only Recurrence is common, occurring in 10-55% of following failure of BAE, 8 due to high mortality rates, patients, and outcome is dependent on underlying disease. 34 particularly in the context of significant comorbidities. 2 Minor temporary side effects such as chest pain and dysphagia are common but fortunately neurological Medical treatment complications secondary to spinal cord ischaemia are rare Treatment of the underlying cause should be initiated (1.4-6.5%) and becoming rarer with super selective when possible. catheterisation. 34 Systemic embolization is also a risk. Treatments include antibiotics for infection, antituberculous therapy in the case of TB and systemic Surgery antifungals for Aspergillus lung disease. Surgery is the treatment of choice for AV malformations, Bleeding abnormalities should be treated with the trauma including pulmonary artery rupture and treatment appropriate blood product. In the case of alveolar

Figure 4. Bronchial artery angiography. These images demonstrate bronchial artery angiography pre and post embolization using embolization particles.

haemorrhage, management should be in conjunction with Case Outcome renal physicians and may include immunosuppression and The patient was treated with analgesia, intravenous tranexamic plasma exchange. 37 acid, intravenous terlipressin and intravenous ceftazidime and Although tranexamic acid is often used, the evidence colomycin. She remained haemodynamically stable, and was for its use is limited mainly to CF. 38 Intravenous subsequently transferred to a specialist centre with a view to vasopressors have been advocated but currently their use is bronchial artery embolization. probably best restricted to patients with CF in specialist She proceeded to undergo contrast CT of the thorax/aorta centres. 39 (Figure 3), which demonstrated multiple enlarged bronchial arteries The treatment of pulmonary embolism and significant with two arteries identified for embolization. The CT also haemoptysis provides an uncomfortable situation for the demonstrated severe bronchiectasis and small airway disease. clinician and risk benefit ratios of immediate Angiography and bronchial artery embolization were undertaken anticoagulation will have to be considered. (Figure 4). She had no further episodes of haemoptysis during that admission but repeat embolization was required 3 months later following a similar admission with recurrent haemoptysis.

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