Massive haemoptysis

Massive haemoptysis is coughing up blood in a volume or at a rate that threatens life by asphyxiation, usually taken as over 100 to 200 mL in 24 hours (definitions range up to 600 mL). Any bleeding that causes airway compromise, hypoxaemia or haemodynamic instability counts, whatever the volume. Death is nearly always from asphyxia and not exsanguination.

Source. About 90% arises from the bronchial arteries (high pressure systemic supply), and most of the rest from the pulmonary arteries or non-bronchial systemic collaterals. Pulmonary artery bleeding, as from a Rasmussen aneurysm or pulmonary artery pseudoaneurysm, is high volume and has a high mortality.

Priorities. Protect the airway and the non-bleeding lung, locate the source, stop the bleeding by embolisation or surgery, and treat the cause.

Key distinctions. Haemoptysis is frothy, bright red, alkaline and mixed with sputum. Haematemesis is dark, acidic and mixed with food. Epistaxis and oral bleeding cause blood to be coughed up after being aspirated from the upper airway.

History.

• Volume, colour, frequency, duration, previous episodes, side felt.
• Fever, cough, sputum, weight loss, night sweats, contact with tuberculosis, previous tuberculosis (cavitation, aspergilloma), HIV infection.
• Chronic lung disease (bronchiectasis, cystic fibrosis), smoking, occupational exposure, cancer, previous radiotherapy.
• Chest pain, calf pain, recent surgery or immobility (pulmonary embolism), cardiac disease (mitral stenosis).
• Renal symptoms, haematuria, rash, sinus disease, joint pain (vasculitis).
• Anticoagulants, antiplatelets, cocaine use, recent bronchoscopy or biopsy, trauma, mouth ulcers or genital ulcers (Behcet disease), travel (paragonimiasis), livestock contact (hydatid disease).

Examination.

• Airway patency, respiratory rate, saturation, work of breathing, ability to speak, haemodynamic state.
• Chest: crackles, wheeze, bronchial breathing, signs of consolidation or effusion, murmurs (mitral stenosis), clubbing, lymphadenopathy, telangiectasia (hereditary haemorrhagic telangiectasia), vasculitic rash, pulsatile mass.
• Nose and mouth to exclude upper airway sources.

Red flags.

• Over 100 mL in a single episode or over 200 mL in 24 hours.
• Saturation under 90%, stridor, increasing respiratory rate, drowsiness, shock.
• Bilateral crackles after bleeding (blood in the contralateral lung).
• Haemoptysis with anticoagulation.

Special groups. Pregnancy: pulmonary embolism, mitral stenosis and tuberculosis. Children: foreign body, cystic fibrosis, congenital heart disease. Region specific: tuberculosis (the commonest cause worldwide), chronic pulmonary aspergillosis after tuberculosis, paragonimiasis, hydatid disease.

Infective.

• Pulmonary tuberculosis (cavitary, post tuberculous bronchiectasis, Rasmussen aneurysm), chronic pulmonary aspergillosis with aspergilloma, necrotising pneumonia, lung abscess.
• Bronchiectasis (including cystic fibrosis), acute bronchitis (rarely massive), paragonimiasis, hydatid disease.

Neoplastic. Primary lung cancer (squamous cell carcinoma, bronchial carcinoid), pulmonary metastases.

Vascular.

• Pulmonary embolism with infarction, pulmonary arteriovenous malformation, hereditary haemorrhagic telangiectasia.
• Aortic aneurysm with aortobronchial fistula, pulmonary artery pseudoaneurysm, Behcet disease with pulmonary artery aneurysm.
• Mitral stenosis, left heart failure with pulmonary oedema, pulmonary hypertension.

Immune and diffuse alveolar haemorrhage. Granulomatosis with polyangiitis, anti-glomerular basement membrane disease, systemic lupus erythematosus, microscopic polyangiitis.

Traumatic and iatrogenic. Chest trauma, bronchial or tracheal injury, foreign body, bronchoscopic or transthoracic biopsy, pulmonary artery catheter injury.

Haematological and toxic. Anticoagulant effect, thrombocytopenia, coagulopathy, cocaine related alveolar haemorrhage.

Cryptogenic. No source found in a minority.

Immediate. Saturation, blood gas, full blood count, urea, creatinine, electrolytes, coagulation, group and crossmatch, ECG. Chest radiograph (localises the side in about half; it may be normal).

Source and cause.

• CT angiography of the chest as soon as the patient is stable enough: identifies the bleeding site, bronchial and non-bronchial systemic arteries, pulmonary artery aneurysm, cavity, mass, bronchiectasis and aspergilloma, and plans embolisation.
• Bronchoscopy: rigid bronchoscopy for ongoing massive bleeding (large suction channel, ventilation and therapeutic tools), flexible bronchoscopy for localisation and treatment once the airway is secure. Bronchoscopy is also valuable when CT is not available.
• Sputum for acid fast bacilli, nucleic acid amplification test, culture, fungal culture and Aspergillus IgG. Blood cultures if sepsis.
• Autoimmune tests (ANCA, anti-glomerular basement membrane antibody, antinuclear antibody, complement), urinalysis for blood and protein, and renal function when vasculitis or alveolar haemorrhage is suspected.
• Echocardiography for mitral stenosis, pulmonary hypertension and right heart strain, and CT pulmonary angiography for pulmonary embolism.

Limitations. Bronchoscopy cannot always locate the source when bleeding is brisk. CT may be limited by motion and contrast risk in kidney impairment, though this should not delay imaging in a life threatening bleed. Chest radiographs can be misleading since aspirated blood causes infiltrates in the non-bleeding lung.

Airway first.

• Position the patient with the bleeding side down (lateral decubitus) when the side is known, to protect the other lung. Suction. High flow oxygen.
• Intubate for hypoxaemia, exhaustion or ongoing massive bleeding, using a large bore tube (8.0 mm internal diameter or more) to allow bronchoscopy. Selective intubation of the non-bleeding main bronchus, or a bronchial blocker or double lumen tube, isolates the bleeding lung.
• Keep sedation minimal when not intubating, and avoid cough suppressants and opioid doses that depress respiration in a non-intubated patient.
• Ventilate with the lowest effective pressures and positive end expiratory pressure as tolerated.

Circulation. Two large bore cannulae and resuscitate with blood products for shock.

Correct coagulopathy:

• Warfarin: 4 factor prothrombin complex concentrate 25 to 50 units/kg IV plus vitamin K 5 to 10 mg IV slowly.
• Platelets to over 50 x 10^9/L.
• Calcium chloride 10% 10 mL IV after every 4 units of red cells.
• Fibrinogen concentrate 2 to 4 g IV when under 1.5 g/L.

Stop antiplatelets and anticoagulants (except when life threatening thrombosis needs them, then specialist decision).

Local haemostasis.

• Tranexamic acid (evidence is limited): nebulised 500 mg (5 mL of 100 mg/mL) every 8 hours, or 1 g IV over 10 minutes every 8 hours for up to 5 days.
• Monitor for thrombosis and seizures; reduce IV dose in kidney impairment (CrCl 30 to 50 mL/min give 1 g every 12 hours).
• Avoid nebulised use in a ventilated circuit without filters because of clot formation.
• Bronchoscopic measures: iced isotonic saline aliquots of 50 mL, topical adrenaline 1 in 10,000 in 1 to 2 mL aliquots (monitor for tachycardia and hypertension), endobronchial balloon blockade, and laser or argon plasma coagulation for visible lesions.

Definitive control.

• Bronchial artery embolisation: first line for most causes, with immediate control in about 70 to 99% and recurrence in about 10 to 55% (higher in aspergilloma, cystic fibrosis and incomplete embolisation). Non-bronchial systemic artery and pulmonary artery embolisation (coils or plugs) for pulmonary artery sources. Spinal artery involvement is a rare serious risk.
• Surgery (lobectomy or pneumonectomy) when embolisation fails or is not available, for aspergilloma with recurrent bleeding, and for localised lesions in a patient with enough lung reserve. Operative mortality is high when done as an emergency.
• Radiotherapy for inoperable tumour bleeding.

Cause directed treatment.

• Tuberculosis suspected: isoniazid 5 mg/kg (maximum 300 mg), rifampicin 10 mg/kg (maximum 600 mg), pyrazinamide 25 mg/kg (maximum 2000 mg) and ethambutol 15 mg/kg (maximum 1600 mg), all orally once daily for 2 months, then isoniazid and rifampicin for 4 months.
• Give pyridoxine 10 to 25 mg daily; send sputum before starting; monitor liver tests, visual acuity and uric acid.
• Adjust for liver and kidney impairment; check rifampicin interactions with anticoagulants and antiepileptics.
• Granulomatosis with polyangiitis, anti-glomerular basement membrane disease or lupus with alveolar haemorrhage: methylprednisolone 500 to 1000 mg IV daily for 3 days with early specialist immunosuppression (rituximab or cyclophosphamide, plasma exchange for anti-glomerular basement membrane disease); monitor glucose, blood pressure and infection.
• Pulmonary embolism with infarction: anticoagulation decided by the multidisciplinary team in the presence of bleeding, with a vena caval filter if anticoagulation is impossible.
• Pneumonia or abscess: amoxicillin with clavulanic acid 1.2 g IV every 8 hours for 5 to 7 days as a minimum, extended for abscess; adjusted to cultures.
• Mitral stenosis: diuretic and rate control while arranging valvuloplasty or surgery.

Monitoring. Continuous saturation, ECG, arterial pressure, urine output, serial haemoglobin and gas. Chest radiograph after intubation and after any procedure. Daily review of blood loss volume.

Referral and escalation. Critical care, interventional radiology, thoracic surgery and respiratory medicine on first recognition. ENT if the bleeding is from the upper airway. Infectious diseases or tuberculosis services where relevant.

Admission criteria. All patients with massive haemoptysis go to critical care or high dependency. Patients with mild haemoptysis (under 20 mL in 24 hours), normal observations and a quick diagnostic plan may be managed as outpatients.

Discharge and safety netting. Discharge when bleeding has stopped for at least 24 to 48 hours, the cause is defined and a plan exists. Patients are told to sit upright, to lie with the bleeding side down if bleeding recurs, and to call emergency services at once for any large haemoptysis, breathlessness or dizziness. Smoking cessation, adherence to tuberculosis treatment and follow up CT or bronchoscopy at 6 weeks are arranged.