Management of an unusual cause of life-threatening haemoptysis: a multidisciplinary approach

Abstract

We present to you a case of life-threatening haemoptysis secondary to non-cystic fibrosis bronchiectasis complicated by bronchial artery pseudoaneurysms. We discuss this patient’s emergency medical management using intravenous tranexamic acid, which resulted in successful resuscitation and eventual survival, and evaluate the need for urgent anaesthetic and interventional radiology input in such a case.

Background

Haemoptysis is defined as the expectoration of blood from the tracheobronchial lobe or lung and is further divided into massive and nonmassive categories depending on the volume lost.^{1 2} More recently, the term ‘life-threatening haemoptysis’ has superseded the use of ‘massive haemoptysis’, now focusing this definition of haemoptysis on causing either haemodynamic or respiratory compromise.³ Haemoptysis accounts for 10%–15% of all respiratory consultations.⁴ The most typical causes of haemoptysis are bronchiectasis, malignant lung tumours, bronchitis and pneumonia.⁵ Using this unique case, we hope to highlight the sole, successful use of tranexamic acid to achieve haemostasis in patients with life-threatening haemoptysis of suspected multifactorial origin, namely, non-cysticfibrosis bronchiectasis and bronchial artery pseudoaneurysms. To the best of our knowledge, there is very little literature pertaining to the management of this rare, combined aetiology of haemoptysis.

Case presentation

A 76-year-old woman presented to the emergency department (ED) resuscitation area via urgent ambulance transfer with a 2-day history of persistent and progressively increasing frank haemoptysis and shortness of breath. Initially in small amounts up to 6 times a day, this had now increased to a volume of 250 mL with tissue, and was witnessed on two further occasions in the emergency department.

She denied any features consistent with vasculitis or constitutional upset, such as weight loss or fever. There was a background of chronic cough for the preceding year. She disclosed having haemoptysis on one occasion 2 years before, which was managed as a pulmonary embolism, but was not on anticoagulation prior to this admission.

Her previous medical history included chronic obstructive pulmonary disease (COPD) and a previous stroke with minimal residual deficits. She was a current smoker with a >50 pack year history. Her WHO performance status score was 2.⁶ Her exercise tolerance was normally less than 100 yards, and she used a walking stick indoors and a wheelchair for outdoors.

Investigations

Arterial blood gases were taken, showing a pH 7.38, PaO2 of 6.9 kPa and haemoglobin of 104 g/L. Laboratory blood tests later revealed a similar haemoglobin level of 107 g/L, and a raised white cell count of 11.2×10⁹/L. There was no thrombocytopaenia or coagulopathy that required reversal.

Portable chest X-ray revealed right lower zone ground glass air space opacity (figure 1). An urgent CT angiogram, performed prebronchial and postbronchial arterial phase, revealed focal fibrosis and mild bronchiectasis in the right upper lobe (figure 2). In addition, there were two tiny areas of abnormal arterial phase enhancement in the apical segment of the right upper lobe, suggestive of bronchial artery pseudoaneurysms and subtle filling defects of the posterior walls of the central bronchial divisions, in keeping with minimal blood in airways due to haemoptysis (figure 3).

Figure 1.

Portable anteroposterior chest radiograph showing patchy ground glass air space opacity in the right lung lower zone (white arrow).

Figure 2.

Contrast-enhanced CT scan of chest, axial image, lung window showing a focal area of fibrosis and bronchiectasis in the right upper lobe (white arrow).

Figure 3.

Coronal reconstructed maximum intensity projection image from thoracic CT angiogram showing right bronchial artery branches (white arrow) and two tiny contrast filled structures within the fibrotic segment of right upper lobe (black arrow) suspicious for tiny pseudoaneurysms.

Differential diagnosis

In view of the large volume nature of the haemoptysis, immediate diagnoses considered were bronchial/pulmonary arterial haemorrhage and subsequently, pulmonary embolism. Other differential diagnoses considered were chronic lung infection, haemorrhagic lung malignancy or pulmonary vasculitis.

Treatment

The patient was alert and able to expectorate, thus maintaining her airway while being placed in the right lateral decubitus position. An urgent anaesthetic review was arranged in case of deterioration. Oxygen delivery was optimised via Venturi mask at 60%, maintaining oxygen saturations at 93%. Intubation and ventilation were not attempted due to her normal Glasgow coma scale score and ability to expectorate and therefore, ability to clear her airway independently.

The trust major haemorrhage protocol was activated to ensure the prompt provision of blood products, and red blood cell concentrate transfusion was commenced. The patient was transfused with two units before admission to an acute respiratory ward. Intravenous tranexamic acid was initiated immediately, and broad-spectrum antibiotics were prescribed because of right lower zone consolidative appearances on the chest X-ray. A combined review from the intensive care and anaesthetic teams was undertaken to ensure that airway support could be provided to facilitate urgent treatments such as bronchial artery embolisation.

The on-call interventional radiologist was consulted, and a bronchial artery embolisation was attempted. However, it could not be completed due to the procedure being technically unfeasible, and no clear abnormal vessel was being identified. Tranexamic acid was therefore continued in this case alongside antibiotics and oxygen therapy, given the likely cause of the bleeding was from the focal area of bronchiectasis in the right upper lobe.

Outcome and follow-up

The patient was transferred to a medical ward and gradually improved with conservative medical management comprising broad-spectrum antibiotics and tranexamic acid. Her haemoptysis gradually subsided, and her oxygen requirements slowly improved. Given that her admission occurred during the COVID-19 global pandemic, SARS-CoV2 was not detected from a reverse transcriptase polymerase chain reaction (rtPCR) nasopharyngeal swab. No pathological micro-organisms were detected in sputum samples, and immunology screen testing for common pulmonary vasculitides showed no convincing immunological evidence of active vasculitis. She was discharged successfully after 8 days with support from community teams and a care package.

Discussion

In this case, the aetiology of the significant haemoptysis was complex and could not beentirely attributed to one causative pathophysiological process. From the radiological evidence, bronchiectasis was the most likely cause. Bronchiectasis and smoking can cause significant haemoptysis due to the formation of bronchial artery pseudoaneurysms and bronchial wall inflammation, respectively.⁷

Significant haemoptysis often occurs from the bronchial arterial circulation due to its high vascular pressure as these vessels arise from the descending aorta.⁸ The presence of chronic infections or inflammatory lung diseases leads to an increased risk of bleeding as the normal vascular anastomoses between bronchial arteries and lower pressure pulmonary vessels become more prominent, dilated and tortuous, thereby becoming prone to spontaneous haemorrhage.⁹

Successful management of this respiratory emergency relied on timely and accurate recognition of the aetiology, as the management of common causes such as arterial or aneurysmal rupture or embolism proves to be dichotomous. Prioritising appropriate investigation such as CT angiogram over CT pulmonary angiogram could be pivotal, so a CT angiogram was prioritised over CT pulmonary angiogram, as this would alter management and require the input of interventional radiology to perform bronchial artery embolisation. This procedure is now an established treatment for bleeding from bronchial arteries and can control bleeding in 66%–90% of patients.¹⁰ Surgery is, however, still recommended in select cases, such as a prior history of chest trauma or iatrogenic rupture of the pulmonary arteries.¹¹

Tranexamic acid, a synthetic derivative of lysine, exhibits an antifibrinolytic effect by blocking the interaction of plasminogen with the lysine residues of fibrin.¹² It was used in this case to achieve haemostasis in the assumed area of haemoptysis. Tranexamic acid has been shown in other retrospective studies to reduce in-patient mortality and length of stay in patients presenting with haemoptysis.¹³ This case highlights its potential efficacy in the management of life-threatening haemoptysis, where bronchial artery embolisation is not technically feasible or immediately available as a specialist, interventional radiology service.

It is also important to consider other causes of significant volume haemoptysis. Haemoptysis of similar severity can be seen in chronic pulmonary infections, particularly those causing cavitating lung diseases such as pulmonary tuberculosis and pulmonary aspergillomas.^{14 15} Diffuse alveolar haemorrhage (DAH) secondary to systemic vasculitides, such as Anti-Neutrophilic Cytoplasmic Autoantibodies (ANCA)-associated vasculitis (AAV), is an important differential but may be less common than DAH secondary to extrapulmonary diseases such as glomerulonephritis.¹⁶ Alveolar haemorrhage in AAV has important implications as it is an indication for intensive immunosuppressive treatment with high-dose glucocorticoids, cyclophosphamide and plasma exchange due to its life-threatening potential.¹⁷ Specific drug therapies such as dual antiplatelet therapy can be a rare cause of life-threatening haemoptysis and should be considered in similar presentations.¹⁸

Multidisciplinary team (MDT) input is vital in complex cases of life-threatening haemoptysis such as this. Early discussion with intensive care and anaesthetic colleagues is crucial as airway support may be needed to facilitate any procedural intervention. Respiratory and local interventional radiology services require early consultation to determine the suitability and feasibility of bronchial arterial embolisation. While it is unlikely that this patient would have benefited from prolonged invasive ventilation for an exacerbation of her COPD due to her frailty and functional baseline, it was agreed that short-term intubation to offer potentially curative treatment of life-threatening haemoptysis would have been appropriate. Therefore, efforts should always be taken to initiate early MDT discussion and decision-making with close patient involvement, as this will ideally provide the level of care in the patient’s best interests.¹⁹

Patient’s perspective.

I was initially worried about how unwell I was at first, and it was really frightening. Though it has taken a long time to recover from what happened, I am happy that I am doing alright now.

Learning points.

• This case shows that tranexamic acid is a useful adjunct conservative management of life-threatening haemoptysis following unsuccessful interventional radiological input.

• In this case, the challenges of diagnosing and managing an emergency with dichotomous aetiologies required careful treatment planning, prioritisation and the expertise of multiple specialties

• This case highlighted the role of effective and timely teamwork and communication between the multi-disciplinary team members in the emergency department setting for the implementation of safe patient-centred care.