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Learning points Radiological features consistent with interstitial pneumonitis have been reported in 0.7% of patients receiving BCG immunotherapy for carcinoma of the bladder. 1 It is debated as to whether the interstitial pneumonitis associated with intravesical BCG is predominantly a hypersensitivity reaction or is a manifestation of miliary disease caused by BCG, or both.

Early diagnosis of pulmonary artery hypertension (PAH) is diagnostically challenging given the extent of pulmonary vascular remodeling required to bring about clinical signs and symptoms. Exercise testing can be invaluable in this setting, as stressing the cardiopulmonary system may unmask early disease. This report describes a young patient with a positive family history of PAH in whom contemporaneous invasive cardiopulmonary exercise testing and surgical lung biopsy reveal the novel association between exercise pulmonary hypertension (ePH) and early histological changes of PAH. Exercise PH currently carries no pathological correlates which means the hemodynamic effects of early pulmonary vascular remodeling remain unknown. Following the recent proceedings from the World Symposium in Pulmonary Hypertension 2018, which broaden the hemodynamic definition of PAH, this report suggests an important association between ePH and early pulmonary vascular remodeling supporting a role for exercise hemodynamic evaluation in patients at increased familial risk of PAH.

A 77-year-old retired engineer presented to accident and emergency with deteriorating shortness of breath that had been troubling him for several months. At that time, he was being investigated by a chest physician who had identified bilateral diaphragmatic paralysis on ultrasound and was awaiting further imaging. Clinical assessment and nerve conduction studies on this admission were compatible with a diagnosis of motor neuron disease but specialist neurology input recommended an MRI to rule out cord pathology. This proved problematic as the patient was non-invasive ventilation dependent and unable to lay supine as this further compromised his respiratory function. To ensure that a potentially reversible cause for his symptoms was identified, the patient was intubated for an MRI which subsequently demonstrated multi level spinal epidural empyema. The benefits of neurosurgical intervention were judged to be uncertain at best, and following discussion with the family, active care was withdrawn. The patient passed away shortly thereafter.

Discussion Apical lung herniation in adults is rare particularly in the absence of penetrating lung injury or chest wall disease. 1 2 It is due to a defect in the suprapleural membrane (Sibson's fascia), and small incidental apical parietal pleural defects have been described which may be present prior to the development of a larger defect. 3 4 Tearing of the fascia and spontaneous hernias have been described in players of wind instruments, weightlifters and those with a chronic cough, which may have occurred in our patient. 2 5 The defect is usually large enough to prevent trapping and incarceration of the lung. 3 5 6 Repair may be necessary in patients with incarceration, symptoms of local compression, for example, dysphagia from oesophageal compression, or for cosmetic purposes. 6 7 The diagnosis can be easily missed both clinically and radiologically if examination is not made during a Valsalva manoeuvre.

Pulmonary functions tests should not be performed in patients with pneumothoraces, recent myocardial infarction, unstable angina, recent thoracic-abdominal surgery, and recent eye surgery. 1 The production of a flow volume curve requires the patient to expel air forcefully as quickly and for as long as possible from a maximal inspiration-a forced vital capacity manoeuvre. The preferred treatment for tracheal stenosis is tracheal resection with reanastomosis. 5 6 In patients with multiple comorbidities who are unsuitable for formal surgical repair, dilatation of the stenotic area combined with neodymium:yttrium aluminium garnet (Nd:YAG), laser, or tracheal stenting may be indicated. 7 8 9 10 A multidisciplinary approach involving thoracic surgeons; ear, nose, and throat surgeons; anaesthetists, and chest physicians is essential in the management of these patients. 5 Patient outcome Rigid bronchoscopy confirmed an area of tracheal stenosis 4 cm below the vocal cords causing 80% tracheal obstruction.

A 77-year-old retired engineer presented to accident and emergency with deteriorating shortness of breath that had been troubling him for several months. At that time, he was being investigated by a chest physician who had identified bilateral diaphragmatic paralysis on ultrasound and was awaiting further imaging. Clinical assessment and nerve conduction studies on this admission were compatible with a diagnosis of motor neuron disease but specialist neurology input recommended an MRI to rule out cord pathology. This proved problematic as the patient was non-invasive ventilation dependent and unable to lay supine as this further compromised his respiratory function. To ensure that a potentially reversible cause for his symptoms was identified, the patient was intubated for an MRI which subsequently demonstrated multi level spinal epidural empyema. The benefits of neurosurgical intervention were judged to be uncertain at best, and following discussion with the family, active care was withdrawn. The patient passed away shortly thereafter.

1 2 3 4 5 They can also occur in patients with chronic lung infections who have associated lung necrosis or spontaneous pneumothorax, particularly in the presence of underlying parenchymal disease. 2 6 7 Postoperative bronchopleural fistulas can occur at any time but are most common during the second week after surgery. 8 Fistulas that present before this time may be related to inadequate closure of the bronchial stump. 8 Small amounts of air and fluid, visible on a chest radiograph, are normally seen in the pleural space after resection, but these are gradually reabsorbed and the space left after pneumonectomy completely obliterated by three weeks to seven months. 8 A decrease in the fluid level on a chest radiograph combined with the production of frothy sputum suggests the loss of fluid through a bronchopleural fistula into the bronchial tree. Short answer Factors associated with an increased risk of postoperative bronchopleural fistula include right pneumonectomy, immunocompromise, preoperative pleuropulmonary infection or radiotherapy, mechanical ventilation, and residual carcinoma at the bronchial margin. 1 9 Long answer Other risk factors associated with the development of postoperative bronchopleural fistulas include preoperative anaemia, Haemophilus influenzae in sputum, postoperative fever, and a raised white blood cell count. 10 Surgical factors include a long bronchial stump, which predisposes to pooling of secretions and increased risk of infection; tight sutures; and excessive dissection, which may result in tissue ischaemia. 8 Fistulas are more common after right pneumonectomy, and the risk is higher for main and intermediate bronchial stumps than for lobar bronchi. 1 Risk factors for unsuccessful closure of bronchopleural fistulas include multiple comorbidities, infection, and poor nutritional status; these last two problems should be rectified and patients' comorbidities optimised before repairs are attempted. 3 How should this patient be managed?

Low molecular weight heparin should be continued for at least five days and until the international normalised ratio is two or more for at least 24 hours. 11 Thrombolytic treatment is indicated in patients with haemodynamic compromise-systolic blood pressure less than 90 mm Hg-in the absence of a major risk factor for bleeding. 11 A high risk of death exists when a pulmonary embolus is associated with hypotension and cardiogenic shock. 12 13 Thrombolysis in these patients results in a better outcome with more rapid thrombus lysis, resolution of perfusion abnormalities, and improvement in right ventricular dysfunction and pulmonary angiogram pressures compared with heparin alone. 11 Agents that can be used include alteplase (10 mg given as an intravenous (IV) bolus followed by 90 mg infusion over two hours), reteplase (10 U IV bolus followed by another 10 U IV bolus after 30 minutes), or tenecteplase (0.5 mg/kg as an IV bolus with maximum of 50 mg). Screening with a transthoracic echocardiogram is a useful non-invasive test to see if there is evidence of raised pulmonary pressures, but a normal transthoracic echocardiogram does not exclude pulmonary hypertension. [...]confirmation with right heart catheterisation is essential to determine true pulmonary artery pressures.

Cardiac catheterisation showed minor coronary artery disease, with equal diastolic pressures in the left and right atria and ventricles. A tuberculin skin test was performed (2 tuberculin units in 0.1 ml solution for injection), and the palpable raised area measured 22 mm. An interferon gamma test was not performed because the test was not available at the time of presentation. Magnetic resonance imaging can show the pericardium more clearly because this technique has excellent resolution and multiplanar capability. 1 2 The accuracy of magnetic resonance imaging in measuring pericardial thickness is 93%; as such, this approach is a useful non-invasive tool to diagnose constrictive pericarditis. 3 4 5 6 Furthermore, magnetic resonance imaging can be used to assess myocardial motion by various techniques. The average duration of symptoms before definitive diagnosis is 11-20 months. 11 12 Some patients present with liver disease secondary to hepatic congestion. Cardiovascular examination might show raised jugular venous pressure, sinus tachycardia, pericardial knock, paradoxic pulse (pulsus paradoxus), and Kussmaul's sign. Pericardial knock is described as an early diastolic sound that occurs when ventricular filling is abruptly halted by the constricted fibrotic pericardium, whereas paradoxic pulse is defined as an inspiratory fall in systolic blood pressure of greater than 10 mm Hg. Echocardiography might also show pericardial thickening, as well as demonstrate characteristic haemodynamic changes such as septal notching, ventricular septal shift with respiration, and dilation of the inferior vena cava without inspiratory collapse. 14 15 16 Pericardial thickening is identified most accurately with cardiac magnetic...