Clinical medicine (London, England)
-
Hepatopulmonary syndrome (HPS) is often not considered as a cause of breathlessness in patients with liver cirrhosis. It consists of a triad of liver disease, intrapulmonary shunting and hypoxaemia. Here we discuss HPS in a patient presenting with acute on chronic breathlessness and typical features of orthodeoxia and platypnoea. Evidence of intrapulmonary shunting on imaging was essential to making the diagnosis, which carries a poor prognosis and was an urgent indication for liver transplantation.
-
Non-invasive ventilation (NIV) given to the right patient, in the right setting, in the right way and at the right time improves outcomes. However, national audits reveal poor practice in patient selection, clinical judgement, treatment initiation and availability of trained staff. NIV is indicated for persistent acute hypercapnic respiratory failure (AHRF) with acidosis after usual medical management in chronic obstructive pulmonary disease (COPD) exacerbation and even without acidosis in neuromuscular disorders or other restrictive conditions eg obesity hypoventilation or kyphoscoliosis. ⋯ Any presentation with AHRF is a poor prognostic indicator and outpatient respiratory follow-up is indicated following discharge. For selected patients with COPD who remain hypercapnic 2 weeks after an exacerbation, domiciliary NIV can reduce admissions and improve survival. For patients with neuromuscular disorders or kyphoscoliosis a presentation with AHRF almost always indicates the need for domiciliary NIV.