Researchers discovered that the outcome of cardiopulmonary resuscitation (CPR) on patients with idiopathic pulmonary fibrosis (IPF) with in-hospital cardiac arrest (IHCA) remains poor, according to a study published in Pulmonary Circulation.

Patients with IPF are at a higher risk of dying should a cardiac arrest occur. Studies monitoring the progress of patients with IPF who suffer IHCA reported a 90-day survival rate of only 6%. However, newer and more targeted PAH therapies offer hope for a better prognosis.

Yang and colleagues decided to study the survival rate of patients with PAH after IHCA. The venue of the study was their own institution, which is equipped with advanced PAH-targeted therapies. They reviewed patients who had a diagnosis of pulmonary hypertension or PAH. They must not have a ‘do not resuscitate’ order or have undergone lung transplantation.

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Once a patient experiences cardiopulmonary arrest, a critical care team will be present to see the patient as soon as possible. Care is carried out according to standard resuscitation protocols and carefully documented. The cardiac arrests were classified as either respiratory, circulatory, neurological, or dysrhythmic.

Read more about IPF epidemiology 

The research team managed to review 91 patients who satisfied the inclusion criteria. Below were some of their findings: 

  • Return of spontaneous circulation occurred in just 23% of patients, while in-hospital mortality was more than 95%. 
  • In the last 2 decades or so, around 13 new PAH-targeted medications have been approved. 
  • In the last 7 years or so, physicians have begun prescribing upfront combination therapy to most patients with PAH. 
  • While extracorporeal support during CPR (e-CPR) has gained popularity over the years, it remains controversial and 10-year survival rates remain poor.

Although PAH therapies have undergone tremendous improvements over the years, it remains a chronic, progressive, and incurable disease. This means that, regardless of the short-term success of any therapeutic strategy, a time may come for the need to have end-of-life discussions with the patient and the patient’s family. In such bleak moments, it is important to remind patients that palliative care allows them “to identify and achieve their goals in their own care and end-of-life wishes,” Yang et al said.


Jang ZY, Odish MF, Mathers H, et al. Outcomes of cardiopulmonary resuscitation in patients with pulmonary arterial hypertension. Pulm Circ. Published online March 23, 2022. doi:10.1002/pul2.12066