Older Age, Male Sex Linked to Worse IPAH Survival in Korean Study

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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People with pulmonary arterial hypertension of unknown cause, or idiopathic disease, who are older, male, or of low socioeconomic status are at higher risk of death, according to a study from Korea.

Researchers also found an overall survival rate of more than 50% among newly diagnosed patients.

The study, “A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea,” was published in the Journal of Korean Medical Sciences.

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In Korea, despite the use of effective therapies that started in the early 2000s, scarce studies have assessed long-term survival, and risk and cause of death in people with idiopathic pulmonary arterial hypertension (IPAH).

Using the Korean National Health Insurance Service, the researchers identified data for 9,017 people who were newly diagnosed with IPAH from 2006 to 2017. Among these patients, the mean age was 62.3 years. Roughly 60% of patients were female, and males were significantly younger than females on average.

The overall mortality rate for the study population was 35.5%, with the common causes of death being cardiovascular disease, cancer, and diseases of the respiratory system.

Statistical analyses demonstrated that the one-year survival rate was 89% in the overall group. The survival rates at three and five years were 79.8% and 72.3%, respectively. At 10 years, the overall survival rate was 57%.

The researchers noted that the rates at one and three years “correspond well with those of other studies” done previously in the U.S., Europe, and other locations. However, the survival rates at five years are somewhat higher than has been reported in prior research. The team suggested that one of the factors explaining this difference may be a higher proportion of IPAH patients with moderate or severe disease in a previous analysis.

“Given the expanded life expectancy thanks to novel treatment medications, we think that more effort should be taken for the continued surveillance” in patients with unexplained breathlessness, to diagnose and then treat IPAH patients as early as possible, the team added.

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In further analyses, the researchers looked for factors associated with survival outcomes. Age was an important factor: among patients diagnosed with IPAH in their 60s, the 10-year survival rate was 65.3%. In comparison, for patients diagnosed at 80 or older, the 10-year survival rate was 15.6%.

Male sex, low socioeconomic status, and a history of heart attack, diabetes, or cancer were also factors associated with significantly worse survival. These differences “should be considered in future research designs and policies for cardiovascular healthcare services,” the researchers concluded, emphasizing in particular the importance of taking into account the role of biological sex.