Age, gender impact survival with IPAH, South Korean study finds

With the sparse data available on survival rates in patients with idiopathic pulmonary arterial hypertension (IPAH) in the Republic of Korea, researchers have offered new insights into long-term survival and the factors that impact survival. survival.

Their findings recently appeared in Journal of Korean Medical Sciences.

Based on more than a decade of data, the researchers found that the 10-year survival for more than 9,000 patients with IPAH was 57%. Among patients, lower survival rates were observed if they were male, had lower income, and had certain comorbidities.

“Our reported models of survival rate and risk of death for IPAH should be considered in future research designs and policies for cardiovascular health care services, with particular attention paid to gender,” said concluded the researchers. “Furthermore, the results of the current study may provide new insights into the contemporary prevalence of IPAH and will be useful in establishing health care policy regarding this rare, incurable, but clinically important disease.”

At 1 year, the patient survival rate was 89% and the 3-year survival was 79.8%. The researchers note that these results were comparable to those of other studies, including the US registry REVEAL, which showed 1- and 3-year survival rates of 91% and 74%, respectively, in patients with IPAH. between 2006 and 2009.

The researchers cautioned that their data came from National Health Insurance benefit records, which may not include patients with IPAH who did not seek medical services or who paid out-of-pocket medical costs, which may have led to an under- or over-estimation of survival rate and risk of death.

Among their cohort, the researchers found that the most common cause of death was diseases of the circulatory system, accounting for almost 1 in 3 deaths, followed by malignant neoplasms (16.8%) and diseases of the respiratory system (13. 4%). Diabetes (HR, 1.29; 95% CI, 1.17-1.42), history of myocardial infarction (HR, 1.51; 95% CI, 1.24-1.83), fibrillation atrial (HR, 1.40; 95% CI, 1.15-1.70), history of stroke ischemia (HR, 1.44; 95% CI, 1.25-1.64), prior hemorrhagic stroke (HR, 1.48; 95% CI, 1.02-2.14) and malignancy (HR, 2.83; 95% CI, 2.47-3.23) were all associated with increased risk.

Notably, hypertension (HR, 0.87; 95% CI, 0.80-0.94) was associated with a reduced risk. According to the researchers, resting systolic blood pressure of less than 110 mm Hg is associated with poorer outcomes in IPAH, suggesting that baseline hypertension may explain the better outcomes in these patients in the current study. .

With age, the risk of survival decreases. Between ages 60 and 69, the adjusted RR for the IPAH was 1.81 (95% CI, 1.26-2.59), which jumped to 7.73 (95% CI, 5.43- 11.0) in patients aged 70 to 79 years. The researchers noted that because their data largely included pediatric and older patients, both groups can easily be misdiagnosed when echocardiography alone is used. Men also had an increased risk, with an adjusted RR of 1.15 (95% CI, 1.06-1.24).

“Male sex is a well-known risk compared to women with cardiovascular disease and lower life expectancy than women with other diseases,” the researchers explained. “In the REVEAL 2.0 calculator, male age >60 is also a risk factor for PAH prognosis, which is a similar finding to our study.”

In men, the 1, 3, 5, and 10-year survival rates were 88.4%, 78.9%, 71.3%, and 55.9%, respectively, compared to 89.4%, 80.4 %, 72.9% and 57.8% for men. females.


Jang S, Kim E, Huh J, et al. A retrospective population-based survival study of idiopathic pulmonary hypertension in Korea. J Korean Med Sci. 2022;37(10):e80

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