Current status of pulmonary arterial hypertension in Korea

Abstract

Pulmonary arterial hypertension (PAH) is characterized by remodeling of the distal pulmonary arteries resulting in high pulmonary vascular resistance and, eventually, right ventricular heart failure. Although current advances in PAH therapy have improved outcomes, poor survival remains a reality worldwide, including Korea. One of the most important issues in PAH is the late diagnosis, since screening or diagnostic efforts are often overlooked due to the rarity of disease. Data from Korean registries and observational cohorts show that delayed detection leads to increased morbidity. Additionally, low percentages of Korean patients are committed to intensive PAH-targeted therapy. Current Korean health insurance policies’ lack of coverage for new PAH-targeted drugs and upfront combination therapy may also hamper the improvement of treatment outcomes. Understanding individual variability in response to therapeutics through deep phenotyping is a novel strategy that should be considered when treating PAH. Overall, early detection of PAH by proactive screening together with early, intensive, individualized PAH therapy using deep phenotyping is crucial for improving prognoses for PAH patients in Korea.

Prevalence and clinical features of bone morphogenetic protein receptor type 2 mutation in Korean idiopathic pulmonary arterial hypertension patients: The PILGRIM explorative cohort

“…It is most likely that this is by chance considering the small sample size and short follow-up. However, the trend is consistent with Japanese studies [10] that share a common east Asian heritage [34]. Also, the BMPR2 mutation carriers in the current study were approximately 8 years younger than those in the meta-analysis [7].…”

“…Although the present study showed similar trends of higher mPAP at diagnosis in BMPR2 mutation carriers compared to non-carriers, the uniquely high mPAP warrants further investigation. These results also indicate the importance of early screening for BMPR2 for the initiation of aggressive treatment in patients to ensure better outcomes [10]. This has been previously suggested for the Japanese population which possesses similar genetic background [30].…”

“…Targeted therapy with the use of endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostanoids have markedly improved the likelihood of survival [10]. Despite such advances, poor prognosis is a reality for most individuals, in part due to the lack of understandings regarding individual phenotypic variability [11,12].…”

Echocardiographic Screening Methods for Pulmonary Hypertension: A Practical Review

“…3)4) In PAH, the impacts of early screening and upfront combination therapy on long-term survival have recently been reported. 5) Initial combination target therapy in specific PAH populations has shown prolonged survival and delay of disease progression. The prognostic implication of reduction of mPAP on long-term survival in Groups 2, 3, and 4 PH has also been emphasized.…”

“…Accordingly, the role of echocardiography in screening PH patients is emerging. 5) Therefore, it is of great importance for physicians to understand echocardiography in screening and evaluating PH patients. In this article, we review echocardiographic measurements that are used in PH patient evaluation.…”