Clinical Characteristics and Survival of Korean Idiopathic Pulmonary Arterial Hypertension Patients Based on Vasoreactivity

다국어 초록 (Multilingual Abstract)

We aimed to identify a vasoreactive subset of patients with idiopathic pulmonary arterialhypertension (IPAH) in Korea and to show their clinical characteristics and prognosis. Dataon patients who were diagnosed with IPAH at Asan Medical Center between January 1994and March 2013 were retrospectively collected. Acute vasodilator testing was performedwith inhaled nitric oxide during diagnostic right heart catheterization. A positive acuteresponse was defined as a reduction in mean pulmonary arterial pressure (PAP) ≥ 10 mmHgto an absolute level of mean PAP < 40 mmHg without a decrease in cardiac output.

Among a total of 60 IPAH patients included for analysis, 9 (15%) showed a positive acuteresponse to acute vasodilator testing. Acute responders showed significantly lower peakvelocity of a tricuspid regurgitation jet on echocardiography (4.1 ± 0.3 m/s vs. 4.6 ± 0.6m/s; P = 0.01) and significantly lower mean PAP hemodynamically (47 ± 10 mmHg vs.

63 ± 17 mmHg; P = 0.003) than non-responders at baseline. The survival rate of acuteresponders was 88% at 1, 3, 5, and 10 yr, respectively, which was significantly higher thanthat of non-responders (85%, 71%, 55%, and 40%, respectively; P = 0.029). In conclusion,Korean IPAH patients with vasoreactivity showed better baseline hemodynamic featuresand survival than those without vasoreactivity.

참고문헌 (Reference)

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