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Abstract
Pulmonary arterial hypertension (PAH) is often difficult to diagnose and challenging to treat. Untreated, it is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular failure and death. The past decade has seen remarkable improvements in therapy, driven largely by the conduct of randomized controlled trials. Still, the selection of most appropriate therapy is complex, and requires familiarity with the disease process, evidence from treatment trials, complicated drug delivery systems, dosing regimens, side effects, and complications. We tried to provide evidence‐based treatment recommendations for physicians involved in the care of these complex patients. Due to the complexity of the diagnostic evaluation required, and the treatment options available, it is strongly recommended that consideration be given to referral of patients with PAH to a specialized center.
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Figure 1.
특발성 폐동맥고혈압 환자의 치료:“제3회 폐동맥고혈압 세계심포지엄 2003-07-23, 베니스”(가) 치료 알고리듬은 뉴욕심장협회 기능적분류 III, Ⅳ기 환자에 중점을 두고 있다. II기 환자는 칼슘통로 차단제 치료에 적합하지 않거나 실패한자로 치료에 효과가 있을 것으로 보고 있다. (나) 단기작용 혈관확장제 검사에서 유의한 반응은 산화질소 흡입또는, epoprostenol 또는 Adenosine 정맥주사시 평균 폐동맥압이 10mmHg 이상 감소하여 40mmHg 이하를 유지하는것으로 정의한다. 이때 심박출량은 변화하지 않거나 증가하여야 한다. (다) 경구칼슘통로차단제에 대한 지속적인 반응은 치료수개월 후 뉴욕심장협회 기능적 분류가 I 또는 Ⅱ 로 향상되며 혈역학적으로 거의 정상화 되는 것으로 정의한다. (라) 뉴욕심장협회 기능적 분류 Ⅳ의 불안정한 환자는 Epoprostenol정주를 최우선의 치료로 권장한다. (마) 뉴욕심장협회 기능적 분류 III 환자의 첫번째 치료에는 엔도텔린수용체 길항제 (Bosentan) 또는 Epoprostenol 지속정주 또는 프로스타노이드 유사체 (Treprostinil 피하주사, Iloprost 흡입)를 포함시킨다. (바) 모든 치료약제에 실패하거나, 적합하지 않은 폐동맥 고혈압환자 는 sildenafil 을 고려한다. Grade of Recommendation :Noted in []: A, strong; B, moderate; C, weak; D,negative; I, inconclusive (no recommendation possible); E/A, strong recommendation based on expert opinion only; E/B, moderate recommendation based on expert opinion only; E/C, weak recommendation based on expert opinion only; E/D, negative recommendation based on expert opinion only. IPAH= Idiopathic pulmonary arterial hypertension; PAH= Pulmonar arterial hypertension; PDE= Phosphodiesterase.
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