教育演講4:肺動脈高血壓分類及治療的新進展
Recent advance in the classification and management of pulmonary arterial hypertension 

程 序 表

E4-4
肺動脈高壓治療策略及療效評估之新進展
吳懿哲
馬偕醫學院醫學系生物醫學所
馬偕紀念醫院醫學教育部內科部心臟內

  The treatment strategy for PAH patients cannot be considered as a mere prescription of drugs, but is characterized by a complex strategy that includes the initial evaluation of severity and the subsequent response to treatment.
  The initial approach includes general measures (physical activity and supervised rehabilitation, pregnancy, birth control and post-menopausal hormonal therapy, elective surgery, infection prevention, psychosocial support, adherence to treatments, genetic counseling and travel), supportive therapy (oral anticoagulants, diuretics, O2, digoxin), referral to expert centers and acute vasoreactivity testing for the indication of chronic CCB therapy.
  The second step includes initial therapy with high-dose CCB in vasoreactive patients or drugs approved for PAH in non-vasoreactive patients according to the prognostic risk of the patient.
  The third part is related to the response to the initial treatment strategy; in the case of an inadequate response, the role of combinations of approved drugs and lung transplantation are proposed.
  Different from previous guidelines, 2015 ESC/ERS guidelines for PH suggests through comprehensive assessment, the patient can be classified as low, intermediate or high risk for clinical worsening or death. Although reliable individual predictions are always difficult, patients categorized as low risk have an estimated 1-year mortality of 5%. Basically these patients present with non-progressive disease in WHO-FC I or II with a 6MWD of 440m and no signs of clinically relevant RV dysfunction. The estimated 1-year mortality in the intermediate-risk group is 5–10%. These patients typically present in WHO-FC III, with moderately impaired exercise capacity and signs of RV dysfunction, but not with RV failure. Patients in the high-risk group have an estimated 1-year mortality of 10%. These patients present in WHO-FC III or IV with progressive disease and signs of severe RV dysfunction, or with RV failure and secondary organ dysfunction.
  Initial monotherapy or initial oral drugs combination therapy is recommended in treatment naive, low or intermediate risk patients with PAH.
  Sequential drugs combination therapy is recommended in PAH patients with inadequate treatment response to initial monotherapy or to initial oral drugs combination therapy. Initial combination therapy including an intravenous prostacyclin analogue is recommended in high risk PAH patients.
  The overall treatment goal in patients with PAH is achieving a low risk status which is usually associated with good exercise capacity, good quality of life, good RV function and a low mortality risk. Specifically, this means bringing and/or keeping the patient in WHO-FC II whenever possible. In most patients, this will be accompanied by a near-normal or normal 6MWD. The present guidelines adopt a threshold of >440 m as suggested during the 5th World Symposium on Pulmonary Hypertension, because this number was derived from the largest cohort investigated so far. Nevertheless, individual factors must be considered and lower values may be acceptable in elderly patients or patients with co-morbidities, whereas even values >440 m may not be sufficient in younger.
  Patients with PAH require sensible advice about general activities of daily living and need to adapt to the uncertainty associated with a serious chronic life-threatening disease. The diagnosis usually confers a degree of social isolation. Encouraging patients and their family members to join patient support groups can have positive effects on coping, confidence and outlook.? Immunization of PAH patients against influenza and pneumococcal infection, avoid pregnancy and psychosocial support are highly recommended.