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表21 小児PAH の重症度/ 予後評価に重要な指標

低リスク予後規定因子高リスクなし右心不全ありなし症状の進行ありなし失神あり正常発育発育不良IまたはII WHO機能分類IIIまたはIV軽度上昇BNP,NT-proBNP高度上昇>1200 pg/mL

軽度右室右房拡大右室収縮低下なし指標: RV/LVes ratio<1 TAPSE正常(z>-2) S/D ratio<1.0(TR jet) PAAT>100 ms(>1歳)心エコー検査高度右室右房拡大右室収縮低下あり指標: RV/LVes ratio>1.5 TAPSE低下(z<-3) S/D ratio>1.4(TR jet) PAAT<70 ms(>1歳)心嚢液貯留CI>3.0 L/min/m2 mRAP<10 mmHg mPAP/mSAP<0.5急性血管反応試験反応あり心臓カテーテル検査CI<2.5 L/min/m2

mRAP>15 mmHg mPAP/mSAP>0.75 PVRi>15 WU・m2

WHO; World Health Organization, BNP; brain natriuretic peptide, NT-proBNP; N terminal pro BNP, RV; right ventricle, LV; left ventricle, es; end-systolic, TAPSE; tricuspid annular plane systolic excursion, S/D ratio; systolic/diastolic duration ratio by Doppler echocardiography, TR; tricuspid regurgitation, PAAT; pulmonary artery acceleration time by transthoracic Doppler echocardiography, CI; cardiac index, e.s.; end-systolic, mRAP; mean right atrial pressure, mPAP; mean pulmonary artery pressure, mSAP; mean systemic artery pressure, PVRi; pulmonary vascular resistance index, WU; Wood unit (Hansmann G, et al. 2019 215)より) ©2019 Published by Elsevier Inc. on behalf of International Society for Heart and Lung Transplantation.