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• SupportiveOxygen: maintain S aO 2 90–92% (reduces vasoconstriction)Diuretics: T RV wall stress and relieve RHF sx; gentle b/c RV is preload dependentDigoxin: control AF, ? counteract neg. inotropic effects CCBDobutamine and inhaled NO for decompensated PHTAnticoagulation: TVTE risk of RHF; ? prevention of in situ microthrombi; ? mort. benefit(Circ 1984;70:580; Chest 2006;130:545)• Vasodilatorsacute vasoreactivity test: use inhaled NO, adenosine, or prostacyclin to identify Pts likelyto have a long-term response to oral CCB ( vasoreactive response defined as T PAP10 mm Hg to a level 40 mm Hg with c or stable CO); 10% Pts are acuteresponders; no response S still candidates for other vasodilators (NEJM 2004;351:1425)Vasodilators CommentsOral CCBIf acute vasoreactive response; 1 ⁄2 will be long-term responderNifedipine, diltiazem (NYHA I/II & near-nl hemodynamics) & have T mortality. Side effects:HoTN, lower limb edema. (NEJM 1992;327:76, Circ 2005;111:3105)IV Prostacyclin Vasodilation, T plt agg, T smooth muscle proliferation; benefits cEpoprostenol; Flolan w/ time (? vascular remodeling). c 6MWT, c QoL, T mortality. Sideeffects: HA, flushing, jaw/leg pain, abd cramps, nausea, diarrhea,catheter infxn. (NEJM 1996;334:296 & 1998:338:273; Annals 2000;132:425)Prostacyclin Same mechanism as prostacyclin IV, but easier to take, T side effects,analoguesand w/o risk of catheter infxn. T sx, c 6MWT; trend to T clinical eventsIloprost (inhaled) w/ iloprost but not treprostinil. Beraprost w/o sustained outcomeTreprostinil (IV or SC) improvement (n/a in U.S.). (NEJM 2002;347:322; AJRCCM 2002;165:800)Beraprost (PO)Endothelin receptor T Smooth muscle remodeling, c vasodilation, T fibrosis. T sx, c 6MWT, Tantagonists (ERAs) clinical events. Side effects: c LFTs, headache, anemia, edema,Bosentan, ambrisentan teratogen. (NEJM 2002;346:896; JACC 2005;46:529; Circ 2008;117:3010)PDE-5 InhibitorSildenafil, tadalafilc cGMP ScNO S vasodilation, T smooth muscle proliferationT sx, c 6MWT, no clinical outcomes. Low side effect profile: HA,vision ‘s, sinus congestion. (NEJM 2009;361:1864)• Treat underlying causes of 2 PHT; can use vasodilators, although little evidence• Refractory PHTballoon atrial septostomy: RSL shunt causes c CO, T S aO 2, net c tissue O 2 deliverylung transplant (single or bilateral); heart-lung needed if Eisenmenger physiologyFigure 2-5 Treatment of PAHPHT 2-17Anticoag ± diuretics ± O2 ± dig⊕Oral CCBsustainedresponse?yesContinueCCBnoAcute vasoreactivity testing& lower risk & higher riskERA or PDE-5 inhibEpoprostenol ortreprostinil (IV)IloprostTreprostinil (SC)Investigational protocolsAtrial septostomyLung transplantationinadequateresponseEpoprostenolor treprostinil (IV)IloprostERA or PDE-5 inhibTreprostinil (SC)inadequateresponseinadequateresponseCombination RxHigher risk: presence of any of the poor prognostic risk factors listed below. Modified from Circ 2009;119:2250.Management of ICU patient• Avoid overly aggressive volume resuscitation• Caution with vasodilators if any L-sided dysfunction• May benefit from inotropes/chronotropes• Consider fibrinolysis if acute, refractory decompensationPrognosis• Median survival after dx 2.8 y; PAH (all etiologies): 2-y 66%, 5-y 48% (Chest 2004;126:78-S)• Poor prognostic factors: clinical evidence of RV failure, rapidly progressive sx,WHO(modified NYHA) class IV, 6MWT 300 m, peak VO 2 10.4 mL/kg/min, c RA or RVor RV dysfxn, RA 20 or CI 2.0, c BNP (Chest 2006;129:1313)• Lung transplant: 1-y survival 66–75%; 5-y survival 45–55% (Chest 2004;126:63-S)