Treatment-pulmonary hypertension

• Principles: 1) prevent & reverse vasoactive substance imbalance and vascular remodeling 2) prevent RV failure: ↓ wall stress (↓ PVR, PAP, RV diam); ensure adeq systemic DBP
• Supportive Oxygen: maintain SaO2 >90–92% (reduces vasoconstriction) Diuretics: ↓ RV wall stress and relieve RHF sx; gentle b/c RV is preload dependent Anticoag: not routinely used; ↓ VTE risk of RHF; ? prevention of in situ microthrombi; ? mortality benefit even if in NSR, no RCTs (Chest 2006;130:545) Supervised exercise training; aggressive apnea/hypoventilatory Rx w/ CPAP/BiPAP
• Vasodilators (ideally right heart catheterization prior to initiation; NEJM 2004;351:1425) acute vasoreactivity test: use inh NO, adenosine or prostacyclin to identify Pts likely to have long-term response to CCB (⊕ response = ↓ PAP ≥10 mmHg to <40 mmHg w/ ↑ or stable CO); ~10% Pts acute responders; no response → still candidate for other vasodilators

Vasoactive Agents as Treatment-pulmonary hypertension

• Upfront combination Rx (PDE-5 inhibitor + ERA vs. monotherapy): ↓ sx, ↓ NT-proBNP, ↑ 6MWT, ↓ hospitalizations (NEJM 2015;373:834)

• Treat underlying causes of 2° PHT; can use vasodilators, although little evidence

• CTEPH: riociguat. Pulm endarterectomy potentially curative (AJRCCM 2011;183:1605) vs. balloon pulmonary angioplasty in non-operative Pts (Circ Outcomes 2017;10:e004029).

• Refractory PHT: balloon atrial septostomy: R→L shunt causes ↑ CO, ↓ SaO2, net ↑ tissue O2 delivery; lung txp (single or bilateral; heart-lung needed if Eisenmenger physiology)

• PHT risk stratification based on CHF symptoms, syncope, WHO functional class, 6MWT, CPET, NTproBNP, imaging, hemodynamics (Eur Heart J 2016;37:67)