Diagnostic-studies-workup-pulmonary hypertension

• High-res chest CT: dilat. & pruning of pulm arteries, ↑ RA & RV; r/o parenchymal lung dis.
• ECG: RAD, RBBB, RAE (“P pulmonale”), RVH (Se 55%, Sp 70%)
• PFTs: disproportionate ↓ DLCO, mild restrictive pattern; r/o obstructive & restrictive lung dis.
• ABG & polysomnography: ↓ PaO2 and SaO2 (espec w/ exertion), ↓ PaCO2, ↑ A-a gradient; r/o hypoventilation and OSA
• TTE: ↑ RVSP (but estimate over/under by ≥10 mmHg in ½ of PHT Pts; Chest 2011;139:988) ↑ RA, RV, & PA; ↑ pressure → interventricular septum systolic flattening (“D” shape) ↓ RV systolic fxn (TAPSE <1.6 cm); TR, PR; r/o LV dysfxn, MV, AoV, congenital disease
• RHC: ↑ RA, RV, & PA pressures; ✓ L-sided pressures and for shunt if PAH: nl PCWP, ↑ transpulmonary gradient (mean PAP-PCWP >12–15), ↑ diastolic pulmonary gradient (PA diastolic – PCWP >7), ↑ PVR, ± ↓ CO if 2° to L-heart disease: PCWP (or LVEDP) >15; if PVR nl → “passive PHT”; PVR >240 suggests mixed picture: if ↓ PCWP → ↓ PVR, then “reactive” PHT; if no Δ, then “fixed”
• CTA (large/med vessel), V/Q scan (small vessel to r/o CTEPH), ± pulm angio if ↑ concern
• Labs: ANA (~40% ⊕ in PAH), anti-Scl-70, anti-RNP; LFTs; HIV
• 6-min walk test (6MWT) or cardiopulmonary exercise testing to establish fxnl capacity