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Table 2. Individual data of the 4 patients who did not undergo pulmonary endarterectomy.

Variables Case 1 Case 2 Case 3 Case 4*
Sex Female Female Female Female
Age, years 66 71 40 36
Follow-up period, months 69 64 119 44
Functional class
 At diagnosis III III III IV
 Last evaluation IV II II III
6MWD, m
 At diagnosis 338 416 387 221
 Last evaluation 286 390 429 390
Level of pulmonary artery stenosis by selective angiography# Distal Distal Distal Proximal (by CTA)
Cardiac catheterization
 RAP, mmHg 15 8 10 8
 SPAP, mmHg 123 68 65 96
 MPAP, mmHg 74 43 46 61
 PAWP, mmHg 12 14
 CO, L/min 2.5 3.8 4
 CI, L/min·m2 1.9 2.7 2.7
 PVR, WU 29.2 5.3 8.2 8.0
 MvO2, % 51 72 73
 SaO2, % 88 92 95 77 (SpO2)
Reasons of no PEA Patient’s refusal Surgical inaccessible Surgical inaccessible Co-morbidities of SLE and APS
PAH-specific drugs Sildenafil Riociguat Riociguat Bosentan + sildenafil
Survival outcome Expired Alive Alive Expired

* Hemodynamic data by echocardiography; # Proximal lesions indicate main or lobal pulmonary artery stenosis, whilst distal lesions indicate segmental or subsegmental stenosis.

APS, antiphospholipid syndrome; CI, cardiac index; CO, cardiac output; CTA, CT angiography of chest; MPAP, mean pulmonary artery pressure; MvO2, mixed venous oxygen saturation; PAH, pulmonary arterial hypertension; PAWP, pulmonary artery wedge pressure; PEA, pulmonary endarterectomy; PVR, pulmonary vascular resistance; RAP, right atrial pressure; SaO2, arterial oxygen saturation; SLE, systemic lupus erythematosus; SPAP, systolic pulmonary artery pressure; SpO2, oxyhemoglobin saturation by pulse oximetry; 6MWD, 6-minute walk distance.