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.