Table.
Review of Published Cases.
| Reference | Age | Gravity Parity | SLE activity | PAH severity | CS, Immunosapressants | Vasodilators | Delivery | Maternal outcome | Fetal outcome |
|---|---|---|---|---|---|---|---|---|---|
| (4) | 19 | NR | NR | PAP 90/30 mmHg after delivery | small dose of PSL | None | Caesarean | Death on day 6 postpartum | NR |
| (5) | 25 | G3P0 | Absence of lupus features | before delivery, RVSP 79 mmHg with moderate to severe TR, mRAP 10 mmHg, RV and RA dilation with RV hypokinesis, mild to moderate sized pericardial effusion | PSL 10 mg every two days → PSL 15 mg/day | None | Caesarean | Death on day 4 postpartum | Alive |
| (6) | 30 | G2P1 | No features of active SLE | at delivery, mPAP 70-80 mmHg | NR | during operation, iNO | Caesarean | Death less than 24 hours after delivery | Alive |
| (7) | 23 | NR | Good condition | No evidence of RHF, PAP 52/26 mmHg, PVR 143 dyn.sec.cm-5 | NR | IV Epoprostenol | Caesarean | Alive | Alive |
| (8) | 29 | NR | SLEDAI 2 → 14 at delivery | at delivery, NYHA III, sPAP 45 mmHg, mPAP 21 mmHg, PVR 250 dyn.sec.cm-5 | prednisone 25 → 50 mg/day at delivery+ HCQ 200 mg/day, AZ 100 mg/day | Sildenafil 150 mg/day, Inhaled Iloprost 50 → 100 μg/day at delivery | Caesarean | Alive | Alive |
| Present case | 31 | G1P0 | before pregnancy, SLEDAI 0; during pregnancy SLEDAI 7 → 4 → 10 | before pregnancy, WHO FC I, estimated sPAP 43 mmHg; during pregnancy, WHO FC II, PAP 45-66/16-35 mmHg; right after delivery, sPAP 75 mmHg | during pregnancy, PSL 25 → 60 mg/day; after delivery, mPSL 40 → 36 mg/day, MZR 150 mg/day | during pregnancy, Sildenafil 60 mg/day, IV Epoprostenol 2.5 ng/kg/min+ after delivery, iNO 20 ppm | Caesarean | Alive | Alive |
SLE: systemic lupus erythematosus, PAH: pulmonary arterial hypertension, CS: corticosteroids, NR: not reported, PAP: pulmonary arterial pressure, PSL: prednisolone, RVSP: right ventricular systolic pressure, TR: tricuspid regurgitation, mRAP: mean right atrial pressure, RV: right ventricular, RA: right atrial, iNO: inhaled nitric oxide, RHF: Right heart failure, PVR: pulmonary vascular resistance, IV: intravenous, SLEDAI: systemic lupus erythematosus disease activity index, NYHA: New York Heart Association, sPAP: systolic pulmonary arterial pressure, HCQ: hydroxychloroquine, AZ: azathioprine, WHO FC: World Health Organization functional class, MZR: mizoribine