Longterm Survival Among Patients with Scleroderma-associated Pulmonary Arterial Hypertension Treated with Intravenous Epoprostenol

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Figure 1.
The progress of patients through the study.
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Figure 2.
Survival probabilities of patients receiving epoprostenol. Survival rate: the percentage of subjects from the total population alive at the start of the year interval. Patients at risk: the number of subjects assessed at the start of the year interval. Deaths: the number of subjects who died during the following year interval. Censored: the number of subjects who dropped out of the study (lost to followup) during the following year interval.

Table 1.

List of criteria for patient termination from the extension study.

I. FDA approval of epoprostenol for use in patients with severe PAH/scleroderma spectrum of disease.
II. Discontinuation of patient from the study due to one of the following reasons:
1. Infusion was discontinued for more than 30 days
2. Patient received a lung or heart-lung transplant
3. Patient withdrew consent to participate in the study
4. Patient received commercially available epoprostenol
5. Patient was lost to followup
6. The patient died

Table 2.

Patient demographics and characteristics in the extension study per survival outcome.

Characteristic	53 Survivors^*	44 Deaths
Age, yrs, median (range)	56.8 (23–77)	56.8 (31–78)
Sex (%)
Female	48 (91)	39 (89)
Male	5 (9)	5 (11)
Race (n, %)
Caucasian	42 (79)	41 (93)
African American	4 (8)	2 (5)
Hispanic	1 (2)	0 (0)
Asian	6 (11)	1 (2)
Height, cm, median (range)	163.0 (145–187)	164.5 (146–182)
Weight, kg, median (range)	73.0 (42.0–126.8)	69.35 (38.5–95.9)
NYHA class (n, %)
II	5 (9)	0 (0)
III	39 (74)	38 (86)
IV	9 (17)	6 (14)
Vasodilator use (%)
No	15 (28)	15 (34)
Yes	38 (72)	29 (66)
SSD diagnosis (n, %)
Limited scleroderma	37 (70)	32 (73)
Overlap syndrome	6 (11)	4 (9)
Features of SSD	3 (6)	2 (5)
Systemic sclerosis	7 (13)	6 (14)
Scleroderma history, mo, median (range)	46 (0–504)	60 (2–360)
PAH history, mo, median (range)	9 (1–96)	7 (1–84)

↵* Includes patients alive at study conclusion and patients withdrawn. NYHA: New York Heart Association; PAH: pulmonary arterial hypertension; SSD: Scleroderma spectrum of disease.

Table 5.

Summary of adverse events with a fatal outcome.

Body System Event	Epoprostenol, N = 97
No. of subjects with ≥ 1 adverse events (%)	44 (45)
Cardiovascular system (%)
Any event	27^* (28)
Right-heart failure	21 (22)
Hypotension	3 (3)
Bradycardia	1 (1)
Heart arrest	1 (1)
Shock	1 (1)
Vasculitis	1 (1)
Respiratory system (%)
Any event	7 (7)
Lung disorder	3 (3)
Respiratory disorder	3 (3)
Dyspnea	1 (1)
Body as a whole (%)
Any event	6 (6)
Reaction unevaluable	2 (2)
Sepsis	2 (2)
Sudden death	2 (2)
Urinogenital system (%)
Any event	2 (2)
Kidney failure	2 (2)
Digestive system (%)
Any event	1 (1)
Hemorrhage gastrointestinal	1 (1)
Metabolic and nutritional disorder (%)
Any event	1 (1)
Hyperkalemia	1 (1)