LETTERS TO THE EDITOR Prostacyclin-analog therapy in sickle cell pulmonary hypertension Sickle cell disease (SCD) is associated with pulmonary hypertension (PH) which results in high morbidity and mortality.1 There are well-established therapies for pulmonary arterial hypertension (PAH), but few reports about their use in SCDPH.2 We report the clinical course of 11 SCDPH patients on maximal supportive therapy including

other pulmonary vasodilators, who received compassionate therapy with prostacyclin-analogs at four PH treatment centers. Inclusion criteria for this retrospective study included PH diagnosis by standard right heart catheterization (RHC), and prostacyclin-analog therapy via any route of delivery for at least four weeks. Data were collected subject to availability in the medical record, including type of prostacyclin-analog therapy, maximal dose and duration, and follow-up data at least 100 days after initiating therapy. Statistical analyses were performed using GraphPad Prism version 5.0 (La Jolla, CA, USA), using paired t-test and

Table 1A. Clinical features prior to initiation of prostacyclin therapy and on therapy.

Sex

1 2a 2b 3a 3b 4 5 6 7 8 9 10 11

m f f f f m f f m m f f f mean SEM

Age (year)

CTEPH

35 27 29 51 54 52 48 59 18 42 32 32 52 41 4

N Y Y Y Y Y N Y Y N N Y N

Other PH Meds

Hgb (g/L)

HS HB BT HB HB HA HBS HBS BS HB HBS H HB

104 80 88 70 89 110 100 88 85 64 59 96 86 86 4

6MWD (m)

RVSP (mmHg)

TAPSE

NT-proBNP (pg/mL)

Pre

On

Pre

On

Pre

On

Pre

On

502 318 327 398 324 318 369 375 194 218 280 329 26

436 367 388 394 360 309 366 342 360 369 12

95 53 60 45 39 57 82 54 55 109 92 32 64 7

43 37 43 28 38 59 64 33 40 69 45 4

1.9 2.5 1.9 2 2.1 0.1

1.8 2 2.5 1.9 2.2 2.3 2.1 0.1

9099 239 239 209 205 1554 153 210 673 10 1259 882

151 270 186 154 311 382 226 188 258 651 87 260 46

Table 1B. Cohort hemodynamics prior to initiation of prostacyclin therapy.

1 2a 2b 3a 3b 4 5 6 7 8 9 10 11

Phenotype

Prostanoid

Route

RAP (mmHg)

mPAP (mmHg)

PCWP (mmHg)

TPG (mmHg)

PVR (dyn·s·cm-5)

CO (L/min)

CI (L/min/m2)

SS S-bothal S-bothal SS SS SC SS SS SS SS SS SS SS

Treprostinil Treprostinil Treprostinil Treprostinil Treprostinil Treprostinil Treprostinil Treprostinil Treprostinil Treprostinil Treprostinil Epoprostenol Treprostinil

SQ INH SQ INH SQ SQ IV INH SQ SQ SQ IV INH mean SEM

16 14 12 9 9 13 3 11 15 8 7 7 7 10 1

45 43 45 29 28 44 35 42 59 32 28 43 35 39 3

11 15 12 13 15 15 11 15 11 11 12 7 11 12 1

34 28 33 16 13 29 24 27 48 21 16 36 24 27 3

289 304 160 344 315 342 200 582 214 185 457 274 306 35

9.4 7.4 8.0 3.0 7.4 5.6 10.8 6.6 7.8 6.9 6.3 7.0 7.2 0.6

5.0 4.5 4.5 3.3 3.5 5.65 3.7 2.7 3.4 4.3 4.6 4.1 0.3

Hgb: hemoglobin; SS: homozygous sickle cell; S-b0thal: sickle beta-0-thalassemia; SC: double heterozygous hemoglobin S and C; RAP: right atrium pressure; mPAP: mean pulmonary artery mean pressure; PCWP: pulmonary capillary wedge pressure; TPG: transpulmonary pressure gradient; PVR: pulmonary vascular resistance; CO: cardiac output; CI: cardiac index; TRV, tricuspid regurgitant velocity; RVSP: right ventricular systolic pressure; CTEPH: chronic thromboembolic pulmonary hypertension; PH: pulmonary hypertension; SQ: subcutaneous; INH: inhaled; IV: intravenous; H: hydroxyurea; A: ambrisentan; B: bosentan; S: sildenafil; T: tadalafil; NT-proBNP: amino-terminal pro-brain natriuretic peptide. On-therapy measures include first recorded measurement, where applicable, at least 12 weeks after initiation of therapy. Dashes indicate missing data.

haematologica 2017; 102:e163

LETTERS TO THE EDITOR Pearson product-moment correlation. P