Abstract
The long-term effects of chronic blood exchange transfusions (BETs) on pre-capillary pulmonary hypertension complicating sickle cell disease (SCD) are unknown.
13 homozygous SS SCD patients suffering from pre-capillary pulmonary hypertension and treated by chronic BETs were evaluated retrospectively. Assessments included haemodynamics, New York Heart Association Functional Class (NYHA FC), 6-min walk distance (6MWD) and blood tests.
Before initiating BETs, all patients were NYHA FC III or IV, median (range) 6MWD was 223 (0–501) m and median (range) pulmonary vascular resistance (PVR) was 3.7 (2–12.5) Wood Units. After a median number of 4 BET sessions, all patients had improved to NYHA FC II or III. Significant improvements in haemodynamics were observed, including a decrease in PVR (p=0.01). There was a trend to higher 6MWD (p=0.09). Median (range) follow-up time after initiation of BETs was 25 (6–53) months. During this period, two patients decided to stop BETs. One of them died from acute right heart failure and the other experienced worsening pulmonary hypertension. Two other patients died during follow-up at 25 and 54 months after BET initiation.
Chronic BETs may be a potential therapeutic option in pre-capillary pulmonary hypertension complicating SCD, leading to significant clinical and haemodynamic improvements. These data must be confirmed in a prospective study.
Abstract
Chronic exchange transfusions may be a potential therapeutic option in pre-capillary pulmonary hypertension complicating sickle cell disease http://ow.ly/BrMj30loRBY
Footnotes
Conflict of interest: M. Turpin has nothing to disclose.
Conflict of interest: C. Chantalat-Auger has nothing to disclose.
Conflict of interest: F. Parent has nothing to disclose.
Conflict of interest: F. Driss has nothing to disclose.
Conflict of interest: F. Lionnet has nothing to disclose.
Conflict of interest: A. Habibi has nothing to disclose.
Conflict of interest: B. Maître has nothing to disclose.
Conflict of interest: A. Huertas has nothing to disclose.
Conflict of interest: X. Jaïs reports grants and personal fees from Actelion and GSK, personal fees from MSD, grants from Bayer, outside the submitted work.
Conflict of interest: J. Weatherald reports personal fees and nonfinancial support from Actelion and Bayer, personal fees from Novartis, and grants from the Canadian Vascular Network, outside the submitted work.
Conflict of interest: D. Montani reports grants and personal fees from Actelion and Bayer, personal fees from BMS, GSK, MSD and Pfizer, outside the submitted work.
Conflict of interest: O. Sitbon reports grants, personal fees and nonfinancial support from Actelion, Bayer, Merck and GSK, personal fees from Acceleron and Arena, outside the submitted work.
Conflict of interest: G. Simonneau reports grants, personal fees and nonfinancial support from Actelion, Bayer, Merck and GlaxoSmithKline, personal fees from Acceleron and Arena, outside the submitted work.
Conflict of interest: F. Galactéros has nothing to disclose.
Conflict of interest: M. Humbert has relationships with drug companies including Actelion, Bayer, GSK, Novartis, Pfizer and United Therapeutics. In addition to being investigator in trials involving these companies, relationships include consultancy service and membership of scientific advisory boards.
Conflict of interest: P. Bartolucci has nothing to disclose.
Conflict of interest: L. Savale reports grants and personal fees from Actelion and Bayer, personal fees from MSD and GSK, during the conduct of the study.
- Received February 6, 2018.
- Accepted August 10, 2018.
- Copyright ©ERS 2018