Chronic blood exchange transfusions in the management of pre-capillary pulmonary hypertension complicating sickle cell disease - Archive ouverte HAL
Article Dans Une Revue European Respiratory Journal Année : 2018

Chronic blood exchange transfusions in the management of pre-capillary pulmonary hypertension complicating sickle cell disease

1 CHU Tenon [AP-HP]
2 SU FM - Sorbonne Université - Faculté de Médecine
3 service de Médecine Interne et d'Immunologie Clinique [AP-HP Hôpital Bicêtre]
4 LA3M - Laboratoire d'Archéologie Médiévale et Moderne en Méditerranée
5 Inrap - Institut national de recherches archéologiques préventives
6 HPPIT - Hypertension pulmonaire : physiopathologie et innovation thérapeutique
7 Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
8 Centre de Référence des Syndromes Drépanocytaires Majeurs
9 IMRB - Institut Mondor de Recherche Biomédicale
10 Hôpital Henri Mondor
11 Service de physiologie, explorations fonctionnelles [Mondor]
12 UP11 - Université Paris-Sud - Paris 11
13 Hôpital Bicêtre
14 Pôle des Cardiopathies Congénitales du Nouveau-Né à L'adulte - Centre Constitutif Cardiopathies Congénitales Complexes M3C, Groupe Hospitalier Paris Saint-Joseph, Hôpital Marie-Lannelongue, Inserm U999, Université Paris-Saclay
15 UP11 UFR Médecine - Université Paris-Sud - Paris 11 - Faculté de médecine
16 Faculte de Medecine
17 U955 Inserm - UPEC - IMRB - "Transfusion et Maladies du Globule Rouge" [Créteil]
18 Laboratory of Excellence GR-Ex " The red cell : from genesis to death "
19 UPEC - Hôpital Henri Mondor AP-HP - Unité des maladies génétiques du globule rouge
20 ICAN - Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases
21 Unité INSERM 955
22 Service d'Anesthésie et de Soins Intensifs [Bicêtre]
23 Université Paris-Saclay
24 Faculté de Médecine - Université Paris-Sud [Le Kremlin-Bicêtre]
25 HPPIT - Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique
Jason Weatherald

Résumé

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.

Dates et versions

hal-04526569 , version 1 (29-03-2024)

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Citer

Matthieu Turpin, Christelle Chantalat-Auger, Florence Parent, Françoise Driss, François Lionnet, et al.. Chronic blood exchange transfusions in the management of pre-capillary pulmonary hypertension complicating sickle cell disease. European Respiratory Journal, 2018, 52 (4), pp.1800272. ⟨10.1183/13993003.00272-2018⟩. ⟨hal-04526569⟩
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