• Al KahfSalmaSINSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hôpital Marie Lannelongue, 92350 Le Plessis-Robinson, France; Université Paris-Saclay, Faculté de Médecine, Pulmonary Hypertension: Pathophysiology and Novel Ther, Anne Roche, Audrey Baron, Christelle Chantalat-Auger, and Laurent Savale.
• INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hôpital Marie Lannelongue, 92350 Le Plessis-Robinson, France; Université Paris-Saclay, Faculté de Médecine, Pulmonary Hypertension: Pathophysiology and Novel Therapies 94276 Le Kremlin-Bicêtre, France; Assistance Publique - Hôpitaux de Paris (AP-HP), Service de Pneumologie et Soins Intensifs Respiratoires, Hôpital Bicêtre, 94270 Le Kremlin-Bicêtre, France.
• Presse Med. 2023 Dec 1; 52 (4): 104209104209.

AbstractSickle cell disease (SCD) is a complex genetic disorder that has long challenged both patients and healthcare professionals. One of its chronic and debilitating complications is pulmonary hypertension (PH). SCD-associated PH is often post-capillary, secondary to left heart disease. It can also sometimes be pre-capillary with multiple and often interrelated mechanisms including obstructive remodeling of the pulmonary vascular bed secondary to hemolysis, endothelial dysfunction, thrombosis, hypoxia, or associated risk factors like portal hypertension. Screening symptomatic patients with echocardiographic signs of PH is crucial to determine those who should undergo right heart catheterization, the cornerstone exam to diagnose and categorize patients with PH. The workup following the diagnosis relies on identifying the cause of PH to personalize treatment. Ongoing efforts are made to treat this complex condition, starting with treating the underlying disease with hydroxyurea or chronic blood exchange transfusion. Robust data on the efficacy of PAH-specific therapies are lacking in this specific population. Initiation of such therapies must be made by an expert center after a case-by-case assessment of the benefit-risk ratio according to the phenotype and the mechanisms involved in the development of PH. Efforts are also poured into studying the interventional and medical therapies used on chronic thromboembolic PH for patients presenting with a thrombotic form. The management of those patients requires a multidisciplinary approach, with conjoint efforts from PH and SCD specialists.Copyright © 2023. Published by Elsevier Masson SAS.

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