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Publications

Journal Article Ahead of print

PubMed: 42373376 DOI: 10.1053/j.seminhematol.2026.05.007

The many facets of cardiopulmonary complications in sickle cell disease.

Semin Hematol

Authors: Thomas d'Humières, Sylvain Le Jeune, Gonzalo de Luna, Thibaut Moulin, Laurent Boyer, Haytham Derbel, Sihem Iles, Nicolas Lellouche, Antonio Fiore, Geneviève Derumeaux, Pablo Bartolucci, Armand Mekontso Dessap, Laurent Savale

Affiliations

  • Physiology Department, FHU SENCODE and REDGENE, Henri-Mondor Hospital, Assistance Publique-Hôpitaux de Paris, INSERM IMRB U955, Team 8, Université Paris Est (UPEC), Créteil, France; Member of the Coordinating Referral Center for Sickle Cell Disease and Red Blood Cell Disorders - UMGGR - Assistance Publique-Hôpitaux de Paris, Créteil, France; Réseau Erythrocyte Drépanocytose RED, Créteil, France.
  • Réseau Erythrocyte Drépanocytose RED, Créteil, France; Department of Internal Medicine, Avicenne Hospital, Assistance Publique-Hôpitaux de Paris, Université Sorbonne Paris Nord, INSERM U955, Institut Mondor de Recherche Biomédicale, Créteil, France.
  • Member of the Coordinating Referral Center for Sickle Cell Disease and Red Blood Cell Disorders - UMGGR - Assistance Publique-Hôpitaux de Paris, Créteil, France; Réseau Erythrocyte Drépanocytose RED, Créteil, France; Department of Internal Medicine, Henri-Mondor University Hospital-UPEC/Assistance Publique-Hôpitaux de Paris, INSERM U955, Université Paris Est Créteil, Créteil, France - Member of the ERN Eurobloodnet, Créteil, France.
  • Cardiology Department, Rhythmology unit, Henri-Mondor Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Est (UPEC), Créteil, France.
  • Physiology Department, FHU SENCODE and REDGENE, Henri-Mondor Hospital, Assistance Publique-Hôpitaux de Paris, INSERM IMRB U955, Team 8, Université Paris Est (UPEC), Créteil, France.
  • Radiology Department, Henri-Mondor Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Est (UPEC), Créteil, France.
  • Cardiac Surgery Department, Henri-Mondor University Hospital-UPEC/Assistance Publique-Hôpitaux de Paris, INSERM U955, Université Paris Est Créteil, Créteil, France.
  • Intensive Care Unit, Henri-Mondor Hospital, Assistance Publique-Hôpitaux de Paris, INSERM IMRB U955, RHU CARMAS, Université Paris Est (UPEC), Créteil, France.
  • INSERM UMR_S 999 « Pulmonary Hypertension: Pathophysiology and Novel Therapies », Hôpital Marie Lannelongue, Le Plessis-Robinson, France; Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, School of Medicine; Le Kremlin-Bicêtre, France-ERN LUNG, FHU André Cournand, Le Kremlin-Bicêtre, France.

Abstract

Sickle cell disease (SCD) is a systemic vasculopathy, which progressively remodels the heart and pulmonary circulation through chronic anemia, hemolysis, and endothelial dysfunction. This review integrates current evidence on the mechanisms, clinical spectrum, and management of SCD-related cardiopulmonary complications. Chronic anemia drives a high-output state characterized by cardiac chamber dilation leading to eccentric remodeling that, over time, may evolve toward diastolic dysfunction, heart failure with preserved ejection fraction (HFpEF), and postcapillary pulmonary hypertension (PH). Concomitant hemolysis, oxidative stress, and microvascular injury promote diffuse myocardial fibrosis that acts as an arrhythmogenic substrate underlying both atrial and ventricular arrhythmias (VAs). Beyond left-sided cardiac disease, SCD also contributes to pulmonary vascular injury and "relative systemic hypertension", both reflecting a vasculopathy process that amplifies target organs damage. Transthoracic echocardiography (TTE) remains the cornerstone of diagnosis and prognosis of SCD-related cardiomyopathy, enabling longitudinal assessment of ventricular performance, chamber remodeling and hemodynamics including pulmonary pressures, while cardiac magnetic resonance (CMR) adds information on tissue characterization and quantification of diffuse fibrosis. Emerging data suggests that curative therapies. Such as hematopoietic stem cell transplantation and gene therapy can reverse cardiac remodeling and fibrosis yet necessitate structured long-term follow-up. Recognizing SCD-related cardiomyopathy as a dynamic and potentially reversible cardiopulmonary continuum is essential for establishing disease-specific diagnostic criteria and guiding physiological grounded therapeutic strategies.

MeSH

Cells, Hypertension, Vascular System Injuries, Hematology, Microscopy, Electron, Scanning Transmission, Anemia, Sickle Cell, Anemia, Fibrosis, Heart, Heart Failure, Diastolic, Echocardiography, Hemolysis, Therapeutics, Cardiomyopathies