Preprint Article Version 1 Preserved in Portico This version is not peer-reviewed

Haemolytic Anaemia-Related Pulmonary Hypertension

Version 1 : Received: 1 December 2023 / Approved: 4 December 2023 / Online: 5 December 2023 (06:18:52 CET)

A peer-reviewed article of this Preprint also exists.

Karyofyllis, P.; Demerouti, E.; Tsetika, E.-G.; Apostolopoulou, S.; Tsiapras, P.; Iakovou, I.; Tsiapras, D. Haemolytic Anaemia-Related Pulmonary Hypertension. Life 2024, 14, 876, doi:10.3390/life14070876. Karyofyllis, P.; Demerouti, E.; Tsetika, E.-G.; Apostolopoulou, S.; Tsiapras, P.; Iakovou, I.; Tsiapras, D. Haemolytic Anaemia-Related Pulmonary Hypertension. Life 2024, 14, 876, doi:10.3390/life14070876.

Abstract

Haemolytic anaemia represents a risk factor for the development of pulmonary hypertension (PH), currently classified as World Health Organization group 5 PH and data regarding appropriate therapeutic strategy are limited. A total of 28 patients, 85.7% with thalassemia and 14,3% with sickle cell disease, with a diagnosis of PH confirmed by right heart catheterization, were included in the study. The patients were divided into three groups according to haemodynamics and overall diagnostic approach: 42,9% had precapillary PH (Pulmonary Arterial Hypertension -PAH group), 25% post-capillary PH and 32.1% Chronic Thromboembolic PH (CTEPH) (29% of b-thalassemia and 50% of SCD patients). The therapeutic approach in each group and its impact on the outcome and haemodynamics was recorded. PAH-specific drug therapy received 82.1% of patients and Balloon Pulmonary Angioplasty (BPA) was performed in 6 patients with CTEPH. There were statistically significant differences in baseline mPAP and PVR values between the CTEPH-haemolytic anaemia group and other groups. PAH-specific drug therapy resulted in haemodynamic improvement for PAH-group. Patients who underwent BPA improved Pulmonary Haemodynamics. Median survival time was 162 months and the survival rate was 1yr-100%, 2,3,4,5,6 years 96%, 9yrs 90% and 13yrs 78%. In patients with haemolytic anaemia, the wide spectrum of induced PH highlights the importance of a correct diagnosis. BPA in CTEPH patients and specific-PAH drug therapy for PAH patients represent potential therapeutic strategies, however the management should be offered in expert PH centers under patients’ individualized approach.

Keywords

Pulmonary Hypertension; Haemolytic anemia; Haematological Disorders; Chronic Thromboembolic Pulmonary Hypertension; Pulmonary Arterial Hypertension; Balloon Pulmonary Angioplasty

Subject

Medicine and Pharmacology, Pulmonary and Respiratory Medicine

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