Pathophysiology of cardiovascular disease in haemoglobinopathies and rare anaemias 3rd Pan European Conference on haemoglobinopathies and rare anaemias 24-26 October 1912 Limassos Cyprus A. Aessopos, M.D. A. Aessopos, M.D. First Dept. of Internal Medicine, First Dept. of Internal Medicine, University of Athens, Medical School University of Athens, Medical School
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Pathophysiology of cardiovascular disease in haemoglobinopathies and rare anaemias
Pathophysiology of cardiovascular disease in haemoglobinopathies and rare anaemias 3rd Pan European Conference on haemoglobinopathies and rare anaemias 24-26 October 1912 Limassos Cyprus . A. Aessopos , M.D. First Dept. of Internal Medicine, University of Athens, Medical School. - PowerPoint PPT Presentation
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Pathophysiology of cardiovascular disease in
haemoglobinopathies and rare anaemias
3rd Pan European Conference on haemoglobinopathies and rare
anaemias 24-26 October 1912 Limassos Cyprus
A. Aessopos, M.D.A. Aessopos, M.D.First Dept. of Internal Medicine, University of First Dept. of Internal Medicine, University of
Athens, Medical SchoolAthens, Medical School
Pathophysiology of cardiovascular disease in rare anaemias
Rare anemias:
They have a global prevalence of less than 5 per 10,000 individuals and encompass almost 90 different conditions
Pathophysiology of cardiovascular disease in rare anaemias
Rare anemias:
•Due to their extremely low prevalence, their complications are not extensively explored.
•In some of those anemias such as hemoglobinopathies, their local prevalence is significantly high and those diseases are exposed to a better observation.
• Regarding the cardiovascular system, most of the rare anemias share some basic features that can affect the system. Thus, the accumulated knowledge from hemoglobinopathies can be useful for the rest of the rare anemias.
Pathophysiology of cardiovascular disease in rare anaemias
•Common features exist in most of the rare anemias
•Different degree of severity of pathogenetic mechanisms for cardiovascular injury
•Cardiovascular CLINICAL CONSEQUENCES
Pathophysiology of cardiovascular disease in rare anaemias
Common features exist in most of the rare anemias
•Anemia•Bone marrow expansion, extra-medullar hematopoiesis, hepato-splenomegaly•Transfusion needs• Increased intestinal iron absorption• Red blood cell defects –hemolysis• Impaired immune competence
Pathophysiology of cardiovascular disease in rare anaemias
Pathophysiology of cardiovascular disease in rare anaemias
6.Susceptibility to infections•Anemia
•Iron load
•Chelation therapy
Immune competence is impaired. Infections: Viral(pericarditis and myocarditis) – Bacterial infections (siderophore bacteria, such as yersinia and klebsiella )
Mechanisms of cardiovascular injury in RA
Farmakis D. et al. Med Sci Monit 2003
Pathophysiology of cardiovascular disease in rare anaemias
Manfre L. AJR. 1999 Karimi M…Rachmilewitz EA., AJ H. 2008
• Leg ulcers, gastrointestinal hemorrhage Anemia- Elastic tissue damage Aessopos et al. Haematologica 2007
65 year-old patient with SSD PXE-like syndrome and GI angiodysplasias (pill camera)
Cardiovascular CLINICAL CONSEQUENCES
1.Vascular complications - Veins1.Vascular complications - Veins Thrombosis and Thromboembolic complications:Thrombosis and Thromboembolic complications:
In In 4.3% and 5.2%4.3% and 5.2% of TM and TI respectivelyTM and TI respectivelyandand in in 3030% % of TI pts with splenectomy of TI pts with splenectomy
• 319 patients iron loaded patients (TM)• T2* >20 ms, normal RVEF in 98% of cases• T2*<20 ms, progressive decline of RVEF, as with LVEF
Alpendurada et al, Eur Heart J 2010
Cardiovascular CLINICAL CONSEQUENCES
3.Left-sided heart dysfunction
• High output state• Increased arterial stiffness • Iron deposition(systolic and diastolic dysfunction) • Endocrine abnormalities • Arrhythmias - Atrio-ventricular conduction abnormalities• Valvular disorders (regurgitation - stenosis) • Infections (Myocarditis)
Cardiac valvular calcifications: a 50-year old thalassemia intermedia patient
Valvular injuryValvular injury
Aessopos et al. Blood 2001
Kremastinos et al, Circulation 1995;91:66-71
Myocarditis in b-thalassaemia major A cause of heart failure
Cardiovascular CLINICAL CONSEQUENCES
•Viral infections•Iron load
4.Pericardial involvement
Pericarditis in TM 50% Engle a tall : Circulation 1964 in well treated TM 5% Aessopos a tall: Eur.J. Haematol 2004
Figure 1a.Operative field in a 27 year old male TM patient with a history of recurrent pericarditis and effusive constrictive pericarditis. Figure 1b biopsy from the same patient demonstrating significant pericardial thickening with severe iron deposition and a small amount of muscle in the left hand corner which contains iron (Prussian Blue Stain).
1a. 1b.
Mechanisms of heart injury in thalassemia majorMechanisms of heart injury in thalassemia major
Pathophysiology of cardiovascular disease in rare anaemias
Conclusions•Common features exist in most of the rare anemias •Their degree of the severity may affect in different way the cardiovascular system and one or the other may dominate the clinical picture
•Any cardiovascular finding has to be considered related to the main disease before is thought to be as a coincidence
•Particular features of each disease may represent additional mechanisms of cardiovascular injury
Thank you!
Pathophysiology of cardiovascular disease in rare anaemias
Rare anemias:
• According to the definition of the European Commission(EC), Rare anemias have a global prevalence of less than 5 per 10,000 individuals.• Encompass a large and markedly heterogeneous group of nearly 90 different conditions, mostly congenital or genetically determined