Stroke and presence of patent foramen ovale in sickle cell disease

Journal of Thrombosis and Thrombolysis - Tập 52 - Trang 889-897 - 2021
Constantina Aggeli1, Kali Polytarchou1,2, Yannis Dimitroglou1, Dimitrios Patsourakos1, Sophia Delicou3, Sophia Vassilopoulou4, Eleftherios Tsiamis1, Kostas Tsioufis1
1First Department of Cardiology, National and Kapodistrian University of Athens, Medical School, Athens, Greece
2First Department of Cardiology, Henry Dunant Hospital Center, Athens, Greece
3Thalassemia Unit, Hippokrateio Hospital, Athens, Greece
4Department of Neurology, National and Kapodistrian University of Athens, Medical School, Athens, Greece

Tóm tắt

Sickle cell disease (SCD) is an inherited monogenic hemoglobinopathy characterized by formation of sickle erythrocytes under conditions of deoxygenation. Sickle erythrocytes can lead to thrombus formation and vaso-occlusive episodes that may result in hemolytic anemia, pain crisis and multiple organ damage. Moreover, SCD is characterized by endothelial damage, increased inflammatory response, platelet activation and aggravation, and activation of both the intrinsic and the extrinsic coagulation pathways. Cerebrovascular events constitute an important clinical complication of SCD. Children with SCD have a 300-fold higher risk of acute stroke and by the age of 45 about 25% of patients have suffered an overt stoke. Management and prevention of stroke in patients with SCD is not well defined. Moreover, the presence of patent foramen ovale (PFO) increases the risk of the occurrence of an embolic cerebrovascular event. The role of PFO closure and antiplatelet or anticoagulation therapy has not been well investigated. Moreover, during COVID-19 pandemic and taking into account the increased rates of thrombotic events and the difficulties in blood transfusion, management of SCD patients is even more challenging and difficult, since data are scarce regarding stroke occurrence and management in this specific population in the COVID-19 era. This review focuses on pathophysiology of stroke in patients with SCD and possible treatment strategies in the presence of PFO.

Tài liệu tham khảo

Bunn HF (1997) Pathogenesis and treatment of sickle cell disease. N Engl J Med 337:762–769 Brittenham GM, Schechter AN, Tom Noguchi C (1985) Hemoglobin S polymerization: primary determinant of the hemolytic and clinical severity of the sickling syndromes. Blood 65:183–189. https://doi.org/10.1182/blood.v65.1.183.bloodjournal651183 Rees DC, Williams TN, Gladwin MT (2010) Sickle-cell disease. The Lancet 376:2018–2031 Kato GJ, Piel FB, Reid CD et al (2018) Sickle cell disease. Nat Rev Dis Primers 4:1–22 Naik RP, Streiff MB, Lanzkron S (2013) Sickle cell disease and venous thromboembolism: what the anticoagulation expert needs to know. J Thromb Thrombolysis 35:352–358 Ansari J, Moufarrej YE, Pawlinski R, Gavins FNE (2018) Sickle cell disease: a malady beyond a hemoglobin defect in cerebrovascular disease. Expert Rev Hematol 11:45–55. https://doi.org/10.1080/17474086.2018.1407240 Pavlakis SG, Bello J, Prohovnik I et al (1988) Brain infarction in sickle cell anemia: magnetic resonance imaging correlates. Ann Neurol 23:125–130. https://doi.org/10.1002/ana.410230204 Ohene-Frempong K, Weiner SJ, Sleeper LA et al (1998) Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 91:288–294. https://doi.org/10.1182/blood.V91.1.288 Prussien KV, Jordan LC, Debaun MR, Compas BE (2019) Cognitive function in sickle cell disease across domains, cerebral infarct status, and the lifespan: a meta-analysis. J Pediatr Psychol 44:948–958. https://doi.org/10.1093/jpepsy/jsz031 DeBaun MR, Jordan LC, King AA et al (2020) American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv 4:1554–1588. https://doi.org/10.1182/bloodadvances.2019001142 Estcourt LJ, Kimber C, Hopewell S et al (2020) Interventions for preventing silent cerebral infarcts in people with sickle cell disease. Cochrane Database Syst Rev 4:CD012389 Lechat P, Mas JL, Lascault G et al (1988) Prevalence of patent foramen ovale in patients with stroke. N Engl J Med 318:1148–1152. https://doi.org/10.1056/NEJM198805053181802 Alsheikh-Ali AA, Thaler DE, Kent DM (2009) Patent foramen ovale in cryptogenic stroke: incidental or pathogenic? Stroke 40:2349–2355. https://doi.org/10.1161/STROKEAHA.109.547828 Mojadidi MK, Zaman MO, Elgendy IY et al (2018) Cryptogenic stroke and patent foramen ovale. J Am Coll Cardiol 71:1035–1043 Elgendy AY, Saver JL, Amin Z et al (2020) Proposal for updated nomenclature and classification of potential causative mechanism in patent foramen ovale-associated stroke. JAMA Neurol 77(7):878–886. https://doi.org/10.1001/jamaneurol.2020.0458 Lee PH, Song JK, Kim JS et al (2018) Cryptogenic stroke and high-risk patent foramen ovale: the DEFENSE-PFO trial. J Am Coll Cardiol 71:2335–2342. https://doi.org/10.1016/j.jacc.2018.02.046 Miranda B, Fonseca AC, Ferro JM (2018) Patent foramen ovale and stroke. J Neurol 265:1943–1949. https://doi.org/10.1007/s00415-018-8865-0 Shatzel JJ, Daughety MM, Prasad V, DeLoughery TG (2018) PFO closure for secondary stroke prevention: is the discussion closed? J Thromb Thrombolysis 46:74–76 DeBaun MR, Armstrong FD, McKinstry RC et al (2012) Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood 119:4587–4596 Debaun MR, Kirkham FJ (2016) Central nervous system complications and management in sickle cell disease. Blood 127:829–838 Hirtz D, Kirkham FJ (2019) Sickle cell disease and stroke. Pediatr Neurol 95:34–41 Kato GJ, Steinberg MH, Gladwin MT (2017) Intravascular hemolysis and the pathophysiology of sickle cell disease. J Clin Investig 127:750–760 Ansari J, Gavins FNE (2019) Ischemia-reperfusion injury in sickle cell disease: from basics to therapeutics. Am J Pathol 189:706–718 Proenca̧-Ferreira R, Brugnerotto AF, Garrido VT et al (2014) Endothelial activation by platelets from sickle cell anemia patients. PLoS ONE 9(2):e89012. https://doi.org/10.1371/journal.pone.0089012 Adams R, Nichols F, Carl E et al (1992) The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med 326:605–610. https://doi.org/10.1056/NEJM199202273260905 Prohovnik I, Hurlet-Jensen A, Adams R et al (2009) Hemodynamic etiology of elevated flow velocity and stroke in sickle-cell disease. J Cereb Blood Flow Metab 29:803–810. https://doi.org/10.1038/jcbfm.2009.6 Fields ME, Guilliams KP, Ragan DK et al (2018) Regional oxygen extraction predicts border zone vulnerability to stroke in sickle cell disease. Neurology 90:e1134–e1144. https://doi.org/10.1212/WNL.0000000000005194 Václavů L, Petr J, Petersen ET et al (2020) Cerebral oxygen metabolism in adults with sickle cell disease. Am J Hematol. https://doi.org/10.1002/ajh.25727 Hulbert ML, Ford AL (2014) Understanding sickle cell brain drain. Blood 124:830–831. https://doi.org/10.1182/blood-2014-06-582403 De Franceschi L, Cappellini MD, Olivieri O (2011) Thrombosis and sickle cell disease. Semin Thromb Hemost 37:226–236. https://doi.org/10.1055/s-0031-1273087 Switzer JA, Hess DC, Nichols FT, Adams RJ (2006) Pathophysiology and treatment of stroke in sickle-cell disease: present and future. Lancet Neurol 5:501–512. https://doi.org/10.1016/S1474-4422(06)70469-0 Al-Awadhi A, Adekile A, Marouf R (2017) Evaluation of von Willebrand factor and ADAMTS-13 antigen and activity levels in sickle cell disease patients in Kuwait. J Thromb Thrombolysis 43:117–123. https://doi.org/10.1007/s11239-016-1418-4 Ito MT, da Silva Costa SM, Baptista LC et al (2020) Angiogenesis-related genes in endothelial progenitor cells may be involved in sickle cell stroke. J Am Heart Assoc 9(3):e014143. https://doi.org/10.1161/jaha.119.014143 Guilliams KP, Fields ME, Dowling MM (2019) Advances in understanding ischemic stroke physiology and the impact of vasculopathy in children with sickle cell disease. Stroke 50:266–273. https://doi.org/10.1161/STROKEAHA.118.020482 Dowling MM, Quinn CT, Ramaciotti C et al (2017) Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke. Br J Haematol 176:300–308. https://doi.org/10.1111/bjh.14391 Lawrence C, Webb J (2016) Sickle cell disease and stroke: diagnosis and management. Curr Neurol Neurosci Rep 6(3):27. https://doi.org/10.1007/s11910-016-0622-0 Hines PC, McKnight TP, Seto W, Kwiatkowski JL (2011) Central nervous system events in children with sickle cell disease presenting acutely with headache. J Pediatr 159(3):472–478. https://doi.org/10.1016/j.jpeds.2011.02.009 Silva GS, Vicari P, Figueiredo MS, Junior HC, Idagawa MH, Massaro AR (2006) Migraine-mimicking headache and sickle cell disease: a transcranial Doppler study. Cephalalgia 26(6):678–683. https://doi.org/10.1111/j.1468-2982.2006.01092.x Steen RG, Reddick WE, Mulhern RK et al (1998) Quantitative MRI of the brain in children with sickle cell disease reveals abnormalities unseen by conventional MRI. J Magn Reson Imaging 8(3):535–543. https://doi.org/10.1002/jmri.1880080304 Adams RJ (2000) Lessons from the stroke prevention trial in sickle cell anemia (STOP) study. J Child Neurol 15(5):344–349 Ware RE, Davis BR, Schultz WH et al (2016) Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia-TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, noninferiority trial. Lancet 387:661–670 Klijn CJ, Paciaroni M, Berge E, Korompoki E, KõrvJ Lal A, Putaala J, Werring DJ (2019) Antithrombotic treatment for secondary prevention of stroke and other thromboembolic events in patients with stroke or transient ischemic attack and non-valvular atrial fibrillation: a European Stroke Organisation guideline. Eur Stroke J 4(3):198–223. https://doi.org/10.1177/2396987319841187 Ataga KI, Kutlar A, Kanter J et al (2017) Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med 376:429–439 Aggeli C, Verveniotis A, Andrikopoulou E et al (2018) Echocardiographic features of PFOs and paradoxical embolism: a complicated puzzle. Int J Cardiovasc Imaging 34(12):1849–1861. https://doi.org/10.1007/s10554-018-1406-1 Pristipino C, Sievert H, D’Ascenzo F et al (2019) European position paper on the management of patients with patent foramen ovale. General approach and left circulation thromboembolism. Eur Heart J 40(38):3182–3195. https://doi.org/10.1093/eurheartj/ehy649 Zhao H, Yue Q, Wang T, Wang L, Pang Z, Dong H, Yang J, Li Y, Li S (2019) Sensitivity of contrast-enhanced transthoracic echocardiography for the detection of residual shunts after percutaneous patent foramen ovale closure. Medicine 98(4):e14276. https://doi.org/10.1097/md.0000000000014276 Takaya Y, Watanabe N, Ikeda M et al (2020) Importance of abdominal compression Valsalva maneuver and microbubble grading in contrast transthoracic echocardiography for detecting patent foramen ovale. J Am Soc Echocardiogr 33(2):201–206. https://doi.org/10.1016/j.echo.2019.09.018 Mas JL, Derex L, Guérin P et al (2019) Transcatheter closure of patent foramen ovale to prevent stroke recurrence in patients with otherwise unexplained ischaemic stroke: expert consensus of the French Neurovascular Society and the French Society of Cardiology. Arch Cardiovasc Dis 112(8–9):532–542. https://doi.org/10.1016/j.acvd.2019.06.002 Dowling MM, Lee N, Quinn CT et al (2010) Prevalence of intracardiac shunting in children with sickle cell disease and stroke. J Pediatr 156(4):645–650. https://doi.org/10.1016/j.jpeds.2009.10.012 Razdan S, Strouse JJ, Naik R et al (2013) Patent foramen ovale in patients with sickle cell disease and stroke: case presentations and review of the literature. Case Rep Hematol 2013:516705. https://doi.org/10.1155/2013/516705 Giblett JP, Abdul-Samad O, Shapiro LM, Rana BS, Calvert PA (2019) Patent foramen ovale closure in 2019. Interv Cardiol Rev 14(1):34–41. https://doi.org/10.15420/icr.2018.33.2 Yaghi S, Ishida K, Torres J et al (2020) SARS2-CoV-2 and stroke in a new york healthcare system. Stroke. https://doi.org/10.1161/strokeaha.120.030335