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• W2914882023 abstract "A 26-year-old woman with hemoglobin (Hb) SC disease was admitted to the hospital with a sickle cell crisis. She had pain in her legs and arms but denied chest pain or dyspnea. Her oxygen saturation (Sao2) was 97% while breathing room air. She was treated with intravenous fluids, hydroxyurea, and hydromorphone (Dilaudid). Approximately 36 h after admission she was transferred to the ICU because of a decreased level of consciousness and respiratory distress. At this time she had a heart rate of 145 beats/min, blood pressure of 107/85 mm Hg, respiratory rate of 48 breaths/min, temperature of 38.8°C, and Sao2 of 87% while receiving supplemental oxygen at 6 L/min. She was unresponsive to verbal commands but withdrew from painful stimuli. There were no focal neurologic defects, and her pupils were equal and reactive to light. A postintubation chest radiograph showed minimal basilar atelectasis. A focused cardiac ultrasound was performed because of hypoxemia and marked tachycardia (Videos 1, 2). A transthoracic echocardiogram (TTE) 4 months earlier had produced normal results. Question: Given the clinical presentation, what is the likely cause of the changes seen on the bedside ultrasound, and how should it be treated? Answer: Fat embolism syndrome; treated by urgent red blood cell exchange transfusion. The ultrasound shows marked dilation of the right ventricle and a flattened intraventricular septum, giving a “D” shape (Videos 1, 2, Narration Video). Given the previously normal TTE results, these changes are indicative of acute cor pulmonale due to a marked increase in pulmonary vascular resistance. Acute cor pulmonale in sickle cell crisis could be due to either pulmonary thromboembolism or fat embolism syndrome (FES). Pulmonary thromboembolism was excluded by CT angiography, and the patient was treated by urgent red blood cell exchange transfusion for presumptive FES. Cerebral fat embolism was confirmed by brain MRI (Fig 1) and a dilated eye examination revealed retinal perivascular whitening and cotton wool spots consistent with ocular fat embolism. Because of the extensive cerebral FES, a repeat focused cardiac ultrasound was performed with injection of 10 mL of agitated saline to look for a right-to-left shunt. Numerous microbubbles entered the left side of the heart within one or two cardiac cycles, indicating the presence of an intracardiac shunt, most likely from a patent foramen ovale (PFO) (Video 3, Narration Video). An atrial septal aneurysm was suspected, based on the shape of the interatrial septum. A follow-up bedside ultrasound 4 days later showed persistent dilation of the right side of the heart and better demonstrated the atrial septal aneurysm (Video 4, Narration Video). Fat embolism syndrome is a recognized complication of sickle cell disease. The pathophysiology of FES in sickle cell disease is thought to be related to an acute vasoocclusive event with bone marrow necrosis, promoting embolization of fat and other bone marrow contents into the venous system.1Dang N.C. Johnson C. Eslami-Farsani M. Hawood L.J. Bone marrow embolism in sickle cell disease: a review.Am J Hematol. 2005; 79: 61-67Crossref PubMed Scopus (69) Google Scholar Once in the venous system, the fat emboli may cause damage through mechanical obstruction and an inflammatory response.1Dang N.C. Johnson C. Eslami-Farsani M. Hawood L.J. Bone marrow embolism in sickle cell disease: a review.Am J Hematol. 2005; 79: 61-67Crossref PubMed Scopus (69) Google Scholar According to the mechanical theory, fat particles that lodge in the pulmonary microvasculature lead to a ventilation-perfusion mismatch and an acute increase in pulmonary vascular resistance. A second, biochemical mechanism postulates that the pulmonary fat emboli initiate a cascade of inflammation, direct endothelial damage, and platelet aggregation.1Dang N.C. Johnson C. Eslami-Farsani M. Hawood L.J. Bone marrow embolism in sickle cell disease: a review.Am J Hematol. 2005; 79: 61-67Crossref PubMed Scopus (69) Google Scholar Clinically, the pulmonary manifestations of FES include hypoxemia, with or without radiographic opacities, and acute cor pulmonale that in extreme cases may cause sudden death.1Dang N.C. Johnson C. Eslami-Farsani M. Hawood L.J. Bone marrow embolism in sickle cell disease: a review.Am J Hematol. 2005; 79: 61-67Crossref PubMed Scopus (69) Google Scholar, 2Vichinsky E. Williams R. Das M. et al.Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia.Blood. 1994; 83: 3107-3112Crossref PubMed Google Scholar Although the pulmonary system is primarily affected, fat emboli can enter the systemic circulation and cause injury to various organs, including the brain, skin, eye, and kidney. The diagnosis of fat embolism is primarily clinical as there is no single pathognomonic finding. A diagnosis of FES is usually suspected when a patient with long bone fracture or sickle cell crisis develops acute hypoxemia, and especially when accompanied by fever and altered mentation. Indeed, unexplained acute neurologic changes in a patient with acute chest syndrome is strongly suggestive of FES.2Vichinsky E. Williams R. Das M. et al.Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia.Blood. 1994; 83: 3107-3112Crossref PubMed Google Scholar Since its features are nonspecific, other causes of hypoxemia such as pneumonia, aspiration, pulmonary edema, and thromboembolism must be excluded. More specific evidence of FES may be provided by the presence of abundant fat in macrophages obtained by bronchoalveolar lavage,3Maitre B. Habibi A. Roudot-Thoraval F. et al.Acute chest syndrome in adults with sickle cell disease.Chest. 2000; 117 (1386-139)Abstract Full Text Full Text PDF PubMed Scopus (92) Google Scholar brain MRI that shows a typical “starfield” pattern of diffuse, punctate, hyperintense lesions on diffusion weighted imaging,4Godoy D.A. Di Napoli M. Rabinstein A.A. Cerebral fat embolism: recognition, complications, and prognosis.Neurocrit Care. 2018; 29: 358-365Crossref PubMed Scopus (20) Google Scholar or retinal lesions on dilated eye examination. In the present case, the finding of acute cor pulmonale on bedside cardiac ultrasound with a negative CT pulmonary angiogram provided compelling evidence for FES and led to prompt institution of exchange transfusion, a potentially life-saving intervention in patients with catastrophic FES.1Dang N.C. Johnson C. Eslami-Farsani M. Hawood L.J. Bone marrow embolism in sickle cell disease: a review.Am J Hematol. 2005; 79: 61-67Crossref PubMed Scopus (69) Google Scholar, 5Greaves P. Mathew V. Peters C. Rowe S. Amos R.J. Tsitsikas D.A. Successful outcome of three patients with sickle-cell disease and fat embolism syndrome treated with intensive exchange transfusion.Clin Case Rep. 2016; 5: 39-43Crossref PubMed Google Scholar The incidence of right-side heart strain in sickle cell-related FES is unknown, but our experience would support a role for focused cardiac ultrasound in the evaluation of patients with sickle cell crisis who develop either hypoxemia or hemodynamic instability. Neurologic manifestations of cerebral FES are variable, ranging from mild confusion to coma. In patients with FES, the presence of a right-to-left intracardiac shunt through a PFO increases the risk of cerebral complications.6Forteza A.M. Koch S. Campo-Bastillo I. et al.Transcranial Doppler detection of cerebral fat embolism and relation to paradoxical embolism: a pilot study.Circulation. 2011; 123: 1947-1952Crossref PubMed Scopus (22) Google Scholar The presence of an atrial septal aneurysm (as seen in this patient) increases the likelihood that a PFO will be associated with a clinically significant right-to-left shunt and paradoxical embolism in the setting of elevated right atrial pressure.7Shirani J. Zafari A.M. Roberts W.C. Morphologic features of fossa ovalis membrane aneurysm in the adult and its clinical significance.J Am Coll Cardiol. 1995; 26: 466-471Crossref PubMed Scopus (24) Google Scholar The degree of right-to-left shunting through a PFO is dynamic and is determined primarily by the relationship between right and left atrial pressures, and to a lesser extent by body position.8Tobis J.M. Narashimha D. Abudayyeh I. Patent foramen ovale closure for hypoxemia.Interv Cardiol Clin. 2017; 6: 547-554Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar Fat embolism can lead to a sudden, marked increase in pulmonary vascular resistance that leads to acute dilation of the right side of the heart and increased right atrial pressure, encouraging fat particles to cross the PFO and enter the systemic circulation.8Tobis J.M. Narashimha D. Abudayyeh I. Patent foramen ovale closure for hypoxemia.Interv Cardiol Clin. 2017; 6: 547-554Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar Given the increased risk of neurologic injury during subsequent sickle cell crises, percutaneous closure of a PFO should be considered in patients who have experienced sickle cell-related cerebral FES.9Ntalios G. Papavasileiou V. Sagris D. et al.Closure of patent foramen ovale versus medical therapy in patients with cryptogenic stroke or transient ischemic attack: updated systematic review and meta-analysis.Stroke. 2018; 49: 412-418Crossref PubMed Scopus (80) Google Scholar The patient in this case had Hb SC, a variant of sickle cell disease that is usually associated with a milder clinical course than Hb SS. However, a recent literature review found that 81% of reported cases of FES in sickle cell disease occurred in patients without the homozygous SS genotype and that SC disease was the single most common variant, accounting for 43% of cases.10Tsitsikas D.A. Gallinella G. Patel Sneha Seligman H. Greaves P. Amos R.J. Bone marrow necrosis and fat embolism syndrome in sickle cell disease: increased susceptibility of patients with non-SS genotypes and a possible association with human parvovirus B19 infection.Blood Rev. 2014; 28: 23-30Abstract Full Text Full Text PDF PubMed Scopus (71) Google Scholar The majority of patients had minimal or no previous complications related to their sickle cell disease, and in a number cases a diagnosis of sickle cell disease was established only after an episode of catastrophic FES that was sometimes fatal.10Tsitsikas D.A. Gallinella G. Patel Sneha Seligman H. Greaves P. Amos R.J. Bone marrow necrosis and fat embolism syndrome in sickle cell disease: increased susceptibility of patients with non-SS genotypes and a possible association with human parvovirus B19 infection.Blood Rev. 2014; 28: 23-30Abstract Full Text Full Text PDF PubMed Scopus (71) Google Scholar The reason for the apparently higher incidence of severe FES in patients with non-SS genotypes is unknown. A role for parvovirus infection in sickle cell-related FES has been postulated.10Tsitsikas D.A. Gallinella G. Patel Sneha Seligman H. Greaves P. Amos R.J. Bone marrow necrosis and fat embolism syndrome in sickle cell disease: increased susceptibility of patients with non-SS genotypes and a possible association with human parvovirus B19 infection.Blood Rev. 2014; 28: 23-30Abstract Full Text Full Text PDF PubMed Scopus (71) Google Scholar A tracheostomy was placed and the patient was transferred to a long-term acute care facility, where she received additional exchange transfusions. Over the following month she regained consciousness, with return to her prior level of cognitive function. She was discharged home and is monitored in the hematology clinic where she receives regular exchange transfusions. A repeat TTE 3 months later showed normal right ventricular size and function with normal estimated peak systolic pulmonary arterial pressure. She has been referred for percutaneous closure of her PFO guided by transesophageal echocardiography. 1.Patients with acute sickle cell crisis can develop bone marrow necrosis with fat embolism syndrome, and the risk of this complication is higher in those with Hb SC disease than the Hb SS genotype.2.Recognition of FES in sickle cell disease is important so that red blood cell exchange transfusion can be implemented promptly.3.When patients with sickle cell crisis develop significant hypoxemia, a point-of-care cardiac ultrasound that shows acute cor pulmonale may provide a rapid presumptive diagnosis of FES once pulmonary thromboembolism is excluded by CT pulmonary angiography.4.Neurologic manifestations of FES are more common and tend to be more severe in patients who have a right-to-left shunt due to a PFO; the latter may be demonstrated by point-of-care cardiac ultrasound by imaging while injecting agitated saline. Author contributions: K. M. L., J. R. B., M. A. S., and J. W. L. had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis, including and especially any adverse effects. Financial/nonfinancial disclosure: None declared. Other contributions: CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met. Additional information: To analyze this case with the videos, see the online article. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiIzOGU5ZDljZjU4MGQwMjA2ZjdhNGEyYzNlMTlkZDRhMiIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4NTM3MTE0fQ.EBwiHhZkoB_THMIua_kymkIpmtVNE7gj7086M1eP5NOkJN71PNDVjwkobzEjKto5LJ0RCG_Gw8I11EkbdzTxPjhgh-siLujjx4jn_IFdeTZH9AD3YXiGieZtoXJu4l_50WQa1Gqb1oUkXGTo7-nRPZsch9sx9Ta426Zp_zV0Jx-63pKc4iW6v9i6yOGPi6ANPVaA7NronUAjPFVbF0Ki7osiYHlFV1taxG8n8zcSFJD4bAjTPkg_tkdo0Ie9fWRZUY3KtnjYQ_u8KB5N0cX0ECeMAjsHwDu0zgANMkeTTNidauGLNqwTTG0dW4aoqaVFgcCzA9fgj7uWkkokVVYBMw Download .mp4 (1.71 MB) Help with .mp4 files Video 1Focused cardiac ultrasound (subcostal), showing marked dilation and decreased function of right ventricle.eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJmMzAxNTZiM2NmYjBhOWZmYTI0MGYzNGMzZDM4OTVjMSIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4NTM3MTE0fQ.MVrZTKVl9c6pPlHx-kUwLYcrMhQVpGROPEdmpPz-aWcNysmrK3A_5ZT7xj8xdtEkDni9_brm8SoZ0bsPMepao7e-9MqvtelDeOGuCpnHR9_Q_pARAEm29vtZts7n2cRX4FHTC3w22QoPGdXwHqyklFHExiXEfy1CawTrwphk78tFD9vRLMxbZbsBIHHj4ZKPmKxoMyQY9Z1xNbt6c9k2C6QrMeWIOao9UAkj0-M-oOa8sZfr2DNtbA7ayh2qem-AEaWI25ZcwyOrP2na3P6GRWnMdSw2NMCn1UjRVWiJRBpq0c06hKfvPwXp7s2G5_YXhpRrdgeLUjp5G2TmaybpbA Download .mp4 (1.71 MB) Help with .mp4 files Video 2Focused cardiac ultrasound (parasternal, short axis), showing septal flattening, giving a “D” shape.eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJhZjkxZGNhZjE3MjRlYTlmYjExMDQ1NzQxNTNhY2M2YyIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4NTM3MTE0fQ.Kok4qFfqcD3_2fe2mzqCqan7XHyuJU0N4hZaF8jL-Si1gow445itiny6mjPrhRkl2nSJlqUshhIFBVxo0EtCauIMa7fF7kh9ikfIuyPEgL-L00_Y51cF6dqW8DD1EbecyJFmsRFAc2C9NXkjipMNomYk20Vy8bQjtcQCfZ52gSGpoB_T4ClCPW1lDaRuclNhyYONPdodm_6A-pM_mUVIL03Jksqn1GnWBL4k01tnBwuT6t_0yroJYqPyMAQpfrUco5RARMZELXAgMRK1mjSznMdICDKXaTeOlRL45rIYYesvITMj_TooQKQzka5USuvpEQ5CtiNZ-C25Q7qaa7N4Xw Download .mp4 (1.73 MB) Help with .mp4 files Video 3Focused cardiac ultrasound (subcostal) during injection of agitated saline, demonstrating numerous microbubbles entering the left side of the heart.eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJiZGRjNjY0ZDljZTBmYjAzNzk2YWRmZTRlYjE2ZTQ4OCIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4NTM3MTE0fQ.Biq7o09JSXbm6quq8O74iziKuHPlAdGDR5CkjrHIbqrH-rG7Nhz35SlWcKOfwbj8Kr7eRRpvqe1pmJ1NUf6YNHAMHajetbEjyFkef9mjGLUHz5Aw7gIgfNEoY-BVFlaFMbbCt1CrBQg51vMbWw5-vapQ-GfNtyKWpwqGUpbCD6euz0tmLw-udqrMJuDJz12RQ9VUvJjEwLQe3UkPo0FfZ1WHq1B-WEmh4HOgbDK7S2mIMK0Jiz2d1WG6upHANOW-dqqnhqLfqnQrSRpl0cqBXo0H20RjpzLggTRJWEbp9KlmSo1BhoYJNp0-2qKDSpzkYR_kjfjQDIbeB6Rj87GYJA Download .mp4 (0.63 MB) Help with .mp4 files Video 4Focused cardiac ultrasound (apical), showing clear evidence of atrial septal aneurysm.eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiI4ODQ0OWYxYTNkY2NkODQwNzQyMGQ1MzcyOGZkMTFkNCIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4NTM3MTE0fQ.g9BN1gIb8hYIaYI8VKV4mIKXlErL6RV6tybfBdu6Tc4oa-PF4vtYIHZvxrodFWBzeIUVGFMR3HXtdhscBrGLCJkFTabU8iZ4xwANTQ3bhMlhBjwoJXhrQ4-w3lbHPDSNWDXSc1R-up3CBIQ9ES8dmDKyjpbOwxSFq8pbs-3eiaDw3eaUKnPnYQEKXVUvJEGRS94UnyseCndhaXO-uPDunnx2uoAHjl_PCR7wYKg-0HhTY-HWEAS4S7go21YdQ1ERI_-zWP86enHDnDt5v3OebvS4Je5VJ34y6uCw0JvpPjU3z_SZ-0pI_elcneTanME80pccHX1A0HClZKOEqpOkkg Download .mp4 (27.59 MB) Help with .mp4 files Discussion Video" @default.
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• W2914882023 date "2019-02-01" @default.
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• W2914882023 title "Acute Hypoxemia and Coma in a Patient With Hemoglobin SC Disease" @default.
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