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Posterior reversible encephalopathy syndrome in a patient submitted to extracorporeal membrane oxygenation for acute fulminant myocarditis
International Journal of Cardiology 172 (2014) e329–e330
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Posterior reversible encephalopathy syndrome in a patient submitted to extracorporeal membrane oxygenation for acute fulminant myocarditis Roberto Lorusso a,⁎, Enrico Vizzardi c, Lorenzo Pinelli b, Sandro Gelsomino a a b c
Cardiac Surgery Units, Community Hospital, Brescia, Italy Neuroradiology Units, Community Hospital, Brescia, Italy Cardiology Units, Community Hospital, Brescia, Italy
a r t i c l e
i n f o
Article history: Received 16 November 2013 Accepted 29 December 2013 Available online 10 January 2014 Keywords: Extracorporeal membrane oxygenation Acute myocarditis Cardiogenic shock Neurological complication
Neurological complications in patients undergoing extracorporeal membrane oxygenation (ECMO) are usually secondary to diffuse hypoxia, hemorrhage, or emboli [1–3]. We herewith report a case of a patient submitted to ECMO for fulminant myocarditis who showed neurological impairment on awakening with brain tissue abnormalities at diagnostic workup compatible with a condition denominated “Posterior Reversible Encephalopathy Syndrome” (PRES) [4,5]. A 32 year-old female patient was admitted to the emergency department for acute chest pain. On arrival, the patients was in cardiogenic shock, immediately intubated, and promptly brought to the cath lab for coronary visualization. ECG showed diffuse ischemic-like changes. The coronary angiography showed normal coronary arteries. Transesophageal echocardiography documented severe biventricular contractile compromise and left ventricular (LV) ejection fraction of 10%. Following several transient episodes of cardiac arrest and persistent hypotension, ECMO was applied by femoral vessel cannulation at the right groin (size 19 for arterial cannula and 25 for venous return, respectively), with distal ipsilateral femoral artery perfusion, and intra-aortic balloon counterpulsation at the contra-lateral femoral artery. Despite apparent effective LV unloading with full ECMO flow, echocardiographic signs of blood stagnation in the LV and absence of aortic valve opening during
⁎ Corresponding author at: Cardiac Surgery Unit, Community Hospital, P.le Spedali Civili, 1 25125 Brescia, Italy. Tel.: +39 030 3995636; fax: +39 030 3995004. E-mail address: [email protected] (R. Lorusso). 0167-5273/$ – see front matter © 2014 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2013.12.275
cardiac systole were observed. High dosages of catecholamine (adrenalin and noradrenalin) were maintained during the first 3 days of mechanical support with disappearance of LV blood stasis, and reinstitution of opening of the aortic valve and pulsatile systemic arterial pressure curve. Initial cardiac contractile improvement was observed at day 4, and full heart contractile recovery at day 7. All vital parameters (systemic venous and arterial blood pressures, activated clotting time, blood pH, blood oxygen and carbon dioxide content, red blood cell count) were within the normal ranges, and no system dysfunction or macroscopic thrombosis was observed throughout the ECMO run. Adenovirus-related myocarditis emerged from blood tests. IABP was maintained for additional 3 days after ECMO withdrawal. On sedation discontinuation, seizures, marked visual disturbances, lethargy and slowed motor responses of the four limbs were noticed. At magnetic resonance imaging (MRI) acute subdural hematoma on the right side and vasogenic edema of the left frontoparietal–occipital white matter were shown (Fig. 1). Furthermore, vasospasm of both internal carotid arteries as well as of both anterior and middle cerebral arteries was also visible (Fig. 1). Appropriate pharmacological therapy was instituted together with aggressive phyõsical rehabilitation for 3 weeks. The patient fully recovered from the neurologic deficits, with complete resolution of functional impairment and disappearance of previous cerebral abnormalities at 6-month MRI control (Fig. 2). Acute severe biventricular failure remains one of the most frequent indications for ECMO [2]. Acute fulminant myocarditis may represent one of the potential scenarios for short-term mechanical cardio-circulatory support in this setting [2] Although ECMO has shown to enhance cardiac recovery in patients with acute myocarditis, several complications may anyhow develop and impact ultimate patient outcome [1–3,6]. Cerebrovascular events have been shown to affect from 20% to 30% of supported patients [1,2], with a mortality reaching almost 100% [3]. Furthermore, only 50% of all cerebral adverse events in patients undergoing ECMO are clinically detected [6]. Neurologic events usually arise from well known ECMO-based “pathophysiology” (embolism, bleeding, and hypoperfusion), but several other factors may influence the integrity of cerebral structures. In our case, the neurologic complication was secondary to a vasogenic insult, although limited hemorrhagic lesion was also associated. Unstable circulatory conditions and altered vasodynamic regulations in the brain are likely part of the global cardiocirculatory compromise and intimately linked to ECMO conditions
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R. Lorusso et al. / International Journal of Cardiology 172 (2014) e329–e330
Fig. 2. Brain magnetic resonance imaging, axial FLAIR (fluid attenuated inversion recovery) image at 3 months from ECMO implantation. Both the right subdural hematoma and the left fronto-parietal–occipital white matter edema completely disappeared.
(blood flows, fluids, vasoactive agents, pH, blood gases-related abnormalities, anemia), besides well known brain-related injuries. Drug-induced or anomalous functional reaction to altered flow states, together with other predisposing factors, is also responsible of a recently described neurologic syndrome called PRES [4,5]. Transient leakage of the blood/brain barrier, secondary to vascular spasm, systemic arterial hypertension, or other conditions impacting the blood/brain barrier integrity, leading to vasogenic edema usually observed at occipital portions, are clinically characterized by seizures, visual disturbances and/or headache [4,5]. Final outcome of PRES has been often shown to be favorable [4,5]. Our case confirms that ECMO patients are at risk for a wider spectrum of neurologic complications then previously considered [1,6]. The pathophysiology of the ECMO/brain interaction is still poorly understood, although clotting, bleeding, and hypoperfusion apparently account for the majority of neurologic events [1,3,6]. The herein reported case, to our knowledge, represents the first evidence of PRES, and brain-related arterial vasospasm, during an ECMO run, but also indicates that favorable outcome may still be achieved in these complex conditions. In addition, the relative high rate of neurologic adverse events in ECMO patients underlines the need of better brain monitoring and aggressive diagnostic workup in these settings. References
Fig. 1. A: Brain magnetic resonance imaging, axial FLAIR (fluid attenuated inversion recovery) image. Hemispheric acute subdural hematoma on the right side (arrows) and diffuse hyperintensity of the left fronto-parietal–occipital white matter with cortical sparing, suggesting vasogenic edema. B: Brain magnetic resonance imaging, ADC (Apparent Diffusion Coefficient) map at the same level as A. Left fronto-parietal–occipital white matter is hyperintense due to a high ADC value, confirming vasogenic (potentially reversible) edema. C: Brain magnetic resonance imaging angiography, MIP (Maximum Intensity Projection) algorithm. There is a clear-cut narrowing of both internal carotid arteries at the level of the carotid siphons (arrows); both anterior and middle cerebral arteries show alternating short segments of tight and less severe stenosis suggestive of cerebral vasospasm.
[1] Risnes I, Wagner K, Nome T, et al. Cerebral outcome in adult patients treated with extracorporeal membrane oxygenation. Ann Thorac Surg 2006;81:1401–6. [2] Smedira NG, Moazami N, Golding CM, et al. Clinical experience with 202 adults receiving extracorporeal membrane oxygenation for cardiac failure: survival at five years. J Thorac Cardiovasc Surg 2001;122:92–102. [3] Kasirajan V, Smedira NG, McCarthy JF, Casselman F, Boparai N, McCarthy PM. Risk factor for intracranial hemorrhage in adults on extracorporeal membrane oxygenation. Eur J Cardio-Thorac Surg 1999;15:508–14. [4] Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494–500. [5] Bartynski WS. Posterior reversible encephalopathy syndrome: fundamental imaging and clinical features. Am J Neuroradiol 2008;29:1036–42. [6] Rastan AJ, Lachman N, Walther T, et al. Autopsy findings in patients on postcardiotomy extracorporeal membrane oxygenation (ECMO). Int J Artif Organs 2006;29:1121–31.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Posterior reversible encephalopathy syndrome in a patient submitted to extracorporeal membrane oxygenation for acute fulminant myocarditis Roberto Lorusso a,⁎, Enrico Vizzardi c, Lorenzo Pinelli b, Sandro Gelsomino a a b c
Cardiac Surgery Units, Community Hospital, Brescia, Italy Neuroradiology Units, Community Hospital, Brescia, Italy Cardiology Units, Community Hospital, Brescia, Italy
a r t i c l e
i n f o
Article history: Received 16 November 2013 Accepted 29 December 2013 Available online 10 January 2014 Keywords: Extracorporeal membrane oxygenation Acute myocarditis Cardiogenic shock Neurological complication
Neurological complications in patients undergoing extracorporeal membrane oxygenation (ECMO) are usually secondary to diffuse hypoxia, hemorrhage, or emboli [1–3]. We herewith report a case of a patient submitted to ECMO for fulminant myocarditis who showed neurological impairment on awakening with brain tissue abnormalities at diagnostic workup compatible with a condition denominated “Posterior Reversible Encephalopathy Syndrome” (PRES) [4,5]. A 32 year-old female patient was admitted to the emergency department for acute chest pain. On arrival, the patients was in cardiogenic shock, immediately intubated, and promptly brought to the cath lab for coronary visualization. ECG showed diffuse ischemic-like changes. The coronary angiography showed normal coronary arteries. Transesophageal echocardiography documented severe biventricular contractile compromise and left ventricular (LV) ejection fraction of 10%. Following several transient episodes of cardiac arrest and persistent hypotension, ECMO was applied by femoral vessel cannulation at the right groin (size 19 for arterial cannula and 25 for venous return, respectively), with distal ipsilateral femoral artery perfusion, and intra-aortic balloon counterpulsation at the contra-lateral femoral artery. Despite apparent effective LV unloading with full ECMO flow, echocardiographic signs of blood stagnation in the LV and absence of aortic valve opening during
⁎ Corresponding author at: Cardiac Surgery Unit, Community Hospital, P.le Spedali Civili, 1 25125 Brescia, Italy. Tel.: +39 030 3995636; fax: +39 030 3995004. E-mail address: [email protected] (R. Lorusso). 0167-5273/$ – see front matter © 2014 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2013.12.275
cardiac systole were observed. High dosages of catecholamine (adrenalin and noradrenalin) were maintained during the first 3 days of mechanical support with disappearance of LV blood stasis, and reinstitution of opening of the aortic valve and pulsatile systemic arterial pressure curve. Initial cardiac contractile improvement was observed at day 4, and full heart contractile recovery at day 7. All vital parameters (systemic venous and arterial blood pressures, activated clotting time, blood pH, blood oxygen and carbon dioxide content, red blood cell count) were within the normal ranges, and no system dysfunction or macroscopic thrombosis was observed throughout the ECMO run. Adenovirus-related myocarditis emerged from blood tests. IABP was maintained for additional 3 days after ECMO withdrawal. On sedation discontinuation, seizures, marked visual disturbances, lethargy and slowed motor responses of the four limbs were noticed. At magnetic resonance imaging (MRI) acute subdural hematoma on the right side and vasogenic edema of the left frontoparietal–occipital white matter were shown (Fig. 1). Furthermore, vasospasm of both internal carotid arteries as well as of both anterior and middle cerebral arteries was also visible (Fig. 1). Appropriate pharmacological therapy was instituted together with aggressive phyõsical rehabilitation for 3 weeks. The patient fully recovered from the neurologic deficits, with complete resolution of functional impairment and disappearance of previous cerebral abnormalities at 6-month MRI control (Fig. 2). Acute severe biventricular failure remains one of the most frequent indications for ECMO [2]. Acute fulminant myocarditis may represent one of the potential scenarios for short-term mechanical cardio-circulatory support in this setting [2] Although ECMO has shown to enhance cardiac recovery in patients with acute myocarditis, several complications may anyhow develop and impact ultimate patient outcome [1–3,6]. Cerebrovascular events have been shown to affect from 20% to 30% of supported patients [1,2], with a mortality reaching almost 100% [3]. Furthermore, only 50% of all cerebral adverse events in patients undergoing ECMO are clinically detected [6]. Neurologic events usually arise from well known ECMO-based “pathophysiology” (embolism, bleeding, and hypoperfusion), but several other factors may influence the integrity of cerebral structures. In our case, the neurologic complication was secondary to a vasogenic insult, although limited hemorrhagic lesion was also associated. Unstable circulatory conditions and altered vasodynamic regulations in the brain are likely part of the global cardiocirculatory compromise and intimately linked to ECMO conditions
e330
R. Lorusso et al. / International Journal of Cardiology 172 (2014) e329–e330
Fig. 2. Brain magnetic resonance imaging, axial FLAIR (fluid attenuated inversion recovery) image at 3 months from ECMO implantation. Both the right subdural hematoma and the left fronto-parietal–occipital white matter edema completely disappeared.
(blood flows, fluids, vasoactive agents, pH, blood gases-related abnormalities, anemia), besides well known brain-related injuries. Drug-induced or anomalous functional reaction to altered flow states, together with other predisposing factors, is also responsible of a recently described neurologic syndrome called PRES [4,5]. Transient leakage of the blood/brain barrier, secondary to vascular spasm, systemic arterial hypertension, or other conditions impacting the blood/brain barrier integrity, leading to vasogenic edema usually observed at occipital portions, are clinically characterized by seizures, visual disturbances and/or headache [4,5]. Final outcome of PRES has been often shown to be favorable [4,5]. Our case confirms that ECMO patients are at risk for a wider spectrum of neurologic complications then previously considered [1,6]. The pathophysiology of the ECMO/brain interaction is still poorly understood, although clotting, bleeding, and hypoperfusion apparently account for the majority of neurologic events [1,3,6]. The herein reported case, to our knowledge, represents the first evidence of PRES, and brain-related arterial vasospasm, during an ECMO run, but also indicates that favorable outcome may still be achieved in these complex conditions. In addition, the relative high rate of neurologic adverse events in ECMO patients underlines the need of better brain monitoring and aggressive diagnostic workup in these settings. References
Fig. 1. A: Brain magnetic resonance imaging, axial FLAIR (fluid attenuated inversion recovery) image. Hemispheric acute subdural hematoma on the right side (arrows) and diffuse hyperintensity of the left fronto-parietal–occipital white matter with cortical sparing, suggesting vasogenic edema. B: Brain magnetic resonance imaging, ADC (Apparent Diffusion Coefficient) map at the same level as A. Left fronto-parietal–occipital white matter is hyperintense due to a high ADC value, confirming vasogenic (potentially reversible) edema. C: Brain magnetic resonance imaging angiography, MIP (Maximum Intensity Projection) algorithm. There is a clear-cut narrowing of both internal carotid arteries at the level of the carotid siphons (arrows); both anterior and middle cerebral arteries show alternating short segments of tight and less severe stenosis suggestive of cerebral vasospasm.
[1] Risnes I, Wagner K, Nome T, et al. Cerebral outcome in adult patients treated with extracorporeal membrane oxygenation. Ann Thorac Surg 2006;81:1401–6. [2] Smedira NG, Moazami N, Golding CM, et al. Clinical experience with 202 adults receiving extracorporeal membrane oxygenation for cardiac failure: survival at five years. J Thorac Cardiovasc Surg 2001;122:92–102. [3] Kasirajan V, Smedira NG, McCarthy JF, Casselman F, Boparai N, McCarthy PM. Risk factor for intracranial hemorrhage in adults on extracorporeal membrane oxygenation. Eur J Cardio-Thorac Surg 1999;15:508–14. [4] Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494–500. [5] Bartynski WS. Posterior reversible encephalopathy syndrome: fundamental imaging and clinical features. Am J Neuroradiol 2008;29:1036–42. [6] Rastan AJ, Lachman N, Walther T, et al. Autopsy findings in patients on postcardiotomy extracorporeal membrane oxygenation (ECMO). Int J Artif Organs 2006;29:1121–31.