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Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke
Journal of Clinical Neuroscience xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Report
Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke Kelly L. Bertram a,b,⇑, Anoop Madan c, Judith Frayne a,b a
Stroke Unit, Level 4, Central Block, Alfred Hospital, Commercial Road, Melbourne, VIC 3004, Australia Faculty of Medicine Nursing and Health Sciences, Monash University, Melbourne, VIC, Australia c Department of Radiology, Alfred Hospital, Melbourne, VIC, Australia b
a r t i c l e
i n f o
Article history: Received 21 September 2015 Accepted 2 January 2016 Available online xxxx Keywords: Pulmonary arteriovenous malformation Stroke Young onset stroke
a b s t r a c t Young onset stroke is uncommon, and may be due to conditions other than traditional vascular risk factors. A 42-year-old woman with an ischaemic stroke was found to have left atrial bubble study positivity on transthoracic echocardiogram (TTE) suggestive of patent foramen ovale, however she also had low peripheral oxygen saturation. Investigation revealed an isolated pulmonary arteriovenous malformation (PAVM), visible on admission chest radiograph. This can cause embolic stroke and is an alternate cause of the TTE findings. The PAVM was able to be closed via endovascular intervention, removing the shunt and therefore removing her risk of recurrent stroke events. This is a rare cause of embolic stroke in young people which can be easily missed on investigation yet is amenable to treatment. Ó 2016 Elsevier Ltd. All rights reserved.
1. Case report A 42-year-old woman presented with left sided face, arm and leg weakness which resolved in 2 hours. She had previously experienced two brief episodes of altered facial sensation on the right. She had no known medical problems, took no medications or illicit substances, drank minimal alcohol and was a nonsmoker who exercised regularly. On examination a subtle left upper motor neurone facial weakness was present, otherwise she was neurologically intact. MRI of the brain showed diffusion weighted imaging changes on the right consistent with ischaemic stroke. Workup included negative vasculitic and thrombophilia screen, and normal carotid Dopplers. Transthoracic echocardiogram showed normal cardiac size and function, but Doppler was reported to show evidence of a small patent foramen ovale (PFO). She was not hypertensive. Her peripheral oxygen saturations were noted to be lower than normal, 92% on room air. Arterial blood gas on room air showed a pH of 7.48 (normal 7.38–7.45), pCO2 of 30 mmHg (normal 35–45) and pO2 of 58 mmHg (normal 75–99). Plain chest radiograph showed prominence of hilar vessels and an abnormality in the right mid-lung field (Fig. 1). Chest CT scan showed an extensive arteriovenous malformation (AVM) (Fig. 2). Pulmonary embolisation was undertaken with femoral vein puncture and catheterisation of the right pulmonary artery. The feeding artery of the AVM was 7 mm in diameter with two draining veins. ⇑ Corresponding author. Tel.: +61 3 9076 5493; fax: +61 3 9076 2671. E-mail address: [email protected] (K.L. Bertram).
Multiple coils (Detach 18 and Nester fibered coils, Cook Inc., Bloomington, IN, USA) were deployed through a microcatheter leading to total occlusion (Fig. 3). Following endovascular intervention her PaO2 returned to the normal range and her exercise tolerance improved. Post-procedure transthoracic echocardiogram saline contrast study showed delayed entry into the left atrium suggesting small residual fistula not a PFO. Cardioembolic stroke accounts for one-third of young onset stroke [1]. In addition to traditional vascular risk factors, other rare aetiologies producing stroke in patients under the age of 50 years include vasculitis, prothrombotic states, illicit drug use, arterial dissection and reversible cerebral vasoconstriction syndrome [2]. Paradoxical emboli are rare, and can be due to congenital cardiac abnormalities, patent ductus arteriosus, and pulmonary arteriovenous malformation (PAVM). PFO are common in the general population and their role in producing ischaemic stroke is unclear. Given recent studies show no reduction in future events with closure of PFO [3], current guidelines from the American Heart Association and American Stroke Association do not recommend routine closure [4]. AVM occur in 15% of people with hereditary haemorrhagic telangiectasia (Rendu–Osler–Weber syndrome), an autosomal dominantly inherited disease, and multiple AVM are often seen. Isolated PAVM are rare, often presenting with symptoms of hypoxia, including platypnoea, orthodeoxia or dyspnoea; or complications of the right-left shunt including stroke or transient ischaemic attacks; or brain abscess [5]. Up to 30% of PAVM can be associated with neurological complications.
http://dx.doi.org/10.1016/j.jocn.2016.01.006 0967-5868/Ó 2016 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Bertram KL et al. Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.01.006
2
Case Report / Journal of Clinical Neuroscience xxx (2016) xxx–xxx
Fig. 1. (Left) Anteroposterior and (right) lateral chest radiographs showing prominence of the hilar vessels and an abnormality in the right mid-lung field.
Fig. 2. (Left) Anteroposterior and (right) axial chest CT scan showing an arteriovenous malformation in the right lung field, corresponding to the abnormality seen on the radiographs in Figure 1.
Fig. 3. Fluoroscopy showing the arteriovenous malformation (left) before and (right) after coil embolization.
Please cite this article in press as: Bertram KL et al. Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.01.006
Case Report / Journal of Clinical Neuroscience xxx (2016) xxx–xxx
Isolated PAVM is a rarely documented cause of paradoxical ischaemic stroke [6]. A number of these patients have been shown to have both PAVM and PFO. Both produce left atrial air bubbles on transthoracic echocardiogram, often leading to the recommendation for closure of the PFO with the assumption it is the causative pathology, whilst the PAVM has initially been missed potentially resulting in repeat stroke [7]. Thrombus may form within the PAVM or elsewhere in the venous system, including deep venous thrombosis in the legs. The presence of PAVM in this circumstance allows passage of the thrombus from the venous to the arterial circulation, resulting in stroke or other arterial occlusion. Doppler ultrasound of the legs should be performed to determine the immediate risk of repeat event or other complication of deep venous thrombosis such as pulmonary embolism or post-thrombotic syndrome. PAVM, although rare, is a treatable cause of ischaemic stroke in the young which may otherwise be asymptomatic and therefore requires diagnostic consideration by the clinician.
3
References [1] Li Y, Margraf J, Kluck B, et al. Thrombolytic therapy for ischemic stroke secondary to paradoxical embolism in pregnancy: a case report and literature review. Neurologist 2012;18:44–8. [2] Maaijwee NA, Rutten-Jacobs LC, Schaapsmeerders P, et al. Ischaemic stroke in young adults: risk factors and long-term consequences. Nat Rev Neurol 2014;10:315–25. [3] Udell JA, Opotowsky AR, Khairy P, et al. Patent foramen ovale closure vs medical therapy for stroke prevention: meta-analysis of randomized trials and review of heterogeneity in meta-analyses. Can J Cardiol 2014;30:1216–24. [4] Kernan WN, Ovbiagele B, Black HR, et al. Guidelines for the prevention of stroke in patients with stroke and transient ischemic attack: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2014;45:2160–236. [5] Meek ME, Meek JC, Beheshti MV. Management of pulmonary arteriovenous malformations. Semin Intervent Radiol 2011;28:24–31. [6] Kimura K, Minematsu K, Nakajima M. Isolated pulmonary arteriovenous fistula without Rendu–Osler–Weber disease as a cause of cryptogenic stroke. J Neurol Neurosurg Psychiatry 2004;75:311–3. [7] Chessa M, Drago M, Krantunkov P, et al. Differential diagnosis between patent foramen ovale and pulmonary arteriovenous fistula in two patients with previous cryptogenic stroke caused by presumed paradoxical embolism. J Am Soc Echocardiogr 2002;15:845–6.
Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
Please cite this article in press as: Bertram KL et al. Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.01.006
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Report
Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke Kelly L. Bertram a,b,⇑, Anoop Madan c, Judith Frayne a,b a
Stroke Unit, Level 4, Central Block, Alfred Hospital, Commercial Road, Melbourne, VIC 3004, Australia Faculty of Medicine Nursing and Health Sciences, Monash University, Melbourne, VIC, Australia c Department of Radiology, Alfred Hospital, Melbourne, VIC, Australia b
a r t i c l e
i n f o
Article history: Received 21 September 2015 Accepted 2 January 2016 Available online xxxx Keywords: Pulmonary arteriovenous malformation Stroke Young onset stroke
a b s t r a c t Young onset stroke is uncommon, and may be due to conditions other than traditional vascular risk factors. A 42-year-old woman with an ischaemic stroke was found to have left atrial bubble study positivity on transthoracic echocardiogram (TTE) suggestive of patent foramen ovale, however she also had low peripheral oxygen saturation. Investigation revealed an isolated pulmonary arteriovenous malformation (PAVM), visible on admission chest radiograph. This can cause embolic stroke and is an alternate cause of the TTE findings. The PAVM was able to be closed via endovascular intervention, removing the shunt and therefore removing her risk of recurrent stroke events. This is a rare cause of embolic stroke in young people which can be easily missed on investigation yet is amenable to treatment. Ó 2016 Elsevier Ltd. All rights reserved.
1. Case report A 42-year-old woman presented with left sided face, arm and leg weakness which resolved in 2 hours. She had previously experienced two brief episodes of altered facial sensation on the right. She had no known medical problems, took no medications or illicit substances, drank minimal alcohol and was a nonsmoker who exercised regularly. On examination a subtle left upper motor neurone facial weakness was present, otherwise she was neurologically intact. MRI of the brain showed diffusion weighted imaging changes on the right consistent with ischaemic stroke. Workup included negative vasculitic and thrombophilia screen, and normal carotid Dopplers. Transthoracic echocardiogram showed normal cardiac size and function, but Doppler was reported to show evidence of a small patent foramen ovale (PFO). She was not hypertensive. Her peripheral oxygen saturations were noted to be lower than normal, 92% on room air. Arterial blood gas on room air showed a pH of 7.48 (normal 7.38–7.45), pCO2 of 30 mmHg (normal 35–45) and pO2 of 58 mmHg (normal 75–99). Plain chest radiograph showed prominence of hilar vessels and an abnormality in the right mid-lung field (Fig. 1). Chest CT scan showed an extensive arteriovenous malformation (AVM) (Fig. 2). Pulmonary embolisation was undertaken with femoral vein puncture and catheterisation of the right pulmonary artery. The feeding artery of the AVM was 7 mm in diameter with two draining veins. ⇑ Corresponding author. Tel.: +61 3 9076 5493; fax: +61 3 9076 2671. E-mail address: [email protected] (K.L. Bertram).
Multiple coils (Detach 18 and Nester fibered coils, Cook Inc., Bloomington, IN, USA) were deployed through a microcatheter leading to total occlusion (Fig. 3). Following endovascular intervention her PaO2 returned to the normal range and her exercise tolerance improved. Post-procedure transthoracic echocardiogram saline contrast study showed delayed entry into the left atrium suggesting small residual fistula not a PFO. Cardioembolic stroke accounts for one-third of young onset stroke [1]. In addition to traditional vascular risk factors, other rare aetiologies producing stroke in patients under the age of 50 years include vasculitis, prothrombotic states, illicit drug use, arterial dissection and reversible cerebral vasoconstriction syndrome [2]. Paradoxical emboli are rare, and can be due to congenital cardiac abnormalities, patent ductus arteriosus, and pulmonary arteriovenous malformation (PAVM). PFO are common in the general population and their role in producing ischaemic stroke is unclear. Given recent studies show no reduction in future events with closure of PFO [3], current guidelines from the American Heart Association and American Stroke Association do not recommend routine closure [4]. AVM occur in 15% of people with hereditary haemorrhagic telangiectasia (Rendu–Osler–Weber syndrome), an autosomal dominantly inherited disease, and multiple AVM are often seen. Isolated PAVM are rare, often presenting with symptoms of hypoxia, including platypnoea, orthodeoxia or dyspnoea; or complications of the right-left shunt including stroke or transient ischaemic attacks; or brain abscess [5]. Up to 30% of PAVM can be associated with neurological complications.
http://dx.doi.org/10.1016/j.jocn.2016.01.006 0967-5868/Ó 2016 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Bertram KL et al. Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.01.006
2
Case Report / Journal of Clinical Neuroscience xxx (2016) xxx–xxx
Fig. 1. (Left) Anteroposterior and (right) lateral chest radiographs showing prominence of the hilar vessels and an abnormality in the right mid-lung field.
Fig. 2. (Left) Anteroposterior and (right) axial chest CT scan showing an arteriovenous malformation in the right lung field, corresponding to the abnormality seen on the radiographs in Figure 1.
Fig. 3. Fluoroscopy showing the arteriovenous malformation (left) before and (right) after coil embolization.
Please cite this article in press as: Bertram KL et al. Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.01.006
Case Report / Journal of Clinical Neuroscience xxx (2016) xxx–xxx
Isolated PAVM is a rarely documented cause of paradoxical ischaemic stroke [6]. A number of these patients have been shown to have both PAVM and PFO. Both produce left atrial air bubbles on transthoracic echocardiogram, often leading to the recommendation for closure of the PFO with the assumption it is the causative pathology, whilst the PAVM has initially been missed potentially resulting in repeat stroke [7]. Thrombus may form within the PAVM or elsewhere in the venous system, including deep venous thrombosis in the legs. The presence of PAVM in this circumstance allows passage of the thrombus from the venous to the arterial circulation, resulting in stroke or other arterial occlusion. Doppler ultrasound of the legs should be performed to determine the immediate risk of repeat event or other complication of deep venous thrombosis such as pulmonary embolism or post-thrombotic syndrome. PAVM, although rare, is a treatable cause of ischaemic stroke in the young which may otherwise be asymptomatic and therefore requires diagnostic consideration by the clinician.
3
References [1] Li Y, Margraf J, Kluck B, et al. Thrombolytic therapy for ischemic stroke secondary to paradoxical embolism in pregnancy: a case report and literature review. Neurologist 2012;18:44–8. [2] Maaijwee NA, Rutten-Jacobs LC, Schaapsmeerders P, et al. Ischaemic stroke in young adults: risk factors and long-term consequences. Nat Rev Neurol 2014;10:315–25. [3] Udell JA, Opotowsky AR, Khairy P, et al. Patent foramen ovale closure vs medical therapy for stroke prevention: meta-analysis of randomized trials and review of heterogeneity in meta-analyses. Can J Cardiol 2014;30:1216–24. [4] Kernan WN, Ovbiagele B, Black HR, et al. Guidelines for the prevention of stroke in patients with stroke and transient ischemic attack: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2014;45:2160–236. [5] Meek ME, Meek JC, Beheshti MV. Management of pulmonary arteriovenous malformations. Semin Intervent Radiol 2011;28:24–31. [6] Kimura K, Minematsu K, Nakajima M. Isolated pulmonary arteriovenous fistula without Rendu–Osler–Weber disease as a cause of cryptogenic stroke. J Neurol Neurosurg Psychiatry 2004;75:311–3. [7] Chessa M, Drago M, Krantunkov P, et al. Differential diagnosis between patent foramen ovale and pulmonary arteriovenous fistula in two patients with previous cryptogenic stroke caused by presumed paradoxical embolism. J Am Soc Echocardiogr 2002;15:845–6.
Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
Please cite this article in press as: Bertram KL et al. Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.01.006