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Table of Contents    
Year : 2017  |  Volume : 65  |  Issue : 4  |  Page : 891-892

An unusual combination of patent foramen ovale with pulmonary arteriovenous malformation in a young patient with stroke

1 Department of Neurology, Institute of Neurosciences, Indraprastha Apollo Hospitals, New Delhi, India
2 Department of Internal Medicine, Institute of Neurosciences, Indraprastha Apollo Hospitals, New Delhi, India

Date of Web Publication5-Jul-2017

Correspondence Address:
Pushpendra N Renjen
Institute of Neurosciences, Indraprastha Apollo Hospitals, New Delhi - 110 076
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/neuroindia.NI_817_16

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How to cite this article:
Renjen PN, Chaudhari D, Meman M. An unusual combination of patent foramen ovale with pulmonary arteriovenous malformation in a young patient with stroke. Neurol India 2017;65:891-2

How to cite this URL:
Renjen PN, Chaudhari D, Meman M. An unusual combination of patent foramen ovale with pulmonary arteriovenous malformation in a young patient with stroke. Neurol India [serial online] 2017 [cited 2021 Jun 20];65:891-2. Available from:


Patent foramen ovale (PFO) is a common condition with an incidence as high as 25% in the general population.[1] In some studies, young patients who present with neurological events show a higher prevalence of PFO associated with a significant increase in recurrent ischemic events.[2],[3],[4]

Pulmonary arteriovenous malformations (PAVMs) form as a result of a direct communication between the pulmonary artery and vein via a thin-walled aneurysmal sac and are often present undetected.[5] PAVM is well recognized as a cause of paradoxical brain embolism. In a majority of cases, PAVM is associated with hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disorder that has an incidence as high as 1 in 2351 in selected population groups.[6],[7]

A 44-year old, right-handed male patient presented with sudden onset weakness of the left side of the body for a duration of 24 hours. At admission, the magnetic resonance imaging (MRI) of the brain showed a large acute infarct in the right middle cerebral artery territory with early hemorrhagic transformation. A complete work up for a hypercoagulable status did not reveal any abnormality except for elevated serum homocysteine levels (42.5 umol/L). A transthoracic echocardiographic study failed to find any source of the emboli. Further work up with a trans-esophageal echocardiography (TEE) revealed the presence of a PFO with an intermittent right-to-left shunt that could be further delineated by injecting saline contrast and by encouraging the patient to cough.

To rule out other differential diagnoses and to unequivocally ascertain PFO as the cause of stroke, we reviewed the patient's history in detail, which revealed that he had a history of hemoptysis 5 years back, the cause of which was undetermined. Therefore, we performed a computed tomography of the chest, which revealed the presence of a pulmonary arteriovenous fistula (PAVF) in the right upper lobe of the pulmonary artery, with a 4-mm defect. We then performed a pulmonary angiography, which further delineated the isolated PAVF in the right pulmonary artery, with a direct connection to the left atrium via the right superior pulmonary vein [Figure 1] and [Figure 2] He did not have any clinical manifestations suggestive of a HHT. Successful embolization of the PAVF was done using a 12 × 40 PDA (patent ductus arteriosus) occlusion device [Figure 3]. Subsequently, no recurrence of stroke was noted in the follow-up period.
Figure 1: Chest CT Pulmonary Angiography: An isolated right PAVF in the right pulmonary artery, with a direct connection to the left atrium via right superior pulmonary vein

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Figure 2: Chest CT Pulmonary Angiography: The isolated right PAVF in the right pulmonary artery, with a direct connection to the left atrium via right superior pulmonary vein is again visualized

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Figure 3: Chest CT Pulmonary Angiography (Post PAVF Repair): Successful embolisation of PAVF using 12*40 PDA Occlusion Device

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Percutaneous closure of a PFO is a procedure that is gaining popularity in the adult population with cardiological ailments. In selected patients, it is an attractive alternative to surgery for elimination of significant arteriovenous shunts and in the reduction of the consequential neurologic events due to the precipitation of paradoxical emboli.[8] Although case series consistently show very low rates of stroke recurrence after the device placement and unequivocally suggest a benefit in primary closure of the PFO rather than persisting with medical therapy,[9] two studies—RESPECT [10] (Randomized evaluation of recurrent stroke comparing PFO closure to establish the current standard-of-care treatment) and the PC (percutaneous closure of patent foramen ovale in cryptogenic embolism) Trial [11]—failed to show that percutaneous closure of PFO significantly reduces the risk of recurrent stroke.

Paradoxically, PAVMs and PFO share similar mechanisms in the development of brain infarction through a right-to-left shunt. The shunt flows through the PAVMs continuously whereas it can pass though PFO only intermittently at the time of right atrial pressure increase. In this context, PAVM is regarded as having a higher risk than PFO for being the source of of paradoxical embolism. The size of right-to-left shunt may not be a critical factor in determining stroke occurrence.[12] Moussouttas et al.,[13] documented that brain infarction most likely occurs in PAVM patients who have the presence of feeding arteries more than 3 mm in diameter, and not in those where these arteries are of a smaller size. Accordingly, they recommended embolization therapy for all PAVMs in which the diameter of the feeding artery exceeds 3 mm.

In the literature, only one case report has been published of PFO and occult PAVM, where the authors detected a PAVM of 4.8 mm after surgical closure of the PFO, and embolization of PAVM resulted in a good outcome.[8] To our knowledge, this is the second published case of a similar entity, and as per the current recommendation, we did not consider surgical closure of PFO. The patient is free from neurological events since the last 6 months. We conclude that PAVM was the cause for the stroke and may have been responsible for the development of the PFO as well because functional closure of the PFO occurred in the follow-up period, once the PAVM was corrected. To our knowledge, this is the first case reporting the functional closure of PFO after surgical closure of the PAVM. Hence, the importance of ruling out other causes of stroke in patients with PFO and in eliciting a detailed clinical history is emphasized.

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There are no conflicts of interest.

  References Top

Hagen P, Scholtz D, Edwards W. Incidence and size of patent foramen ovale during the first decade of life: An autopsy study of 965 normal hearts. Mayo Clin Proc 1984;59:17-20.  Back to cited text no. 1
Overell JR, Bone I, Lees KR. Interatrial septal abnormalities and stroke: A meta-analysis of case-control studies. Neurology 2000;55:1172-9.  Back to cited text no. 2
Bogousslavsky J, Garazi S, Jeanrenaud X, Aebischer N, Van Melle G, Lausanne stroke with paradoxical embolism study group. Stroke recurrence in patients with patent foramen ovale: The Lausanne study. Neurology 1996;46:1301-5.  Back to cited text no. 3
Lechat P, Mas JL, Lascault G, Theard M, Klimczac M, Drobinski G, et al. Prevalence of patent foramen ovale in patients with stroke. N Engl J Med 1988;318:1148-52.  Back to cited text no. 4
White RI. Endovascular repair of PAVM by embolotherapy: In a patient with a transient ischemic attack or stroke, a positive contrast echocardiogram does not always mean a patent foramen ovale. [Last accessed on 2017 May 27].  Back to cited text no. 5
Khalil A, Farres MT, Mangiapan G, Tassart M, Bigot JM, Carette MF, et al. Pulmonary arteriovenous malformations. Chest 2000;117:1399-403.  Back to cited text no. 6
Guttmacher AE, McKinnon WC, Upton MD. Hereditary hemorrhagic telangiectasia: A disorder in search of the genetics community. Am J Med Genet 1994;52:252-3.  Back to cited text no. 7
Schussler JM, Phillips SD, Anwar A. Pulmonary arteriovenous fistula discovered after percutaneous patent foramen ovale closure in a 27-year-old woman. J Invasive Cardiol 2003;15:527-9.  Back to cited text no. 8
Khairy P, O'Donnell CP, Landzberg MJ. Transcatheter closure versus medical therapy of patent foramen ovale and presumed paradoxical thromboemboli: A systematic review. Ann Intern Med 2003;139:753-60.  Back to cited text no. 9
Meier B, Kalesan B, Mattle HP, Khattab AA, Hildick-Smith D, Dudek D, et al. PC Trial Investigators. Percutaneous closure of patent foramen ovale in cryptogenic embolisation. N Engl J Med 2013;368:1083-91.  Back to cited text no. 10
Carroll JD, Saver JL, Thaler DE, Smalling RW, Berry S, MacDonald LA, et al.; RESPECT Investigators. Closure of patent foramen ovale versus medical therapy after cryptogenic stroke. N Engl J Med 2013;368:1092-100.  Back to cited text no. 11
Todo K, Moriwaki H, Higashi M, Kimura K, Naritomi H. A small pulmonary arteriovenous malformation as a cause of recurrent brain embolism. AJNR Am J Neuroradiol 2004;25:428-30.  Back to cited text no. 12
Moussouttas M, Fayad P, Rosenblatt M, Hashimoto M, Pollak J, Henderson K, et al. Pulmonary arteriovenous malformations: Cerebral ischemia and neurologic manifestations. Neurology 2000;55:959-64.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3]


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