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Cryptogenic Stroke Caused by Pulmonary Arterial Venous Malformation with Massive Right-to-Left Shunt: A Case Report
Jie Zhan1,2, Cong Dong2, Mei Li2
1Postdoctoral research station, Guangdong Provincial Hospital of Chinese Medicine, The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, 510120, China.
Background:
Pulmonary arterial venous malformation (PAVM) is an abnormal vascular malformation between pulmonary arteries and veins characterized by varying degrees of right-to-left shunts (RLS). Cryptogenic stroke (CS) due to paradoxical embolism (PE) caused by PAVM is relatively rare in the clinic.
Case Presentation:
We report the case of a 54-year-old right-handed woman who presented with sudden-onset left-sided limb weakness for 2 h. A physical examination revealed normal vital signs but weakness in her left upper and lower limbs, graded as 1/5 using the Medical Research Council scale. Her National Institutes of Health Stroke Scale (NIHSS) score was 8, and her modified Rankin scale (mRS) was 4. Brain diffusion-weighted imaging showed acute infarction in the right basal ganglia and the radiation crown but brain magnetic resonance angiography found no obvious abnormality. A transcranial Doppler ultrasound with bubble study (TCD-b) found the rain curtain sign of microbubbles in the left middle cerebral artery, reflecting significant RLS. Transesophageal echocardiography (TEE) and transthoracic echocardiography (TTE) were conducted to distinguish between intra- and extracardiac shunts. A pulmonary computerized tomography angiogram (CTA) demonstrated a PAVM. We considered the patient had CS due to PE caused by PAVM. Thrombolytic therapy within the time window was performed. Then, transcatheter device occlusion of the arteriovenous fistula was successfully undertaken, and the patient carried on with rehabilitation training. At a 15-month follow-up, there were no catheter-related complications or recurrent stroke, and her NIHSS and mRS scores were both 0.
Conclusions:
PAVM is an important risk factor for PE and CS and should not be ignored as a possible etiology in stroke patients without any other risk factors. CTA of the pulmonary artery is the recommended gold standard for diagnosing and locating a PAVM. Thrombolytic therapy within the time window combined with transcatheter device occlusion of arteriovenous malformation and rehabilitation training may benefit the recovery of patients with CS caused by PE resulting from PAVM.
Insights
Pulmonary arterial venous malformation (PAVM) can cause cryptogenic stroke (CS) through paradoxical embolism (PE). Early diagnosis via pulmonary CTA and prompt treatment including thrombolysis and device occlusion can lead to full recovery.
Area of Science:
- Cardiology
- Neurology
- Radiology
Background:
- Pulmonary arterial venous malformation (PAVM) involves abnormal connections between pulmonary arteries and veins, potentially causing right-to-left shunts (RLS).
- Cryptogenic stroke (CS) resulting from paradoxical embolism (PE) due to PAVM is a rare but significant clinical occurrence.
Purpose of the Study:
- To present a case of CS attributed to PE from a PAVM.
- To highlight the diagnostic utility of pulmonary computed tomography angiography (CTA) for PAVM.
- To demonstrate the efficacy of combined therapeutic interventions for this condition.
Main Methods:
- A 54-year-old woman with acute stroke symptoms underwent comprehensive neuroimaging and vascular assessments.
- Diagnostic tools included brain MRI, transcranial Doppler with bubble study (TCD-b), echocardiography (TEE/TTE), and pulmonary CTA.
- Treatment involved timely thrombolytic therapy, transcatheter device occlusion of the PAVM, and rehabilitation.
Main Results:
- The patient presented with acute ischemic stroke, with TCD-b indicating significant RLS and pulmonary CTA confirming PAVM.
- Successful transcatheter device occlusion of the PAVM was performed after thrombolysis.
- At 15-month follow-up, the patient showed complete neurological recovery (NIHSS and mRS scores of 0) with no complications.
Conclusions:
- PAVM is a critical, often overlooked, risk factor for PE and CS in patients lacking other stroke risk factors.
- Pulmonary artery CTA is the gold standard for diagnosing and localizing PAVM.
- A multimodal treatment approach combining thrombolysis, transcatheter occlusion, and rehabilitation offers significant benefits for patients with CS secondary to PAVM.
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