Intern Med. 2013;52(10):1091-4.

Hereditary hemorrhagic telangiectasia with pulmonary arteriovenous malformations and embolic strokes treated successfully with video-assisted thoracoscopic resection.

Akiyama S, Hanada S, Uruga H, Takaya H, Miyamoto A, Morokawa N, Kurosaki A, Fujii T, Kohno T, Kishi K.

Department of Respiratory Medicine, Respiratory Center, Toranomon Hospital, Japan.

 

Abstract

A 67-year-old hypoxic woman was admitted following two episodes of cerebral infarction. Based on the clinical presentation and radiological findings, a diagnosis of hereditary hemorrhagic telangiectasia was made and the cerebral ischemic complications were considered to have been caused by paradoxical embolizations related to pulmonary arteriovenous malformations (PAVMs). We performed video-assisted thoracoscopic surgery (VATS) and identified turbulent thrombi in one of the PAVMs that were capable of circulating systemically and inducing embolic strokes. The condition of the patient improved immediately following VATS. This case suggests that VATS may be a preferred therapeutic option in the treatment of patients with PAVM accompanying recurrent episodes of life-threatening complications such as strokes.

PMID: 23676596

 

Additional description:

Paradoxical embolism due to pulmonary arteriovenous malformations (PAVMs) is the main cause of cerebral infarction in patients with hereditary hemorrhagic telangiectasia (HHT). Transcatheter embolization has been recognized to be effective and to have a good safety profile, with rare PAVM-related complications during long-term follow-up. International guidelines recommend that clinicians treat PAVMs with transcatheter embolization as a first-line therapy. Although surgery, including pneumonectomy, lobectomy, segmentectomy, wedge resection, or vascular ligation, was the preferred treatment till the seventies, experts nowadays agree there is no role for surgical management of PAVMs, other than in the management of life-threatening bleeding.

In this case, recurrent episodes of strokes implied that the PAVMs could contain some thrombi, which were pathologically observed in one of the PAVMs. These thrombi were capable of circulating systemically and inducing cerebral infarctions. As shown in the supplemental figure, the aim of transcatheter embolization is to occlude all the PAVM feeding arteries (afferent vessels) by performing selective catheterization of pulmonary arteries. However, transcatheter embolization alone would not be sufficient for treatment of PAVMs with recurrent embolic stroke events. We needed to manage not only the afferent but also the efferent vessels of PAVMs to eliminate the risk of ischemic episodes. Therefore, we selected video-assisted thoracoscopic surgery (VATS) (which is less invasive than thoracotomy) as the treatment to remove both the PAVM vessels.

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Figure 1. The thrombus in the pulmonary arteriovenous malformations (PAVM) may be a cause of paradoxical embolization, causing cerebral infarctions. The risk of recurrent strokes persists in the patients treated with transcatheter embolization unless we manage the efferent vessels. In contrast, video-assisted thoracoscopic surgery (VATS) can remove not only the afferent but also the efferent vessels of PAVMs which lead to prevent circulation of the thrombus.

PAVM = pulmonary arteriovenous malformations

VATS = video-assisted thoracoscopic surgery

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