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Talla A. Rousan, Christopher Parker, Mazen Abu-Fadel
Transcatheter Embolization of a Pulmonary Arteriovenous Malformation in a Patient with Hereditary Hemorrhagic Telangiectasia Talla A. Rousan, Christopher Parker, Mazen Abu-Fadel University of Oklahoma Health Sciences Center Oklahoma City, Oklahoma
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Outline Case presentation Investigations Management Discussion
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Case Presentation 74 year-old male with CHF, paroxysmal atrial fibrillation and COPD presented with decompensated heart failure (NYHA class III-IV).
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Case Presentation Significant physical examination findings:
O2 saturation 88% on 4L by NC. Bilateral lower extremity edema. Jugular venous distention to the ankle of the mandible. Numerous small telangiectasias on the lips and tongue.
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Investigations (CTA)
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Investigations (CTA)
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Investigations (angiography)
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Investigations - RHC A pulmonary wedge pressure catheter balloon was used for complete occlusion of the right PAVM for five minutes: The mean pulmonary artery pressure remained stable (36-40 mmHg). Mixed venous saturation climbed from 38% to 46% and his pulse oximetry increased to over 90%.
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Management The right lower pulmonary artery was selectively engaged using an internal mammary diagnostic catheter. A size 10 Vascular lug Type II (St. Jude Medical. St. Paul, Minnessota, U.S.A.) was then deployed into the neck of the AVM over a wire.
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Management
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Follow-up On a post-discharge follow-up two months later, the patient reported significant symptomatic improvement from his pre-intervention condition (NYHA II).
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Discussion PAVMs are communications between the pulmonary arterial and venous systems creating a right-to-left shunt. Most pulmonary AVMs are associated with HHT, a disease characterized by the development of arteriovenous malformations in virtually any organ system. The gold standard for diagnosis of PAVM is direct pulmonary angiography.
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Discussion Curacao criteria for HHT diagnosis:
The presence of spontaneous and recurrent epistaxis. Multiple telangiectasias present on lips, oral cavity, fingers, and nose. Presence of visceral AVMs (GI, Pulmonary, hepatic, cerebral, or spinal. One first degree relative with HHT according to these criteria. Definitive HHT: 3 or more. Possible HHT: 2. Unlikely: < 2
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Discussion - Management
Initially, the treatment for PAVM was surgical resection, first performed in 1942. Since the advent of percutaneous, catheter-based interventions, embolotherapy has become the treatment of choice for closure of PAVMs. Balloon and coil embolization are the most commonly utilized techniques with coils being more prevalent. Composite data from several reported case series indicates greater than 99% efficacy in initial occlusion for both techniques and less than 2% recanalization rates
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Discussion - Management
Amplatzer Vascular Plugs (AVP): Composed of self-expanding nitinol mesh with radio-opaque platinum and iridium markers. AVP provide direct obstruction of vascular flow with the advantages of: The ability to reposition the plug prior to final release. More distal occlusion which spares normal tissue. Better rates of single device occlusion. Predicting the required size of the device needed is of less concern.
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(St. Jude Medical. St. Paul, Minnessota, U.S.A.)
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Discussion - Management
The current guidelines recommend treating all AVMs with a feeding artery greater than 3 mm. Symptomatic individuals should be considered for embolotherapy (regardless of AVM size). For all patients with PAVMs, treated or untreated, the guidelines recommend frequent follow-up and antibiotic prophylaxis when a procedure risks bacteremia.
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References Trerotola SO, Pyeritz RE. PAVM embolization: an update. AJR Am J Roentgenol 195: Zukotynski K, Chan RP, Chow CM, Cohen JH, Faughnan ME. Contrast echocardiography grading predicts pulmonary arteriovenous malformations on CT. Chest 2007; 132:18-23 White RI, Jr., Pollak JS, Wirth JA. Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy. J Vasc Interv Radiol 1996; 7: Hsu CC, Kwan GN, Thompson SA, van Driel ML. Embolisation therapy for pulmonary arteriovenous malformations. Cochrane Database Syst Rev:CD008017 Hundt W, Kalinowski M, Kiessling A, Heverhagen JT, Eivazi B, Werner J, et al. Novel approach to complex pulmonary arteriovenous malformation embolization using detachable coils and Amplatzer vascular plugs. Eur J Radiol Tapping CR, Ettles DF, Robinson GJ. Long-term follow-up of treatment of pulmonary arteriovenous malformations with AMPLATZER Vascular Plug and AMPLATZER Vascular Plug II devices. J Vasc Interv Radiol 22: Hart JL, Aldin Z, Braude P, Shovlin CL, Jackson J. Embolization of pulmonary arteriovenous malformations using the Amplatzer vascular plug: successful treatment of 69 consecutive patients. Eur Radiol 20:
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