Management of Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia Patients

Conclusion

Strategies for PAVM screening, characterization, and treatment have evolved significantly in recent years due to an enhanced understanding of the molecular and clinical determinants of PAVM presentation and behavior. Patients with HHT should be evaluated at least once in Centers of Excellence for HHT (HHT.org) for thorough screening and comprehensive HHT-related care; however, many HHT-related PAVMs can be treated at community hospitals thereafter. Clinical interventional radiologists play a pivotal role in caring for HHT patients and their families, and are well served by remaining up to date with the most current recommendations arising from the cumulative experience of physicians caring for this patient population.

• References

• 1 Cottin V, Plauchu H, Bayle J-Y, Barthelet M, Revel D, Cordier J-F. Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. Am J Respir Crit Care Med 2004; 169 (9) 994-1000
• 2 van Gent MWF, Post MC, Snijder RJ, Westermann CJJ, Plokker HWM, Mager JJ. Real prevalence of pulmonary right-to-left shunt according to genotype in patients with hereditary hemorrhagic telangiectasia: a transthoracic contrast echocardiography study. Chest 2010; 138 (4) 833-839
• 3 Faughnan ME, Palda VA, Garcia-Tsao G , et al; HHT Foundation International—Guidelines Working Group. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet 2011; 48 (2) 73-87
• 4 Gossage JR, Kanj G. Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med 1998; 158 (2) 643-661
• 5 Faughnan ME, Thabet A, Mei-Zahav M , et al. Pulmonary arteriovenous malformations in children: outcomes of transcatheter embolotherapy. J Pediatr 2004; 145 (6) 826-831
• 6 Moussouttas M, Fayad P, Rosenblatt M , et al. Pulmonary arteriovenous malformations: cerebral ischemia and neurologic manifestations. Neurology 2000; 55 (7) 959-964
• 7 Shovlin CL, Jackson JE, Bamford KB , et al. Primary determinants of ischaemic stroke/brain abscess risks are independent of severity of pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia. Thorax 2008; 63 (3) 259-266
• 8 Brillet P-Y, Dumont P, Bouaziz N , et al. Pulmonary arteriovenous malformation treated with embolotherapy: systemic collateral supply at multidetector CT angiography after 2-20-year follow-up. Radiology 2007; 242 (1) 267-276
• 9 Trerotola SO, Pyeritz RE, Bernhardt BA. Outpatient single-session pulmonary arteriovenous malformation embolization. J Vasc Interv Radiol 2009; 20 (10) 1287-1291
• 10 Velling TE, Brennan FJ, Hall LD. Pulmonary angiography with use of the 5-F omniflush catheter: a safe and efficient procedure with a common catheter. J Vasc Interv Radiol 2000; 11 (8) 1005-1008
• 11 White Jr RI. Pulmonary arteriovenous malformations: how do I embolize?. Tech Vasc Interv Radiol 2007; 10 (4) 283-290
• 12 Milic A, Chan RP, Cohen JH, Faughnan ME. Reperfusion of pulmonary arteriovenous malformations after embolotherapy. J Vasc Interv Radiol 2005; 16 (12) 1675-1683
• 13 Trerotola SO, Pyeritz RE. Does use of coils in addition to Amplatzer vascular plugs prevent recanalization?. AJR Am J Roentgenol 2010; 195 (3) 766-771
• 14 Donaldson JW, Hall IP, Hubbard RB, Fogarty AW, McKeever TM. Peri-Procedural Complications Associated with Transcutaneous Embolisation for Pulmonary Arteriovenous Malformations: A Systematic Review and Meta-Analysis. 10th HHT Scientific Conference, Hematology Reports 3 2013;(Suppl 1):34–35
• 15 Lee WL, Graham AF, Pugash RA , et al. Contrast echocardiography remains positive after treatment of pulmonary arteriovenous malformations. Chest 2003; 123 (2) 351-358
• 16 Hayashi S, Baba Y, Senokuchi T, Nakajo M. Efficacy of venous sac embolization for pulmonary arteriovenous malformations: comparison with feeding artery embolization. J Vasc Interv Radiol 2012; 23 (12) 1566-1577 , quiz 1581