Summary
A 46–year old woman experienced an episode of arterial desaturation despite administration of 100% oxygen during anesthetization for an elective laparoscopy. Further evaluation revealed a giant pulmonary arteriovenous malformation (PAVM) with right–left shunt associated with previously undiagnosed hereditary hemorrhagic telangiectasia (HHT, Morbus Osler– Weber–Rendu). The PAVM was treated interventionally with an Amplatzer® duct occluder. Transcatheter embolization of the PAVM was well tolerated with symptomatic and hemodynamic improvement. CT scan after six months demonstrated correct position of the duct occluder in the left pulmonary artery with nearly complete occlusion of the feeding vessel.
PAVMs are rare direct communications between pulmonary arteries and veins, associated with HHT in the majority of cases and often presenting with dyspnea or major neurological complications due to paradoxic embolism. In this case report, we present a rational and stepwise diagnostic workup for this rare medical condition and show that transcatheter embolization is an appropriate treatment for larger malformations.
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McAllister KA, Grogg KM, Johnson DW et al (1994) Endoglin, a TGF–β binding protein of endothelial cells, is the gene for hereditary hemorrhagic telangiectasia type 1. Nature Genet 8:345–351
Johnson DW, Berg JN, Baldwin MA et al (1996) Mutation in the activin receptor– like kinase 1 gen in hereditary hemorrhagic telangiectasia type 2. Nature Genet 13:189–195
Shovlin CL, Letarte M (1999) Hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations: issues in clinical management and review of pathogenic mechanisms. Thorax 54:714–729
Gossage J, Kanj G (1998) Pulmonary arteriovenous malformations: a state of the art review. Am J Resp Crit Care Med 158:643–661
Bourdeau A, Dumont DJ, Letarte M (1999) A murine model of hereditary hemorrhagic telangiectasis. J Clin Invest 104:1343–1351
Moussouttas M, Fayad, Rosenblatt MM, Hashimoto M, Pollak J, Henderson K, Ma TYZ, White RI (2000) Pulmonary arteriovenous malformations: Cerebral ischemia and neurologic manifestations. Neurology 55:959–963
Kjeldsen AD, Oxhoj H, Andersen E, Green A, Vase P (2000) Prevalence of pulmonary arteriovenous malformations (PAVMs) and occurrence of neurological symptoms in patients with hereditary hemorrhagic telangiectasia (HHT). Journal of Internal Medicine 248:255–262
Gallitelli M, Guastamacchia E, Resta F, Guanti G, Sabba C (2005) Pulmonary arteriovenous malformations, heriditary hemorrhagic telangiectasie, and brain abscess. Respiration 21 [Epub ahead of print] 314:1–5
Lee DW, White RI, Egglin TK et al (1997) Embolotherapy of large pulmonary arteriovenous malformations: long term results. Ann Thorac Surg 64:930–940
Esplin MS, Varner MW (1997) Progression of pulmonary arteriovenous malformation during pregnancy. Obstet Gynecol Surv. 52:248–253
Hughes JMB, Allison DJ (1990) Pulmonary arteriovenous malformations: the radiologist replaces the surgeon. Clin Radiol 41:297–298
Gorenflo M, Bettendorf M, Ulmer HE, Brockmeier K (2000) Oxygenmediated pulmonary vasodilation and plasma levels of endothelin–1, atrial natriuretic peptide and cyclic GMP in patients with left–to–right shunt and pulmonary hypertension. Z Kardiol 89:100–108
Kjeldsen AD, Oxhoj H, Andersen PE, Elle B, Jacobsen JP, Vase P (2000) Pulmonary arteriovenous malformations: screening procedures and pulmonary angiography in patients with hereditary hemorrhagic telangiectasia. Chest 118:566–567
Chaing ST (1968) Anomogram for venous shunt (Qs–Qt) calculation. Thorax 23:563–565
Nanthakumar K, Graham AT, Robinson TI, Grande P, Pugash RA, Clarke JA, Hutchison SJ, Mandzia JL, Hyland RH, Faughnan ME (2001) Contrast echocardiography for detection of pulmonary arteriovenous malformations. Am Heart J 141:243–246
White RL, Lynch–Nyhan A, Terry P et al (1988) Pulmonary arteriovenous malformations: techniques and long term outcome of embolotherapy. Radiology 160:663–669
Swanson KL, Prakash UBS, Stanson AW (1999) Pulmonary arteriovenous fistulas: Clinic Experience, 1982– 1997. Mayo Clin Proc 74:671–680
Gupta P, Mordin C, Curtis J, Hughes JMB, Shovlin CL, Jackson JE (2002) Pulmonary arteriovenous malformations: Effect of embolization on rightto– left–shunt, hypoxemia and exercise tolerance in 66 patients. Am J Roentgenol 179:347–355
Khurshid I, Downie GH (2002) Pulmonary arteriovenous malformation. Postgrad Med J 78:191–197
Marshall JK, Hunt RH (1997) Hormonal therapy for bleeding gastrointestinal mucosal vascular abnormalities: a promising alternative Eur J Gastroenterol Hepatol 9:521–525
Undas A, Bazan–Socha S, Swadzab J, Musial J (2002) Hereditary hemorrhagic telangiectasia, factor V Leiden and antiphospholipid syndrome: a case report. Blood Coagul Fibrinolysis 13:53–56
Guttmacher AE, Marchuk DA, White RI (1995) Hereditary hemorrhagic telangiectasia. N Engl J Med 333:918– 924
Haitjema T, Disch F, Overtoom TTc et al (1995) Screening family members of patient with hereditary hemorrhagic telangiectasia. Am J Med 99:519–524
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Hinterseer, M., Becker, A., Barth, A.S. et al. Interventional embolization of a giant pulmonary arteriovenous malformation with right–left–shunt associated with hereditary hemorrhagic telangiectasia. Clin Res Cardiol 95, 174–178 (2006). https://doi.org/10.1007/s00392-006-0345-3
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DOI: https://doi.org/10.1007/s00392-006-0345-3