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CASE REPORT
Coexistence of pulmonary arteriovenous malformation and inferior vena cava agenesis in a patient presenting with hemoptysis
1
Pulmonary Department, Aristotle University of Thessaloniki, G.H. “G. Papanikolaou”, Thessaloniki, Greece
2
Thoracic Surgery Department, G.H. “G. Papanikolaou”, Thessaloniki, Greece
3
Radiology Department, G.H. “G. Papanikolaou”, Thessaloniki, Greece
Corresponding author
Despina Papakosta
Pulmonary Department, Aristotle University of Thessaloniki, “G. Papanikolaou” Hospital, 57010 Exohi, Thessaloniki, Greece
Pulmonary Department, Aristotle University of Thessaloniki, “G. Papanikolaou” Hospital, 57010 Exohi, Thessaloniki, Greece
Pneumon 2017;30(4):255-260
KEYWORDS
ABSTRACT
Introduction:
Pulmonary arteriovenous malformations (PAVMs) are abnormal vascular connections, mostly associated with hereditary hemorrhagic teleangiectasia (HHT), an autosomal vascular disorder. Inferior vena cava (IVC) agenesis is a rare congenital abnormality, reported to be associated with idiopathic deep venous thrombosis and pulmonary embolism in young patients. A coexistence of pulmonary arteriovenous malformation with IVC agenesis has only been described once in published literature.
Case presentation:
We present the case of a 24-year-old, Greek male with a history of recurrent episodes of hemoptysis. Evaluation confirmed the coexistence of a pulmonary arteriovenous malformation with IVC agenesis. The patient underwent a right lower lobectomy and remains asymptomatic at follow-up.
Conclusion:
PAVMs are rare causes of hemoptysis. Given the very limited epidemiologic evidence of IVC agenesis, we could not theorize that this entity presents a true association. As unanswered questions remain regarding the pathogenetic correlation of these vascular malformations, further investigation is needed on PAVMs and their pathogenesis.
Pulmonary arteriovenous malformations (PAVMs) are abnormal vascular connections, mostly associated with hereditary hemorrhagic teleangiectasia (HHT), an autosomal vascular disorder. Inferior vena cava (IVC) agenesis is a rare congenital abnormality, reported to be associated with idiopathic deep venous thrombosis and pulmonary embolism in young patients. A coexistence of pulmonary arteriovenous malformation with IVC agenesis has only been described once in published literature.
Case presentation:
We present the case of a 24-year-old, Greek male with a history of recurrent episodes of hemoptysis. Evaluation confirmed the coexistence of a pulmonary arteriovenous malformation with IVC agenesis. The patient underwent a right lower lobectomy and remains asymptomatic at follow-up.
Conclusion:
PAVMs are rare causes of hemoptysis. Given the very limited epidemiologic evidence of IVC agenesis, we could not theorize that this entity presents a true association. As unanswered questions remain regarding the pathogenetic correlation of these vascular malformations, further investigation is needed on PAVMs and their pathogenesis.
REFERENCES (19)
1.
Shovlin CL. Pulmonary arteriovenous malformations. Am J Respir Crit Care Med 2014; 190:1217–28.
2.
Nakayama M, Nawa T, Chonan T, et al. Prevalence of pulmonary arteriovenous malformations as estimated by low-dose thoracic CT screening. Intern Med 2012; 51:1677–81.
3.
Circo S, Gossage JR. Pulmonary vascular complications of hereditary haemorrhagic telangiectasia. Curr Opin Pulm Med 2014; 20:421–8.
4.
Swanson KL, Prakash UB, Stanson AW. Pulmonary arteriovenous fistulas: Mayo Clinic experience, 1982-1997. Mayo Clin Proc 1999; 74:671-80.
5.
Cartin-Ceba R, Swanson KL, Krowka MJ. Pulmonary arteriovenous malformations. Chest 2013; 144:1033-44.
6.
Abernethy J. Account of two instances of uncommon formation in the viscera of the human body. Philosophical Transactions of the Royal Society of London 1793; 83:59-66.
7.
Lambert M, Marboeuf P, Midulla M, et al. Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature. Vasc Med 2010; 15:451-9.
8.
Namisaki H, Nishigami K, Murakami M, et al. Congenital absence of inferior vena cava with azygos continuation revealed by vascular echo in a patient with pulmonary thromboembolism and deep vein thrombosis: a case report. Ann Vasc Dis 2013; 6:195-7.
9.
Ruggeri M, Tosetto A, Castaman G, et al. Congenital absence of the inferior vena cava: a rare risk factor for idiopathic deep-vein thrombosis. Lancet 2001; 357:441.
10.
D’Aloia A, Faggiano P, Fiorina C, et al. Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism. Int J Cardiol 2003; 88:327-9.
11.
Chee YL, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young. Br J Haematol 2001; 114:878-80.
12.
Hawass ND, Kolawole TM, Badawi MG. Concomitant pulmonary arteriovenous and inferior vena cava malformations. A case report. Eur J Radiol 1988; 8:1025.
13.
Santhirapala V. Orthodeoxia and postural orthostatic tachycardia in patients with pulmonary arteriovenous malformations: a prospective 8-year series. Thorax 2014; 69:1046-7.
14.
Shovlin CL, Guttmacher AE, Buscarini E, et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 2000; 91:66-7.
15.
Mcinnes RR. Developmental Biology: Frontiers for Clinical Genetics Vascular malformations: localized defects in vascular morphogenesis. J Cell Physiol 2003:340-51.
16.
Gill SS, Roddie ME, Shovlin CL, Jackson JE. Pulmonary arteriovenous malformations and their mimics. Clin Radiol 2015; 70:96-110.
17.
Reslan OM, Raffetto JD, Addis M, Sundick S. Congenital Absence of Inferior Vena Cava in a Young Patient with Iliofemoral Deep Venous Thrombosis Treated with Ultrasound-accelerated Catheter-directed Thrombolysis: Case Report and Review of the Literature. Ann Vasc Surg 2015; 29:1657.e9-1657.e15. doi:10.1016/j.avsg.2015.05.018.
18.
Shah NL, Shanley CJ, Prince MR, et al. Deep venous thrombosis complicating a congenital absence of the inferior vena cava. Surgery 1996; 120:891-6.
19.
Ramanathan T, Hughes TMD, Richardson AJ. Perinatal inferior vena cava thrombosis and absence of the infrarenal inferior vena cava. J Vasc Surg 2001; 33:1097-9.
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