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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.
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