greater than 80% of pavms are congenital , and approximately 50% of are associated with hereditary hemorrhagic telangiectasia ( hht ) . in addition to common complaints , such as dyspnea and epistaxis , a pavm can cause hemoptysis , hemothorax , and serious neurologic complications , such as stroke , seizures , and brain abscesses . ebstein 's anomaly ( ea ) is a congenital cardiac malformation characterized by downward displacement of the attachment of the septal and posterior leaflets of the tricuspid valve . ea is also a rare disorder , and patients with ea may have various additional cardiovascular anomalies . although the causes of a pavm and ea have not been established , embryogenic or genetic factors might give some contributions . however , there are no reports concerning the co - existence of pavms or hht with ea . we present a case of a pavm with ea that is suspected to have hht . she had experienced spontaneous recurrent epistaxis for the last several years , with no other remarkable medical history . a chest radiograph showed cardiomegaly and a well - demarcated , 12-mm nodular opacity in the left lung field ( fig . the electrocardiogram showed tall and broad p waves , as well as an incomplete right bundle - branch block . on transthoracic echocardiography , the displacement index ( distance between the mitral annulus and tricuspid annulus , divided by the body surface area ) was 15.6 mm / m , and it fulfilled the displacement index criteria for the diagnosis of ea ( 8 mm / m ) . moreover , tethering of the septal and posterior leaflets of the tricuspid valve was observed with central coaptation failure and severe regurgitation . a portion of the right ventricle was atrialized because of apical displacement of the tricuspid valve . she was diagnosed with ea , and agreed to undergo surgical treatment . to evaluate the nodule in the left lung , a 12-mm serpiginous vascular structure connecting the left interlobar pulmonary artery to the left inferior pulmonary vein was detected in the superior segment of the left lower lobe ( fig . no part of her medical history could explain the causes of secondary pavm , such as chest trauma , thoracic surgery , long - standing hepatic cirrhosis , metastatic carcinoma , mitral stenosis , or infections . three - phase contrast ct scans of the liver and mr angiography of the brain were performed to identify other visceral arteriovenous malformations . a liver ct showed diffuse dilatation of the hepatic arteries and veins with multifocal arteriovenous malformations , suggesting hht ( fig . the brain mr showed no evidence of vascular malformation . on the 7th day of admission , after discharge , she has remained stable and comfortable , and will undergo percutaneous embolotherapy of the pavm in the near future . a pavm is a rare pulmonary vascular anomaly with an incidence of 2 - 3 per 100,000 population.1 ) pavms may be single or multiple , and the left lower lobe of the lung is the most common location of solitary pavm.2 ) greater than 80% of pavms are congenital , and approximately 50% of these are associated with hht , also known as rendu - osler - weber disease.2)3 ) a single pavm < 2 cm in diameter on chest radiography usually does not cause symptoms.4 ) the most common complaint is epistaxis . a pavm can cause dyspnea , cyanosis , hemoptysis , hemothorax , and serious neurologic complications , such as strokes , seizures , and brain abscesses.4)5 ) patients with co - existing hht tend to have multiple arteriovenous malformations , rapid disease progression , and a higher complication rate.2 ) the brain , lungs , and liver are the most frequently involved organs in patients with hht.6 ) ea is a congenital cardiac malformation characterized by downward displacement of the attachment of the septal and posterior leaflets of the tricuspid valve.7 ) ea is also a rare disorder occurring in 1 per 200,000 live births.7 ) most cases are sporadic , and the embryologic and genetic contributors are unclear,8 ) but the ea gene might be located on chromosome 9.9 ) the genetic linkages to hht are located on chromosome 9 or 12.10 ) patients with ea may have various additional cardiovascular anomalies.8)11 ) however , there are no reports concerning the co - existence of a pavm or hht with ea . although it is unclear whether or not a concurrent pavm and ea has an embryologic or genetic relationship , we report a patient with a pavm and ea who was suspected to have hht according to the curaao criteria.12 ) further genetic and embryonic studies are needed to detect a possible relationship between the two medical conditions .
a pulmonary arteriovenous malformation ( pavm ) is a rare pulmonary vascular anomaly presenting as dyspnea or recurrent epistaxis . 
 ebstein 's anomaly ( ea ) , a congenital cardiac malformation , is also a rare condition . 
 there have been no reports concerning the co - existence of pavm with hereditary hemorrhagic telangiectasia ( hht ) and ea . 
 a 40-year - old woman was admitted with a 2-month history of increasing dyspnea and several years of recurrent epistaxis . on transthoracic echocardiography , she was diagnosed with ea and agreed to undergo surgical treatment . 
 a chest ct angiography showed a 12-mm serpiginous vascular structure suspicious for a pavm and a liver ct suggested htt . 
 although it is unclear whether or not a concurrent pavm and ea have an embryologic or genetic relationship , we report a case of a pavm with ea . 
 further genetic and embryonic studies are needed to identify a possible relationship of the two medical conditions .