Diffuse pulmonary arteriovenous fistulae secondary to patent ductus venosus

Sugio, Yoshitsugu; Shimizu, Ryoichi; Tanaka, Hiroko; Kondoh, Osamu; Sugio, Yoko; Tsukahara, Masato

doi:10.1007/s00431-003-1169-z

Cited by 4 publications

(6 citation statements)

References 5 publications

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“…Case reports in childhood revealed that a persistent DV has the same physiopathologic consequences as an acquired portocaval shunt in adults. If the shunt rate is more than 30%, all the subsequent conditions can be found: hypergalactosemia-hyperbilirubinemia-hyperammonemia, [2][3][4]8,9 decreased Fisher's ratio of branchedchain to aromatic amino acids, 3 elevated bile acids, 3,[8][9][10] liver dysfunction, 2,4,8-10 autoimmune disorders, 10 hepatopulmonary syndrome, 2,10 and hepatic tumorlike lesions. [2][3][4] If the shunt rises more than 60%, hepatic encephalopathy can occur.…”

Section: Discussionmentioning

confidence: 99%

“…9 Analysis of families with DV have disclosed some typical pathological findings also in apparently asymptomatic younger siblings. [8][9][10] During the neonatal age, mild symptoms can be undistinguished by those pathologies due to the prematurity itself (e.g., hyperbilirubinemia). Interestingly, in the rare circumstance of congenital absence of portal vein, where a maximal portosystemic shunt occurs, an hepatic lesion is almost always found.…”

Section: Discussionmentioning

confidence: 99%

“…There are few reports of global or left-lobe fatty degeneration without regenerative nodules on histological examination of the liver due to portocaval shunt. [8][9][10] Once the shunt vessel is closed, biochemical findings and both fatty liver and FNH improve immediately, and the liver grows to a normal size. 5,8,9 Fatty infiltration is demonstrated by US not only as diffuse or focal hyperechoic liver alteration but also as focal sparing of segment IV.…”

Section: Discussionmentioning

confidence: 99%

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Transient Hepatic Nodular Lesions Associated with Patent Ductus Venosus in Preterm Infants

Schierz

Placa²,

Giuffrè³

et al. 2010

Amer J Perinatol

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We report on two cases of low-birth-weight preterm infants with patent ductus venosus associated with hepatic hypoechoic lesions of the fourth segment in an otherwise normal liver. Although tumorlike hepatic lesions have been previously reported in association with portosystemic shunts in children and adults, they were never described in preterm infants during physiological patency of ductus venosus. In our patients, hepatic lesions disappeared shortly after the spontaneous ductus closure. Physiopathologic interactions are discussed regarding altered portal blood supply caused by ductus venosus shunt.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Transient Hepatic Nodular Lesions Associated with Patent Ductus Venosus in Preterm Infants

Schierz

Placa²,

Giuffrè³

et al. 2010

Amer J Perinatol

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“…A similar pathophysiology may be seen in those patients with diffuse acquired pulmonary arteriovenous fistulas secondary to a patent venous duct, a malformation which results in a congenital portosystemic shunt. [61][62][63] Ligation of the patent venous duct has been followed by resolution of the pulmonary arteriovenous fistulas. Banding of the patent venous duct has also proved beneficial in this regard in another patient.…”

Section: Intersection With the Hepatopulmonary Syndromementioning

confidence: 99%

“…Banding of the patent venous duct has also proved beneficial in this regard in another patient. [61][62][63] Pulmonary arteriovenous fistulas, the Glenn shunt, the Fontan and total cavopulmonary connection: the role of hepatic venous exclusion…”

Section: Intersection With the Hepatopulmonary Syndromementioning

confidence: 99%

The biological “scrabble” of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery

2004

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Pulmonary arteriovenous fistulas are vascular malformations, which, by virtue of producing abnormal vascular connections proximal to the units of gas exchange, result in intrapulmonary right-to-left shunting. These malformations or fistulas reflect at least in part disordered angiogenesis, and less commonly recruitment and dilation of pre-existing vascular channels. Pulmonary arteriovenous fistulas occur in a number of diverse clinical settings. Such fistulas are a well-established feature of the Weber-Osler-Rendu complex, or hereditary haemorrhagic telangiectasia, an autosomal dominant vascular dysplasia characterized by mucocutaneous telangiectasis, epistaxis, gastrointestinal haemorrhage, and arteriovenous malformations in the lung, brain, liver and elsewhere. They are also seen in the patient with acute or chronic liver disease, disease that is usually but not invariably severe, or those with non-cirrhotic portal hypertension. They may occur as congenital malformations, single or diffuse, large or small in isolation, and when large or extensive enough may result in hypoxaemia, clinical cyanosis, and heart failure. Cerebral vascular accidents are also a well-known complication of this disorder. An extensive literature has accumulated with regard to the pulmonary arteriovenous fistulas seen in the setting of the Weber-Osler-Rendu complex, and there is considerable information on the genetics, basic biology, clinical findings, complications and therapeutic interventions of these malformations in the setting of this syndrome. These issues, however, are not the primary considerations of this review, although some aspects of this fascinating disorder will be discussed later. Rather the focus will be on pulmonary arteriovenous malformations that develop in the setting of cavopulmonary surgery, and their relationship to the pulmonary arteriovenous fistulas occurring in the hepatopulmonary syndrome. The complex tapestry of these overlapping and intersecting clinical observations will be unfolded in the light of their chronology.

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Pulmonary Arteriovenous Fistula

Hoffman

2009

The Natural and Unnatural History of Congenital Heart Disease

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Diffuse pulmonary arteriovenous fistulae secondary to patent ductus venosus

Cited by 4 publications

References 5 publications

Transient Hepatic Nodular Lesions Associated with Patent Ductus Venosus in Preterm Infants

Transient Hepatic Nodular Lesions Associated with Patent Ductus Venosus in Preterm Infants

The biological “scrabble” of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery

Pulmonary Arteriovenous Fistula

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