Hepatic Venous Blood and the Development of Pulmonary Arteriovenous Malformations in Congenital Heart Disease

Srivastava, Deepak; Preminger, Tamar J.; Lock, James E.; Mandell, Valerie S.; Keane, John F.; Mayer, John E.; Kozakewich, Harry P.; Spevak, Philip J.

doi:10.1161/01.cir.92.5.1217

Cited by 341 publications

(221 citation statements)

References 18 publications

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“…Pediatric congenital heart surgical experiences favor similar hypotheses. [12][13][14] What circulating mediators should or could be measured? We await such innovation, discoveries, and genetic profiles.…”

Section: Commentsmentioning

confidence: 99%

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Krowka

2001

Liver Transplantation

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This report describes the unique development of pulmonary vascular dilatation and hypoxemia associated with portosystemic shunt in a pediatric liver transplant recipient. Ligation of the shunt resulted in resolution of hypoxemia. The outcome suggests that hepatic venous return to the pulmonary circulation is important in maintaining normal pulmonary vascular caliber.(2) Hepatopulmonary syndrome in inferior vena cava obstruction responding to cavoplasty. De BK, Sen S, Biswas PK, Sanyal R, Majumdar D, Biswas J. Gastroenterology 2000;118:192-196. (Reprinted with permission.) AbstractReports show that hepatopulmonary syndrome mostly occurs in the setting of advanced hepatic dysfunction, with the associated vasoactive substance imbalance believed to be responsible for its pathogenesis. However, hepatopulmonary syndrome has also been reported in cases of mild hepatic dysfunction or noncirrhotic portal hypertension, indicating that portal hypertension also plays a part in the pathogenesis. Liver transplantation remains the only therapeutic option of proven benefit. We describe 2 cases of hepatopulmonary syndrome in the setting of inferior vena cava (suprahepatic) obstruction, but with minimal hepatic dysfunction. After balloon cavoplasty, 1 patient showed, in addition to improvement of the features of hepatic outflow obstruction, significant reduction of dyspnea, cyanosis, and hypoxemia with arterial blood gas normalization within 2 weeks and intrapulmonary shunt reversal within 8 weeks. This implies that hemodynamic alterations (such as portal hypertension) independently contribute to the pathogenesis of hepatopulmonary syndrome in at least some of the cases. Therapies aimed at correcting these abnormal hemodynamics may be important in the treatment of this condition, especially when the hepatic functional status by itself does not warrant a liver transplant.

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Section: Commentsmentioning

confidence: 99%

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Krowka

2001

Liver Transplantation

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“…This is not the forum to explore all these issues, but a few words about pulmonary arteriovenous malformations are germane. [131][132][133][134][135][136][137][138][139][140][141][142] The etiology for the development of pulmonary arteriovenous malformations after the Fontan operation remains uncertain. Clinical practice currently suggests that exclusion of the hepatic veins and hepatic venous effluent from the pulmonary circuit may be causal to the development of pulmonary arteriovenous malformations and that inclusion of the hepatic venous blood into the pulmonary circuit may reverse their formation.…”

Section: Right Heart Bypass and The Fontan Experiencementioning

confidence: 99%

Pediatric Cardiology and Cardiovascular Surgery: 1950-2000

Freedom

Lock²,

Bricker³

2000

Circulation

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“…Abnormal, thin-walled parenchymal vessels have been reported in the lungs of patients [2,17] and experimental animals [19] following CPA. However, it is unclear whether these vascular structures are of pulmonary or systemic origin or whether they mediate PAVS.…”

Section: Discussionmentioning

confidence: 99%

“…Although PAVS is believed to occur via pulmonary arteriovenous malformations (PAVMs) that develop following creation of a Glenn shunt, the vascular structures responsible for PAVS have not been clearly characterized. It is commonly believed that PAVMs develop because of the absence of a putative hepatic venous 'factor' in the pulmonary circulation of these patients [9,15,17]. Identification and morphologic analysis of the structural components responsible for PAVMs may provide a better understanding of abnormal vascular developmental processes in these patients and facilitate isolation of putative circulating etiological substances.…”

mentioning

confidence: 99%

“…Recruiting additional systemic blood flow may provide a source of putative hepatic factor [3] necessary for normal pulmonary vascular development. It is intriguing that CPA induces changes in both the bronchial and the pulmonary arterial circulations while PA banding does not.Abnormal, thin-walled parenchymal vessels have been reported in the lungs of patients [2,17] and experimental animals [19] following CPA. However, it is unclear whether these vascular structures are of pulmonary or systemic origin or whether they mediate PAVS.…”

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confidence: 99%

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Morphological Studies of Pulmonary Arteriovenous Shunting in a Lamb Model of Superior Cavopulmonary Anastomosis

McMullan¹,

Reddy

Gottliebson

et al. 2007

Pediatr Cardiol

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We sought to identify and characterize the abnormal vascular structures responsible for pulmonary arteriovenous shunting following the Glenn cavopulmonary shunt. Superior cavopulmonary shunt is commonly performed as part of the staged pathway to total cavopulmonary shunt to treat univentricular forms of congenital heart disease, however, clinically significant pulmonary arteriovenous malformations develop in some patients after the procedure. The causes of pulmonary arteriovenous malformations and other pulmonary vascular changes that occur after cavopulmonary shunt are not known. Using a juvenile lamb model of superior cavopulmonary anastomosis that reliably produces pulmonary arteriovenous malformations, we performed echocardiography and morphological analyses to determine the anatomic site of shunting and to identify the vascular structures involved. Pulmonary arteriovenous shunting was identified by contrast echocardiography in all surviving animals (n = 40) following superior cavopulmonary anastomosis. Pulmonary vascular corrosion casts revealed abnormal tortuous vessels joining pulmonary arteries and veins in cavopulmonary shunt animals but not control animals. In conclusion, unusual channels that bridged pulmonary arteries and veins were identified. These may represent the vascular structures responsible NIH Public Access KeywordsHeart defects; Congenital pulmonary circulation; Arteriovenous malformationThe classic Glenn surgical superior cavopulmonary anastomosis (CPA) is commonly performed as part of the staged Fontan pathway to treat univentricular forms of congenital heart disease [6,7]. Up to 60% of these patients develop pulmonary arteriovenous shunting (PAVS), which may lead to progressive and clinically significant hypoxia [1][2][3]11]. Although PAVS is believed to occur via pulmonary arteriovenous malformations (PAVMs) that develop following creation of a Glenn shunt, the vascular structures responsible for PAVS have not been clearly characterized. It is commonly believed that PAVMs develop because of the absence of a putative hepatic venous 'factor' in the pulmonary circulation of these patients [9,15,17]. Identification and morphologic analysis of the structural components responsible for PAVMs may provide a better understanding of abnormal vascular developmental processes in these patients and facilitate isolation of putative circulating etiological substances. Using a juvenile lamb model that reliably produces pulmonary arteriovenous malformations, we performed echocardiographic, angiographic, and morphologic analyses to identify and characterize the vascular structures involved in PAVS following the Glenn shunt. Materials and MethodsThese studies were performed in compliance with animal welfare regulations of the University of California-San Francisco and USDA guidelines. Furthermore, these studies conformed to the "Position of the American Heart Association on Research Animal Use," adopted by the American Heart Association on November 11, 1984, and were approved by the University o...

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Hepatic Venous Blood and the Development of Pulmonary Arteriovenous Malformations in Congenital Heart Disease

Cited by 341 publications

References 18 publications

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Pediatric Cardiology and Cardiovascular Surgery: 1950-2000

Morphological Studies of Pulmonary Arteriovenous Shunting in a Lamb Model of Superior Cavopulmonary Anastomosis

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