Redirection of hepatic venous drainage after total cavopulmonary shunt in left isomerism

Uemura, Hiroji; Yagihara, Toshikatsu; Hattori, Reiji; Kawahira, Youichi; Tsukano, Shinya; Watanabe, Ken

doi:10.1016/s0003-4975(99)00665-7

Cited by 59 publications

(37 citation statements)

References 17 publications

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“…If the patient's systemic outflow tract exhibits signs of potential stenosis, the double-barrel Damus-Kaye-Stansel operation is also In either case of right or left isomerism, a hepatic to pulmonary connection is mandatory to avoid the development of pulmonary atriovenous fistula. 81 Congenital absence of the portal vein is a rare but noteworthy complication of left isomerism. 82 Pulmonary arterial hypertension may develop because of massive portosystemic shunt via the ductus venosus or other venous collaterals.…”

Section: Left Isomerismmentioning

confidence: 99%

Human Heterotaxy Syndrome

Shiraishi

Ichikawa

2012

Circ J

121

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Section: Left Isomerismmentioning

confidence: 99%

Human Heterotaxy Syndrome

Shiraishi

Ichikawa

2012

Circ J

121

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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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“…Though interrupted inferior caval vein may predispose these anomalous veno-venous shunts [6], not all patients with polysplenia present with interrupted inferior caval vein like our patient. Therefore, we must keep these shunts in mind in any patients with heterotaxy syndrome.…”

Section: Commentsmentioning

confidence: 50%

Portal-systemic encephalopathy after Fontan-type operation in patient with polysplenia syndrome

Koteda

Suda

Kishimoto

et al. 2009

European Journal of Cardio-Thoracic Surgery

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An 18-year-old patient, who had polysplenia and single ventricle, presented with altered mental status 9 years after a Fontan-type operation and pacemaker implantation. He underwent replacement of common atrioventricular valve and aortic valve plasty 1 year previously and has been placed on multiple medications including beta-blocker for his poor ventricular function. Blood chemistry revealed hyperammonemia of 2420 microg/l as a cause of this altered mental status disturbance. Superior mesenteric arteriography revealed large portal-systemic shunts in venous phase as a cause of hyperammonemia. To control blood ammonia level, we placed him on low protein diet, oral polymixin B, and lactulose instead of closing shunt with device. This case illustrates that portal-systemic shunt may result in hyperammonemia leading to altered mental status long after a Fontan-type operation.

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Redirection of hepatic venous drainage after total cavopulmonary shunt in left isomerism

Cited by 59 publications

References 17 publications

Human Heterotaxy Syndrome

Human Heterotaxy Syndrome

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Portal-systemic encephalopathy after Fontan-type operation in patient with polysplenia syndrome

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